Beyond the Puzzle of LVAS

By Tami Esmay, Oregon H&V Parent Guide

Enlarged Vestibular Aqueduct Syndrome (LVAS) is a puzzle to all; the parents, the audiologists, the educators, and certainly the child growing up with fluctuating, unpredictable hearing and all that goes along with it. The author acquired this information in bits and pieces when her son Riley was diagnosed. Her desire is to reach out to the next family who heard this diagnosis for the first time and give them information upfront to help them to better understand the syndrome in context to the whole child.

Riley Esmay

My ten year old son Riley was found to have a possible hearing loss in his left ear through the EDHI (Early Detection of Hearing in Infants) screening. He was initially diagnosed with a slight mild conductive hearing loss in his left ear and normal hearing in his right ear. By three years of age, his hearing loss “progressed” to moderate to moderately severe in his left ear and from typical hearing to a moderate loss on the right.  With that progression, he was then fitted with his first set of hearing aids. (He was very excited to pick out the color of his very first set of ear molds: red!)  A year later, the audiologist noticed that the sensitivity in each ear had started to change again, going to a mild sloping hearing loss in the right ear and a mild to severe loss in the left. The diagnosis of Enlarged Vestibular Aqueduct Syndrome(LVAS) gave this unpredictable picture a name when he was five years old.  Along with this diagnosis, we also had him evaluated by an occupational therapist to see what she could do to help him increase his balance and gross motor skills. After receiving occupational therapy services for a couple of years, his balance as well as his coordination improved tremendously and put him within his age group developmentally. Now at ten years of age, his right ear has stayed within the mild to moderate range while his left ear now slopes from mild to profound levels.

Some families restrict activity due to the risk of progressive hearing loss if a child should fall or experience a bump to the head with this syndrome. We had chosen to limit him from participating in only a few extracurricular activities. Now that he is older, he is more aware of the risks and chooses his own activities with some guidance. Riley has always used the proper safety equipment when needed.  He has played baseball since he was five years old as well as tried soccer and basketball.  Due to the high concussion rates in soccer and football, he has decided not to play those sports. 

Perhaps most difficult, Riley experiences episodes of vertigo (dizziness) which started off as only minor when he was younger, but as he has grown, the frequency as well as the intensity has increased. The last couple of years he has experienced nausea with vomiting during episodes of vertigo. During these periods, he can’t walk very well and is also very sensitive to light. When his episodes occur, they usually last a couple of days with the first day being the most severe and the second with minor dizziness and no other related symptoms. Our ENT has informed us that his experience is a rarity. Generally a low percentage of people with LVAS have these vertigo episodes.

Riley’s hearing tends to fluctuate on a weekly basis, and can be as frequently as a daily occurrence.  He does not have the maturity yet to identify this, but it is obvious due to the nature of the “what” that he asks more often, or not responding when he normally would.  Now that he is older, he is starting to share more openly about his hearing loss. He recently told me that he is scared to lose his hearing and feels left out at times being the only one with hearing aids/hearing loss. He does have a younger brother with a unilateral loss but it just is not comparable. To try to ease his anxiety level about the unknown future, we have started to learn ASL. We also participate in all Hands & Voices family events to give him lots of exposure to other kids and adults on a similar journey. I have shown and discussed with him information about cochlear implants. He is currently not interested as his focus is learning more ASL.

One of the biggest frustrations that my husband and I have encountered is being able to get professionals within the educational setting to understand the nature of his hearing loss. I know it is a challenge to understand the subtleties and changing nature of his abilities in the classroom. The ongoing task to make sure that his hearing aids and personal FM system are working properly is one thing; continually assessing his understanding and processing of all material presented to him, and to keep the level of social awareness/maturity on our radar screens on a regular basis is another challenge.   

His audiogram only explains a little bit about him--it’s not everything. Though he certainly has many challenges, he has been very successful in his academics.  He is also expanding his willingness to try many new things as he gets older and more confident. Many people comment on how good-natured he is amongst others.  I think much of Riley’s success can be attributed to his ability to guide us in any way he can to what works for him as he makes this lifelong journey.       

More About LVAS

Enlarged Vestibular Aqueduct Syndrome also known as LVAS or EVAS is a condition related to a progressive type of hearing loss. The hearing loss is primarily sensorineural with a conductive component, which makes it difficult to diagnose. Sudden hearing loss and/or fluctuating hearing with a conductive hearing loss component often accompany the diagnosis of the syndrome. The cochlear aqueduct is the narrow canal that runs through the skull, connecting the inner ear to the cranial cavity. Enlarged aqueducts (and enlarged lymphatic ducts and sacs) can’t be seen in a physical examination, but only detected through the use of a CT scan or MRI. To help reduce the potential of further hearing loss, children are encouraged to avoid head impact sports and are encouraged to wear head protection when involved with any impact sports. Since a change in barometric pressure can also cause further change in hearing, activities such as scuba diving are discouraged. 

Along with this being a progressive hearing loss, a person’s hearing can fluctuate from day to day from near normal, to significant loss, back to near normal in a matter of days.  Due to the nature of this hearing loss, alternate or supplemental means of communication are generally encouraged which may include sign language or cued speech as well as using assistive devices such as hearing aids or cochlear implants.

Since the hearing loss affects the vestibular (balance) system, some people can experience episodes of vertigo.  Episodes can be very minor with minimal side effects from minor nausea to vomiting and dizziness.

There has not been much research on LVAS but there is an ongoing study with NIDCID (National Institute on Deafness and other Communication Disorders).  No proven treatments have been discovered thus far.   http://www.nidcd.nih.gov/health/hearing/Pages/eva.aspx

Two chat groups featuring information from fellow parents and professionals can be found on Facebook and the yahoo group: LVAS@yahoogroups.co.