by Neil Bauman, Ph.D.
© April 2007 (latest revision March, 2019),
- What Is a Reverse-Slope (or Low Frequency) Hearing Loss?
- How Common Are Reverse-Slope (or Low Frequency) Hearing Losses?
- Causes of Reverse-Slope (or Low Frequency) Hearing Losses
- How Reverse-Slope (or Low Frequency) Hearing Losses Progress
- Characteristics of Reverse-Slope (or Low Frequency) Hearing Losses
- Hearing Testing and Reverse-Slope (or Low Frequency) Hearing Losses
- Amplification for Reverse-Slope (or Low Frequency) Losses
- Hearing Aids and Reverse-Slope Losses
- Fitting Hearing Aids to People with Reverse-Slope Losses
- Cochlear Implants and Reverse-Slope Losses
- Assistive Devices and Reverse-Slope Losses
- Literature Cited
- End Notes
Imagine a person with a hearing loss so severe he can’t hear thunder rumbling overhead, yet, at the same time, has hearing so acute he can hear a pin drop; or imagine a person that can’t hear you talking just 4 feet away, yet clearly hears a whisper from across a large room; or imagine a person that can’t hear a car motor running right beside him, yet can hear a single dry leaf skittering along in the gutter 50 feet away.
“Impossible,” you say, “a person could never have such good and bad hearing at the same time!”
Surprise! It’s true. Welcome to my world—the bizarre world of people with extreme reverse-slope hearing losses.
What Is a Reverse-Slope (or Low Frequency) Hearing Loss?
Hearing losses are sometimes classified according to the shape they form on an audiogram. They commonly go by strange names such as ski-slope loss, cookie-bite loss, flat loss, reverse cookie-bite loss and reverse-slope (or reverse curve) hearing loss. (My article Kinds of Hearing Losses explains these different hearing losses and illustrates the various shapes they form on audiograms.)
By far the most common kind of hearing loss is the typical ski-slope loss where the line on the audiogram slopes downto the right. In contrast, a reverse-slope loss (as its name implies) does the reverse and slopes up to the right.
As a result this kind of hearing loss is sometimes referred to as an up-sloping loss, a rising loss, or even a low frequency hearing loss, but by far the most common name is the reverse-slope (or reverse curve) hearing loss.
Don’t make the mistake of thinking that all reverse-slope losses are the same. Nothing could be further from the truth. There is an enormous difference in hearing between a mild gently-sloping reverse-slope hearing loss, and a severe or profound steeply-sloping reverse-slope loss.
For practical purposes, we can group reverse-slope hearing losses into three basic classes.
Fig. 1. Class 1. Audiogram of a mild reverse-slope hearing loss. Class 1. Perhaps the most common form of this relatively-rare loss is a gently up-sloping line in the standard audiometric frequencies between 250 and 8,000 Hz (Fig. 1). In this class, the worst low-frequency hearing loss typically lies somewhere between mild and moderately-severe.
Ella’s hearing fits this class. Her audiogram shows a 40 dB loss at 250 Hz sloping up to around 25 dB at 1,000 Hz and reaching 10 dB by 4,000 Hz (Fig. 1).
Fig. 2. Audiogram of Diane’s mild reverse-slope hearing loss. Diane has a bit worse reverse-slope loss. Her hearing loss ranges from 50 dB at 500 Hz to 20 dB at 4,000 Hz (Fig. 2): Diane’s mild reverse-slope loss).
Class 2. Rarer, is a fairly-steep up-sloping line in the standard audiometric frequencies. In this class, there is a moderate to severe hearing loss in the frequencies below 1,000 Hz, but at the same time, hearing becomes virtually normal somewhere in the range of 2,000 to 6,000 Hz (Fig. 3: Severe reverse-slope loss).1
Fig. 3. Class 2. Audiogram of a severe reverse slope hearing loss. Lorene’s hearing loss falls into this category. She explains, “The audiogram for my right ear starts at 90 dB at 250 Hz and rises to 30 dB at 8000 Hz.”
Class 3. The rarest form of the reverse-slope loss reveals a steep up-sloping line ranging from 70 to 110 dB in the low frequencies to incredible “dog-ears” hearing in the very high frequencies (those frequencies above 8,000 Hz) (Fig. 4: Neil”s extreme reverse-slope loss).
Fig. 4. Class 3. Audiogram of Neil’s extreme reverse slope hearing loss. Years ago when I was around 20, my hearing ranged from 75 dB at 1,000 Hz to -30 dB above 16,000 Hz (Fig. 4). (Note: numbers above the 0 dB line represent super-acute hearing, and are expressed as negative numbers.) Hearing at -30 dB means you can hear sounds so faint that people with normal (perfect) hearing can’t hear them at all. In fact, my very high frequency hearing (well above 10,000 Hz) was at one time so sensitive that the energy needed to produce the faintest sound I could hear needed to be multiplied 1,000 times before a person with “perfect” hearing could even begin to hear it! That’s some incredible hearing. Since I could easily hear “silent” dog whistles, some said I had “dog ears” hearing.
Reverse-slope hearing losses give rise to various degrees of “weird” hearing. This is caused by two main factors. The first factor is the amount (degree) of hearing loss we have in the low frequencies. This indicates what sounds we will not hear. For example, if we have severe or worse low-frequency hearing losses, we will not hear common loud sounds such as thunder, car and truck motors running, fans whirring, and the speech sounds that give speech most of its volume and fullness.
The second factor is the difference in volume (measured in decibels) between the softest low-frequency sound we can hear and the softest high-frequency sound we can hear. The greater this range, the weirder our hearing losses become.
Notice that the total range of the reverse-slope loss in Class 1 is not all that much—somewhere in the range of 30 dB. In the previous examples, both Ella’s and Diane’s hearing losses only range 30 dB between their worst and best frequencies. At this point, hearing loss is not all that strange, but people with these losses begin to exhibit some of the characteristics of those with more severe reverse-slope losses.
In Class 2 the range increases dramatically to somewhere between 60 and perhaps 90 dB, and with this increased range, there is a marked increase in the degree of hearing loss “weirdness.” Notice that Lorene’s hearing loss has a range of 60 dB.
However, it is in Class 3, with its incredible range of over 100 dB between the faintest low-frequency sound heard and the faintest high-frequency sound heard that truly bizarre hearing is the most pronounced. My hearing loss spanned an incredible range of 105 dB. No wonder people were always confused about what I could, and could not, hear!
Incidentally, since hearing is normally only tested to 8,000 Hz (because the typical audiometers in use today are only calibrated to 8,000 Hz), an audiogram of people with incredible high-frequency hearing gives a false impression of what that person really hears. In fact, people with my kind of hearing loss have been sent to “shrinks” because the medical professionals were so sure they were lying about what they could hear!
Therefore, if a person has a Class 2 or 3 reverse-slope loss, it is imperative that their hearing be tested to at least 16,000 Hz. (and preferably to the highest frequency they can hear), and to the softest sound they can hear, in order to properly diagnose their reverse-slope loss.
How Common Are Reverse-Slope (or Low Frequency) Hearing Losses?
Reverse-slope hearing losses are not common. Most people with reverse-slope losses tend towards the milder form, but I have run across a few people with severe to extreme forms like mine.
In fact, significant reverse-slope hearing losses like mine are very rare. Out of roughly 38 million hard of hearing people in the USA and Canada, only about 3,000 of us have this unusual hearing loss according to Dr. Charles Berlin, formerly the head of the Kresge Institute in Louisiana, one researcher that has studied this kind of hearing loss fairly extensively.
This means that only one person out of every 12,000 hard of hearing people has a significant reverse-slope loss. No wonder hearing health care professionals seldom see such cases. Consequently, there is little authoritative information written on this subject.
Causes of Reverse-Slope (or Low Frequency) Hearing Losses
There are a number of causes of reverse-slope losses, but the two most common causes seem to be certain genetic (hereditary) abnormalities and Meniere’s Disease.
Meniere’s Disease
When people think of causes of reverse-slope losses, typically they think about Meniere’s disease. Classic Meniere’s disease does indeed often, but not always, result in a reverse-slope hearing loss, at least in the beginning stages. From what I have seen, reverse-slope losses due to Meniere’s Disease tend to be more of the Class 1 kind of curve.
Heredity
Probably the most common cause of reverse-slope hearing losses, particularly in Classes 2 and 3, is of genetic origin. Hereditary losses seem to run in our families more often than not. For example, Dolores explained, “I think my reverse-slope hearing loss is genetic. My mother and sister have it too.”
Reverse-slope hearing loss runs in my family—at least it has for the past five generations. Those that I know of include my maternal grandfather, my mother, myself, my brother, my younger daughter and my brother’s older son and his son. My hearing loss is the worst in the tribe, with my daughter’s poor hearing coming in a close second. In addition, I know of other people who also have reverse-slope hearing losses running in their families—at least for the past 4 or 5 generations.
From the above, it appears that extreme reverse-slope hearing losses are a dominant genetic trait. It certainly is in my family. Each person born in my family has a 50% chance of having this kind of hearing loss.
In contrast, most other cases of genetic hearing loss seem to stem from recessive genes. In such cases, there is only a 25% chance of each child having a hearing loss (although there is a 50% chance of any child being a carrier, but not having a hearing loss). Furthermore, if the hearing loss is caused by a recessive gene, it may appear to skip several generations. This is because in order for it to show up, both parents have to be carriers of the same recessive gene.
Another interesting characteristic of severe or extreme reverse-slope hearing losses such as mine is that they are non-syndromic—that is, they don’t have any other conditions or syndromes associated with them. This is also in contrast to many cases of hearing loss from recessive genes where various syndromes are associated with these hearing losses.
I think many people, including doctors, stretch to find obvious causes for these hearing losses—and fail to realize that they really may be hereditary. For example, my mom was knocked down and run over by a street car when she was 5 or 6. When they noticed that she had poor hearing after that, they attributed it to the head trauma she sustained in that accident—yet it is now obvious that she really had an hereditary reverse-slope loss that she passed on to me and my brother.
Other Causes
Various childhood diseases are also thought to sometimes result in reverse-slope hearing losses. For example, Debbie wrote, “I acquired my loss from a rare illness when I was four years old. My daughter also shares my type of loss, although it has never been concluded that it is genetic in nature. Like me, she was not born with a hearing loss but acquired it from complications of chicken pox at age 2. The complications led to the same serious disease that I had when I was four.”
Judith explained, “My hearing loss apparently was the result of measles at the age of 2. My audiologist says I have reverse-slope hearing loss.”
Sometimes otosclerosis can show as a reverse-slope loss, as can enlarged vestibular aqueduct syndrome (EVAS/LVAS).
A rare condition called Susac syndrome can also cause a reverse-slope hearing loss. Hearing loss in Susac syndrome is primarily in the low frequencies and usually occurs relatively suddenly. It may affect both ears (bilateral). Its severity can range from mild to severe. In some cases, hearing loss may occur before other symptoms of Susac syndrome develop. Hearing loss is often accompanied by intense ringing of the ears (tinnitus).
How Reverse-Slope Hearing (or Low Frequency) Losses Progress
Whether reverse-slope losses get worse with time depends on what caused the loss in the first place. In general, it doesn’t seem that reverse-slope losses progress much differently from other kinds of hearing losses with two notable exceptions.
Meniere’s Disease
One of the characteristics of Meniere’s Disease is that it results in a progressive, fluctuating step-wise hearing loss. Thus if you have Meniere’s Disease, initially you may have a Class 1 type of reverse-slope loss. Over time, as your Meniere’s Disease progresses, you will likely find that this reverse-slope loss slowly evolves into a reverse cookie-bite or flat loss and ultimately into some degree of a severe or profound ski-slope loss.
Hereditary losses
With hereditary reverse-slope losses, we seem to go through three distinct stages.
Stage 1: The first stage occurs from birth to around 5 years of age. It appears that although there is some degree of hearing loss at birth, hearing in the lower frequencies rapidly decreases until around age 5 or so.
When my daughters were born, we were expecting one of them to be hard of hearing. Thus, we carefully observed them from birth in order to watch for any developing hearing loss. As a result, my hard of hearing daughter had several audiograms in her early childhood years which documented her increasing hearing loss from birth to age 5.
Furthermore, it seems that because of our excellent high-frequency hearing and because of our good speechreading skills at a very early age (of which our parents are typically totally unaware), our hearing losses do not become apparent until something traumatic happens as was the case with my mother (mentioned above), or something happens to drive home the fact that we cannot hear well.
For example, my parents didn’t discover I had a hearing loss until I was about 4 or 5. One day my dad, who was standing behind me where I couldn’t speechread him, asked, “Do you want to come for a ride in the car with me?” I totally ignored him, and continued playing on the floor. He knew something was wrong because I loved riding in the car! (It was a ‘29 Buick in those days.) Another time he asked me if I wanted some ice cream—which I still love—and again I ignored him. It was at this point that my parents had my hearing tested and discovered I had a severe hearing loss!
Stage 2: The second stage goes from about age 5 to around age 50 (if there are no other factors involved such as hearing loss from noise damage or from taking ototoxic drugs, for example).
The good news is that in stage 2, hearing loss doesn’t change much if at all. It remains stable through childhood, and early and middle adulthood. Debbie explained, “My hearing has for the most part, remained stable for as long as I can remember.” This has been my experience too, and my mother’s and daughter’s experience as well.
Thus, once we learn to adapt to our strange hearing losses, our coping strategies can remain the same for most of our lives.
Stage 3: The third stage kicks in about age 50 and continues for the rest of our lives. This is not really our reverse-slope hearing loss progressing, but rather, the effects of aging dramatically impinging on our precious high-frequency hearing. Here’s what happens.
Fig. 5. Average hearing loss with increasing age. As people age, they typically begin to lose their high-frequency hearing. This happens slowly and insidiously over many years. Fig. 5 plots “average” curves showing increasing high-frequency hearing loss from ages 40 (top line) to age 80 (bottom line).
The fact that people normally lose much of their high-frequency hearing as they age has some important ramifications for those of us with reverse-slope losses.
Notice that people with the typical ski-slope losses have already lost their high-frequency hearing (but retain their low-frequency hearing). Thus as they age, they don’t have much high-frequency hearing left to lose.
In contrast, those of us with extreme reverse-slope losses have most of our residual hearing in the high (and very high) frequencies, yet it is these very frequencies that people typically lose as they get older. As a result, somewhere around age 50 or so we begin to notice a significant drop in our hearing. Our reverse-curve loss rapidly begins to flatten and in time becomes more or less a flat curve.
Fig. 6. Neil’s reverse-slope loss at age 59. For example, between age 50 and age 60, I lost a lot of my excellent high-frequency hearing. As a result, I don’t hear much at all any more—but this is the result of aging, rather than from a progressive reverse-slope hearing loss.
To verify this, compare my audiogram taken at around age 21 (Fig. 4) with my current audiogram (Fig. 6) taken at age 59. As you can see, I have lost much of my high-frequency hearing. I no longer hear many of the familiar high-frequency sounds I used to depend on. Now I am having to learn other coping skills to make up for this loss. My world is rapidly becoming more and more silent. Eventually, if I follow my mother’s pattern, I’ll probably have a flat “curve” at around 80 dB or so.)
Characteristics of Reverse-Slope (or Low Frequency) Hearing Losses
What It’s Like to Live with a Reverse-Slope Hearing Loss
Having a Class 2 or 3 reverse-slope loss makes for some interesting experiences. Here are some of the things that we hear, or don’t hear, and how we cope with it.
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- We don’t hear normal sounds that tell us that certain appliances are running. For example, I have to put my hand on some of my household appliances and feel the vibrations in order to know if they are running, or watch them for movement. Furthermore, I can’t hear the low-frequency hum of the furnace, fridge, washer, drier, dishwasher, etc. so, likewise, in order to know if they are running, I have to put my hand on them (or look at the dials). However, the interesting thing is that we can easily hear the faint clicks of the relays kicking in/out to start/stop all these appliances. Thus, we can tell when they start or stop—we just don’t know whether they just started or just stopped. Lorene echoes my experiences when she explains, “I can hear the fridge kicking in, but can’t hear it when it is running.”
- When hard of hearing people get together in a room, those with ski-slope hearing losses complain how the noisy heating and air conditioning systems drown out speech, yet, without our hearing aids, we don’t even know these devices are making sounds at all!” These sounds certainly don’t bother us!
- In noisy conditions such as in mills and manufacturing plants, hearing people have to shout over all the low-frequency noise around them. Since we don’t hear low-frequency sounds well, we clearly hear the people shouting. That’s the one situation where I tell people (tongue in cheek), “You don’t have to yell at me. I’m not deaf!”
- Since loud low-frequency sounds mask softer high-frequency sounds, and since we don’t hear low-frequency sounds well, the softer high-frequency sounds stand out clearly to us. When we hear such soft high-frequency sounds, people think we must have incredible hearing, not that we are almost deaf. No wonder hearing people sometimes don’t think we are hard of hearing at all!
- Our hearing is exactly backwards to that of the typical hard of hearing person. As Debbie explains, “I have near normal hearing in the high frequencies, but my hearing drops off in the low and speech frequencies.” This is just the opposite of what people with ski-slope losses have. Thus most things they hear, we don’t, and most things we hear, they don’t.
- We generally can’t hear car motors running. This can make for some interesting experiences in parking lots. As a result, we need to use our eyes and watch for back-up lights and/or brake lights coming on if we are walking behind cars, or watch for their driving lights coming on if we are in front of them.
- Since I can’t hear my car’s motor running, sometimes when I am parked I may try to start my car a second time thinking it hadn’t started. The suddenly-swiveled heads of the people nearby tell me that I just ground the gears on the starter—again! Now, since modern cars have tachometers, I always look at the tachometer first. If it’s not reading 0, I leave the starter alone!
- I hear squeaking fan belts and squealing brakes very well. Often that is all I hear of motor vehicles approaching me, or running nearby.
- The screech of the wheels of trains on the tracks is so loud to me that it hurts my ears—yet to most people this is not even a loud sound. Imagine not being able to hear the loud roar of a train bearing down on you, yet getting headaches from the painfully-loud screech (to me) of the train wheels against the tracks as the train goes around a curve. This includes modern light rail systems in most big cities.
- We don’t hear the low-frequency sounds of things like big 18-wheelers rumbling beside us, but when their air compressors bleed off with a “pshhhhhh,” that sound is so loud that it is one of the few outside sounds I can hear from inside a building.
- We can hear whispers from considerable distances. For example, in school, I used to hear kids whispering from across the classroom, yet couldn’t hear the teacher talking only a few feet away. (I always had to speechread my teachers to know what they were saying.) It always puzzled me that the teachers never heard all the whispering that to me was so loud.
- We can hear phones ringing—especially if they have the old style bells for ringers—but can’t hear people talking on them. For example, I could clearly hear the phone ringing in my mom’s house while I was standing on the far side of the highway across from her house, even though her house was set back from the road a ways, the phone was mounted on an inside wall and the doors and windows were shut. Debbie has similar hearing to mine and relates, “I hear the alarm and phone ringing even when my hearing husband doesn’t!” With such incredible hearing, people can’t understand why pressing the receiver tight against our ears still doesn’t let us hear and understand the person on the other end unless we have special amplified phones.
- When it comes to using phones, we are at a decided disadvantage. You see, the high frequencies upon which we depend are deliberately chopped off to save “bandwidth.” Standard commercial phones are designed to have a flat frequency response from about 300 Hz to 3,400 Hz. After that, the frequency response drops off sharply. As a result, even when we use special amplified phones to give us the overall volume we need, we still find speech difficult to understand because the sound signal lacks the high-frequency component we so much rely on to give speech its intelligibility.
- We can easily hear sounds most people don’t hear. For example, when I was younger, I could easily hear the inaudible (to most people) 15,734 Hz whine produced by the fly-back transformer of a TV from anywhere in the house, and even from outside the house, yet I had to put my ear about 6 inches from the TV’s speaker in order to understand any speech from it (if the volume was set to normal hearing levels).
- We hear insects that other people don’t seem to hear well, if at all. To me, certain insects chirping from a block away (even just one insect) produce a racket loud enough to drown out the voice of a person standing almost nose-to-nose speaking to me. The “funny” thing (to me) is that people with normal hearing either can’t hear that insect at all, or only hear it very faintly!
- We hear some birds singing and chirping away, but not others. For example, I have never heard the low-frequency sounds of an owl hooting or a Mourning Dove cooing (although I have a flock of Mourning Doves right outside my back door). I cannot hear a large flock of geese honking overhead, unless they are flying almost at ground level, yet I can easily hear a male hummingbird’s high-pitched angry squeaks as it chases off a competitor, or the wonderful trilling sounds it makes as it power dives to impress its prospective mate.
- Water running in the sink typically drowns out any speech for us. We have to turn the water off in order to converse.
- We cannot understand what is said via public address systems. As Lorene explains, “I cannot understand any announcements in stores or malls.” This is my experience too.
- We hear women’s higher-pitched voices better than men’s lower-pitched voices—exactly the opposite of those with the more-common kinds of hearing losses.
- When inside, we do not hear traffic going by outside the house. Traffic noise definitely does not disturb our sleep!
- Road noise does not bother us much, even when the windows are down.
- We can hear sirens if we are outside and they are close by. However, if we are in our cars or houses, we don’t hear a thing. For example, for 10 years I lived right beside the fire hall and the fire trucks could roar out with their sirens blaring and I’d not hear a thing if I was inside the house. (Since I was a fireman, it would have been a bit embarrassing if they had left without me!)
- We hear/don’t hear the strangest things. For example, I can hear a dry leaf skittering down the street from 50 feet away, but can’t hear a car motor running right beside me.
- We do not hear somebody coming up behind us on a bicycle, and, as Lorene relates, “scaring the life out of me as they whiz past.”
- We cannot hear jets flying overhead when we are outside.
- We only hear thunder when it is very loud and almost overhead. We seldom hear the rain beating on the windows—unless it is a violent storm driving the rain into the glass, or there is hail or ice in the rain. Even then, we only hear the high-frequency component of the sound hail makes when hitting the windows.
- Although we have severe hearing losses in the speech frequencies, we can easily hear faint high-frequency sounds such as a pin dropping on a table or hard floor. Sarah explains, “I have a 60-80 dB reverse-slope hearing loss. I can hear a pin drop, but normally can’t hear thunder! I’m the same. I can easily hear pins dropping, but not thunder unless it is very loud.
- We don’t hear strange noises outside. (What? You mean there are strange noises outside!) That’s like the hearing person who asked a group of us (hard of hearing people) if it bothered us when people talked about us behind our backs and we replied, “They do?”
- Sound deadening tiles deaden the higher-frequency sounds we normally hear. Thus, we find it more difficult to hear in rooms with acoustical tile since the purpose of the tile is to absorb the high-frequency noises most people find annoying, but to us are critical to understanding what we are hearing.
- Adjusting the treble on a radio or stereo changes the volume for us. As strange as it my seem, for years I couldn’t tell much difference between the treble control and the volume control on a radio or stereo. Turning up the treble made the sound louder, just like turning up the volume control did. I can remember when my wife would turn on the radio, and if it was bothering me I’d just turn down the tone until I couldn’t hear it—and both of us were happy. (I could also precisely tune (zero-beat) a radio to an FM station I couldn’t even hear. I would just tune it for no “hiss” or “whistle” and when it was in perfect tune, I couldn’t hear a thing! My wife could never do that with her normal hearing.
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Reverse-Slope Losses vs. Ski-Slope Losses
There are some very real differences between how we perceive speech as compared to how people with ski-slope losses hear speech. Naturally, the worse our hearing is, the more pronounced these differences become.
These differences are largely the result of the following 6 basic facts concerning speech and hearing. I think you will find this very enlightening.
- Speech is composed of vowels and consonants. Vowels are loud sounds while consonants generally are softer sounds. In fact, roughly 95% of speech energy goes into producing the 5 vowel sounds. This means that only 5% of speech energy is left to produce the 21 remaining consonants.
- Vowels basically produce lower-frequency sounds, while many consonants produce higher-frequency sounds.
- Most of the volume of speech comes from the (lower-frequency) vowels.
- Most of the intelligibility of speech is contained in the (higher-frequency) consonants. Thus, to a large extent, vowels give speech its volume, while consonants give speech its intelligibility. You can easily prove this. Write out a sentence, then take out all the vowels leaving blanks—and show it to someone, and they, with a bit of reflection, will be able to fill in the blanks and read the sentence correctly. Now try the same sentence, but with all the consonants removed. The sentence is impossible to decipher. If you tried pronouncing it, all you would hear is something akin to an Indian chant or war-cry.
- Low-frequency sounds travel considerable distances in air.
- High-frequency sounds are quickly attenuated in air so they don’t travel very far.
Now let’s put all these facts together and see exactly what it means to those of us with reverse-slope losses as opposed to those with the common ski-slope losses. Many of the coping skills you read about are for people with ski-slope losses, not for those of us with reverse-slope losses.
1. If you have a ski-slope loss you hear the loud vowel sounds, but not the soft consonants. Thus you hear people talking with no problem, but because most of the intelligence of speech is in the consonants, you don’t understand what people are saying. To you, speech sounds muffled because you don’t hear the high-frequency sounds. Thus you want more clarity.
In contrast, those of us with reverse-slope losses hear the soft high-frequency consonant sounds. Thus speech is thin, almost inaudible and often sounds like whispers. For example, as I approach someone talking, the first sounds I hear are the high-frequency voiceless “s” sounds. This is how I know someone is talking. We do not really hear a person talking until we get very close so we can hear the “voiced sounds.” Fortunately, we generally understand speech when it is finally loud enough for us to hear it. Thus we typically need more volume.
One of the “rules” when speaking to a hard of hearing person (really meaning those with ski-slope losses) is that you do not “yell” at them, but speak in a normal volume. Since they need clarity, not more volume, they want you to speak at a normal level, but speak slowly and clearly to enable them to catch the high-frequency consonantal sounds as best they can.
This approach is totally wrong for those of us with reverse-slope losses. We need people to speak louder in order to hear speech in the first place because we do not hear the low-frequency sounds that give speech much of its volume.
2. If you have a ski-slope loss, you can hear people talking from a considerable distance because low-frequency vowel sounds travel well in air. As a result, you can hear a person calling to you from a distance. Thus, you would turn around at a shouted warning (to see what the racket was about).
However, those of us with reverse-slope losses cannot hear people until we are almost nose-to-nose with them because we don’t hear the vowel sounds that give speech its volume and ability to travel far in air. As a result, we don’t hear people calling to us from any distance at all, so we often do not respond to shouted warnings.
3. Another bit of “common wisdom” is that hard of hearing people hear men better than women and children. This is true for people with the typical ski-slope losses because they hear the louder, lower-pitched voices of men better since they hear low-frequency sounds best. Women and children with their higher-pitched (and often softer) voices are much more difficult for them to hear and understand.
This “wisdom” is also totally wrong for those of us with reverse-slope losses. Since we hear the higher-frequency sounds best, we typically hear women’s voices better than men’s voices. As Rusty says, “Men’s voices have been difficult for me to hear.” I, too, much prefer talking to women as their voices are higher pitched and more in tune with my ears. Their high soprano voices are so lovely for me to hear and understand. Debbie explains, “I do not use the phone unless it is someone I can understand, namely a high-pitched female voice.”
Unfortunately for us, women don’t put as much sound energy into their voices as men do. Thus, we can’t hear them from any distance, but close up they are so easy to hear and understand. As a result, I normally stand almost nose-to-nose when I am talking to someone. I remember talking to my hard of hearing friend Elizabeth. We were out in the middle of a big, almost-empty arena—yet we were standing there nose-to-nose enjoying our chat.
4. People with ski-slope losses can’t hear whispers. Surprisingly enough, those of us with reverse-slope losses hear whispers very clearly—even from across a room. My former mother-in-law wouldn’t believe my hearing was as bad as it was because she would whisper when she didn’t want me to hear something, and I would hear her. (She never got it through her head that if she just spoke in a normal voice, I wouldn’t have understood a thing!)
To me, whispering seemed quite loud, so when I used to “whisper,” I’d actually use “low voice” (which to me sounded very faint as compared to whispering). To my chagrin, everyone around me heard me “whispering.” My wife kept telling me to “whisper.” It eventually dawned on me that others couldn’t hear the whispers I so easily heard.
5. If you have a ski-slope loss, you can’t hear in the presence of low-frequency noise.
In contrast, we typically hear better in “noisy” situations (if the noise is all low-frequency sounds) because we don’t hear these louder low-frequency sounds much, and people in noise tend to speak up (or shout) in such situations. Since we need more speech volume anyway, this is exactly what we need in order to hear.
Because people with normal hearing lower their voices in quiet situations so much that they are inaudible to me, I use a sneaky trick to “force” them speak up. For example, if I am outside mowing the grass or rototilling the garden and someone wants to talk to me, I generally leave the motor on—just idling—so they have to speak up (over the noise). That way I can then hear them much better. I discovered that if I turned the motor off, they would lower their voices and I couldn’t hear them at all. This is just one of the tricks I use to cope with my weird hearing loss.
6. A person with a ski-slope loss can hear a car or truck approaching from a distance because they hear the low-frequency sounds of the motor. For example, I was once talking with a hard of hearing farmer with the typical high-frequency hearing loss. He heard a truck approaching while it was still half a mile down the road and turned to see who was coming. In contrast, I never heard a sound even when the truck turned into the farm yard and stopped right in front of me!
Since we don’t hear low-frequency sounds, being around moving vehicles can be dangerous to us. Thus, we have to rely on our eyes. Furthermore, we can’t rely on our ears alone when crossing roads. Crossing streets is particularly dangerous for us when it is foggy and we neither can see nor hear vehicles approaching.
7. People with ski-slope losses don’t hear high-frequency sounds, and thus aren’t bothered by them.
In contrast, high-pitched sounds may hurt our ears but not bother people with normal hearing. Thus, they discount the pain we feel from such sounds. This is particularly true for those of us who have Class 2 or 3 reverse-slope losses. Debbie explains, “Sometimes high-pitched sounds seem so loud they hurt my ears.” I have the same problem. Another lady explained, “The things that bring me out of my seat are the screaming crowds at sports games on TV and small children’s shrill voices.”
Supersensitivity to High-Frequency Sounds
Those of us with Class 3 reverse-slope losses have super-acute hearing in the very high frequencies. For example, Kathi wrote, “I used to be able to hear dog whistles, bats, and all kinds of crazy things that other people couldn’t hear. It never occurred to me that I should tell my ENT doctor about it.” Rusty related, “I hear high-pitched sounds that drive me bats—like the Celtic flute and bagpipes.” Personally, I love listening to bagpipes! (I guess it is the Scot in me.)
Paul revealed, “I could hear a watch alarm go off 100 yards away but couldn’t understand speech at all. I was supersensitive to high frequency sounds.” Grant explained, “I recall as a cub scout having someone come in with some sound equipment. One of the things he did was to try to get people to hear some really high-pitched noise. I could still hear it when all the others couldn’t. Shirley confessed, “Chirping birds drive me nuts! I also respond well to dog whistles!”
I also used to hear “silent” dog whistles and other high-frequency sounds that no one around me could hear. In fact, I had a silent dog whistle years ago—my idea was to give it to my partner when I was timber cruising—and we could communicate like that without disturbing other people around us. However, it didn’t work out in practice. First, it was so shrill to me that it gave me an instant headache. Second, since high-frequency sounds attenuate very fast in air, I couldn’t hear it from any distance at all.
Because we hear sounds that people with normal hearing can’t, it is hard for anyone to believe that we are as deaf as we are. At the same time, these high-frequency sounds may be very loud to us and hurt, and no one can understand why we are cringing, when these sounds seem normal, or faint, to them.
Music Appreciation and Reverse-Slope Losses
Another aspect of Class 2 and 3 reverse-slope hearing losses is that we experience music much differently than do people with normal hearing. For example, when listening to acoustical guitars, all I heard was the pick, pick, pick sound of the pick on the strings, not the tones the strings themselves produced. For many years, I wondered why anyone would make and play such a “stupid” musical instrument—one that didn’t even produce musical sounds!
At the other end of the music spectrum, piccolos and flutes are so shrill and thin and “hissy” that the sounds they produce sound more like fingernails screeching on a blackboard than beautiful music. This was Marge’s experience also. She explains, “I have never really enjoyed, or been able to hear, music that was pleasant to me. I hear only high tones, so ‘real music’ is all irritating and ‘screechy’.”
Since I was born with a severe reverse-slope loss, I don’t know what music really should sound like either. Even so, I love music and enjoy what little music I can hear.
However, to be really enjoyable, music has to be transposed to the octaves that make it come alive for us. For example, one lady with reverse-slope hearing loss tried a special hearing aid designed specifically to transpose low-frequency sounds to higher-frequency sounds. She reported, “So stirring was the music that suddenly I began to cry. The beauty of the sounds I was hearing was almost torture—I simply couldn’t get enough of it.”2
That is my experience too. In one church I used to attend, I once told the organist how my strange hearing only let me really enjoy music played in a certain narrow range, and with no harmony accompanying the melody. From then on, each Sunday, Ruth would transpose and play a piece at the specific pitch my ears hear best—so I too, could really enjoy the music (at least part of the time). Everyone else in the congregation thought she is just utilizing all the different voices the electric piano had—but she really did it for me. That music was so beautiful, tears would well up in my eyes, and my soul really soared listening to it.
Speech Quality and Reverse-Slope Losses
One of the things that surprises many people is that all of us with severe reverse-slope losses have perfectly-normal or near-normal speech. Imagine a person that is essentially deaf, yet has flawless enunciation, perfectly-formed and well-modulated speech, all without having had any speech therapy. This is one of the blessings of having a severe reverse-slope hearing loss.
Debbie relates, “Both my daughter Heather and I have perfect speech, which is one of the bonuses of having this strange hearing loss.” Shirley explains, “Because I have high-frequency hearing, my voice has never been affected by my hearing loss, although my hearing loss is profound.”
By perfectly-normal speech, I mean speech that is indistinguishable from the speech produced by people with normal hearing. Given the degree of our hearing losses in the speech range, you would expect us to produce the “flat” or “deaf” speech so prevalent in those with severe and profound hearing losses, but we don’t.
In a three-way study comparing the speech of people with normal hearing to people with reverse-slope losses, and to people with ski-slope losses, every person that received an “A” (excellent) for speech (meaning their speech sounded virtually as if the person had no hearing loss at all) either had normal hearing, or had considerable high-frequency hearing (hearing above 8 kHz, i.e. a reverse-slope hearing loss). In contrast, every person that received an “F” (failure) for speech had measurable hearing only in the frequencies below 3 kHz (i.e. had a ski-slope hearing loss).3
Because my speech is also indistinguishable from the speech of people with normal hearing, I’ve had many people refuse to believe how bad my hearing really is. I am talking about hearing health care professionals, not just the man on the street. It is impossible to tell that I have a hearing loss from my speech (unless, on the rare occasion, you hear me mispronounce a word that I’ve never heard spoken aloud before).
Peggy, herself hard of hearing, after hearing me speak, exclaimed, “Do you realize that your speech is absolutely perfect? You must have worked very hard to perfect your tone like that what with growing up hard of hearing.”
The surprising truth is that those of us with severe reverse-slope losses don’t need speech therapy. My perfectly-normal speech just came naturally. I’ve never had, or needed, speech therapy.
In contrast, it is a rare person that has a severe or worse ski-slope hearing loss that speaks normally. Some deaf, or profoundly hard of hearing, people do have excellent speech. In fact, I am amazed how well they speak considering they haven’t heard anything for years. For example, Hilda—totally deaf for years—has excellent speech, but it is not perfect. Her good speech comes from excellent speech memory, plus extensive speech therapy and regular “touch-up” speech therapy sessions from time to time.
All this speech therapy is not necessary for those of us with reverse-slope hearing losses. Back in 1978, Dr. Chuck Berlin realized that “one of the interesting characteristics of people with significant reverse-slope hearing losses in the severe to profound range is that they have unusually good speech. This capability has usually been attributed to post-lingual hearing loss or to unique methods of oral speech training.” However, it seems that the secret to our excellent speech is that we have good hearing in those frequencies above the traditional frequencies tested (i.e. above 8,000 Hz).4
In fact, the real secret to perfectly-normal speech is hearing all speech frequencies, especially the high-frequency consonants such as “s,” “f,” “sh,” “ch,” “t,” and “th.” These high-frequency consonants do not have any “voice.” They are simply produced by air hissing between the teeth, and around the tongue, in various ways.
When you can’t hear these sounds, it is very difficult to produce them properly. In fact, I can tell if a person is hard of hearing just by the way they move their lips when they try to produce these sounds. Think how difficult or impossible it would be to learn to whistle if you couldn’t hear any of the sounds you were trying to produce. In like manner, these voiceless sounds depend so much on aural feedback—meaning you listen to the sound you make, and if it isn’t “right on” you immediately correct it. If you cannot hear it, you don’t get this feedback so you don’t correct these sounds, and your poor speech reflects this.
Since those of us with severe reverse-slope losses hear these “voiceless” sounds the best, we use them correctly in our speech and thus avoid the flat “deaf speech” patterns of those with the typical ski-slope losses.
Let me illustrate the contrast between reverse-slope and ski-slope losses by examining the word “stop.” It is composed of 3 voiceless consonants. These consonants—“s,” “t” and “p”—are actually just air forced out of the mouth without any sound produced by the vocal cords. It contains one vowel “o” that produces vocal sound.
Breaking it down into the sounds of the individual letters, here is what actually happens.
s – air hissing between the teeth—a very high frequency sound.
t – a burst of air released from behind the teeth—another high frequency sound.
o – a loud lower-frequency vowel sound produced by the vocal cords.
p – a puff of air from the cheeks forced between the lips.
Now, if you have the typical ski-slope loss where you hear low-frequency sounds quite well, and do not hear high frequency sounds much, if at all, when someone says the word “stop” all you hear is the loud “short oh” sound “awe” and thus, that is what you repeat.
However, those of us with severe reverse-slope losses hear the voiceless sounds of the “s,” “t” and “p” and, because the “o” is a loud sound, we likely hear a bit of it too. Thus, we hear the whole word correctly—assuming it is loud enough for us to hear in the first place, otherwise we’d hear nothing. Because we hear all the sounds in the word, we also naturally produce them correctly when we speak.
Another interesting characteristic of those of us with severe reverse-slope hearing losses is that while our speech is perfectly normal, we may pitch our voices somewhat higher than normal. Thus, for example, you might think I was a baritone rather than a bass. However, when we wear properly-fitted hearing aids a surprising thing happens. Instantly our voices pitch themselves to normal.
The reason we pitch our voices higher is so we can better hear our own voices when we speak. This is a totally subconscious reflex. I have never been aware of it myself. However, people tell me that when I put my hearing aids on, the pitch of my voice drops instantly.
Dr. Berlin found the same thing in his research. Some years ago, he helped develop a special hearing aid designed specifically for those of us with severe reverse-slope losses. In one case, he noted that whenever a certain young lady wore her special hearing aid, “the fundamental frequency of her voice dropped sharply and the vowel formant frequencies became normal.” He added, “The most remarkable and consistent observation in successful users is the sudden drop in fundamental frequency of the voice, even within the first few seconds of use.”5
Hearing Testing and Reverse-Slope (or Low Frequency) Hearing Losses
Reverse-Slope Losses Often Misdiagnosed
One of the tragic characteristics of having a severe reverse-slope loss is that in the past, hearing health care professionals have often misdiagnosed us. Since ski-slope losses are so common, few hearing health care professionals realize just how differently those of us with severe reverse-slope losses perceive speech. Thus, they treat us as though we had ski-slope losses, and that is wrong!
Dr. Berlin wrote, “Virtually all of these people [with severe reverse-slope losses] have normal or nearly-normal speech, and in good listening conditions act as if they have no hearing impairment; yet with no visual cues, they sometimes appear to be quite deaf. Depending on the slope and the nature of their losses, they are among the most misdiagnosed and mismanaged hearing loss patients.”6
Such wrong diagnoses include malingering (pretending to have a hearing loss when you don’t), central hearing loss (in the brain rather than in the ears), or even normal hearing.7 Sarah wrote, “I’m fed up with having to deal with so many ignorant people who are convinced I’m faking it.”
One audiologist accused a man of lying about his hearing loss because she had not run across a reverse slope loss before. He explained, “The first audiologist I went to told me I was lying about not being able to hear. She had me take the test 3 times and was clearly frustrated with my ‘horrible lies’. To her credit, she apologized after consulting with her colleagues. It was the first time she had seen that type of loss.”
Rusty related, “I could hear the dog’s toenails on the cement walk and would tell my parents that Rex was coming. As a result, they thought my hearing was fine, and it was many years before I got my first hearing test and hearing aids.” I can relate to similar experiences with hearing the dog’s toenails clearly clicking on the floor.
Of course when you can hear a pin drop, or a whisper from across a room, and have perfectly-normal speech, it’s hard for people to believe that you have a severe hearing loss and can’t hear much at all!
Thus, sometimes, instead of believing their own audiological tests, some hearing health care professionals assume we are pretending to have a hearing loss, and thus need a “shrink.” This really happens. In fact, one lady was treated by psychiatrists and psychologists for ten years because they just assumed she had “functional hearing loss”—i.e. was faking it, before finally being diagnosed correctly as having a severe reverse-slope hearing loss.8
This was because she:
- Had a pure-tone average of 80 dB, yet had a speech detection (or reception) threshold (SRT) of only 25 dB. (Obviously if you can detect speech at 25 dB, you can’t have a loss of 80 dB—so they thought.)
- Had virtually perfect articulation of high-frequency consonants such as “s”, “sh”, “f”, “th” and so on. (She acted as if she heard sibilants so well that no one could believe she was hearing impaired.9
- Had superb lip reading ability.
- Would respond to a very faint and deliberate hiss and could be called from the opposite end of her home if someone simply hissed sharply.
- Refused standard hearing aids, and seemed to do much better without them than with them.10
Dr. Berlin continues, “These errors in classification stem from the almost normal sensitivity to environmental sounds that contain broad-band transients or sibilant-like hisses. Their extraordinary hearing outside the standard range also allows them to develop unusually fine speech in both the sibilant articulation areas and even speech timing, provided they are not forced to wear powerful binaural aids, which occlude their ears and mask their good high-frequency hearing. When they take pure-tone audiograms, they appear to have substantial losses though the speech range (300 to 3000 Hz), which is grossly incompatible with their communication abilities.”11
Dr. Berlin then cautions, “These people are difficult to diagnose unless one is vigilant for their salient characteristics: unusually good speech and sensitivity to environmental sounds in the presence of poor speech perception (without visual cues) and severe pure-tone hearing losses in the presence of unusually good speech-detection thresholds. Their audiograms suggest they are hearing impaired or deaf, while their behaviors suggest that they have good hearing sensitivity and speech production, but poor speech perception. Many of these people reject standard hearing aids and often function well without them.”12
A simple way to test for the presence of a reverse-slope hearing loss is to use the Ling 5 sound test (ah, oo, ee, ss, sh). The tester should stand behind the person being tested and using his own natural voice, pronounce “ah, oo, ee, s, sh” with even intonation, asking the person to raise a hand each time anything is heard.
When a person has a fairly-severe reverse-slope loss, you will notice that they hear the “s” and “sh” sounds at much softer sound levels than the “ah, oo, and ee” sounds.13
Here are 8 characteristics that those of us with severe reverse-slope losses generally have. If we exhibit most of these characteristics, we very likely have a severe reverse-slope loss. These characteristics include:
-
- Hearing loss of 70 to 110 dB at 1,000 Hz.
- Better than expected Speech Recognition Threshold (SRT) scores for this degree of hearing loss, i.e. excellent speech detection.
- Do not like wearing conventional hearing aids.
- Remarkably precise articulation (i.e. great speech) without any amplification.14
- Poor pitch control when singing.15 (e.g. I can’t tell when my voice is pitched to a given note when I am singing. In fact, I don’t even have a clue in which “key” I am singing!)
- Higher-pitched voices when not wearing hearing aids, yet virtually perfect articulation, especially of fricative sounds such as “f” “v” “s” and “z”.
- Excellent language expression and comprehension.
- Readily hear selected higher-frequency environmental sounds.16
High-Frequency Testing Is the Answer
Most of the above problems with misdiagnoses could be instantly eliminated if audiometers were designed with our hearing losses in mind—i.e. were sensitive down to at least -30 dB and were calibrated to test hearing up to 20,000 Hz, and if audiologists would consistently test people to the highest frequency, and softest sound, they can hear.
Unfortunately, modern audiometers generally only test to 8,000 Hz, rather than up to 20,000 Hz. Back in the mid 1960s, I was tested with an old audiometer (old in those days too) that was calibrated to cover the frequencies well above 8,000 Hz.
At 1,000 Hz I couldn’t hear anything until the volume was set quite high (probably about 7 or 8 out of a range of 0 to 9). However at the highest frequency tested (16,000 or 20,000 Hz), I could hear all the way down to 0 and it was still loud to me. I bet the tester that I could take the earphones off and hold them at arm’s length, and since the volume was as low as it would go, if she would turn the power switch off and on, I could tell her when it was on or off—and I did. This illustrates the incredible sensitivity of our high-frequency hearing. It’s about time the audiometers we need are readily available to test our hearing!
Amplification for Reverse-Slope (or Low Frequency) Losses
People with reverse-slope hearing losses have completely different amplification needs than people with the much more common ski-slope losses. Thus, effectively fitting hearing aids, cochlear implants and assistive devices to people with reverse-slope losses requires a somewhat different approach than is usually taken.
Hearing Aids and Reverse-Slope Losses
People with reverse-slope losses often ask, “Which is the best hearing aid for my kind of hearing loss?” In the past there were no “best” hearing aids. In fact, there weren’t even any “good” hearing aids for us. No wonder Terry lamented, “Is there any kind of hearing aid for my kind of hearing loss? It seems everything out there is for high frequency loss.”
Hearing aid manufacturers have not concerned themselves with the unique needs of people with reverse-slope hearing losses. They just make hearing aids for the most common kind of hearing loss (the ski-slope loss). From their point of view, there are not enough of us for them to have the financial motive to develop a hearing aid to fit us.
Think about it. Since reverse-slope losses like mine only occur in 1 person in 12,000 hard of hearing people, assuming that all of us with reasonably-severe reverse-slope losses bought such hearing aids, the manufacturers would be able to sell a total of maybe 3,000 or so in North America! Not much of an incentive, is it?
Some people with reverse-slope losses have wondered why hearing aids such as the AVR ImpaCt, a high to low frequency-transposing hearing aid designed specifically for those with severe ski-slope losses, couldn’t be reverse designed to transpose the low frequencies to higher ones.
For example, Patti wrote, “It seems that they should be able to develop a hearing aid that could take low frequencies and compress them into the high frequencies so that we could hear more.”
You may be surprised to learn that some years ago (back in the 1980s), Drs. Berlin, Halperin and Killon did exactly that. They designed some experimental hearing aids that transposed the lower frequencies to the higher frequencies that those of us with reverse-slope losses can hear well. Dr. Halperin ultimately made 50 prototypes of these special translating aids.17
However, not everyone who tried one of these aids liked them because all sounds were higher pitched, making people sound more like Mickey Mouse than human. They took a bit of getting used to.
When one lady began wearing this special translating hearing aid, she wrote, “It sounded very strange and for the first hour or so I fooled around with the translator and direct aid controls. Voices sounded like jabberwocky because they were so different. I opened a door and let it slam. I expected the slam would be harsh but it wasn’t—a surprise! My cousin spent the evening with us and I tried the aid while conversing with him. He has a low voice and I often needed to strain to follow his conversation. Well, the hearing aid unquestionably made his voice clearer and easier to follow. I found myself relaxing while listening to him, and I was able to understand him despite the fact that he has a mustache. Later in the evening I listened to the sounds of a lively New Orleans jazz band; it was loud but not glaring.”18
Fortunately, with new digital hearing aid technology, having frequency-transposing hearing aids is much less of a concern. This is largely because of two innovations. The first is that new digital hearing aids have multiple channels in them (typically between 7 and 16). (Note: channels are basically just a way of dividing up the audio frequency spectrum so that each channel can be programmed independently from each other.) Thus you can have your hearing aids set to exactly match your hearing loss—frequency by frequency. Therefore, theoretically, you can properly program digital hearing aids to match any reverse-slope loss.
The second innovation is the development of wide-band hearing aids. Typical hearing aids cover the frequencies up to 6,000 or 8,000 Hz. The new wide-band hearing aids work up to 16,000 Hz where those of us with reverse-slope losses typically have much of our hearing.
Because we hear relatively well between 4 kHz and 16kHz, we need hearing aids that don’t amplify these high frequencies much—but, at the same time, allow these sounds to come through without obstruction, and yet amplify lower-frequency sounds.19 It is critical that our hearing aids don’t block or mask the high-frequency sounds that are so important to us. We use the very high-frequency sounds to understand speech, and to recognize environmental and warning sounds.
My current hearing aids are the very first wide-band (16,000 Hz) hearing aids America Hears produced. They work well for me. In contrast, Rusty tried the Widex Inteo (Widex IN-19) and is having excellent results with them.
Fitting Hearing Aids to People with Reverse-Slope Losses
Since reverse-slope hearing losses are quite rare, few audiologists have much experience programming hearing aids to meet our needs. The truth is, fitting hearing aids to people with reverse-slope hearing losses is not as straightforward as it first seems. There is no cut and dried method that works for everyone.
Many audiologists rely on the manufacturer’s fitting software. However, programming our hearing aids according to the manufacturer’s theoretical programming protocols seldom (if ever) works for us, especially if we have the more severe reverse-slope hearing losses. Audiologists that do this end up with unhappy patients like Terry, Judith and Dolores.
Terry wrote, “I have a couple of Siemens in-the-ear, programmable aids that have been mostly useless because I can’t get them programmed right.”
Judith explained, “The audiologist I now rely on says I have a “reverse-slope loss”. The hearing aid I now have is less than satisfactory. The audiologists I have been going to have told me it is a very difficult loss to correct, and each would rather the other assist me. It is not fun to spend $2,650.00 for unsatisfactory equipment!”
Dolores was totally upset with her fitting experience. She related, “My audiologist adjusted my hearing aids according to the manufacturer’s fitting protocol. When I walked out, the door squeaked loudly and my footsteps rang in my ears. I started the engine and the roar overpowered the radio. My nerves were shot by then, and I began to cry.”
It is obvious that the programming software just doesn’t make proper provision for people like us. In fact, the audiologist my wife goes to told me she wouldn’t be able to program digital aids to fit me because of the software limitations.
The reason for this is that we perceive sounds somewhat differently than normal—especially if we have had reverse-slope hearing losses from birth. Doreen explained, “My audiologist said reverse-slope losses are harder to fit because each person’s perception of normal sound is so different.” Audiologist Brad Ingrao, Au.D. definitely agrees. He wrote, “The real trick, in my experience, is in unraveling the pattern of loudness and pitch perception in a reverse-curve loss. I haven’t ever found a reliable pattern, so ended up taking each one individually.”
Since this is the case, audiologists need to follow Brad’s example—really listen to us, then specifically set the volume by frequency to what we like—and largely forget about the theoretical fitting curves and suchlike if they want to successfully fit reverse-slope hearing losses. As Doreen discovered, “The computer program tells the audiologist what settings to use based on my audiogram. They set my aid as specified by the computer and it was awful. I ended up with my aids set almost opposite to what the computer said it should be.”
Nancy explained, “Fitting a reverse slope loss is somewhat counter intuitive. My audiologist had to turn off the ‘learning’ programming in my new hearing aids. It would try to adapt my hearing aids to my environment. Unfortunately, the algorithms were developed with the usual high-frequency hearing loss in mind. As a result, it completely messes things up for those of us with reverse slope losses.”
Audiologists can’t follow their fitting algorithms when fitting me either. I don’t understand speech well when my audiologist sets my hearing aids the way they are theoretically supposed to be set. To me it sounds like a person talking with several whole plums in his mouth (if that is possible)! It is bassy, boomy and largely unintelligible.
I need my hearing aids programmed according to how my brain perceives speech, not slavishly following my audiogram. This means programming my hearing aids so everyone sounds more like Mickey Mouse. Since my brain has never heard low-frequency sounds well, it thinks that sounding like Mickey Mouse is normal, so that is where I understand speech the best.
I think the reason my hearing is like it is results from two key factors. First, I had a severe reverse-slope hearing loss from birth. As a result, my brain didn’t know that speech contained a lot of lower-frequency sounds. Second, I did not get hearing aids until well after my brain had “wired” itself to think that normal speech was largely devoid of low-frequency sounds. (Your brain finishes wiring itself around age 6 or 7.)
As Evan Hillman, another person born with a reverse-slope hearing loss explains, “By being born with RSHL, my brain decided over 50 years ago that some portions of spoken sound are useless to me. Amplifying and presenting this portion of speech to my brain does not help because I have not developed neural pathways to utilize it. At best, the sounds will be useless. At worst, the sounds will increase my level of confusion.”
Speech contains a lot of redundancies. “Given the choice, our brains will do as little work as possible. Therefore, as we first learn language, our brains decide what minimum portions of a given sound it will use to decode the sound into meaningful speech. Because of our RSHL, those of us born with it use a different sampling of sounds to decode speech.”
In my case, I didn’t wear a hearing aid until I was 15 years old (although I had tried one for a few months when I was 8). By that time, I understood speech only when the high-frequency sounds were predominant. Thus, I needed my hearing aids set to the way my brain understood speech—not to the way the audiogram indicated my hearing aids should be programmed.
In contrast, my younger daughter, who has a similar reverse-slope loss to mine, began wearing two hearing aids when she was 3 or 4—while the auditory circuits in her brain were still plastic. Thus her hearing aids allowed her brain to wire itself more normally.
Evan explains, “Logically, it makes sense to only amplify the sounds our brains are able to use most effectively. Most audiologists don’t realize that the sounds most useful to a person with reverse-slope hearing loss are different than those of a person with normal hearing, especially a normal hearing loss acquired as an adult. In my opinion this is one of the core reasons we are always fighting with our hearing aid providers.”
Apart from getting fitted with hearing aids at a very young age while the brain is still plastic, there are two other secrets to successfully fitting hearing aids to people with reverse-slope losses. These both address the same problem, but they do it in two different ways.
The problem is that we need to hear the very high-frequency sounds we rely on to understand speech. Unfortunately, the higher frequencies our brains learn to use is prone to a lot of interference from environmental noise (crackling leaves, shuffling papers, running water, etc.) that don’t bother those with normal hearing, but really affect us. As a result it takes us much more effort to filter these sounds out so we can understand speech than it does for those with normal hearing. Therefore, we need our hearing aids fitted to how our brains understand speech the best. If this is not done, we end up as frustrated as Dolores.
She explained, “I’ve tried hearing aids twice before and have once again begun trying another. I’m having an awful time with it. I don’t understand what people are saying, and even with the hearing aid it’s not helping—everything is muffled and the phone is a nightmare.”
Dolores is experiencing problems because her hearing aids and/or ear molds are blocking the very high-frequency sounds she needs for understanding speech. Without these high-frequency sounds, to us speech sounds all muffled.
Therefore, when you fit hearing aids to people with reverse-slope losses, you need to be aware of the serious deleterious effects that occluding our ear canals has on us. Whenever, or if ever, our ear canals must be occluded by hearing aids or ear molds, you need to provide some other way for us to hear very high-frequency sounds.
There are two ways to do this. The first way is to use non-occluding ear molds. These can be either open-fitted ear molds, or ear molds with vents of sufficient size to allow us to hear the high-frequency sounds we need.
The second way, especially if open-fitted ear molds or vented ear molds will cause feedback, is to fit us with special wide-band hearing aids that amplify sounds up to 16 kHz or higher, rather than topping out at 6 or 8 kHz as regular hearing aids do.
The secret here is to program the aids to “pass through” these very high-frequency sounds, even though we have normal hearing in this range, because the ear molds are blocking us from otherwise hearing them.
If this is not done, we will complain that you are giving us too much low-frequency amplification, and not enough high-frequency amplification.
Traditional hearing aids both mask and occlude the high-frequency portion of the hearing spectrum. This forces us to use the less effective, low-frequency hearing we have at our disposal,20 rather than our good high-frequency hearing on which we generally rely, and that frustrates us.
This was Missy’s problem. She wrote, “The trouble I have experienced is when they give me what I need to boost my lows, it overpowers my highs.”
Liz has a similar problem. She writes, “Part of my problem is that my aids have been programmed with too much lows and not enough highs.”
Liz further states, “My audiologist doesn’t want to fix that because she feels that I need the lows, and that my highs are essentially normal, but I told her I’m not getting the consonants to help me differentiate between words.”
Liz is absolutely right. Her audiologist is programming in too much low-frequency amplification.
This stems from two different fitting philosophies. The better NAL fitting philosophy is to equalize, rather than normalize loudness relationships across speech frequencies. If all the speech frequencies are amplified so that they are heard equally loud, speech intelligibility is maximized.21
However, when other fitting methods try to preserve this normal relationship of loudness among the speech frequencies, they tend to prescribe too much gain for the low frequencies.22
Therefore, the key is to amplify less at frequencies where there is severe to profound hearing losses, which in the case of reverse-slope losses means the low frequencies, and amplify more at the frequencies of better hearing.23 This is because the frequency regions of poorest hearing will contribute the least towards speech intelligibility.24 In fact, this is the principle of effective audibility.
As Ted Venema, Ph.D. explains, “Effective audibility refers to how much information can be extracted from speech sounds once they are audible. As hearing loss increases, for people with severe or greater hearing loss, a small sensation level might give some amount of information, while a high sensation level will not necessarily add much more information for understanding speech. Thus for those with profound hearing loss, audibility might be accompanied with virtually no added effective audibility.25
This seems to be the problem with the fitting philosophy in Missy’s and Liz’ cases.
In fact, for properly fitting reverse-slope losses, the NAL-NL1 fitting protocol does not target any gain below 500 Hz, and reduced gain at 500 Hz itself, because these frequencies will not contribute to effective audibility.26
Thus for effectively fitting reverse-slope losses, we need reduced low-frequency amplification so it does not overpower the high-frequency and very high-frequency amplification on which we so much depend.
If you are an audiologist or hearing aid fitter (or are just interested), you would do well to listen to this 15 minute presentation by Don Schum of Oticon USA (DJS@Oticonusa.com). It gives an excellent overview of why traditional methods of fitting hearing aids to those of us with reverse-slope hearing losses don’t work. He then explains the principles of effectively fitting hearing aids to people with reverse slope hearing losses . He uses graphs and charts/audiograms to explain these fitting protocols and emphasizes much of what I have just said on how to properly fit hearing aids to us, including the technical details that you need for a successful fitting.
Finally, never forget about the effects of recruitment (where certain sounds are perceived as much too loud). Recruitment can be a real problem for those of us with reverse-slope losses. At some frequencies, my recruitment is quite severe. Unfortunately, these are the very frequencies I need to understand speech. Thus by setting the compression levels for comfort, it means I no longer have as good as discrimination as otherwise—but at least I can stand the recruiting sounds, so this is a mixed blessing.
To be effective, at least in my experience, compression needs to be programmed into our hearing aids frequency by frequency. Unfortunately, few audiologists program compression this way. Francis Kuk, Ph.D. in his study of people with reverse-slope hearing loss concluded:
- A digital multi-channel nonlinear hearing aid has more features than a linear hearing aid to match the gain requirement of people who have a low-frequency hearing loss.
- Use of wide dynamic range compression (WDRC) with a low compression threshold (CT) and high level compression may be more effective (than linear or WDRC with high CT) in preserving audibility and maintaining comfort across listening environments.
- People with a reverse-slope audiogram do prefer amplification in the low frequencies. However, their gain preference, compared to the recommendations of some proprietary fitting targets, may vary depending on input levels.
- A broad bandwidth, including amplification in the normal or near-normal frequency region, is desirable. However, the specific amount needs individual customization.27
One word of caution in relation to Dr. Kuk’s conclusions: from what I can determine, the people he studied generally fell into the Class 1 reverse-slope category. Therefore, his conclusions need to be modified to include such things as wide band hearing aids and allowing pass-through very high frequency sounds, as I have explained above, in order to apply more particularly to people with Class 2 and Class 3 reverse slope losses.
Note: just because a person has a reverse-slope hearing loss doesn’t guarantee that a hearing aid will help him. For example, people with Auditory Neuropathy/Auditory Dys-synchrony (AN/AD) often have reverse-slope hearing losses, yet such people generally do not benefit from wearing hearing aids (with some notable exceptions, although they often benefit from cochlear implants). Therefore, it is important to test middle ear muscle reflexes, otoacoustic emissions and auditory brainstem response to ensure a proper diagnosis of the problem.28
Fig. 7. Reverse-slope loss of a person with AN/AD. The salient features of people with AN/AD include:
- Otoacoustic emissions are, or at one time were, present.
- Auditory Brainstem Response (ABR) is absent or grossly abnormal.
- Middle ear reflexes are absent.29
People that have AN/AD fit the above conditions, yet they may have a very pronounced reverse-slope audiogram (Fig. 7). According to Dr. Berlin, “In AN/AD the audiogram is virtually meaningless, so before you try to treat a reverse-slope loss, you have to rule out AN/AD because no hearing aid easily helps it.”30
Cochlear Implants and Reverse-Slope Losses
Since not many hard of hearing people have reverse-slope losses, it follows that not many have received cochlear implants (CI) either. Thus, for those with extreme reverse-slope losses, there is little history of how well cochlear implants will perform.
This has led to concern on the part of people with reverse slope losses that they won’t get good results with a cochlear implant. For example, Lorene wrote, “I am scared that if the CI didn’t work, I would not be able to use what is left of my residual hearing in my right ear.”
Part of this concern stems from the fact that ski-slope losses and reverse-slope losses have residual hearing at opposite ends of the cochlea. The cochlea is shaped like a snail, and consists of 2½ turns. High frequency hearing occurs at the base and low frequency hearing at the tip (apex).
With the normal ski-slope loss you basically have no high-frequency hearing left, so when the doctor inserts the cochlear implant electrodes into the base of the cochlea where they would normally destroy any residual high-frequency hearing, there is none there left to lose. Also, since the electrodes don’t quite reach the tip where the low frequencies reside, some residual low-frequency hearing may remain.
Furthermore, with a reverse-slope loss, the residual (high-frequency) hearing is at the base of the cochlea where it is destroyed when the electrodes are inserted. Thus, people with reverse-slope losses have more to risk in that sense if the cochlear implant should fail to work well for them.
However, if you wait until your hearing is so poor and garbled that you can’t understand much anyway, you really don’t have much to lose, so why worry about keeping any residual hearing?
As noted in the previous section, in Dr. Berlin’s experience, people with AN/AD often do well with a cochlear implant, in spite of having reverse-slope losses. For example, the person whose audiogram is shown in Fig. 7 “benefited remarkably from a cochlear implant” according to Dr. Berlin.
Programming a cochlear implant for a person with a long-standing extreme reverse-slope hearing loss likely will be quite a challenge since cochlear implant fitting protocols are set up for those that have the typical ski-slope loss. When people with a ski-slope loss get a CI, they typically tell how all voices then sound high-pitched and Mickey-Mousey. This is because they haven’t heard high-frequency sounds for years. Now, all of a sudden, the CI gives them those sounds.
In contrast, a person with a reverse-slope loss, when turned on, not having heard low-frequency sounds for years or a lifetime, will likely find the low-frequency sounds overpowering. In fact, this is exactly the case with one who reported that voices now sounded very low-pitched and growly and were mostly incomprehensible.
I can well believe this for when my hearing aids are programmed for “normal” I also feel like I am being drowned in a sea of loud, low-frequency incomprehensible noise. A partial solution seems to be in turning off a number of the low frequency electrodes. Only time will tell what the real solution will ultimately prove to be.
Assistive Devices and Reverse-Slope Losses
If you have a more severe reverse-slope loss, you will soon find that a number of the wonderful assistive devices to help hard of hearing people hear better are not so wonderful after all. This is because they have been engineered in such a way that we cannot hear and understand speech well when using them.
This is especially true when we try to find an amplified phone we can hear on. For example, Karen wrote: “We were at a seminar where a Clarity (new name for the old Ameriphone/Walker company) representative was heavily pitching their “Clarity Power” telephones as hot new items that are to replace the old Ameriphone line. When asked from the audience “why was there no tone control with Clarity Power” the engineer said that tone controls are obsolete, and that it is much better to have the tone embedded in the volume control so that as the volume increases, so does the frequency emphasis. He was pretty strong in his having “engineer expertise” so no one challenged him.”
Karen then asked: “Am I missing something in my understanding of what the combined tone/volume control does? How would someone get the right amplification in their frequency range? I’m pretty sure I’m right, but I’m not an engineer.”
This is a classic example of engineers doing shoddy research into the needs of hard of hearing people.
If he had said that the Clarity products were designed only for hard of hearing people with ski-slope losses, I would have agreed with him that having an integrated volume and tone control might be a smart idea.
However, such a design is stupid for people with flat losses and cookie-bite losses, and is totally asinine for those of us with severe reverse-slope losses. If he had ears like mine, he would never have made such a ridiculous statement.
This is much the same kind of thinking that went into the design of a wonderful phone for hard of hearing people—the Williams Sound Teletalker™ phone. To their credit, the designers included both a volume and a tone (they call it an enhance) control.
Unfortunately, they bought into the above misconception about what all hard of hearing people need. Although this phone is billed as having 55 dB of gain (which would be wonderful for my hearing loss), instead of putting all the power into the volume control, they assumed that hard of hearing people would never need more than 25 dB of overall gain, so they put 25 dB gain into the volume control, and then put the other 30 dB of gain into their tone (enhance) control.
Since I need much more than 25 dB of basic gain, I need to crank the tone control way up to get the required volume, but, by the time I have enough volume, all that extra high-frequency gain so distorts people’s voices that I can’t understand them.
Thus I have a wonderful phone designed specifically for hard of hearing people, yet I can’t use it. I’ve suggested to Williams Sound that they put all the gain on the volume control, but so far, they have ignored my suggestion.
As an effective workaround for both the TeleTalker and the Clarity phone I now use, I have added the Ameriphone HA-40 in-line amplifier. This neat little gizmo has separate volume and tone controls. Thus, I can add in the extra volume I need without distorting the higher frequencies.
To be sure, there are a number of other assistive devices that have straight volume or have both volume and tone controls that work pretty well for us. For example, the Williams PockeTalker personal amplifier has both volume and tone controls and works great for me.
Since our perception of speech is so different from other hearing losses, it is imperative that we try out any assistive devices before we buy them to be sure they will work for us (or be allowed to return them if they prove unsatisfactory).
(Back to Table of Contents)
________________________________Literature Cited
Berlin, Charles I., et. al. 1978. Superior Ultra-Audiometric Hearing: A New Type of Hearing Loss Which Correlates Highly with Unusually Good Speech in the Profoundly Deaf. ORL.
Berlin, Charles I. 1980. Ultra-Audiometric Hearing in the Hearing Impaired and the Use of Upward-Shifting Translating Hearing Aids. Kresge Hearing Research Laboratory of the South. Louisiana State University Medical Center. New Orleans, Louisiana.
Berlin, Charles I. 1985. Unusual Forms of Residual High-Frequency Hearing. In: Seminars in Hearing—Volume 6, Number 4. Kresge Research Laboratory, New Orleans, Louisiana.
Berlin, Charles I., et. al. 2002. Chapter 8, The Physiological Bases of Audiological Management. In: Hair Cell Micromechanics and Otoacoustic Emissions. Singular.
Berlin, Charles I., et. al. 2003. Auditory Neuropathy/Dyssynchrony, Its Diagnosis and Management. Pediatr. Clin. N. Am. 50 (2003) pp. 331-340.
Kuk, Francis, et. al. 2003. Changing with the Times: Managing Low-Frequency Hearing Loss. Hearing Review, November 2003. http://devdomino.widexpro.com/images/researchArticles/DAR61.pdf.
Venema, Theodore. 2001. The NAL-NL1 Fitting Method. Audiology Online. http://www.audiologyonline.com/articles/article_detail.asp?article_id=253.
________________________________
1 Berlin, 1985. p. 389.
2 Berlin, 1980. p. 50.
3 Berlin, 1980. p. 48.
4 Berlin, 1978. p. 111.
5 Berlin, 1985. p. 392.
6 Berlin, 1985. p. 393.
7 Berlin, 1980. p. 53.
8 Berlin, 1978. p. 112.
9 Berlin, 1985. p. 391.
10 Berlin, 1985. p. 389.
11 Berlin, 1980. pp. 53-54.
12 Berlin, 1980. p. 54.
13 Berlin, 1985. pp. 393-394.
14 Berlin, 1980. p. 50.
15 Berlin, 1978. pp. 111-112.
16 Berlin, 1980. pp. 44-45.
17 Berlin, 1985. p. 392.
18 Berlin, 1980. p. 50.
19 Berlin, 1980. p. 46.
20 Berlin, 1980. p. 49.
21 Venema, 2001. p. 2.
22 Venema, 2001. p. 3.
23 Venema, 2001. p. 4.
24 Venema, 2001. p. 7.
25 Venema, 2001. p. 4.
26 Venema, 2001. p. 5.
27 Kuk, 2003. p. 6.
28 Berlin, 2002. p. 139.
29 Berlin, 2003. p. 333.
30 Berlin, 2006. Personal communication.
I have a reverse-slope hearing loss. My right ear has severe mixed hearing loss in the lows (80 dBHL), rising to within normal limits, sloping to moderate at 8 KHz; and left ear, mild low frequency hearing loss (55 dBHL) rising to within normal limits. I cannot use the telephone on my right ear and rely considerably on lip reading. This poses a challenge at my workplace. I also have some difficulty with accents. I am looking for some direction regarding whether there are hearing aids that can help me.
Thank you,
Betty
According to multiple hearing tests I have a similar yet rare hearing loss. At low frequencies, my loss is substantial. Additionally, at high frequencies it is substantial as well. At more medium frequencies, I have a much lower hearing loss. I haven’t thought much about it until my audi recently explained it. However, this is tough. I am 22 and have had severe hearing loss since birth. I find that both audiologists I have had both had difficulty programming my hearing aids to properly suit me. Because of the odd loss at opposite frequencies, my hearing loss has always been a difficult one to correct.
Hi Peter:
You have what is commonly called a reverse cookie-bite hearing loss. This curve looks like someone took a bite out of the bottom of your audiogram. You can see audiograms of the various kinds of hearing losses in my article at http://hearinglosshelp.com/blog/kinds-of-hearing-losses/ .
As you have noted, this is a particularly difficult kind of hearing loss to effectively fit hearing aids.
Cordially,
Neil
When I was first diagnosed with my RSHL in 2010 (when I was 27) I found this website and I come back from time-to-time to re-read the information, mostly when I start feeling crazy when I talk about my hearing loss.
This website really made old conversations or comments jump out in my memory. Like my ex-BIL who was astounded I couldn’t hear the loud music thumping out the car driving down the road. Or now when my daughter is surprised when I can hear her very clearly whisper to her sister to ask me something and I just answer “no”.
Your resources really helped me when I was explaining to husband that it really was that I couldn’t hear him and I wasn’t ignoring him.
Thank you for this resource. I am loathe to get hearing aids because I am so young and it’s a pretty big expense for something that may not work. The auditory technologist that diagnosed me had never seen hearing loss like mine and so she didn’t have much information that could help me. She just said I was a candidate for hearing aids (but I’m not sure they would help).
Thank you again!
Hello, my mother has reverse slope hearing loss that started in her 50’s and has worsened over the years. She is now 75 and is beginning to feel isolated in group settings. No one has been able to help her. We live in Middle-Ga. Could you refer her to someone that understands her type of hearing loss? Any help would be greatly appreciated!
Thanks
Hi Mark:
I well know the feeling of being isolated in group settings. I always have been because hearing aids aren’t the solution in such settings. That is a VERY difficult setting in which to hear and understand much UNLESS everyone agrees to speak up, speak clearly and one at a time. And hearing people just won’t do this. They talk over each other and there is laughing and joking and one liners–and we just can’t understand speech through all that.
So what you are asking is beyond any hearing aid. To be sure, some hearing aids will help her hear more, but she will still miss a lot. The secret is to use assistive listening devices and have the microphone at the speaker’s mouth. Then she could hear clearly. But again, hearing people are loathe to pass a mic around–so typically that doesn’t work either.
What I recommend in that case is to clip a lapel FM microphone onto the person that does the most of the talking anyway (the life of the party). Then you mom can hear what that person says via a FM receiver she wears. Of course, if someone else is talking at length, then pas the FM mic to that person and your mom will hear them.
One cool little FM system that I use sometimes is the Comfort Contego that you can see/purchase at http://hearinglosshelp.com/shop/comfort-contego-personal-fm-system/ . Your mom can hear via her hearing aids and t-coils, or by wearing headphones or earbuds.
Cordially,
Neil
Thanks for you help, Neil. We’ll check out your suggestion.
Wow! I’m just in shock! Came across your article and for the 1st time in over 30 years, I feel normal again after reading your article. Everything mentioned above fits my loss to a T. I’ve gone through multiple hearing aids since I was 10 with no help whatsoever. It wasn’t til a few years ago I gave up and decided nothing will help. Audiologists kept turning up the lower frequency making it even more distorted. I’d go back week after week re-programming them. Only to realize I would be better off without them. I knew my hearing loss was opposite of others, but truly did not get clarity until you broke it down for me. I knew I could hear high pitched noises others couldn’t. We had a mouse in our garage so we used a device that plugged into the walls that used high frequency tones to scare them away. Every time I went near it I became nauseous and have a slight headache but never could explain why. Anyhow, thank you so much for this. I never leave comments but this was worth it. I hope one day the manufactures will see a need for low frequency devices. Until then, you gave me a great direction to start researching even more. Only one question for you that was not mentioned here. Any connection between tinnitus and lfhl? They tend to ring more around allergy season. So much to look into after this and it gives me new hope! Thank you!
Hi Amy:
Glad my article helped you. Few know much about the subject of Reverse-slope or low-frequency hearing loss.
I think tinnitus accompanies this kind of hearing loss just as much as it does with any other kind of hearing loss. Most people with hearing loss have tinnitus as a result. The frequency of the tinnitus may vary with the kind of loss. Typically, tinnitus is near the frequency of your greatest loss, so we should have low-frequency tinnitus, but in my experience we are just the opposite and have a very high frequency tinnitus.
Cordially,
Neil
Your writing is remarkably informative. I suffer normal hearing loss but my wife suffers from Reverse Slope. I’ve joined the Reverse Slope FB page and the group at Yahoo. Is there a fitter you can recommend in Las Vegas (our home) or Southern CA or the Northern AZ or Phoenix areas?
Hi Havard:
Thanks for the kind words
Sorry, but I don’t know of any really good audiologists regarding RSHL apart from Dr. Charles Berlin who is down in Florida.
I think your best bet is to take my article to an audiologist, and if the audiologist is willing to read it and follow the principles I give near the end, then work with that audiologist to get the best fitting. If an audiologist won’t even read it, you know they are not a good fit for you. They probably are fixed in their own ideas of what we need, and are not willing to listen to you or work with you. I’d run from them.
Cordially,
Neil
In most cases of reverse slope moderate to moderately severe hearing loss, wearing hearing aids works (especially RIC open fit/ little less effect but considerable if short mold half open used). Note: It is not good to use custom closed shell for tinnitus. I am wearer of RIC short semi-open mold.
I was referred to your article by an audiologist. I have seen several ENT doctors, even had hearing aids a number of years go. I have never found anything at all that actually helped with my loss.
Two weeks ago, my boss suggested that I “go get hearing aids.” Because I was being perceived as a bully by some newer nurses who thought I was ignoring them when they tried to start conversations with me. It echoed a common theme in my life, people who don’t believe I have a loss, and then say “Well why don’t you get hearing aids?”
I thought, well, maybe the technology is better. It has been 10 years. What the heck, I will try them. My experience in the past was that I would have to take the hearing aids out in the OR, or anytime someone was talking. So they were essentially useless.
I keep having to wipe my eyes as I read your article, because I am tearing up. Finally someone understands. I had a stapedectomy at the age of 17, I’ve had hearing aids, I have tried everything. My mothers entire family has similar hearing loss. Some worse than others, but about 80% of her family has some form of loss.
I have an appointment on next Thursday with an ENT and I am so hopeful, for the first time I can walk in the door knowing what the problem is.
Thank you so much!!
Hi Jessica:
Just remember that success with wearing hearing aids if you have a reverse slope loss is having the set the way I say in that article, NOT the way audiologists typically want to set them. So your audiologist needs to read the last section in my article about principles for setting hearing aids and use that as a base and then the two of you work together from there.
If your audiologist won’t follow those principles (because they “know” how to set hearing aids, dump him/her and find someone who will follow them. Almost no audiologist is an expert in setting hearing aids for reverse slope losses because they see so few in their whole career.
Cordially,
Neil
This is very frustrating, I admit. They all want me to just trust them, and drop a couple thousand for hearing aids. I don’t have a couple thousand to spend in the hopes they will get it right.
One audiologist says I need a fitted mold (tried those before and hated them) another says I need the pass through, but won’t program them differently than for standard hearing loss.
I would really like to get hearing aids, but I am gun shy about the expense after already having surgery and hearing aids that have failed in the past.
Hi Jessica:
When you have a reverse slope loss, you NEED to be fitted differently than people with ski-slope losses. The hearing losses are diametrically opposite. Any audiologist that doesn’t understand this shouldn’t be practicing. So you need to go to someone that is willing to work with you and set your aids the way you want/need them to be set.
My last hearing aids I got from Sam’s Club. I was told for years by audiologists that my low frequency loss was too great for open fit earmolds which I wanted, but the dispenser at Sam’s Club said he thought it would work for me–and surprise–it did. I got their aids and he even let me program them as it was faster for me to do it while he watched that for him to do it.
They are not perfect by any means–but neither have been any aids I’ve worn in the past 50+ years. However, I think they are the best so far.
Some nice things about Sam’s Club (and CostCo) is they are not high pressure outfits, they give you 90 days to try them out, they give you ALL your money back if they don’t work for you, they work with you to get the best fit and they don’t charge you an arm and a leg either.
Cordially,
Neil
I recently did the 23and me genetic testing, and it said that I am a carrier for nonsyndromic hearing loss and deafness, DFNB1 (GJB2 related)
Is this somehow what is causing my hearing loss? The test was only $200 and I would love to be able to that for my nieces and nephews and see if they are going to have similar problems. My nieces ENT doctor told my sister that her daughter was perfectly normal, but she has all the same symptoms I show (not able to hear you talking when the water is running, high pitched mickey mouse speech, etc) I just don’t trust the ENT’s word on this, and I know my son wasn’t diagnosed until he was 9.
Hi Jessica:
DFNB1 is a recessive gene. Thus, if you are a carrier only, you should not have hearing loss from this cause. This is because as a carrier, you only have one of the gene pairs needed to make hearing loss happen. In order to actually have DFNB1 related hearing loss, you would have to have BOTH of the gene pairs–one from your mother and one from your father.
By the same token, you alone cannot cause any of your children to be deaf. Any of your children can be carriers like you, but unless your husband ALSO has the DFNB1 gene, none of your children will have deafness as a result. But, as I said, there is a 50% chance that any of your children would be carriers. And when they grow up and marry, if they marry a person who is a carrier or has the deafness (both gene pairs) themselves, then there is a 50% chance your grandchildren will be deaf. However, if their spouse does not carry the DGNB1 gene, then there is a 50% chance that any of your grandchildren will be carriers like you are. It is interesting that 1 in 33 people are carriers of the DFNB1 gene.
As a result, the deafness (or hearing loss) may surface and then skip one or several generations before it surfaces again.
Note that hearing loss from the DFNB1 gene is present at birth and hearing loss does not get worse as you get older unless other factors interfere such as taking ototoxic drugs, expose your ears to loud sounds, the effects of aging (presbycusis) , etc.
So, if your extended family members don’t have any hearing loss when tested by an audiologist, you don’t have to worry about them getting/having hearing loss from this gene.
Cordially,
Neil
This is by far the best article written about RSHL. I too, refer to it many times. but it clearly explains what I go through on a daily basis. Although, I have been diagnosed, I still get backlash for not talking on the phone, or for not answering someone when they are talking to me. I appreciate this article so much, and wish I could hand it out every time I am in company with people.
Wow, this article really hit home with me. I was first told I had reverse slope hearing loss when I visited an ENT about 12 years ago after having several episodes of severe rotational vertigo. I went into the hearing test thinking my hearing wasn’t that bad and can still remember the feeling I got when I sat there waiting for the test on my right ear to start. It took forever to start hearing the tones. I had had trouble hearing since childhood, but it was the first time I learned I had an actual problem.
In my case, I have fluctuating hearing loss. I’ve had a few tests over the years. In some cases, the loss of hearing in my right ear is minimal. In that very first test, though, it was a pretty severe reverse slope dip most resembling Figure 3 Class 2 above. Which is strange because I’ve been told I probably have Meniere’s, which according to your comment above, doesn’t seem to fit (I also have tinnitus, pressure in the ear at times, and vertigo). I do know that stress plays a big part in how good or bad my hearing will be. I was dealing with a lot of stress when I took that first test so many years ago.
Anyway, the reason this article made so much sense to me is that even with my hearing loss, I also have hypersensitivity to certain sounds! For example, whenever someone sneezes or coughs at a certain frequency, it goes straight into my ear.
Hi Sunny:
You may still have Ménière’s disease, even though you have a more severe reverse slope loss. Ménière’s results vary all over the place. From everything you say, it looks like you do have Ménière’s disease.
If you’d like to get rid of your Ménière’s, a couple of years ago, I have discovered the simple cure for it. You can read about it in my article at http://hearinglosshelp.com/blog/atlas-adjustments-alleviate-menieres-disease/
Cordially,
Neil
Dr. Bauman,
My 30 year old son has a reverse slope hearing loss. He is looking for an audiologist in the Philadelphia area with expertise in hearing aid fitting for this type of loss. Might you have any recommendations?
Hi Cyndi:
Sorry, I don’t keep track of who fits what kind of losses. What I suggest is you ask your potential audiologist how much experience (and successes) they hhave had in fitting reverse slope losses.
Even if they haven’t had much experience, if they’re willing to read my article and follow the principles outlined in the section near the end, and work with your son to get the best fit, I’d say they’re a good person and work with them.
Cordially,
Neil
I am thrilled to have been guided to this information! I have been working tirelessly to reach a place of comfort with my hearing deficit since my diagnosis in 2014. I haveven Kaiser insurance which covers HearX Hear Usa audiologists. I have seen 5 AUD’S to date, spent an exorbitant amount of money, and not one has been successfulooking at setting me or mentioned the words “reverse slope loss”. I have felt so often that I was not taken seriously when voicing my discomfort and constant need to be seen yet again to reset my aids. I asked the AUD’S time and time again if they were sure they knew how to accommodate my type of loss. I would be assured each time like the last that they knew what they were doing and the one before them must not have. I am so tired of demanding to be seen by another that truly does not know how to handle my loss and once and for all would like to find someone who does.
Can you recommend a competent, educated, experienced AUD in the Orange County area?
Hi Cherity:
I can’t help you there–don’t know of anyone that is truly competent in reverse slope losses apart drom Chuck Berlin, but I think he’s retired or semi-retired now.
The way I’d do it is to print out my article and have your audiologist read it and follow the fitting principles in the section near the end. If they are willing to do that, work with him/her and slowly tweak the settings to get it right. If your audiologist refuses to follow the principles I set out and work with you, they don’t have a clue (and are proud of their ignorance) and you need to dump them and find someone who will.
Cordially,
Neil
Hi, Dr Bauman,
Thanks for publishing this great resource. My audiogram is reverse slope, but not as severe. Frequencies 2000 and below are all between 35 and 60dB.
Reading through your work makes me wonder if I am best served by just continuing to cope without hearing aids, considering that the special attention (and possibly price) required to get precisely what I would need (not to mention the headache in getting a hearing aid tech to understand all this and to cooperate given their prejudices) are so cumbersome.
Do you agree, or would you suggest something else? Normally for me it really is a matter of volume.
Thanks for any suggestions.
Hi Andy:
If all frequencies above 2000 Hz are normal, then you may find hearing aids are going to be a pain one way or the other. If you just need more volume (I agree with you), you could use an assistive listening device such as a PockeTalker and wear earbuds or headphones to hear in those situations where you have difficulty hearing. Turn the tone to where speech sounds the best. I find that the tone control is critical to best understand speech, once you have enough volume. You can get a PockeTalker at http://hearinglosshelp.com/shop/pocketalker-2-0/
Cordially,
Neil
Thanks very much for your wisdom!
In your reply to Andy above, you mentioned that “If all frequencies above 2000 Hz are normal, then you may find hearing aids are going to be a pain. . . .”
In my right ear, all frequencies 1500 Hz and above are normal up to 8000 Hz (at which it’s at 25 dB). However, I have some high frequency loss in my left ear (it goes from ten dB at 3000 Hz down to 30 dB at 4000 Hz, and continues dropping as the frequencies increase). Does this recommendation apply in this instance? Should I just get a hearing aid in my left ear?
I just realized my “left ear” description may have been misleading. To clarify: I have 40-ish hearing loss from 250-1500 Hz in the left ear, sloping up to 20 dB at 2000 Hz, 10 dB at 3000 Hz, and then dropping down to 30 dB at 4000 Hz.
I’ve thought before that I don’t really want one for my right ear anyway, and that way i’d only be getting one so it would be cheaper– however, at the same time, most places recommend you get two hearing aids for the purpose of better sound location ability.
Thoughts? Thank you. 🙂
Hi Elissa:
When your hearing is sort of on the borderline for getting hearing aids, its a tough call. Should you or shouldn’t you? The best way to make up your mind is to try wearing two hearing aids (or just one) for a month and see what you think. If they help you significantly, then get them. If they don’t, return them for a refund.
Note that many places just give you 30 days so if you miss it by one day, you can’t return them. Also many places charge a return fee of $250.00 or so per hearing aid. That gets expensive. What I’d do is go to a COSTCO store that has a hearing aid department and try them out there. Not only are equivalent aids up. to $2,000 cheaper, but you get 90 days to try them out and best of all, they give you ALL your money back if you return them. (Check to be sure this is all true before you sign on the dotted line.)
Cordially,
Neil
Hello
I came across this article after finding out that my Child 7 months now failed her newborn screen and ABR on the left side.They said her loss is conductive mild 30-40 in low frequencies <=1000 Hz and normal 20db in frequencies above.They v only recommended follow up so far.Please share ur experience with me growing up as I am totally scared as a first time mother if I am making her miss out in language and speech
Thanks
Ree
Hi Ree:
Your child has a conductive loss. Did the doctor/audiologist tell you the cause of this conductive loss? It could be as simple as her ears are clogged up and when they drain, hearing returns to normal. So it may not be a long term thing.
When a child has a hearing loss, you need to change how you talk to them. First, don’t expect her to hear/understand you from another room. So if you call her to come to you and there is no response, don’t assume she is “being bad”, but that she didn’t hear you–not that your child is at that age yet. But another thing to keep in mind is that if you can’t hear well, then you want to see. For example, a child with normal hearing can hear you moving around in another room and knows she is not alone in the house. However, with a hearing loss, she may not hear you, and so think she has been abandoned–say she is in the bedroom and can’t see you. Thus, she wants to see you, rather than hear you.
However, your child’s hearing is not so bad that she can’t hear such sounds. The big thing is to get close and face her when you talk to her so she hears words properly and thus picks up normal speech.
The way to talk to any hard of hearing person is to get close and face them so they can speechread you as well as have a better chance of hearing you. Just doing that will make an enormous difference.
Once you know the cause of her hearing loss and if it proves to be permanent, I can give you more specific pointers on helping her.
Cordially,
Neil
I wear Phonak B90-R hearing aids. My difficulty is with the extreme sounds of the long vowels in speech. They seem so extreme that I have to lower my volume or cringe. My audiologist can’t seem to find a balance to make these sounds sound more normal instead of sounds that sound loud when they are spoken normally and avoid screechiness at high levels of hearing.
Hi John:
To me it seems like you do not have the compression set properly BY FREQUENCY. Often, audiologists just set the compression for general speech–not by each frequency band. In your case, you need the compression set individually for each frequency so no sound can get over your most comfortable level and recruit. The compression needs to be set a few dB below where each of these higher frequency sounds recruit and thus become much too loud.
That’s the first thing I’d try.
Cordially,
Neil
so…I’ve been evaluated and approved for a CI at the University of Washington. But are the risks worth the outcome? Both surgeon and audiologist say I’ll hear better than I do now…but how much? That’s the question. I’m at 14% word recognition. Wearing the new Oticons OPN and like them. Struggle with conversation but in limbo as to whether to go forward with the surgery. Do well with t-coil if a female speaker or man who speaks slow and enunciates. Your take on getting a CI?
Hi Berne:
You are basically ready for a cochlear implant when your hearing aids aren’t significantly helping you anymore. 14% word recognition is pretty poor. So you don’t have much to lose if you get a CI in one ear. What I’d suggest is getting a CI in your worse ear (if you have one) and wear the Oticon OPN in the better ear and see how you do.
If you do great, then consider getting a CI in the other ear as well.
Since you have had a reverse slope loss, you may not find the success that other people with ski-slope losses have because CIs aren’t basically designed for those with reverse slope losses–they are designed to amplify the high frequencies too much, which is what people with ski-slope losses need, but typically not people with reverse slope losses.
Having said that, I know people with reverse-slope losses that did not do well at all with CIs, but I also know people that did great. So it is somewhat of a crap-shoot. That is why I only suggest trying a CI in one ear at first.
I think that the more extreme your reverse-slope loss, the less chance of having good results will be. But if you no longer hear the higher frequencies much anymore, or didn’t have an extreme reverse-slope loss in the first place, then I think it is worth the risk.
My hearing loss is severe enough that I’d be a candidate for a CI, but my word recognition is still way up there, and I get significant help from my hearing aids, besides I had an extreme reverse-slope loss, so in my case, it is NOT worth the risk–at least not at this time.
Cordially,
Neil
Hi Neil,
Very interesting article. I have moderate to severe reverse slope hearing loss. Currently wearing Phonak B90 R’s and struggling to hear speech in background noise. I can turn the HA,s down, but speech also decreases. Do you have any recommendations for increasing the volume of speech in noisy environments? Ps I wear open domes
Thanks
Hi Charlotte:
In noisy places I take my hearing aids off and either rely on speechreading or use my PockeTalker and super directional microphone. That works for me the best. For us, the secret to hearing in noise is to get your ears (or microphone) close to the speaker’s lips so the speech sounds are much louder than the noise level.
Cordially,
Neil
Hi Neil,
I was just wondering if you could recommend a volume and tone amplifying headset that I could use in a call centre role having reverse slope hearing loss. I have the clarity product you mentioned above , but I forced phone can only be plugged into it and not a headset with a mic
Thanks
Hi Charlotte:
If you want an external amplifier with great amplification, and including a six-band equalizer so you can adjust individually for low, middle and high frequency tones, and have a jack to plug in a headset (earphones with boom microphone), and works on any phone with the dial in the base, then the Speech Adjust-A-Tone is the gizmo for you. There are several varieties. The one you want is the second model listed, the HGA-MX. You can see it/order it from http://hearinglosshelp.com/shop/speech-adjust-a-tone-audio-telephone-amplifier/ .
Cordially,
Neil
Hi Neil,
Just ran across your article that describes my reverse slope hearing loss and wanted to share my story with you and others to let them know that they’re not alone, and that the current generation of hearing aids do help a lot, especially for me.
I’m in my mid-40s now, but have known about my hearing loss since I was in elementary school, when they did hearing tests on all the kids, but I was the one held behind afterwards for further testing.
My left ear is still relatively good and is where I get the best speech recognition, while my right ear is pretty bad with speech recognition, where I can hear words but they sound muffled. Both ears have reverse slope hearing loss.
I got my first hearing aid in high school for just the bad right ear, an in-the-ear analog hearing aid with a little dial to control volume. Imagine how I felt being a teenager wearing a hearing aid, in addition to having glasses and braces.
After I graduated from college and worked for a few years, in my mid-20s, I bought my first set of Widex completely-in-canal digital hearing aids, deciding to get a pair instead because when I only wore one analog aid, I felt this imbalance like being in an airplane where your ears don’t pop. These were a major improvement over the analog aid, but it was just the first generation of digital hearing aids. It was at this time that I learned about having the reverse slope hearing loss, having seen my audiogram results and realizing how different it was from my father’s audiogram. My father has a normal slope hearing loss, where his hearing loss resulted from working in loud environments and old age. Because of how my hearing has not changed much over the years, I’ve concluded that my hearing loss is probably congenital.
About 7 years later, the components in my Widex aids started to wear down and not perform as well, so I got my next set of digital hearing aids, GN Resounds this time, CICs again. I got this from a new audiologist, as my previous one had retired or closed up shop. I did spend a couple of years with my current audiologist before getting the hearing aids, trying to get him to understand my reverse slope hearing loss. When I asked him how many of his clients had a reverse slope hearing loss, he said I was the only one. After I spent about 2 years building up trust in him to understand my reverse slope hearing loss, then I finally decided to buy the GN Resounds. These worked great, but being completely-in-canal designs, I could only have one setting, and I had it programmed to maximize speech recognition, but it obviously also amplified ambient noise. I have been using these for the past 12 years, having components replaced every 3-4 years whenever they wore out.
In the past month, I finally decided to get a new pair, after 3 years of saving money in a Health Savings Account that my employer offered in my health plan. Opting for Starkey Muse iQs, which my audiologist said was the only completely-in-canal aids that allowed multiple settings, it also has some programming that helps high frequency sounds, which I don’t really need help at. I love the new multi-program settings for 4 different environments, which basically enables different levels of noise reduction to reduce ambient noise.
I’ve had these Starkey Muse iQs for a couple of weeks now. I’ll be going back to my audiologist in a couple of weeks in a follow up and will likely have him boost the power in the speech banana frequencies relating to the m, n, and l consonants, which are the letters I tend to struggle with in speech recognition. I still struggle with soft-spoken speech and strong foreign accents.
With these new aids, I can toggle to “car” mode to increase noise reduction so that the ambient road noise doesn’t overwhelm me anymore when driving, as my previous GN Resounds used to when it amplified everything with its one setting. I don’t know whether I can wear these on airplane flights yet, as that environment gets really loud.
Neil, thanks for your extremely thorough and informative article. My reverse slope hearing loss is not as extreme as those in your article, but it does accurately reflect my situation and makes me feel less alone in the world.
Hi Leland:
If hearing aids are programmed properly for your reverse-slope loss, they can work quite well in certain situations. I’ve always worn BTE hearing aids so I have more features available to me. My current aids have four programs just like yours have. This allows me to switch to whatever program works best in whatever situation I find myself in.
And if none really helps, I just take them off. In some noisy situations I hear better without hearing aids at all since everyone is yelling and I don’t hear the loud background noise like they do, so their voices stand out more to me.
Cordially,
Neil
Hi Neil,
I am extremely grateful for articles and amazed at your continued support and advice for people with hearing conditions all around the world. I am new to this and here is my story –
I am 39 year old Female, I work in a busy office, I use a headpiece for my phone, I always use this on my right ear and have done for years. About 5 weeks ago I notice when speaking to people the sound sounded different, kind of like an echo or vibration, just strange and not comfortable so I changed the earpiece to the other side and didn’t think any more about it, I didn’t believe I had an issue with hearing loss at the time, it just sounded funny.
Then 16 days ago I experienced what I thought was a blockage in that ear, I went to the doctor the next day to check my ears, he gave me antibiotics and said that I had no wax in my ears, he thought that my eardrum looked inflamed, he also gave me nasal spray, my situation didn’t improve and I developed tinnitus and then full blocked ear feeling (which I now know to be deafness) and dizziness, not flat out vertigo but attacks of up to a few minutes of head spinning, floor moving experience to a point where I had to sit down. I went back to the Dr on Friday and explained my condition had no improved, he then said it might be viral and gave me antihistamine but also gave me a referral to an ENT specialist, he said to contact in a few weeks if there was no change, this was on the Friday exactly 6 days after first noticing the blockage.
On the weekend it just got worse, the tinnitus didn’t stop and I experienced a 15 min dizzy spell after a run, I knew something wasn’t right so I booked the specialist on Monday and had an appointment on Tuesday morning, the specialist said I had no fluid or inflamed eardrum and gave me an audio, I will try and attach my results, he explained that I appear to have SSHL and explained that I need to take Oral Steroids immediately to give me a chance of saving my hearing, he prescribed me with 50 mg Prednisone to take immediately but only 25mg the next day until gone, sent me for a MRI the following week and basically said good luck I will see you in a week! After looking on the internet at other prescriptions for SSHL I was concerned my dose was too little so I contacted a top ENT Specialist in one of the best Hospitals in Sydney, he said come in immediately, I went in the next day to see him and was admitted to hospital for intravenous steroids and one intratympanic steroid injection under general antistatic. Strangely the day after I started taking the Oral Steriod’s 50gm on the Tuesday I began to see massive improvement in my tinnitus (it actually disappeared) and my hearing improved significantly and it did so for 2 days but after I came out of hospital on the Friday after the further treatment I started feeling the hearing loss come back and then the tinnitus started again and now 4 days later I have no improvement. I am still having dizzy spells and my hearing has not improved, I went from thinking that I was one of the lucky ones to realising that I think this is going to be long term.
I am just confused by a few things, firstly the first initial symptom of the hearing in that ear a few weeks back, if I have SSHL, what are your thoughts on me having this? Seems to indicate that this wasn’t sudden.
In your opinion, would you suggest that I go for further treatment, steroid injections? Have you heard of symptoms improving vastly like mine a few days after taking the oral but then getting worse? I seem to have levelled off now, I am on day 16 from first blockage.
I am completely paranoid that my good ear is going to go the same way, what do you think the chances are? Every strange noise in the good ear makes me feel scared but I think I am just over reacting because I am conscious of everything now, it’s just that this ear feel a bit clicky as well, I can create clicking sounds when opening my jaw. What I find interesting is that I seem to have RSHL which I understand is very rare, my hearing loss seems to be severe down to almost 70 in 500 Frequency zone, do you think it’s perhaps not SSHL and rather something else because of the first symptoms, it just confused me a little.
Thank you again for all your information and any other advise you have would be much appreciated.
Ange Broughton
Hi Ange:
I found your post in my spam box–hence the delay in answering you.
Sudden hearing loss is defined as a lost of at least 30 dB over three consecutive frequencies within 72 hours. That was not your case. I’d more describe your hearing loss as a rapid hearing loss over the past 2 months or so. So the things that cause true sudden hearing loss probably don’t apply to you.
From what you describe, my first thought is that you may have autoimmune inner ear disease (AIED). You have the symptoms and the typical time frame. Plus you had the sudden recovery with the steroids and then a relapse afterwards.
Rather than answering your questions directly, I’ll ask some of my own. Do you have any known autoimmune disease in your body. If you do, then AIED could be a distinct possibility. Often, you can get it if you already have another immune system problem.
Another possibility given your symptoms is Meniere’s disease. Meniere’s often starts out with a low frequency hearing loss and the balance problems and the tinnitus and feeling of fullness in you ear. It typically occurs some years after trauma to your head such as whiplash from a car accident or something similar.
It gives you periodic episodes of the above symptoms and they subside inbetween. So this is another good possibility.
Read my articles on AIED and Menierer’s and see whether either one rings a bell with you and fits your situation. If one does, then you know what the treatment is for it.
You can read my article about AIED at http://hearinglosshelp.com/blog/autoimmune-inner-ear-disease-aied-2/
You can read my comprehensive article on Meniere’s disease at http://hearinglosshelp.com/blog/atlas-adjustments-alleviate-menieres-disease/
Let me know what you think after you have read these article and considered your situation.
Cordially,
Neil
Hey Ange,
I have the exact same symptoms as you.
I have a blood test and MRI scheduled for this weekend and have just started oral and steroid injection to the ear drum.
Are your ears OK?
What happened since the episode?
Do let me know. Thx!
Hi Neil,
I am extremely grateful for articles and amazed at your continued support and advice for people with hearing conditions all around the world. I am new to this and here is my story –
I am 39 year old Female, I work in a busy office, I use a headpiece for my phone, I always use this on my right ear and have done for years. About 5 weeks ago I notice when speaking to people the sound sounded different, kind of like an echo or vibration, just strange and not comfortable so I changed the earpiece to the other side and didn’t think any more about it, I didn’t believe I had an issue with hearing loss at the time, it just sounded funny.
Then 16 days ago I experienced what I thought was a blockage in that ear, I went to the doctor the next day to check my ears, he gave me antibiotics and said that I had no wax in my ears, he thought that my eardrum looked inflamed, he also gave me nasal spray, my situation didn’t improve and I developed tinnitus and then full blocked ear feeling (which I now know to be deafness) and dizziness, not flat out vertigo but attacks of up to a few minutes of head spinning, floor moving experience to a point where I had to sit down. I went back to the Dr on Friday and explained my condition had no improved, he then said it might be viral and gave me antihistamine but also gave me a referral to an ENT specialist, he said to contact in a few weeks if there was no change, this was on the Friday exactly 6 days after first noticing the blockage.
On the weekend it just got worse, the tinnitus didn’t stop and I experienced a 15 min dizzy spell after a run, I knew something wasn’t right so I booked the specialist on Monday and had an appointment on Tuesday morning, the specialist said I had no fluid or inflamed eardrum and gave me an audio, I will try and attach my results, he explained that I appear to have SSHL and explained that I need to take Oral Steroids immediately to give me a chance of saving my hearing, he prescribed me with 50 mg Prednisone to take immediately but only 25mg the next day until gone, sent me for a MRI the following week and basically said good luck I will see you in a week! After looking on the internet at other prescriptions for SSHL I was concerned my dose was too little so I contacted a top ENT Specialist in one of the best Hospitals in Sydney, he said come in immediately, I went in the next day to see him and was admitted to hospital for intravenous steroids and one intratympanic steroid injection under general antistatic. Strangely the day after I started taking the Oral Steriod’s 50gm on the Tuesday I began to see massive improvement in my tinnitus (it actually disappeared) and my hearing improved significantly and it did so for 2 days but after I came out of hospital on the Friday after the further treatment I started feeling the hearing loss come back and then the tinnitus started again and now 4 days later I have no improvement. I am still having dizzy spells and my hearing has not improved, I went from thinking that I was one of the lucky ones to realising that I think this is going to be long term.
I am just confused by a few things, firstly the first initial symptom of the hearing in that ear a few weeks back, if I have SSHL, what are your thoughts on me having this? Seems to indicate that this wasn’t sudden.
In your opinion, would you suggest that I go for further treatment, steroid injections? Have you heard of symptoms improving vastly like mine a few days after taking the oral but then getting worse? I seem to have levelled off now, I am on day 16 from first blockage.
I am completely paranoid that my good ear is going to go the same way, what do you think the chances are? Every strange noise in the good ear makes me feel scared but I think I am just over reacting because I am conscious of everything now, it’s just that this ear feel a bit clicky as well, I can create clicking sounds when opening my jaw. What I find interesting is that I seem to have RSHL which I understand is very rare, my hearing loss seems to be severe down to almost 70 in 500 Frequency zone, do you think it’s perhaps not SSHL and rather something else because of the first symptoms, it just confused me a little.
Thank you again for all your information and any other advise you have would be much appreciated.
Hi Ange:
My first thought on reading your account is that you are experiencing your first attack of Meniere’s disease. You have all the symptoms, plus you have a low frequency hearing loss which is often the way Meniere’s begins.
With Meniere’s, you get periodic attacks and go back to normal between episodes except your hearing typically goes in a stepwise fashion–two steps down, one step back, then next episode two steps down and one step back. So hearing is on a downward slide. Tinnitus may go away or get less between episodes and gets worse just before an episode begins.
The good news is that if you have Meniere’s,although no doctors kow the cure, I do. I wrote a comprehensive article on Menier’s which you should read. You can see it at http://hearinglosshelp.com/blog/atlas-adjustments-alleviate-menieres-disease/ .
Once you have carefully read it, see if it rings any bells with you. If so, try to find an upper cervical chiropractor to help you. If you are in the UK, you may have problems finding one.
Cordially,
Neil
Susac syndrome is another cause of reverse slope hearing losses. I’m fact low frequency hearing loss is one of the three main symptoms of this disease.
Hi Anthony:
Thanks for the information on Susac syndrome. I’ve updated this article to reflect this information.
Cordially,
Neil
Thanks Neil. As a Susac sufferer myself, I try to get the word out about this rare disease as much as possible. I also really enjoy your writing.
You mention wide-band hearing aids amplifying up to 16khz in this article. I wonder how these worked for you over standard hearing aids and if such a thing is still available?
Hi Anthony:
I don’t think they worked like they could have if I had worn them when I was younger as by the time I got them, my wonderful high-frequency hearing was dropping due to presbycusis. Furthermore, the technology of the time was limited in how much you could specifically adjust them for my hearing loss.
I really don’t know whether there are any current aids that go that high, but the trend is to make hearing aids that amplify higher frequencies than was done in the past.
Cordially,
Neil
I suspect that I also have RSHL but have not begun to research audiologists in my area because I also read how rare it is and I do not want to jump through a bunch of hoops to get to the right AUD that has experience with RSHL and spend a bunch of money out of pocket finding that person until I know for sure that they can help me. I’ve always known that I have a hearing problem, but did not know how bad until my early 20’s when after getting married my husband’s arrival home would scare me quite often because I couldn’t hear him come in the door until he slammed it. It is still that way today. I saw an AUD in my early twenties(I am now 42) and she said my “loss” was from birth and that traditional hearing aids wouldn’t help me much. She was right! I tried an in-ear hearing device and as you described it was AWFUL! Everything high pitched was deafening and I made the mistake of trying it out at a family gathering at a restaurant since those are the settings that I struggle with the most with many people talking, etc. It was so bad, I had to take them out during the meal. Ironically, although I have a difficult time hearing low sounds, the high pitched sounds are overwhelming to me at times. I struggle in places with a lot of noise to the point of feeling the need to excuse myself outside for a few minutes to decompress! I would LOVE to find an AUD in my area(Austin, TX or San Antonio) that is experienced with RSHL. My teenage kids are so tired of me asking them to repeat things all of the time 😦
Hi Brandy:
My wife would scare me when she came home from work if I wasn’t facing the doorway when she suddenly “appeared”. We came up with a solution. She wouldn’t get close to me or speak until she flicked the light in the room I was in to get my attention. Then I’d turn around to see what was the matter–and there she was. There’s lots of little tricks you can use to prevent being startled when you can’t hear.
Another one I use all the time is that in my office I always place my desk in a corner–but facing into the room so I can clearly see all doorways. That way I have walls to my back and thus no one can approach me without being in my direct line of sight. This works wonderfully well for me. My current office has 4 doorways, but I can see them all while I am working at my desk. This gives me peace of mind.
Good luck on finding a truly knowledgeable audiologist for RSHL.
When wearing hearing aids, if you have RSHL, you’ll find the higher-pitched sounds become extremely loud. I have had this problem with my hearing aids all my life. Part of the problem is that most (maybe all) hearing aids are designed for people with high-frequency loss. They are unable to handle the compressed dynamic range that we with RSHL need without reducing our discrimination.
Teach you kids how to speak to you–you know the routine–first, get close–like in your face, second, get your attention before uttering the first syllable, third speak up a bit and fourth, speak slower and clearly. That makes all the difference in the world.
Since it is hard to find an experienced Audiologist, the next best plan is to find one who is willing to work with you and is willing to learn. Too many of them think they know it all–how to program a hearing aid for a person with RSHL–and they don’t need you or me or anyone else to tell them how. So they do it wrong. Those that will read my article and then try to apply the principles I outline and work with you are gems. I think you’ll get the best results from them.
Cordially,
Neil
Hoping you might be able to make sense out of the situation I am facing with my young daughter.
She passed her newborn screen, but was slow to speech and clumsy but not excessively so in either instance. At 18 months, I pushed for additional testing and speech therapy. OAE testing referred on both ears, but ABR showed sensoneural HL in right ear only. Testing in the sound booth led her doctors and audiologist to finally diagnose her with mild to moderate, low frequency, conductive hearing loss in her right ear. As for the shifting diagnosis from sensoneural to conductive, they said they trusted the results from the sound booth.
She had tubes placed in both ears and now wears a hearing aid in her right ear. At 3 (wearing hearing aid for about 5 months now), her speech is improving but she struggles with high frequency sounds. Her speech therapist was surprised to learn that it was low frequency tones that she could not hear as well — she had assumed the opposite based on her speech.
I know that as she gets older we will get better feedback from her, but I feel the information we have now is contradictory. Is this a case of her hearing aid simply not providing the benefit I was hoping for or are there tests I should be pushing for to ensure her diagnosis is accurate? Any other thoughts?
Hi Angela:
I wouldn’t put as much faith in earlier testing as later testing. As a child gets older, the results get more and more accurate.
However, based on what you said, and mother’s know their children better than audiologists ever will, if you say she is having trouble with the higher frequencies, I’ll bet she is. I’d go to a different place to have her tested again (get a second opinion) and see what her latest audiogram shows. You don’t want her aids set to amplify lows if she really needs highs, or maybe she needs both if she has a reverse cookie-bite audiogram (high in the middle and low at both ends). That’s a hard hearing loss to fit. That’s what I’d do. Then you can compare the results–or email be copies of both audiograms and I’ll tell you what I think.
Cordially,
Neil
Hi Neil. Great article. Just wanted to get your opinion on whether or not my hearing loss is genetic or could be noise induced. I only have hearing loss in my left ear I also have tinnitus in that ear. I believe it is noise induced, the tinnitus started Immediately after firing a shot gun round with no protection and I went to the ENT after that to see if there was anything that could be done and found out about the hearing loss. I never noticed any hearing issues prior to that. My hearing loss has stayed the same for the past 5 years from the time I damaged it. my mother is deaf in one ear and hard of hearing in the other but I’m not sure which type of loss she has. She has told me she lost her hearing while going through puberty. My loss is mild to moderate reverse slope hearing loss with the worst hearing dropping to 50 at 1k. I just haven’t seen any other instances of rshl from noise damage. Thanks for your time and all the work you do.
Hi Justin:
From what you say, I wouldn’t be surprised if it is noise-induced. A genetic loss isn’t going to just show up after you fire a shotgun. (You may notice it then and blame it on the shotgun. For example, when my mom was a little girl, she got knocked down and run over by a streetcar. After that they noticed she had a hearing loss and attributed it to the streetcar incident, but in reality she had a genetic loss all along and they just noticed it at that time–when she was about 5 which is when a lot of parents notice their kids have hearing loss. The proof that it was genetic is that I inherited that gene and have hearing loss–and my parents noticed it when I was 4 or 5 but it also was there all along.)
If you mom lost her hearing going through puberty, there is a good chance that she has otosclerosis. If she lost more hearing when you were born, that would almost certainly indicate it was otosclerosis.
For the milder kinds of reverse slope losses, it is hard to tell whether it is genetic or from other factors. Once you get the extreme reverse slope losses, it seems to always be due to genetics.
Cordially,
Neil
Hi! I live in NY and I am looking for a top audiologist in the field of RSHL that I can take my son to to make sure he is fit properly with his hearing aids. If anyone can recommend someone id appreciate it
Thanks so much for this informative blog, which I’m returning to often as I try to address my sudden binaural low frequency loss, which emerged mid Oct 2018 and helped lead to my diagnosis of Susacs. My hearing hovers at around 50 until about 1500 when it leaps up to 10-5 at 2000 and then stays about there for the higher frequencies. A couple of questions
(1) In your “Fitting Hearing Aids to People” section you talk about using “non-occluding ear molds” so that higher frequency sounds aren’t blocked. This youtube video (https://www.youtube.com/watch?v=_f-EjaAUf-k) by “Comparing Hearing Aids” (?) suggests the opposite, suggesting that in the eye models decrease the chance of over-amplification, though still tells you to look for an aid that “eliminates muffled sounds that sometimes accompany in the ear hearing aids”–any thoughts for someone with my hearing?
(2) How is compression being used in your report? I tend to think of compression as making louder noises softer (with expansion as making softer noises more audible), but you seem to suggest that compression can actually transpose a lower frequency into a higher one. How does this relate to your discussion of multi-channel digital hearing aids (and how many channels does a person need and what is actually programmed when you program a frequency–its volume?)
(3) Finally something like tinnitus appears to really influence what I can make out. It’s the low, white noise variety (so not extraordinarily annoyed except that it seems decrease my hearing!) and is especially loud at conferences (in large ballrooms, etc), in the car, and when I’m outside. I understand this type isn’t something you’ve had to deal with: any suggestions about whom to ask.
Again, many, many thanks and best wishes!
Liz
Hi Liz:
1. First, the Comparing Hearing Aids YouTube has a number of errors of fact in it. Thus, it is not credible. For example, it states, “Hearing loss is often classified by frequency, either high or low. Low frequency hearing loss, also known as conductive hearing loss, results from damage to the outer or middle ear.”
This is total nonsense. Hearing loss is classified as sensorineural or conductive or mixed. Hearing loss can also be classified as low-frequency loss, flat loss or high-frequency loss. You CANNOT say that low-frequency losses are conductive. Some may be, but I dare say most cases of low-frequency (reverse-slope) losses are sensorineural. Certainly the more extreme cases are.
How you treat conductive losses is different than how you treat sensorineural hearing losses. With conductive losses, you basically amplify the missing frequencies as the inner ear is still working properly. However, with sensorineural hearing losses, especially reverse-slope losses, you DON’T just amplify the missing frequencies. If you do, a person can’t stand it, and can’t understand speech. You need to modify which frequencies you amplify–not the lows, but mostly in the mids, but at the same time you must NOT block the highs as I point out in my article.
2. You are mixing up two completely different things–loudness compression and frequency compression.
Loudness compression is what you are thinking of. But frequency compression is another animal. Actually, the term “frequency compression” is wrong because you physically can’t compress a frequency. What they really mean is “frequency transposition” where you move sounds from one frequency to another but keep their relative ratios the same–so you recognize it as understandable speech–think changing Darth Vader’s voice to Mickey Mouse’s voice. Both are understandable, but they use different frequencies when you do this.
For people with reverse-slope losses, they don’t hear Darth Vader well or at all, but they certainly hear Mickey Mouse–so transposing low-frequency sounds (which they don’t hear) to higher-frequency sounds which they hear very well can work. Unfortunately, because so few people have sensorineural reverse-slope hearing losses, there isn’t any financial incentive to produce such hearing aids. However, the reverse is not true–there are hearing aids for people with ski-slope losses that transpose sounds down to lower frequencies where they can hear them–think of Mickey Mouse now sounding like Darth Vader. The vast majority of people with hearing losses have sensorineural high-frequency losses, so these hearing aids are financially viable.
As far as the number of channels you need, most hearing aids have 32 or 64 channels and that is adequate in my opinion. You are correct, you program the amount of amplification for each channel.
3. I think what you are experiencing is reactive tinnitus where your tinnitus gets louder in unison with certain frequencies of sound. You can read all about it in my comprehensive article on the subject at http://hearinglosshelp.com/blog/reactive-tinnitus/ .
Cordially,
Neil
I am in Phase 3 now (63 years old) and benefit from cochlear implants, but this article describes my life and difficulties perfectly. I had many doubters and nay-sayers over the years. This article validates my life and hearing history.
PS – when you live in a tropical locale, it sucks to be able to hear mosquitoes!
i am 63 and the 5th child of a large family that most of us have some type of hearing loss. my mom had reverse slope ( document ed back 4 or 5 generations) and dad had ski slope. i am in the process of checking out the newest hearing aids, but after reading this i realize it is more important to find a hearing professional that understands what they are dealing with.
Hi Jim:
You are right–programming an aid correctly is more important that the specific brand you select.
I’m curious what kind of a hearing loss you have. If you married a reverse-slope loss to a ski-slope loss, theoretically you’d end up with a reverse cookie-bite loss.
Cordially,
Neil
I was just diagnosed with reverse slope hearing loss in one ear and ski slope in the other. My mother and all of her seven siblings and her father became severely hard of hearing as they aged. We always thought our father had exceptional hearing, but now that I have studied up on reverse slope hearing loss, I suspect he may have had reverse slope hearing. Until my audio test, I thought my hearing in my left ear was excellent, albeit with some peculiar twists. This is the ear with reverse slope hearing loss.
Hi Paula:
Put your two ears together and you should have normal hearing. LOL
When you have great high-frequency hearing, it is hard for people to realize that you can be very hard of hearing at the same time. So being able to hear faint high-frequency sounds really fools people, hearing professionals included. Such are the joys of having a reverse-slope hearing loss.
Cordially,
Neil
I am ecstatic to have found this article! I am now 54 years of age and have had a lifetime of trouble with hearing aids (finally had a hearing test at age 12). I have cookie bite hearing loss at 250 it reads 35, 500-2k is 65, and slopes up at 4K to 0, at 8k it is back down to 30.
Do you have any specific comments or advice to pass along to an audiologist? You have inspired me to try hearing aids again, perhaps if they are programmed properly, they will actually help.
Hi Paula:
Your audiologist needs to read this article, particularly near the end where I give the principles for programming hearing aids for people with reverse-slope losses.
Another thing you can do IF you have an android phone is join the beta testing for a new kind of hearing aid that is being designed for people with reverse-slope and cookie bite losses. They are looking for people for beta testing right now–I just heard from the head honcho yesterday. Here is what I posted on another list.
______________________
Hi everyone:
If you’re like me, you have longed for a hearing aid that would really help you hear and understand speech when you have a reverse slope hearing loss, rather than struggle to understand speech in many situations and at the same time hope background sounds won’t be so loud that they hurt.
Here’s some good news. A new company, Cocha Labs, is designing a hearing pod that focuses on giving you clear speech no matter what kind of hearing loss you have, rather than slavishly focusing on meeting your target volume by frequency which, as we all know, doesn’t work well for us.
Amy, the founder and CEO at Concha Labs knows a lot of our struggles as she has a very significant cookie-bite hearing loss which is also hard to fit. I got excited as she was telling me about the hearing pods they have designed.
At this point, they are looking for people with RSHL and other weird hearing losses to be beta testers for their new hearing pod and patented Soundscope technology that drives the system. It doesn’t matter whether you currently wear hearing aids or not.These hearing pods, which look like BTE hearing aids, are adjusted via your cell phone so you need to have a cell phone to try them out.
At this time they only need beta testers for Android phones. Those of us with iPhones will have to wait for a bit before they are ready for iPhone beta testers.🙁
If you want to try out and test this cool new technology and see how well it works for you, visit https://www.conchalabs.com or email them at hello@conchalabs.com.
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So that is another option if you have an android phone. The iPhone beta testing will come later. I want to get in on that too since I have an iPhone.
Cordially,
Neil
I’m about positive I have Class 2. My last test result the only range I was in the normal was 6 and 8Khz. After reading this article the causes don’t really fit anything, no childhood illness or Meniere’s etc. Unsure about the genetic cause. My loss didn’t really start til late 30’s. I don’t recall if it was sudden so I was just curious if there is any data supporting workplace noise exposure as I had one job that was noisy, no hearing protection was mandatory. Because my hearing loss does not fall into the workplace exposure profile – reverse slope the claim I have put in for was denied.
Hi Steve:
It’s possible that workplace noise caused your hearing loss, but just hitting the low frequencies without affecting the high frequencies is not likely. This is because the high frequency hair cells are at the base of the cochlea where the sound signals enter the cochlea. Thus, they bear the brunt of the noise damage.
The most likely cause is genetic in my opinion, but it seems that most low-frequency genetic hearing losses occur at birth–and don’t just show up once you are an adult.
It’s also possible that a drug may have cause a low-frequency hearing loss–but again, this isn’t common. Most drugs start at the highest frequency you can hear and work their way down the frequency spectrum.
Cordially,
Neil
Thank you so much for this article. My daughter is 3 months and was initially just diagnosed with severe loss in her right ear. They did a 2nd ABR on her left ear and was diagnosed moderate loss at 500hz, mild at 1000hz and normal at 2000 & 8000hz. Our audiologist were very confused by her results and didn’t always have answers to our questions. We put a hearing aid on the reverse slope ear.
This article gave us some understanding as we search for a cause.
Hi Katie:
Probably the cause is genetic since she is that young. But don’t waste too much time of the cause unless the treatment will be different due to the cause. Otherwise, just accept it and treat her as you would any other child–except with special attention to her hearing needs.
Cordially,
Neil
Hello, hope you can help. I live in the UK and I have tried many times through my audiologist to be tested for reverse slope hearing loss. After reading about rshl via your site I was rather taken back as everything I read related to my deafness including the audiogram. I am totally deaf in 1 ear and have 20% hearing in my other ear. When I have tried to push for a test for rshl I was told by my audiologist that I can’t have rshl as I only have slight deafness in the 1 ear? I feel fobbed off, frustrated and wondering what your opinion is on this? Your advice would be very much appreciated. Many thanks
Hi Emma:
You don’t specifically test for RSHL. You take the standard hearing tests and your audiogram will reveal whether you have a reverse slope hearing loss or not. If you send me a copy of your audiogram attached to an email (my email is at the bottom of every page on this website) I can tell you what kind of hearing loss your have.
Does your good ear have 20% hearing loss or 20% hearing? You can’t have both.
The best way for me to answer your questions is for you to attach your audiogram to and email and fire it off to me.
Cordially,
Neil
Good Afternoon Neil,
I’ve just finished reading your article which was an absolute delight and a breath of fresh air. I’ve been looking for papers on RSHL for some time and rarely have I found any as accurate and understanding as your article is. I am in my mid-twenties, I work as a hearing wellness advisor in the UK and have mild-moderate RSHL. I have seen several audiologists over the last 16 years who all seem to have very little understanding on RSHL and how to fine-tune hearing aids effectively. Is there any advice or tips you could give to help me with this? I work very closely with audiologists who are qualified to fine-tune but they themselves are a little puzzled by RSHL, like many. Absolutely any advice would be greatly appreciated and if by sending my AG would help, let me know.
Kindest Regards,
Georgina
Hi Georgina:
Thanks for you kind words regarding my reverse-slope article.
As I mentioned in the article, few audiologists have much experience and knowledge for properly fitting hearing aids to a reverse-slope loss.
My advice/tips on how to properly fit hearing aids to a RSHL is found near the end of this comprehensive article.
Did you also listen to Don Schum’s 15 minute discourse on fitting RSHL? The link is in the section “Fitting Hearing Aids to People with Reverse-Slope Losses” or you can go directly to it at https://vimeo.com/59940134 .
The sources I have quoted from are the best I can find.
If you want to send me your audiogram via my email address (at the bottom of the page), I’ll give you my opinion on how to best fit to it.
Cordially,
Neil
There is also another short article I wrote at https://hearinglosshelp.com/blog/adjusting-hearing-aids-for-reverse-slope-losses/
WOW!! I cannot believe that I have just found your article!! Thank you for this life changing information. I have programmable Beltone hearing aids that I have just stopped wearing. I have found that all of the constant low tone noises that I don’t typically hear(fans, appliances, etc.) have my attention and confuse me. I made an appointment with my audiologist to try to tweak the programing as you have outlined. THANK YOU SO MUCH!
Hi Neil! I am hoping you can help point me in the right direction and will try to keep this as brief as possible.
I am a 33 year old female who has been suffering from several health issues… that are non specific with no known cause.
Over the last 6 months, I noticed my hearing had changed with tinnitus and left sided feelings of fullness. I finally saw an ENT who diagnosed me with “Bilateral Symmetrical Low Frequency Sensorineural Hearing Loss” which he explained was exceedingly rare and he guessed was genetic… but could not give me any genetic leads and wasn’t really interested in helping.
I feel like this new diagnosis paired with the health issues I’ve had the last several years can’t be a coincidence and I am desperately looking for answers.
Before falling ill, I was a singer… so this new development hits rather hard.
Do you also believe this is genetic or perhaps autoimmune? Any input or suggestions would be appreciated.
Hi Kaitlin:
Are you taking or have been taking any drugs/medications in the past year? So many drugs can cause hearing loss, tinnitus and a feeling of fullness in your ears so I like to rule that out at the start.
Also, if you have a copy of your audiogram, would you send it to me (neil at hearinglosshelp.com) so I can see exactly what your hearing testing showed. Also in the email, tell me about your health issues so I can see whether I think they are coincidences or not.
Often, reverse-slope losses are genetic, especially if they are bilateral. Furthermore, they usually develop by the time you are 6. Other kinds of genetic hearing losses can occur more from your age to 45 or so.
Do you have any autoimmune system conditions? If it was autoimmune related, I’d expect you to also have another autoimmune system disease.
Cordially,
Neil
Thanks so much for this. I developed sudden onset “all the bass vanished” hearing loss in my left ear, with bad tinnitus in same ear. as a result of a hearing damage incident in Summer 2021. Long story short: I had normal hearing. I soaked 1 hr in a salt bath spa with no earplugs and walked around with salt residue deep in both ears and couldn’t hear so well. With the salt clogged ears, I was exposed to a damaging noise and had literal pain and ringing in left ear. As the pain calmed down i realized music all sounded wrong, realized my entire lower frequency was gone.
The tinnitus recently got worse and it is now gently pulsing in time with heartbeat. I’m about to go in for an MRI in case there’s something bodily going on (vascular or other).
So it appears I have a rarity within a rarity: hearing loss that is reverse slope, and (seems to have been) caused by injury and not genetics. If you recommend anything in particular for me to ask the audiologist or look into, I would welcome the guidance. Seems like nobody is familiar with my situation, even the professionals.
Hi Paul:
I take it that your left ear was facing the loud sound so it got the brunt of it? And that is why your right ear is ok?
Has your ringing sound gone now and was replaced by the pulsatile tinnitus?
I’d be interested in seeing your audiogram and tympanometry if you’d send me a copy–just scan it and attach it to an email to me. My email address is at the bottom of this (and every) page on the Center’s website.
Since this happened 3 years ago, I think the hearing loss is permanent.
I assume they looked in your left ear canal and your eardrum is ok, and hopefully so are the 3 bones in your middle ear. Tympanometry should show that.
After I have looked at your audiogram, I may have some suggestions.
Cordially,
Neil