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Causes/Kinds of Hearing Loss

Large Vestibular Aqueduct Syndrome (LVAS)

© June 2002 (revised September 2007, April 2012) by Neil Bauman, Ph.D.

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.

Its 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, 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.

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.

_______________________________

There is a wonderful information and support E-mail list for people (and parents of children) with LVAS. Hundreds and hundreds of parents have received help and support from other parents farther down the LVAS road.


Here is what one of the mothers on this wonderful, supportive list recently said: "Neil, I must say what a wonderful help you are. Your knowledge and input are a blessing to this group. Because of you and this group I go to the doctors appointments well prepared, and sometime more knowledgeable than the doctors. I know just the right questions to ask. I can never say thank you enough for what you and this group do."
—A. R.


If you would like to join this LVAS support group, type your E-mail address in the box below, and then click on the Yahoo Groups button. You can unsubscribe at any time.)
 

Subscribe to the LVAS list


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.