Hearing Loss Help  

by Neil Bauman, Ph.D.

A young mother wrote:

My husband and I just found out over the past few months that our daughter is deaf, due to LVAS [large vestibular aqueduct syndrome]. She passed her newborn hearing test in her right ear and failed in her left, but a month later when we did the ABR [auditory brainstem response] she was not responding to sound at any level in either ear, which we were told is equal to being deaf. We are looking into a cochlear implant and are very hopeful. My question is, how likely is it that this will reoccur in future children? This is our first child, and I had always dreamed of having 3 or 4 children, now I am very scared.

Large Vestibular Aqueduct Syndrome (LVAS) is also known as Enlarged Vestibular Aqueduct Syndrome (EVAS).

With LVAS, doctors think that the extra-large vestibular aqueduct (really just a hole in your skull) allows the contents of the endolymphatic sac (on the inside of your skull) to flow (backwards) though the endolymphatic duct into the inner ear where it somehow causes hearing loss.

LVAS is a relatively new diagnosis, even though the underlying condition has been around for awhile, but no one knew about it until recently. Doctors just classified hearing loss from LVAS under “idiopathic hearing loss” which simply means hearing loss of unknown causes.

The good news is that I know a lot of kids with LVAS that have responded very well to cochlear implants. So I am hopeful that your daughter will have similar results if you choose to go through with it.

Don’t be scared about having more children. They are worth it whether they have hearing loss or not. You can find a lot of help and support on the LVAS list that I set up for people with LVAS. As a matter of interest, most of those on this list are mothers in just your situation. To join this special list, click here then  scroll to the bottom where you can fill in your email address and subscribe to the LVAS list. While you are there read the whole article on LVAS.

Don’t put off having more children just because there is a possibility of them having LVAS. Even if all of them had LVAS and lost their hearing, you would still love them. They will be normal except for not hearing well. I knew before my children were born that one or more of them would be born hard of hearing—and my wife and I are still glad we had them—even though one has a severe hearing loss like me.

Now to answer your question as to how likely any of your future children might have LVAS. (The following works for any other recessive hearing loss syndrome as well for that matter.) Since LVAS is a recessive hereditary trait, both you and your husband must already be carriers of the recessive LVAS gene. Furthermore, both of you need to pass this recessive gene on to your child in order for your child to have LVAS.

Remember, genes come in pairs, and you only pass on one gene of each pair to each child, so there is a 50% chance you will pass on the normal gene and a 50% chance you will pass on the LVAS gene. The chances that both you are your husband will both pass on the LVAS gene at the same time is only 25.

Here are the probabilities (assuming there is only one LVAS gene—but this is not certain, there may be more, so the probabilities may be even less than shown below.

25% chance your child will have LVAS (child receives an LVAS gene from each of you).

50% chance your child will be a carrier of the recessive LVAS gene, but will NOT have LVAS (child only receives an LVAS gene from one of you).

25% chance the child will not have any LVAS genes and thus will not have LVAS, nor be a carrier of LVAS. (child does not receive a LVAS gene from either of you).

So each of your future children only has a 25% chance of actually having LVAS.

Mind you, this is how it works out on the average. However, it could be that all of your future children will have LVAS, or none of them. Think of flipping a coin. On the average you’ll get heads 50% of the time. However, it is possible to flip a coin 10 times and get 10 heads in a row (possible, but extremely unlikely). This is the way it works with having children with LVAS too.

If it were me, I’d have all the children you want. We (hard of hearing people) live happy, successful and fulfilled lives in spite of our hearing losses. There is no reason your children won’t also.

by Neil Bauman, Ph.D.

A lady asked:

I was just diagnosed with bi-lateral LVAS [large vestibular aqueduct syndrome] AND Meniere’s disease, moderate- profound loss. Does the fact that I have both, increase my chances for further hearing loss? Can I expect my hearing to deteriorate further? I know no one can say for certain, I’d just like to know some odds or statistics.

As far as I know, these two conditions do not affect each other. Therefore, look at each of these conditions independently. People who have a hearing loss from LVAS typically have some kind of trauma that results in more hearing loss each time it occurs. Some people with LVAS never seem to have any hearing loss, or are only susceptible to pretty major trauma, so they lose some hearing at one point and then it stays at that level for a long time.

With Meniere’s disease, typically you lose more hearing with every attack you have. Then, after the attack, your hearing returns somewhat, but not to its previous level. So think of your hearing loss typically as going down two steps, then back up one step—then down two steps and back up one with each succeeding attack.

This isn’t written in stone, but the people that I know with Meniere’s typically have this kind of loss, and eventually they lose most of their hearing in the affected ear.

The good news is that Meniere’s typically affects only one ear—but about 20% of the people with Meniere’s have it in both ears or it switches to the other ear later.

You can learn more about LVAS here.

Learn more about Meniere’s Disease and what you can do to help yourself.

by Neil Bauman, Ph.D.

A concerned mother wrote:

I was just exploring your website for information regarding enalapril and healing loss. My son is on Enalapril and a Baby Aspirin each day. He is 3 years old and had a hearing screening at school yesterday. He passed with flying colors on the right and had no response to the test on the left. I am very concerned that there is a link between the ototoxicity of his medications and his hearing loss. Can you advise me about his situation?

I can’t speak specifically to your son’s situation. However, I can tell you that Enalapril can cause hearing loss, tinnitus, ataxia, dizziness and/or vertigo in some people. However, I don’t know how the Enalapril would affect a young child as opposed to an adult.

Also, Aspirin can (and does) cause hearing loss and tinnitus in people. Fortunately, typically, but not always, hearing returns and tinnitus goes away when you stop taking the Aspirin.

I find it a bit strange that one ear is “perfect” and the other one is “dead.” You would think that drugs would affect both ears more or less equally–although I know of a number of cases where only one ear was affected for whatever reason.

Therefore, although it is possible that these drugs are the culprits, with such limited information, I have no way of knowing for sure. At the same time, there is the possibility that neither of these drugs caused the problem and you need to look for an unrelated cause.

For example, have you had your son tested to see if he has enlarged vestibular aqueducts–Large Vestibular Aqueduct Syndrome (LVAS). I know of hundreds of kids with this condition. Interesting enough, a number of them have hearing loss in only one ear. Furthermore, often it isn’t detected until they are tested as was your son. It is at this point that the doctors look further to try to find a cause and discover the LVAS. This is just one of the things you might want his doctors to check out.

You can learn more about LVAS by reading my article, Large Vestibular Aqueduct Syndrome.

by Neil Bauman, Ph.D.

A mother wrote:

My son’s genetecist recently concluded that he has Pendred’s Syndrome. I’m a little sketchy on how they came to that conclusion. When I mentioned that because of the results of his CT scan, he was diagnosed with LVAS, she didn’t know much about it. I’m wondering what, if any, correlation exists between them.

I read on-line the following, “The SLC26A4 gene encodes an anion transporter known as pendrin and is the gene mutant in Pendred syndrome (PDS; 274600), DFNB4 (600791), and enlarged vestibular aqueduct syndrome (EVA; 603545).” If a mutation on that particular gene is for Pendred and/or LVAS, how do they know that you have Pendred and not just LVAS?

Good question. I am not a geneticist, but this is my understanding on the subject.

There are three conditions that all result from the mutation of the same SLC26A4 gene. Thus in one sense, they are all just different manifestations of the same thing.

If the mutation of the SLC26A4 gene results in developmental abnormalities in the cochlea, a sensorineural hearing loss and thyroid problems (goiter), then they call it Pendred syndrome.

If the mutation of the SLC26A4 gene results in an enlarged vestibular aqueduct and sensorineural hearing loss and/or balance problems, they call it Large Vestibular Aqueduct syndrome.

If the mutation of the SLC26A4 gene results in non-syndromic sensorineural hearing loss (and goiter and LVAS are not noticeable) then they call it Sensorineural Non-syndromic Recessive Deafness. (Sometimes a goiter later develops and then they say, “Oh, it must have been Pendreds after all.”)

So for all practical purposes, these three are really one condition having several manifestations.

Look at it this way. In all cases there is a sensorineural hearing loss.

If, in addition, one of the manifestations is an enlarged vestibular aqueduct, then the diagnosis is LVAS.

Or, if, in addition, one of the manifestations is thyroid problems, then the diagnosis is Pendreds.

Or, if neither an enlarged vestibular aqueduct nor thyroid problems are evident, then the diagnosis is sensorineural non-syndromic recessive deafness.

However, a person could have both a thyroid problem and LVAS. Some doctors may simply diagnose this as Pendred syndrome, while others may mention that the person has LVAS.

Maybe it would be easier to understand if we lump all the above together and call it “SLC26A4 Gene Mutation Syndrome” and explain that this syndrome includes sensorineural hearing loss and may also include thyroid problems (goiter) and/or enlarged vestibular aqueducts.

by Neil Bauman, Ph.D.

A couple wrote:

We found your writing on Large Vestibular Aqueduct Syndrome (LVAS) and other topics extremely helpful.

We are wondering if the information on LVAS is up to date, or if there are recent developments since 2002 when you did the write up. We are also wondering which centers in the US, and the world, are working intensively on this problem.

Glad my article on Large Vestibular Aqueduct Syndrome (LVAS) helped you.

I’m not aware of any major breakthroughs in either the understanding of LVAS or its treatment–so the information is generally still up to date.

However, one thing that seems to have changed is the definition of exactly how large the vestibular aqueduct (VA) has to be in order to be considered enlarged. In my article it was defined as being 1.5 mm or larger at the midpoint of the VA. Currently, some doctors say this is much too large, and think the correct definition should be more like 0.8 mm or so.

I really don’t have an opinion on this as there doesn’t seem to be any clear correlation between the size of the VA and the resulting hearing loss. Thus there must be other factors that need to be discovered and evaluated.

The only major study on LVAS of which I’m aware is being conducted here in the USA at the National Institutes of Health. When it is completed (hopefully in the next 3 or 4 years), I think we will have a much better idea about LVAS than we do now. Until then, I guess we just have to wait.

by Neil Bauman, Ph.D.

A man wrote:

My daughter was just diagnosed with Large Vestibular Aqueduct Syndrome (LVAS) in one ear. Is this condition typically limited to only the currently infected ear, or can it “spread” to the other, presently uninfected, ear in the future?

Large Vestibular Aqueduct Syndrome (LVAS) is a hereditary genetic condition so it cannot “spread” or “infect” anything. Either you have it or you don’t.

However, at present, just diagnosing the presence of LVAS seems to be a matter of opinion among doctors. For example, one doctor can read your MRIs or CT scans and declare you have LVAS in one (or both) ears. Another doctor, looking at the same films, can just as emphatically declare you don’t have LVAS.

What this means is that until more is known about LVAS and how it develops in young children, your daughter’s diagnosis is open to interpretation. Thus, it is possible that your daughter may have LVAS in both ears now, although it has not been diagnosed as such at this time.

To read more about LVAS point your browser to http://www.hearinglosshelp.com/articles/lvas.htm.

by Neil Bauman, Ph.D.

A mother asked:

My son has Large Vestibular Aqueduct Syndrome (LVAS). I know that LVAS is genetic, but is it only the result of getting a gene from both the father and mother, or is it now a strong possibility that when my son, who has LVAS, has children, that they will have LVAS even though the mother does not have the gene? If so, is there an estimated percentage of chance that his kids will have LVAS?

Excellent questions. In order to answer this, we need a brief lesson in genetic inheritance. Here’s how I understand genetics and LVAS.

Genetic conditions may be “dominant” or “recessive.” LVAS is thought to be recessive. This means that both the father and the mother have to carry this gene and pass it on, in order for a child to have LVAS.

(In contrast, if the condition was carried by a dominant gene–then only one parent needs to pass it on. For example, I have a dominant genetic hearing loss. Thus, I have, and pass on, this gene (on the average) 50% of the time. In my case, this is exactly what happened. Of my two children, one has normal hearing and the other has my rare kind of hearing loss.)

Here’s how recessive genetic inheritance works. Each parent has two genes for any given characteristic. These genes may both be either dominant (RR), or both recessive (rr) or one of each (Rr). However, each parent only passes on one of these genes in each child. Which gene is passed on is totally random for each child.

Since LVAS is recessive only a person with “rr” would have LVAS. A person with “Rr” would be a carrier of LVAS but would not have any symptoms of it, and a person with “RR” would not have LVAS, nor carry it.

Thus, each child ends up with one of these three possible combinations. Either:

1. The child receives no LVAS gene from either parent. Result, child does not have LVAS, nor is child a carrier of LVAS (RR).
2. The child receives one LVAS gene from one parent and none from the other. Result, child is a carrier of LVAS and thus can pass it on, but does not have LVAS himself (Rr).
3. The child receives one LVAS gene from each parent. Result, child has LVAS himself (rr), and, of course, could also pass it on to his children (but only if the child’s spouse also passes on an LVAS gene at the same time).

Now let’s look at this from the standpoint of the probability of any given child inheriting LVAS. There are five possibilities.

• If neither parent carries an LVAS gene (RR), then, of course, the child will not have LVAS either. The probability of Number 1 (RR) occurring is 100%.

• If One parent carries the LVAS gene (Rr) and the other doesn’t (RR), the child will not have LVAS but may carry the LVAS gene. The probability of Number 1 (RR) occurring is 50% and the probability of Number 2 (Rr) occurring is 50%.

• If one parent has LVAS (rr) and the other does not (RR), then there is no chance of the child having LVAS, but he would be an LVAS carrier (Rr). The probability of Number 2 (Rr) occurring is 100%.

• If one parent has LVAS (rr) and the other is a carrier of LVAS (Rr), then the child will either have LVAS (rr) or be a carrier of LVAS (Rr). The probability of Number 2 (Rr) occurring is 50% and the probability of Number 3 (rr) occurring is 50%.

• If both parents have LVAS (rr), then each parent HAS to pass on an LVAS gene to the child, and thus the child has will have LVAS (rr). The probability of Number 3 (rr) occurring is 100%.

The above make two assumptions. One, that LVAS is carried by a recessive gene (which is almost certainly the case), and two, that there is only one gene involved in causing LVAS (which is not likely to be the case). If there is more than one gene involved in causing LVAS, then the probabilities are obviously much more complicated than shown above.

The fact that your son has LVAS (rr) means that both you and your husband either also have LVAS yourselves (rr), or you are carriers of LVAS (Rr). This means that only possibilities C, D or E apply in your case.

If you want to learn more about LVAS and how it can affect a person’s ears, point your browser to http://www.hearinglosshelp.com/articles/lvas.htm.

by Neil Bauman, Ph.D.

Sometimes I get unusual emails. For example, one person recently wrote, “Two days ago my middle-aged boyfriend had a 20 minute hearing loss after having worked out extraordinarily strenously with his personal trainer. When he mentioned this to his trainer, his trainer told him that one other person, a woman in her 20s had told him the same thing. What’s going on?”

Strenuous exercise, whether it is lifting weights, straining to win a foot race, or even a strenuous yoga session can sometimes result in hearing loss. There are a number of causes. I’ll just mention two of them here.

First, strenuous exercise, or a blow to the side of the head, can cause a membrane in your inner ear to rupture, allowing fluid (called perilymph) to leak from either the oval window or the round window into the middle ear cavity. The fancy name for this is perilymphatic fistula. Sometimes these tears heal up by themselves. Other times they require surgery to fix them. Hearing loss from perilymphatic fistulas may be temporary or permanent. Often much, but not all, hearing returns, leaving you with some degree of permanent loss.

Another cause of hearing loss resulting from strenuous exercise is from a condition called Large Vestibular Aqueduct Syndrome (LVAS). In people with LVAS, the increase in intracranial pressure from the exercise sometimes forces the contents of the endolymphatic sac (sandwiched between the brain and the skull) to flow “backwards” to the cochlea where the extra-high ionic content of the endolymph causes problems in the inner ear. The result is hearing loss.

To learn more about LVAS, point your browser to http://www.hearinglosshelp.com/articles/lvas.htm.