Hearing Loss Help  

by Neil Bauman, Ph.D.

A concerned parent wrote:

Our six-year-old daughter was recently diagnosed with LVAS. Our doctor tells us she has already lost all useful hearing in one ear, so we are obviously very protective of her good ear. We are now struggling with the right balance on activity restrictions. She is an enthusiastic swimmer on our community’s summer swim team. The swimmers are taught to enter the water using a racing dive. We understand that diving is not recommended, but we could use some help with the fine points.

It’s clear to us that she should stay off of the high dive, but what are the risks associated with diving from a low diving board? What about a racing dive from the side of the pool (used in competition)? Are the pressure changes in the ear the main concern (which wouldn’t seem like a risk with a racing dive) or is there a significant risk of hitting the water wrong and causing head injury?

Also, any advice you have on whether or not participation in gymnastics (mat activities like tumbling, not a high balance beam) would be reasonably safe would be very helpful. We are erring on the side of caution right now and not allowing it, but our daughter is doing somersaults and cartwheels in the grass anyways. She may well be safer if she is taught to do them properly and practicing on a better padded surface.

It is interesting that so far in her life, the various “trauma” incidents she has already experienced have only caused your daughter to lose her hearing in one ear, but not the other. To me, this indicates that her good ear is more robust, or not as affected by trauma as her bad ear. This is good news. Perhaps her LVAS will not affect the hearing in her good ear in the future either—but there are no guarantees—hence the need to still be cautious.

As far as pressure is concerned, since your daughter has LVAS, she should only shallow dive—in other words, not let her head go more than 5 or 6 feet underwater. This keeps the pressure change to a minimum and should not aggravate her LVAS which could cause more hearing loss.

When she dives, whether on a diving board or a racing dive, if she has her hands over her head, which breaks the force of the water hitting her head, there shouldn’t be any “trauma”. Thus, as far as I can see, this should be ok.

In regards to doing gymnastics, I’m with you. She should learn to do it right and avoid trauma to her head. If she does not hit her head, or flip with such force that internal pressure builds up in her skull, she should be ok as I see it.

As always, there are no guarantees. If any activities result in hearing loss, those (and kindred) activities are out unless she wants to risk further hearing loss in the future. However, if any activities do not produce any hearing loss (or balance issues), then you should let her continue to do them. In other words, don’t unduly restrict her unless/until that activity (or similar activity) is proven harmful to her ears.

by Neil Bauman, Ph.D.

A mother explained:

My son has had a few bouts of tinnitus followed by a hearing drop in one ear. This always seems to be as a result of, or following, strenuous exercise. So far the ear has always recovered to its old level. I reported the last episode to my doctor who has now raised the question that it could be “labyrinthine hydrops.” The drugs that he recommended we consider are Betahistine or Cinnarizine, but the side effects seem severe. Up until now, these episodes only occur when my son has congestion, and this has thrown even more questions into the pot! I’d appreciate your advice.

Strenuous exercise can raise the internal pressure in the head, just like a blow to the head can. If your son has large vestibular aqueduct syndrome (LVAS), this can cause hearing loss. This is nothing new. Actually, it doesn’t even have to be exercise—any form of extreme straining can cause this in people with LVAS if they are susceptible. Fortunately, not all people with LVAS are susceptible to this.

It’s great that his hearing comes back after each episode. Of course, there are no guarantees that his hearing will always return, but it seems you can go by your son’s previous history—which is, it is a temporary hearing loss.

It could be labyrinthine hydrops or anything else, but if your son has LVAS, that would be the most likely cause. Maybe your doctor considers LVAS to be a form of labyrinthine hydrops?

Personally, I’d not take either Betahistine or Cinnarizine if I were in his shoes. To me, the solution is much simpler—just don’t strain so much when exercising. He should be able to find the level below which this doesn’t happen, and then limit himself to that much straining effort.

Incidentally, labyrinthine hydrops is an inner ear condition, whereas congestion is a middle ear condition.

If his hearing loss only occurs when he is congested, then it could be that the strenuous exercise is causing “gunk” (to use a fancy medical term), to clog up his Eustachian tubes and middle ears causing some degree of conductive hearing loss. When the gunk drains out, his hearing returns to normal. If this is the case, it has nothing whatsoever to do with LVAS.

One way to determine which is which is to go to an audiologist and have an audiogram done as soon as he loses some hearing from straining. If the audiogram shows only a sensorineural hearing loss, then it is LVAS (or another inner ear condition). If it shows a conductive loss, it is likely gunk in the middle ear or Eustachian tube. If he already has a sensorineural hearing loss from LVAS, and it is gunk in his middle ear, the audiogram should show an air-bone gap indicating the conductive component. Once you know what the audiogram shows, then you’ll better know how to proceed.

by Neil Bauman, Ph.D.

A mother asked:

My daughter has bilateral LVAS and is begging to play an instrument. Do you have any advice or experience with what instruments are safe for children with LVAS to play?

Some people with LVAS (Large Vestibular Aqueduct Syndrome, sometimes called EVAS—Enlarged Vestibular Aqueduct Syndrome) are very sensitive to changes in air pressure. For them, even blowing on a brass instrument (trumpet, trombone, tuba, etc.) can result in further hearing loss. For these people, any musical instrument that does not require them to blow is safe in that regard.

As far as I know, hearing loss from people with LVAS playing musical instruments is quite rare. Therefore, playing a woodwind or brass instrument probably won’t affect her hearing.

Fortunately, there is a way you can figure this out. It is simply this. If your daughter’s hearing is affected by changes in air pressure (low or high pressure systems moving in rapidly, by going up or down hills in vehicles, by flying, or exerting or straining herself—running very hard, pressing weights, etc.) then the exertion needed to blow a brass instrument will also likely cause a hearing loss.

However, if the above things do not affect her hearing, then it is highly unlikely that playing a woodwind or brass instrument would affect her hearing either.

In short, if you’ve watched what activities have caused hearing loss in your daughter in the past, then those and similar activities will likely cause more hearing loss in the future if she takes part in them. Other than that, you shouldn’t unduly restrict her activities. You should be able to make a good judgment call on this issue by applying the above to her situation.

Finally, one caution, and this is for everyone, not just people with LVAS, the volume of sounds in bands and orchestras can be dangerous to your hearing. Therefore, it is wise to use ear protectors to keep the sounds down to an acceptable level and thus preserve your hearing. There are special musicians’ ear protectors that reduce the volume without affecting how you hear pitch. Many professional musicians wear them. You would do well to do the same.

by Neil Bauman, Ph.D.

A man explained:

I came across your blog post regarding LVAS and hearing loss, and was looking for assistance or more information.

I am a 48 year old with profound sensorineural hearing loss in both ears. I’ve worn aids for 10 years and had many medical and hearing specialist exams but no explanation of the cause. It keeps getting worse, but mysteriously seems to vary, becoming milder, or more severe, on a weekly basis. I’ve never been able to correlate it to any food or activity.

Recently, I took a vacation during which I paused my regular and typically strenuous aerobic and anaerobic exercise activities. That week, my hearing was much better. Upon return to my normal exhausting exercise, I’m having one of the worst hearing weeks ever. This clued me to the possible correlation between hearing loss and exercise. Your article is the only one I find tying the two together. Is there any treatment or test to verify if this is the cause?

The suggestion I fear is ‘give up exercise’. I play competitive tennis, and train extensively for it. It is a lifelong passion. Making that sacrifice would be a drastic step, but at least I’d like to understand if that could be an answer to a hearing problem that is now affecting my ability to earn a living and support my family.

This is not a well-known subject, so you may have to do your own experimenting. When you stopped exercising for a week and your hearing returned to whatever degree sounds like a positive correlation to me. In order to prove or disprove this theory, do the same thing again. Stop exercising for a week and see if the same thing happens. If so, you know that for you, at least, strenuous exercise negatively affects your hearing.

In order to find out why this is happening, I’d suggest you have an MRI or CT scan specifically looking to see whether you have Large [or Enlarged] Vestibular Aqueduct Syndrome (LVAS). Be sure to have them measure the vestibular aqueducts and give you the results in mm. Some doctors just eyeball them and say—yup, you got LVAS. That is not the way to do it.

As you read in the above-cited article, some people with LVAS lose hearing due to strenuous exercise. You may be one of these. If that is the case, don’t think you have to give up all exercise. What I’d suggest is give up strenuous exercise—where you really exert yourself—cut it down to moderate exercise for a week and see what happens to your ears. What you want to do is cut down the exertion in the exercise to below where it causes hearing loss. Once you find that point, then stay below it if you want to preserve your hearing.

Straining to run faster, lift more, etc. increases your internal body pressure, and this is what causes the problem if you have LVAS. Thus, exercise that doesn’t substantially increase your internal pressure should be OK.

Once you have tried the above and see what the results are, then you can decide whether you want to protect your ears (and to what level), or continue with competitive tennis (and at what level). Perhaps there is a happy medium that will meet both needs.

by Neil Bauman, Ph.D.

A concerned mother asked:

My daughter is 9 years old and she has been hearing impaired since she was about 2½. She has bilaterally enlarged vestibular aqueducts, and she also has only 1½ turns on her cochlea. My question is, do you think it would be safe for her to go into the mountains in Colorado? We live in Illinois and want to drive to Colorado, but I wasn’t sure whether the high altitude would affect her hearing. She already has severe loss in her left ear and moderate to severe loss in her right ear. I don’t want to take any chances with her hearing!

I can understand your dilemma. There are no guarantees that the air pressure changes in the Colorado mountains won’t affect her hearing. However, you can make an educated guess and then act accordingly. Here’s how I would do it.

First, look at her previous hearing history. If her hearing hasn’t changed much, or at all, since she was 2½, then it is unlikely that the change in air pressure will affect her now. However, if every little bang on her head has affected her hearing, that would be different.

Second, if sudden changes in air pressure (fast moving storms, or going up or down hills) cause hearing problems, then the change in altitude could do the same. But if there haven’t been any problems so far, then she will probably be fine.

Third, has she flown before? If so, did it cause any hearing problems? If not, then driving in the mountains in Colorado wouldn’t be much different than flying in a pressurized plane as they pressurize a plane to somewhere below 8,000 feet or so.

Children with LVAS who have stable hearing for a number of years have likely experienced all of the above in the past, and thus aren’t likely to be affected by similar activities in the future, including enjoying the wonders of the high mountains in Colorado.

by Neil Bauman, Ph.D.

More and more children are being found to have a hearing loss due to what is called Large (or Enlarged) Vestibular Aqueduct Syndrome (abbreviated to either LVAS or EVAS).

LVAS is passed on from generation to generation by recessive genes. That much researchers know. How many genes are involved in LVAS? No one knows.

So far researchers have now traced the gene thought to be responsible for LVAS to a location on chromosome 7q31 in a region that overlaps the area containing the gene responsible for Pendred syndrome (PDS).

As I wrote back in 2002, “This suggests that different mutations in the PDS gene can cause a variety of related conditions ranging from nonsyndromic recessive hearing loss (NSRHL) with enlarged vestibular aqueducts (basically LVAS) to the severe Mondini deformity and Pendred Syndrome. Some people with Pendred Syndrome have fluctuating hearing loss similar to that observed in people with LVAS.

Another theory is that a separate gene responsible for LVAS may exist close to the Pendred gene, and that mutations in both are required for full Pendred syndrome, whereas a mutation in only one of these genes may result in hearing loss associated with a variety of cochlear abnormalities.” (1)

The truth is, many more genes may be involved in LVAS than the one or two related to Pendred syndrome that have been discovered so far.

Did you know that of the approximately 20,000 genes in the human body, “more than 4,500 genes participate in the development and maintenance of the human inner ear, making it the most genetically complex organ in the human body.”

Researchers also know that hundreds of genes are involved in hereditary hearing loss, leading to syndromes such as Waardenberg, Usher and Pendred.” (2)

From the above, it is obvious that researchers still have a long ways to go in order to identify all the genes that go into making up LVAS. I say this because hearing loss (and balance problems) do not seem to correlate with the size of the vestibular aqueducts or anything else so far. In fact, some people don’t have an hearing loss at all in an ear with LVAS. Thus some critical information is still missing.

Until then, and with so many unknown variables related to LVAS, it will continue to be very difficult to effectively diagnose and manage LVAS.

(1) Large Vestibular Aqueduct Syndrome (LVAS)by Neil Bauman, 2002.  

(2) In “Advance for Audiologists” (July 22, 2008) by Jess Dancer, Ed. D.

 by Neil Bauman, Ph.D.

A mother asked:

Do you or don’t you restrict your child’s activities due to their LVAS (Large Vestibular Aqueduct Syndrome)?

When my daughter (almost 12) was diagnosed with unilateral LVAS at age 8, the ENT said to avoid loud music (to protect her normal ear), scuba diving, and head trauma.

I have found information saying kids should avoid diving (which my daughter does and enjoys), roller coasters (which my daughter has ridden and loves), doing “headers” in soccer (which she practiced at a recent soccer clinic), contact sports like basketball (which she played for the past 3 years), and playing the trumpet (which she is supposed to start next fall). She is very active.

I am questioning whether I should try to convince her to change the instrument she has selected to play in band next fall. We had a band night where they could try all the instruments, which she did, and she loved the trumpet. The teacher said she was a natural and had done better than any incoming 6th graders he’d heard that night. So naturally we signed her up to play trumpet. But I don’t want to put her hearing at risk either.

Also I’m wanting to know if I should stop her from diving off the diving board this summer, not sign her up to play soccer next fall, and limit her at Six Flags to only non-roller coaster rides. On the other hand, she’s done these activities in the past, and her hearing in her LVAS ear has remained pretty stable. I would hate to limit activities she really enjoys.

You are not alone in trying to determine which activities your LVAS child should or should not do. Many parents of children with Large (or Enlarged) Vestibular Aqueduct Syndrome (LVAS/EVAS) often agonize over this very question.

Fortunately, if a child has a history of LVAS for a few years such as your daughter has, the question is relatively easy to answer—no matter what the doctor gives as guidelines. Let me explain.

All of the above activities have the potential to cause hearing loss in people with LVAS. But just because there is the potential doesn’t mean it will happen. You have to consider each child on a case by case basis.

Since your daughter has been doing a number of these activities for some time, all you have to do is ask yourself, “In the past few years, have any of these activities caused more hearing loss or balance problems in my daughter?” If the answer is “no”, then let her continue to do these (and related kinds of activities), because obviously her ears are not sensitive to these kinds of activities.

However, if your daughter lost more hearing or had balance problems each time she did one of the above, then obviously these activities are not the right ones for her, and you should restrict her to those activities that have not caused hearing loss/balance problems in the past.

In your daughter’s case, you’ve answered your own question. These activities haven’t bothered her in the past, so let her do them in the future—unless you subsequently find that they really are damaging her ears.

by Neil Bauman, Ph.D.

A lady asked:

To have Large Vestibular Aqueduct Syndrome (LVAS), both parents have to have the gene, right? And their parents before them have had to have the gene? I come from a big family, and no other relative has had a hearing issue. If it is inherited, I would think you would see it more in the family history. I’ve started to tell family members about my son’s diagnosis and that it is an inherited gene. They look at me like I have lost my mind! They all say they don’t know of anyone that has a hearing problem in the family.

First, remember this is a recessive trait—so yes, both parents have to have at least one gene of the gene pair to give the child LVAS. However, one parent could have one gene from one of their parents who could have one gene from one of their parents, etc., and LVAS would never show up until finally someone in the family (in this case you) married a person who also carried the LVAS gene.

Second, your son has a mild to moderate hearing loss. Maybe there really are some in your family that also have LVAS but have such a mild hearing loss no one was ever aware of it.

Third, we don’t have a clue how many people are walking around with LVAS and never have a hearing loss—we only know some of those that do have the hearing loss. Until they test the general population for LVAS, they’ll never have a handle on just how often it causes hearing loss vs. not causing hearing loss.

Thus, there are 3 possibilities why your son has LVAS (which is passed genetically) and a hearing loss although it doesn’t appear to be in your family.

This mother concluded, “I’m beginning to believe that I caused my son’s hearing loss because of something I did,”

You are right. You did! You married his father didn’t you? But that wasn’t a bad thing, was it?

by Neil Bauman, Ph.D.

A mother wrote,

My daughter has LVAS [Large Vestibular Aqueduct Syndrome]. My husband and I have not had genetic testing done to determine whether we are carriers. Am I to assume that since my daughter has LVAS, so will my son?

No—unless you both also have LVAS. The fact that your daughter has LVAS indicates that you both are carriers of the LVAS gene(s). Since you are just carriers, then there is a 25% chance your son will have LVAS, a 50% chance he will be a carrier, and a 25% chance he won’t carry the gene(s) at all. At least that is how I understand it. (I’m sure it is more complicated than the above because they don’t even know all the genes involved in LVAS yet—but it should give you a good general idea.)

Also, remember this is the average in a large population. That said, it IS possible you could have 4 kids in a row with LVAS, or have 4 kids in a row without LVAS—but on the average the 25, 50, 25 rule holds—just like you can flip a coin and get 4 heads in a row or 4 tails in a row—but it is not likely.

Just because you already have a child with LVAS doesn’t make succeeding children more (or less) likely to have LVAS. each child has the same 25/50/25 percent chance of having LVAS independent of whatever has occurred before with your other children.

To learn more about LVAS, read this article.

 by Neil Bauman, Ph.D.

A lady wrote:

We just visited our ENT for my son’s biannual hearing test. My husband asked the doctor if he could do another cat scan. He told the doctor we wanted to make sure whether my son had LVAS or not because we want to fly. My doctor told us he has other patients with LVAS and most of the parents take their children on flights. So, to reassure us that it is perfectly fine for our son to fly, he told me I should join a support group and talk to other parents to see what their opinion is. Thus, I decided to join your Large Vestibular Aqueduct Syndrome (LVAS) list because the reality of not flying is starting to sink in.

Your doctor gave you good advice. Few doctors know much about LVAS, but there is a wealth of collective wisdom on the LVAS list.

Here are two rules of thumb.

1. If your child is not affected by rapid pressure changes—such as from driving up or down a mountain, or when a fast-moving high or low pressure weather system moves in, or diving down to 6 feet or so underwater, or blowing on a woodwind or brass musical instrument, then the chances of him having ear problems related to LVAS and flying are almost nil.

2. In order to know what will likely happen in the future, you have to look at your child’s past history. If none of the situations in rule 1 (above) apply, try a flight and see what happens. If he doesn’t have any problems, then you can likely fly to your heart’s content without any problems in the future.

A lot of kids with LVAS do indeed fly, and with no harmful side effects I might add.

We did an informal survey on our LVAS list some months ago. Nineteen people responded that they had flown with a child with LVAS. Of the 19 that have flown, only 2 experienced a hearing loss which may have been related to flying.

The first child lost her hearing 12 days after flying and experienced a permanent loss. The family was unable to identify another reason for her loss such as head trauma or a virus. However, this child had flown twice before with no apparent loss.

The second child was fine on the way to the destination, but suffered a loss on the way home after transferring planes (in a high altitude location—Denver—flying to a low altitude location—Seattle). She suffered a “vestibular attack” (i.e. eyes squeezed shut, wanting to lie prone, vomiting nonstop). An audiogram a week later indicated a drop of 15 dB. Prior to this trip her hearing was progressive in nature. This child had also flown prior to this incident with no apparent hearing loss.

As you can see, most kids with LVAS can fly with no hearing side effects, and even those that did experience side effects had flown before without any problems.

Thus, you do not need to unduly restrict your LVAS child from flying unless past history indicates it is not a wise choice.

If you would like to learn more about LVAS or join the LVAS on-line support group, go to http://www.hearinglosshelp.com/articles/lvas.htm.