by Neil Bauman, Ph.D.
© April, 2012
Question: My child has been diagnosed with Large Vestibular Aqueduct Syndrome (LVAS). Exactly what is LVAS; what causes it; and more importantly, what can I do about it?—A. M.
Answer: Large Vestibular Aqueduct Syndrome (LVAS), also commonly known as Enlarged Vestibular Aqueduct Syndrome (EVAS), and rarely “Vestibular Aqueduct Syndrome” (VAS), is a fairly recently-diagnosed condition. I’m not surprised you haven’t heard of it before. Not a lot is known about it yet but researchers are finding out more and more as time goes on.
Vestibular Aqueduct? What Is That?
The vestibular aqueduct is a narrow bony canal (aqueduct) that runs through the skull, connecting the inner ear (vestibule) to the cranial cavity-hence its name.
Running through this bony canal is a membranous “tube” called the endolymphatic duct. Like other parts of the inner ear, the endolymphatic duct is filled with a fluid, appropriately called endolymph. This duct then courses through the bony vestibular aqueduct and dumps into the endolymphatic sac, which lies between the inside of the skull and the membranes that cover the brain.
Endolymph normally flows away from the organs of the inner ear and towards the endolymphatic sac. In LVAS, the endolymphatic duct and sac are much larger than normal. This enlarged size potentially allows the endolymph to flow from the endolymphatic sac back into the hearing and balance organs.
Back in 1978, this syndrome was dubbed the “Large Vestibular Aqueduct Syndrome” (LVAS) because only the large vestibular aqueduct part of this syndrome showed up on the CT (computed tomography) scans.
Researchers using high-high-resolution magnetic resonance imaging (MRI) techniques can now actually see the endolymphatic duct and sac. They have discovered that when the vestibular aqueduct is enlarged, so too is the endolymphatic duct and sac. It is the enlarged endolymphatic duct and sac that cause the hearing problems, not the large vestibular aqueduct itself. Therefore, some researchers have changed the name of this syndrome to better reflect this reality. They are now calling it “Large Endolymphatic Duct and Sac Syndrome” (LEDS). In addition, some researchers think that LVAS is really just a mild case of Pendred Syndrome (PDS) but I think further research is disproving this. (Pendred Syndrome is characterized by sensorineural hearing loss, goiter and an abnormal perchlorate discharge test.) Whether you call it LVAS or LEDS or EVAS or just VAS, you are talking about exactly the same condition.
You might be surprised at just how small the normal vestibular aqueduct is. At the half-way point, the diameter normally ranges somewhere between 0.5 mm and 1.4 mm and averages 0.8 mm. Back in 1978, researchers Valvassori and Clemis defined LVAS as a vestibular aqueduct with a diameter greater than or equal to 1.5 mm at the midpoint. In some cases of LVAS, the vestibular aqueducts can be as large as 8 mm, although the average is around 3.7 mm.
Then, in 2008, after further investigations into the size of the vestibular aqueducts in children with hearing loss, Dewan, Wippold and Lieu revised downward the size of vestibular aqueducts regarded as “enlarged”. This new criteria is now called the Cincinnati criteria. The Cincinnati criteria defines LVAS as either vestibular aqueducts that are 0.9 mm or greater at the midpoint of the aqueduct, or vestibular aqueducts that are greater than 1.9 mm at the operculum (opening at the larger end).
This, of course, means that many more people have LVAS than was formerly the case. For example, in one study of 130 children with cochlear implants, the Valvassori criterion identified 16% of the vestibular aqueducts as “enlarged” whereas when using the Cincinnati criteria, 45% of the vestibular aqueducts were found to be “enlarged”, a 281% increase.
Note: before 2003, CT scans were done using a 1.0 mm slice, while now they use a 0.6 mm slice. The 1.0 mm slice gave insufficient resolution to accurately measure the vestibular aqueduct to meet the Cincinnati criteria.
As a baby is developing in the womb, the vestibular aqueducts are short, straight and broad. After birth, they continue to develop and change their shape until they reach the normal adult form, usually when a child is about 3 or 4 years old. By that time, each vestibular aqueduct is about 10 mm long and shaped like an inverted “J”.
Because the vestibular aqueduct is one of the last parts of the inner ear to reach maturity, it is most vulnerable to developmental damage as a baby grows.
Inner Ear Deformities and LVAS
Large Vestibular Aqueduct Syndrome (LVAS) is one of a group of deformities that results from this abnormal or delayed development of the inner ear. LVAS is not congenital like doctors originally thought. They now know it occurs sometime after birth and is the result of abnormal postnatal or early childhood development. Recent reports indicate that people who have LVAS are not born with a hearing loss, but merely are predisposed to the development of a hearing loss.
There are many deformities of the cochlea and vestibular system. LVAS is often associated with many of them. In one study, various cochlear deformities were present in 76% of the ears that had LVAS. In this same study, there were also vestibular (balance) abnormalities in 40% of the ears with LVAS. This makes LVAS one of the most common inner ear deformities seen in people with sensorineural hearing loss that occurs in early childhood.
Originally, LVAS was thought to be a variant of the Mondini type of inner ear deformity where the cochlea only develops 1½ turns instead of the normal 2½ turns. Now, researchers recognize LVAS as a completely separate clinical condition.
LVAS may be associated with Mondini’s Syndrome or it may occur by itself. This proves that the resulting hearing loss is caused by the large vestibular aqueduct and not by any other abnormalities.
Diagnosing LVAS
Large Vestibular Aqueduct Syndrome may result in sudden, fluctuating, or progressive sensorineural hearing loss. It is defined as the combination of the clinical presence of sensorineural hearing loss in a child and the identification of the large vestibular aqueduct on a CT scan or the MRI identification of the large endolymphatic duct and sac.
The gross appearance of the CT scan of the inner ear often appears normal. Therefore, doctors need to precisely measure the diameter of the vestibular aqueduct in order to correctly diagnose the presence of LVAS.
In the past, LVAS was felt to be one of the most commonly overlooked causes of hearing loss in children. Even today, many audiologists are unaware of LVAS or do not suspect it, in part due to the presence of a conductive component in the resulting hearing loss. In fact, a fluctuating hearing loss and a conductive component often accompany LVAS and are important audiologic findings when diagnosing this syndrome.
If a drastic change in hearing has occurred in a child following a minor heard injury, change in barometric pressure, or physical exertion, the cause may very well be LVAS.
CT scans are the preferred diagnostic tools of doctors. Credible CT and MRI scans now give the doctors an objective way to test for LVAS. As a result, parents can be informed ahead of time that their child’s hearing could suddenly deteriorate. This gives them a chance to make realistic educational and therapeutic decisions. Most importantly, they can take precautions to try to avoid further hearing loss.
How Common Is LVAS?
Doctors estimate that LVAS occurs in at least 1% to 1.5% of people with sensorineural hearing loss or balance problems. More specifically, some studies have found that somewhere between 5% and 7% of the people with sensorineural hearing loss of unknown causes really have LVAS.
Just because you have a large vestibular aqueduct, doesn’t mean that you will also automatically have a sudden hearing loss. In one study, the incidence of sudden hearing loss among people with LVAS ranged between 11.8 and 19.1%. In another study, the incidence figures were much higher, occurring in up to 60.9% of the people with LVAS. This means that there are a lot of people that have a large vestibular aqueduct, but do not have the associated sudden hearing loss.
It’s in the Genes
Researchers are now discovering that abnormal or delayed development of the inner ear is often caused by genetic defects. In one study, 39% of the cases of LVAS occurred within families indicating that sensorineural hearing loss associated with LVAS is an inherited recessive trait. They have now traced the gene thought to be responsible for LVAS to a location on chromosome 7q31.
More specifically, researchers now know that the LVAS gene lies in the 1.7cM interval between the flanking markers D7S501 and D7S2425. Interestingly, this region overlaps the region containing the SLC26A4 gene responsible for Pendred syndrome (PDS), which was identified recently.
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.
It appears that there are a number of genes, as yet unidentified, that may be associated with LVAS. In one study of children with profound hearing loss, 12 were found to have mutations of either the Connexin 26 or Connexin 30 genes. Using the Cincinnati criteria, 8 of the 12 were identified as having LVAS while 4 had normal vestibular aqueducts.
Thus, LVAS may be associated with Pendred Syndrome or the Connexin 26 or Connexin 30 genes (and who knows how many other syndromes), or it may occur independently from such syndromes. Much of what constitutes LVAS remains a mystery at this time.
Characteristics of LVAS
Large Vestibular Aqueduct Syndrome has a number of characteristics-some of them rather unusual.
1. In routine CT scans, LVAS is found to be a relatively frequent inner ear anomaly among people with sensorineural hearing loss of unknown cause.
2. Hearing loss associated with LVAS is acquired in the years following birth. It is not congenital as was previously thought.
3. Hearing loss is commonly associated with LVAS. For example, in one study of 33 ears with LVAS and no other inner ear deformities, 31 of the 33 ears had sensorineural hearing loss. Eight of these also had a conductive loss. However, the sensorineural hearing loss was the most predominant component. Over time, 65% of the ears had progressive hearing loss.
4. On the average, hearing loss is greater if you have LVAS and do not have any other cochlear deformity.
5. In people with LVAS and no other cochlear deformities, hearing loss is generally much greater in the higher frequencies than in the lower frequencies.
6. If you have LVAS without any other cochlear deformities, your ears are subject to sudden drops in hearing. This does not appear to be the case if your ears have associated deformities such as Mondini’s Syndrome. In these cases, you may have a progressive hearing loss instead. Therefore, it is important to differentiate between cases where the large vestibular aqueduct is the only inner ear malformation seen on CT scans and those with associated cochlear and/or semicircular canal abnormalities.
7. The hearing loss caused by LVAS is primarily a sensorineural hearing loss. However, because other abnormalities are often present, there may also be a conductive component as well. One study indicated that 90% of the time hearing loss was comprised of both sensorineural and conductive losses although the sensorineural hearing loss was the most predominant. As a result, an air-bone gap exists to some degree in almost all people with LVAS. Incidentally, the conductive component can easily be misinterpreted as a middle ear ventilation problem or a type of otosclerosis.
8. LVAS in both ears (bilateral) is much more common (80 to 91%) than LVAS in only one ear (unilateral).
9. If you have LVAS and also injure your head or neck, you may develop Post-traumatic Meniere’s Syndrome.
10. Sudden hearing loss has been observed frequently among children with LVAS. This is one of the important points differentiating LVAS from Meniere’s disease. With Meniere’s disease, the majority of people are middle aged; with LVAS, they are young children. Incidentally, the vestibular aqueducts of people with Meniere’s disease are often rather small. In people with LVAS, they are much larger than normal.
11. The fluctuating sensorineural hearing loss of people with LVAS does not resemble the low frequency loss characteristic of Meniere’s disease. Unfortunately, most health care professionals still associate fluctuating hearing loss with just Meniere’s disease. The hearing losses in LVAS are basically flat or down-sloping high frequency losses.
12. LVAS generally follows a characteristic pattern. People with LVAS hear normally during the first year or few years of life. Progressive sensorineural hearing loss may not develop until the teenage years. Hearing loss usually occurs in early childhood, less commonly in adolescence, and occasionally in adulthood. In one study all but 2 people had hearing loss in early childhood (< 5 years old). One developed hearing loss at 17 and another at 45. In both of these latter cases, the hearing loss was precipitated by head trauma.
13. Another important characteristic is that, most people with LVAS experience sudden hearing loss following a minor head injury or other activity which causes increased intra-cranial pressure (increased cerebrospinal fluid pressure). In one study the figure was 85.7% while another study reported 61%. A mild bump on the head, tripping or falling down or even jumping can jar the head enough to result in more hearing loss. Also, sudden hearing loss can follow a minor illness such as a common cold (although I think this is more likely related to viral activity than to LVAS), strenuous exercise or a sudden change in barometric pressure.
In one study of 12 children with LVAS, five had sudden hearing losses. Three of the episodes followed relatively minor head injuries. The fourth occurred while forcefully playing a trumpet, and the fifth occurred immediately after an airplane flight.
In another case, a six-year-old child ran into a goal post while playing soccer and hit his head on the left side. This resulted in sudden hearing loss in the left ear. Three years later, after mild head injury while on the school playground, the child again reported an immediate hearing loss, this time in the right ear. An audiogram done that same day revealed severe to profound hearing loss in the right ear and profound hearing loss in the left. A CT scan revealed bilateral large vestibular aqueducts with no other inner ear abnormalities. Over the next month, hearing fluctuated between moderate and severe.
After episodes of sudden hearing loss, hearing may recover to the previous level, or much more commonly, it may recover partially to a new “normal.” One girl with LVAS had characteristic attacks of sudden hearing loss following minor head trauma, common colds or exercise. Her high frequency hearing is now almost non-existent but her low-frequency hearing tends to recover shortly after each episode.
14. Hearing loss in LVAS generally follows a step-wise pattern. Each incident causes the hearing to drop another step. The hearing loss will follow one of two patterns. One is a gradual progressive stepwise hearing loss, but with fluctuations. The other is sudden bouts of hearing loss of at least 15 dB that may fluctuate to profound levels following minor head injuries or fluctuations in the pressure of cerebrospinal fluid (CSF). Usually these acute drops in hearing do not recover to previous levels. The end of this downward progression typically is profound hearing loss.
15. If you have LVAS, not only can you experience episodes of sudden hearing loss, but these episodes can also result in damage to the vestibular (balance) system in your inner ears. That is why you may also commonly experience vertigo, balance problems or other symptoms of disequilibrium in addition to sudden hearing loss. In people with LVAS, tests of inner ear balance function are usually abnormal. Approximately one third of the people with LVAS have a history of vertigo. In one study, complaints of dizziness were present in 29% of the cases.
How Does LVAS Cause Hearing Loss?
How does LVASL cause hearing loss? The short answer is, “No one knows for sure.”
The exact role that the endolymphatic duct and sac plays is still uncertain at present. However, some researchers feel they are somehow responsible for regulating endolymphatic pressure within the inner ear. They think the endolymphatic sac could serve as a reservoir for endolymph and, due to its capacity for water absorption, may have a pressure-regulating role. This is because the endolymphatic sac has a surprisingly high protein content, giving it a markedly higher ionic content in relation to the ionic concentration of the endolymph contained in the remainder of the inner ear. Other doctors think it could be the site for active ionic exchange of endolymph with the cerebrospinal fluid.
Doctors do not yet know exactly how LVAS results in hearing loss. In fact, there is doubt whether LVAS actually causes hearing loss itself, or whether it is just a marker for another underlying process that causes the hearing loss. They have proposed several theories and one or more of them may ultimately prove to be true.
One theory is that a sudden increase in cerebrospinal fluid pressure from a minor head injury or other activity could have compressed the dural envelope surrounding the endolymphatic sac thereby forcibly pushing the hyper-concentrated fluid in the endolymphatic sac back through the dilated duct in the large vestibular aqueduct and into endolymphatic circulation. This hyper-concentrated endolymphatic fluid then damages the hair cells of the cochlea, resulting in sudden hearing loss.
The reason this does not normally happen is because the inner ear is buffered from any rapid intracranial pressure changes by the narrow vestibular and cochlear aqueducts. When the vestibular aqueduct is larger than normal while the cochlear aqueduct remains normal in size, any rapid fluctuation in cerebrospinal fluid pressure from minor head trauma causes temporary force imbalances across the cochlear partition. This shearing action could cause damage to the delicate inner ear hearing structures such as the organ of Corti or stria vascularis as well as damaging the vestibular (balance) structures.
Another theory suggests that inner ear fluid movements or pressure changes caused by a relatively minor head injury cause a tear or rupture in an area of congenital weakness in the delicate basilar membrane or Reissner’s membrane in the inner ear, causing permanent damage to the hearing structures.
In support of this theory, the results from one study suggest that hearing loss and vertigo attacks in people with LVAS may be caused by a rupture in the membrane separating the endolymph and perilymph. This is the same mechanism that is thought to occur in Meniere’s disease and in perilymphatic fistulas.
What Can You Do?
Since LVAS is caused by a genetic defect, you can’t just make it go away. You have to live with it. However, there are things that you can do to help your child cope with LVAS.
Prevention
If your child has LVAS, one of the most important things you can do in managing this syndrome is to take the necessary steps to prevent additional hearing loss from occurring. This is especially important if your child has LVAS in both ears.
Doctors recommend avoiding head trauma as it can cause hearing loss to progress faster. There are two exceptions to this rule. One situation is if your child has LVAS in both ears and your child’s hearing has already progressed to a profound loss so that there is little hearing left to lose. The other situation is if your child only has LVAS in one ear and the hearing loss has already reached profound in that ear. In these two situations, since there is nothing left to lose, there is no need to further restrict your child’s activities.
Apart from the above, if your child has LVAS, it may be prudent to restrict your child’s activities. Since minor head trauma can cause episodes of sudden (and sometimes irreversible) hearing loss, your child should avoid contact sports such as wrestling, football, rugby, soccer and hockey, for example. Baseball, basketball and cycling may be okay, although there still is risk. (In spite of LVAS, your child needs to have a life. Therefore in situations like these where there is the possibility of head trauma, your child should always wear head protection.)
Other activities that your child should avoid include things like bumper car rides, roller coasters, dirt bikes, skate board jumping, bungee jumping, radical/strenuous exercise, weightlifting and diving. In addition, you want your child to avoid activities that involve large changes in barometric pressure such as scuba diving. Even playing certain musical instruments is not without risk if they require high expiratory pressures. For example there is a report of a person with LVAS who had a hearing loss resulting from playing a trumpet.
At the same time it is important not to unduly restrict the activities of children with LVAS. They need to be allowed to take part in as many activities as possible having regard for their LVAS. Based on the experiences of hundreds of children with LVAS, here is a good way to tell whether your child can participate in any given activity. All you need to do is look at the child’s past history. Activities that have caused hearing loss in the past will likely cause hearing loss in the future if the child participates in them. It’s that simple.
Some children are so sensitive to LVAS that minor head bumps result in hearing loss. Other children with LVAS can run full tilt into the goal post and not have any resulting hearing loss, even though the episode resulted in concussion! Therefore, the only reliable indicator for your child is his/her past history.
Fortunately, most children are not unduly sensitive to head trauma and changes in air pressure. For example, only a handful out of hundreds polled had hearing loss after flying in commercial planes. Therefore, you can most likely let your child fly—unless he/she has a history of hearing problems from flying.
One doctor wrote a letter to the school on behalf of one child with LVAS. I thought it fairly presented the risks of LVAS and a plea not to unduly restrict childhood activities (see yellow box below). However, ultimately it is the parents who decide those activities in which their child will and will not participate.
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To whom it may concern: XXX has large vestibular aqueduct syndrome (LVAS). This is a congenital anomaly of the inner ear which predisposes him to hearing loss from what can be fairly minor head trauma. In theory, any degree of head trauma can do this and thus to completely protect him would require parents to preclude all physical activity. This is an unreasonable demand for any child. Thus my recommendations to parents are to avoid contact sports, but allow all other athletic activities with the understanding that this involvement does entail risks, but risks that are appropriate given the benefits of such activity. XXX’s parents are well aware of these risks and concur that he should be able to take part in all non-contact sports. Avoiding purposeful contact between soccer balls and his head is obviously a wise choice. It is equally obvious that such contact could occur accidentally, but that would only be preventable by avoiding all sports. I would not choose this extreme measure for my own children and XXX’s parents are in agreement on this. If I can provide any further information, please let me know. Dr. _______ |
Amplification
If your child has a significant hearing loss, have him fitted with hearing aids as soon as possible. (I’d suggest that you get considerably more powerful aids than your child needs at present as his hearing is likely to continue to get worse in the future.)
If the hearing loss progresses to profound where hearing aids are of little or no help, the good news is that Cochlear Implants generally work well in children with LVAS.
Medical Treatment
There are conflicting reports on surgical intervention in people with LVAS. Some doctors report that shunting the endolymphatic sac does not halt the progression of the hearing loss. Indeed, it is actually associated with the significant likelihood of profound deafness being the result. One study found an immediate decrease in hearing in four of seven ears after endolymphatic shunt surgery.
Other doctors have used surgical techniques that seem to work, at least in some cases. In one study, seven people with LVAS had surgery to obliterate their endolymphatic sacs to try to stop further hearing loss. Hearing remained stable in four of the seven, improved in two and continued to get worse in one.
However, it seems that surgical intervention has fallen out of favor now and is seldom undertaken as the benefit/risk ratio is not all that favorable.
Therapy
Hearing loss from LVAS is no different than hearing loss from other causes. If your child is still young, it is important to obtain speech/language therapy during the language learning years so that your child can develop good oral speech and language skills.
In addition, children (and adults) with deteriorating or fluctuating hearing loss may experience emotional problems such as depression, frustration and anger. You should promptly seek appropriate help for you child if this occurs.
Some selected references:
Abe, Satoko, et. al. 1999. Fluctuating Sensorineural Hearing Loss Associated with Enlarged Vestibular Aqueduct Maps to 7q31, the Region Containing the Pendred Gene. American Journal of Medical Genetics 82:322-328 (1999).
Callison, Diana, et. al. 1998. Large Vestibular Aqueduct Syndrome: An Overlooked Etiology for Progressive Childhood Hearing Loss. Journal of the American Academy of Audiology. Volume 9, Number 4. pp. 285-291. August 1998.
Dewan, Karuna, et. al. 2008. Enlarged Vestibular Aqueduct in Pediatric SNHL. Otolaryngol Head Neck Surg. 2009 April; 140(4): 552-558.
Hain, Timothy. 2001. Post-Traumatic Vertigo. http://www.tchain.com/otoneurology/disorders/post/posttrau.html.
Joachims, Zoha, et. al. 2000. Heredity in Large Vestibular Aqueduct Syndrome. The Journal of Otolaryngology. Volume 29, Number 4, pp. 244-246. 2000.
Okumura, Tomoko, et. al. 1995. Sensorineural Hearing Loss in Patients with Large Vestibular Aqueduct. Laryngoscope 105: March 1995.
Tan, T. Y. 1999. Large Endolymphatic Duct and Sac Sydrome—A Case Report. Singapore Medical Journal. Vol 40(05).
Tong, Karen, et. al. 1997. Large Vestibular Aqueduct Syndrome: A Genetic Disease? AJR:168, April 1997.
MY SON HAS LVAS AND I WOULD LIKE TO BE ADDED TO YOUR SUPPORT GROUP. THANK YOU!
The above info. was very informative. Would like to join the lvas group, thanks.
Hi Kate:
I’ve just sent you and invite to join the LVAS group.
Cordially,
Neil
Your article is very informative. Any of the information change as the child becomes an adult? I am a LVAS person that was always seen at the u of mn since I was a child of the 60’s. Would like more in sight if possible.
Hi Holly:
I don’t think there is any substantial difference in the symptoms, etc. of LVAS between being a child or an adult. The big difference is that as an adult, you have much more experience in knowing what kinds of activities make it worse, and which ones do not affect it. When you are starting out as a child, you don’t have any history to go by so don’t know what activities to permit and which to restrict.
Cordially,
Neil
My son has LVAS and I would like to be added to your group. I tried to subscribe but it wouldn’t go through. Please let me know how to do so. Thank You!
Hi Magan:
I don’t know why you can’t sign up. I’ve just sent you and invite to join the LVAS group. Let’s see if that works.
Cordially,
Neil
Hi,
Is there any information for parent available in Spanish? We have a student who was just diagnosed and the parent don’t speak English.
Hi Mahboobeh:
I’m not aware of any–but then I haven’t looked either. Maybe someone else on this list knows something.
Cordially,
Neil
The best explanation of this condition I have found. The link is not working. Can I join this group?
Hi Debra:
Sorry I didn’t have the link fixed when I changed the website over to WordPress. I’ve fixed it so you should be able to join the LVAS group now.
Cordially,
Neil
My 2 yr old son has suspected EVA and is being considered for cochlear implants as his hearing loss is getting worse, he currently has hearing aids
My daughter was diagnosed today. I would love to be a part of your group
Hi Gretchen:
Glad to have you join us. If you have any questions, ask away.
Cordially,
Neil
Hi, Please could you provide more information about the surgery to “obliterate their endolymphatic sacs”? The results sounded positive, which doctor carried this out and when? Are there going to be any more trials or investigations into this that you know of?
Many thanks
Hello! I’m 14 years old, and I have bilateral EVAS. I’m completely deaf in my left ear, but have perfect hearing in my right ear. It’s been this way ever since I was diagnosed at the age of 3. Anyways, I just wanted to know more about it, and I found this very helpful. Thank you!
I am 34 and I am almost deaf in my right ear due to this. It seems to be getting worse as the years go by. I wish that there was something that could be done to help. Does anyone have any hearing aids in their deaf ear that help level out the sound? I was just wondering. I keep looking for some research breakthroughs.
“Recent reports indicate that people who have LVAS are not born with a hearing loss, but merely are predisposed to the development of a hearing loss.” So, if a child fails their NBHS and is subsequently diagnosed with a SN hearing loss, then one could rule out EVAs?
Thank you Dr Bauman for this incredibly informative article. I have read every article on EVA that I could find, and this is by far the most comprehensive. My 2 yr old has Eva and mondini. You referenced in your article that sudden drops in hearing is not typical with mondini, and a progressive pattern is more common. Have you ever seen mondini stabilize or is profound deafness inevitable. My daughter also has balance issues. Could this get worse as her hearing drops. We do notice fluctuations in vestibular function, which is often disheartening. Thank you in advance.
Hi Pam:
I don’t know enough about Mondini’s to be able to answer your question, but I don’t think that profound deafness is inevitable. Each person is different. So you can be optimistic that hearing loss will not progress to the profound level. And even if it does, cochlear implants can probably help improve hearing.
I’ve not heard of balance issues getting worse as time progresses. Often it is the other way around and balance gets better as the brain learns to filter out any bad balance signals it receives.
Unfortunately, you have to wait and see. Only time will ultimately tell what will happen as your daughter grows up.
Cordially,
Neil
My child was diagnosed at 4 with EVA in both ears. Minimal in right with normal hearing at this time. Profound loss in her left ear. She has difficulty with her speech and coordination/balance trouble, gets motion sickness in car. She is getting Speech services. Her hearing tested every 6 months. She talks very loud, trouble with localization and “clumsy”, cries and becomes easily frustrated when communicating but not all the time. The specialist discussed BAHA as an option. I am not ready for her to have this as what if her hearing gets bad on the good side. I just feel its invasive but if she struggles in school we have decided to proceed with the surgery. Any suggestions?
Hi Rebecca:
I’m not surprised she has problems with localization. You can’t localize sound with only one ear. It’s just impossible. So you never say, “I’m in here” as she will never know where “in here” is. You have to be specific, “I’m in the kitchen” or wherever so she knows.
The BAHA is the current darling of the industry, but it is the most expensive option and is somewhat invasive. There are other options. Wearing a regular hearing aid will help her, or wearing a CROS aid to bring sound from her deaf side to her good side, or wearing a bone conduction hearing aid. (The BAHA is basically a fancy bone conduction hearing aid.)
I’m not sure why she talks so loud if she has normal hearing in her good ear. Ditto for having difficulty with her speech.
If you do decide to go with the BAHA, make sure you go with a “postless” kind. The reason I say this is that the post sticks up through the skin and can be an avenue for infection. With a postless version, once the incision heals up, there is no avenue for infection. This is the way I’d go if I were eligible for such a device. The external processor is held in place the same way as a cochlear implant is held in place–with internal and external magnets attracting each other.
Cordially,
Neil
My daughter have LVAS and I would like to be added to your group. I tried to subscribe but it wouldn’t go through. Please let me know how to do so. Thank You!
Hi Gilat:
I don’t know what is wrong. All you do is click on the link that says “Subscribe to LVAS Support Group” that is at the bottom of the article. That should open a blank email in your emailer, then you just press send to send that blank email. It’s that simple. I tried it and works for me.
Cordially,
Neil
Dear Dr. Neil
I’m a little bit confused, and do not know how to proceed.
Please, I’m waiting for your advice.
Rgd,
Gilat
Hi Gilat:
What exactly do you want from me? The above message doesn’t tell me much.
Cordially,
Neil
Must congratulate you on the most comprehensive coverage on the mystery of LVAS. I have very recently submitted a personal account of my own LVAS to the Reader’s Digest and would like to email it to you for your review and/or comment. Can you send me your email address? Sincerely
Hi Geoff:
I’d like to read your LVAS account. My email is at the bottom of every page on the Center’s website.
Cordially,
Neil
Hi Neil
I live in Sydney, Australia and my daughter was diagnosed with LVAS which resulted in profound hearing loss at birth. She had cochlear implants at 9 months old and is now doing really well. She is nearly 3 years old. I am happy to share my experience with any parents that are in the same situation. My point is that with the way modern medicine is developing, children diagnosed with LVAS can lead very normal lives!
Hello, all, this page has been very helpful indeed. Thank you. I’m hoping someone might be able to help me. My son, who is six, has just been diagnosed with LVAS in his right ear. He has no hearing in his right ear and on his left he has a high-frequency loss at 4 kHZ. We don’t know when he lost his hearing in his right ear. He functions normally as his hearing challenges don’t seem to present any obstacles in his life so far. The doctor doesn’t think any amplification is needed for now and has recommended regular hearing tests to check that if left ear doesn’t get worse.
I wondered if anyone could help with a couple of questions:
* Is it normal for children with unilateral LVAS to have sensorineural hearing loss in the other ear? Does anyone have any experience with this?
* His LVAS on his right ear was diagnosed by MRI by a radiologist, who according to the doctor, had a lot of experience in diagnosing this condition. However, the doctor did say that it was possible that my son did have LVAS on the other ear and that it wasn’t showing up on the MRI. Does anyone have an opinion on whether it would be worth pushing for a CT scan, which might better inform as to the presence, or not, of LVAS on the other ear?
Any information or shared experience on these questions would be hugely helpful.
thanks so much in advance!
Hi Al:
It’s certainly not uncommon to have varying degrees of hearing loss in an ear that supposedly does not have LVAS.
Personally, I wouldn’t get the CT scan because of the radiation he’d be exposed to. The MRI results should show enough. But in any case, what difference will it make? You are still going to treat his hearing loss with hearing aids whether he is diagnosed with LVAS or not. You already know he has LVAS in one ear so may be susceptible to drops in hearing from head trauma, etc. Again, you are not going to do anything differently–protect his head, etc, so why bother with the CT scan?
Cordially,
Neil
Hi Neil.
Thank you for the very informative and detailed article. It’s greatly appreciated for those us trying to understand what it all means for our loved ones.
You mentioned the different situations that could cause a deterioration in hearing levels ie air pressure, head trauma etc. I understand the theory behind the resultant loss with head trauma and pressure. And I agree it certainly sounds very plausible. However, I don’t fully understand how illness could cause the subsequent drops. Are you able to shed some light on how this may happen?
In regards to LVAS and it’s ability to affect balance. I’m curious as to whether surgical pressure release of endolymph may positively affect a persons balance. I understand that a person has the ability to adapt as they age and their brains learns to compensate for different movements. However, we witnessed my sons balance improve after bilateral cochlear implants surgery. He had gushers from both ears once the inner ear was breeched which suggests that it was under pressure. After his initial recovery from surgery, we saw a marked improvement in balance including his ability to climb. 12 months later we have seen a decrease in balance which may be attributed to pressure build up again. If this is the case, then this would further support the theory that the endolymph from the endolymphatic sac truly does affect the other internal structures of the ear.
Many thanks
Samantha
Hi Samantha:
In regards to Minor illness, I don’t see an exact connection either. I was just quoting from the literature. However, I just updated this article to reflect my opinion. Personally, I don’t think that the common cold causes a change in hearing due to LVAS itself. Most likely, the hearing loss is due to the viral activity of the cold virus in the inner ear. Viruses can result in sudden hearing losses, but I think this is coincidental to having LVAS, not a result of already having it.
You may be correct that increased pressure in the inner ear may result in balance problems, but I have no direct information about this. If the perilymph in the cochlea and the perilymph in the vestibular part of the inner ear are connected, then this could make perfect sense. This would be a good question to run by your otologist.
Cordially,
Neil
Please someone help me my daughter has EVA she is now 8 and is complaining of these high pitch noises does this mean she is losing more?? I have been reading online all night and can’t find the answers I need. I would appreciate some help so I can stop worrying.
Hi Chalyce:
Stop worrying. Worry won’t help your daughter in the least, and will just cause more problems for your mental and physical health. Yes, you are the mom, so you are concerned. That’s ok. Just don’t worry about it.
Since your daughter has LVAS (or EVAS), I assume she has some hearing loss–that’s how you found out she has LVAS, correct? She had hearing tests at that point, correct? So you have a baseline of her hearing.
If you are concerned that her hearing may be dropping, have her hearing tested again and compare to the baseline audiogram. Then you’ll know whether her hearing is dropping or not.
Typically, tinnitus (the noises she is hearing) accompanies hearing loss. So if she has hearing loss, it is very likely she has tinnitus too. If the tinnitus changes it COULD indicate that she is losing more hearing, but tinnitus itself does not cause hearing loss–it’s the other way around.
What answers do you need? I’ll help you, but I need to know more about her situation.
Cordially,
Neil
My daughter has EVA in both ears and was diagnosed when she was 5 (she’s almost 13 now). She has recently experienced a drastic drop in her right ear. I would love t be included in this group. Kristen
My 5 year old daughter has just been diagnosed with bilateral “EVA”.. she has normal hearing in the low frequencies & then it drops to severe/profound SNHL in her right ear & normal hearing in left ear with some conductive components in the left ear. CT scan shows normal cochlear but 1.7 for right & 1.95 for left. I know the range is 1.0-1.5 mm. My question is … is she a walking time bomb? Is it a matter of not if but when she will lose hearing in both ears & the only thing I can do is monitor her hearing & make decisions as to how & when she loses her hearing & if she starts developing any vestibular issues? Is there anything at all that I can do to be preventative not reactive? And finally how do I empower my daughter from am emotional, educational & social aspect if there’s no true triggers? Thank you in advance for your time. With much gratitude, Eileen – a heartsick mom
Hi Eileen:
Receiving any kind of diagnosis is a shock to the system. now that you know that she has LVAS, quite rightly you want to know what you can do about it. Since LVAS is peculiar to each person, the best way to answer your question is for you to observe what is happened in the past.
If she has had sudden drops in her hearing after various kinds of head trauma or rapid air pressure changes, then you can expect the same thing to happen in the future. If you only notice changes in hearing with a certain activity, then you probably want to restrict that activity but nothing else. You want her to grow up to be a well-rounded child. Thus, she has to be allowed to do as many activities as she would like to do consistent with not dropping her hearing when doing them.
Some children go for years without any further drops and their hearing, while others are much more sensitive and loose hearing from time to time. That is why I say go by past experience as much as you can, because that will give you the best indication of what will likely happen in the future.
You can put your mind at rest. Most children with LVAS do not lose all their hearing. Their hearing may drop to severe, but they don’t go totally deaf. and even if she did lose almost all her hearing, the good news is that you have a Plan B. Since her cochlea is ok, she can probably do very well with cochlear implants if she ever needs them.
As far as what you can do proactively, it’s just observing what activities or incidents cause problems and then figuring ways to avoid them in the future. Until you know, if she bumps her head hard, or gets hit in the head, or falls on her head, make sure she tells you. Then you, on your part, may want to get her hearing checked and see whether there was any drop in hearing. Usually hearing loss will show up either that day or the next day. If there is any hearing loss, your ENT may prescribe Prednisone. For a lot of kids with LVAS, their hearing comes back–at least much of it–after taking a course of Prednisone.
once you’ve established a track record of what can affect her hearing, then avoid those activities as much is possible. Other than that let her be a normal girl. don’t try to over protect her and cause her to become fearful. And on your part, don’t anticipate the worst. It is not good for your mental health either. For all you know her hearing may remain stable for years.
Cordially,
Neil
Hi thank u so much for your helpful essay. Im 16 and i have LVAS. I realy like to join your group. Please
Hi Pari:
What’s stopping you from joining the LVAS group? I’ve just sent you an invitation.
Cordially,
Neil
Hello, I have not yet been diagnosed but am a suspect of EVA from an old MRI. I have significant hearingn loss in right ear and will have another MRI few months later, as I am pregnant now. Now my question is that can EVA be passed down to my children? I was lucky to develope my sudden hearing loss at age 27 and not in childhood but now as a mom i worry about my kids ability to hear as i have the problem.
Can you shed some light on my inquiry?
Hi Nafisa:
There seems to be a genetic component to EVA but they still don’t know what all genes are involved, so it is possible you could pass it on to your child, but I think that EVA is carried recessively. That means BOTH you AND your husband would have to have the same genetic mutation to pass EVA to your children.
Why worry about it at this point? Worry won’t help. You know you have succeeded in spite of your hearing loss. Your child (if he/she ever has a hearing loss) can do the same. You already know the problems and how to largely overcome them, so you are in an admiral position to help you children.
Before I had any children, I told my wife that we would have a 50/50 chance that any children we had would be hard of hearing (because I carry a dominant [not recessive] gene for hearing loss. And that is exactly what happened. We had two girls and one had normal hearing and the other had a severe hearing loss–just like mine. It didn’t bother me at all–she was just like me–and she could overcome just like I did. We were both readers because it is easier to learn from books than from trying to hear people.
I wouldn’t worry. Just rejoice that you are having a child.
Cordially,
Neil
Any updates or research on whether Steroids (Prednisone) help recover the loss when there is a sudden drop?
Hi Tammy:
Nothing new of which I’m aware. Prednisone helps in some cases, and doesn’t help in others.
Cordially,
Neil
Hello, I’m a mom of child with LVAS in China. My daughter was having hearing loss at birth and diagnosed LVAS at 5 months old by MRI. My question is finding the enlarged endolymphatic sac means having LVAS? Since on the MRI report didn’t figure out LVAS, but the audiologist estimated it since he said there is enlarged endolymphatic sac on MRI image.
Also although it’s easy to found what is LVAS and how to prevent the hearing loss, but rarely have the sharing from people who have LVAS, I really want to join your group to learn more but unfortunately doesn’t have access to login to yahoo. Not sure if any other platform that I can touch with your group?
My question is a bit long, but thanks for your understanding of the worry of a mom.
Hi Carol:
There is a Facebook LVAS group if you want to join it if you can access FaceBook from China. Here is the link. https://www.facebook.com/groups/201092251569/?ref=share
Cordially,
Neil
I have a 9 year old son who was diagnosed with EVA 3 years ago. He had unilateral loss until this past checkup which revealed loss in his other ear. I would like to join group if it is still up and running.
Hi Jamie:
It doesn’t have much if any activity anymore. I think Yahoo groups is going the way of the Dodo bird. You can always post comments/questions below this article for others to read.
Cordially,
Neil
Hi Neil,
Our daughter has had moderate hearing loss in both ears since birth. She has been aided since early childhood. During puberty she developed tinnitus. She is a cross country runner and last year started experiencing complete hearing loss and vertigo during races. Luckily it returns after awhile but the ringing is almost unbearable. Should we be concerned about LVAS and have her tested. Her younger brother has the same hearing loss without the ringing but he is only 11.
Hi Randi:
It sure wouldn’t hurt to have both kids tested if you are concerned about LVAS. It is possible that LVAS is the cause of the hearing loss and vertigo when your daughter really exerts herself when racing (or any other activity). Testing could confirm that.
But some people lose hearing when they really exert themselves (apart from LVAS) so just the exertion can be the cause–probably because when you really exert yourself, your body directs your blood to the main muscles and shunts it away from the peripheral organs (which includes ears). The lack of oxygen to the ears can cause the hearing loss, vertigo, etc. When the oxygen returns to normal, hearing returns–unless the exertion was for too long, and some hair cells die. Then some hearing loss will be permanent.
The solution is for her to not “really” exert herself–tone it down a notch to a level that doesn’t cause the hearing loss. Of course, by doing this, she probably won’t win any races. But the choice is between winning and losing hearing, etc.
The same can be true if she has LVAS. Again, the trick is to find the degree of exertion that is just below where it affects her ears–and then stay below that level.
Cordially,
Neil
I was diagnosed 3 years ago, at the age of 49, with EVA in my right ear but with conductive hearing loss. Last year in Jan. I started getting sharp pain deep in my right ear. Cold and wind made it worse. Even in the summer months I would wear earmuffs to try and protect my ear from the wind. It has since gone away, for the most part. My ENT didn’t think it was related to my EVA. She couldn’t find a reason for it. Do you think it could possibly be linked to my EVA in that ear?
Hi Melissa:
Since EVA is a congenital condition, you’ve always had the enlarged vestibular aqueduct, even though you were just diagnosed with it 3 years ago. Since you hadn’t been having pain in your right ear for 46 years, I rather doubt that the pain is associated directly with your EVA. So I tend to agree with your doctor.
What changed in January last year that possibly could be the cause of your ear pain? Any ideas? Did you expose your ears to loud noise, or a sudden sharp sound? Was there any sudden change in your hearing around then?
Cordially,
Neil
Please add me to your list to get your information quickly.
Hi Barb:
Alas, the Yahoo group for LVAS is no more. However, you are free to post your comments/questions below this article.
Cordially,
Neil
Hi,
Just found your interesting blog. Could you please direct me to the reference that LVAS may be developed after birth please. Thank you, Pia
Hi Pia:
I think you are referring to this quote:
“When the vestibular aqueduct develops, it is short, straight and broad. It then continues to refine its shape until the adult form is reached, usually when a child is about 3 to 4 years of age. By this time the vestibular aqueduct is about 10 mm in length and shaped a bit like an inverted ‘J’. Any factor that affects the foetus during the early development of the inner ear may prevent the narrowing and lengthening of the vestibular aqueduct to its adult shape, instead leaving it permanently enlarged.”
This quote came from “The Vestibular Aqueduct Syndrome” Reprinted with permission to the E.A.A Listserver 3/99, from a handout provided by The Deafness Centre, New Children’s Hospital, Westmead, Sidney, Australia. 1999. I got it as a FAX from the UCSF Audiology Clinic.
There was a study “Embryological development and large vestibular aueduct syndrome” by PG M Pyle in Laryngoscope 2000 Nov; 110(11):1837-42 that concluded, “The data suggest that LVAS may result from postnatal and early childdood maldevelopment.”
Cordially,
Neil
Great thank you Neil!
Hello, I was diagnosed with LVAS at 12 years old along with my younger sister. I’m now 36 and pregnant – I can’t seem to find any information on whether childbirth and the associated increase in CSF pressure can increase the chances of further hearing loss? I’ve always assumed a caesarian is the safest option for women with LVAS. Would appreciate any information.
Hi Katia:
I don’t have any data on that either, but you can assume that the increase in CSF pressure could cause hearing loss in some people with LVAS. Just how likely it is depends on your history of LVAS.
You have told me nothing about your LVAS and how it has affected your hearing in your lifetime so far. I assume you have some hearing loss from LVAS and that was why you were checked for it when you were 12, correct?
If over the years, if your LVAS has not affected your hearing when you strain at things–exerting yourself at exercise, or when trying to pick up something heavy, or even straining when going to the bathroom, then the straining at childbirth probably won’t affect your hearing either. However, if the opposite is true, then you can assume that childbirth could also cause additional hearing loss.
Also consider other causes of pressure changes such as when flying, going up and down mountains whether in a car or chairlift or gondola, diving underwater more than about 8 feet down, etc. Have any of these caused additional hearing loss? If not, then your risk of more hearing loss at childbirth is also low and unlikely.
Also consider whether or how much hearing returns after a pressure change. Many people with LVAS have most of the lost hearing return in a few weeks, but often not all the hearing returns, but it is not as bad as it was initially. Thus, the overall change in hearing ultimately may not be all that much.
And looking at it another way, vaginal births as opposed to caesarean births have certain long-lasting health benefits to your baby. So you want to consider that as well.
So, unless you are particularly sensitive to pressure changes/CSF pressure changes, etc., personally I’d opt for a regular vaginal birth over a caesarean birth.
Cordially,
Neil