LVAS

Large vestibular aqueduct syndrome (LVAS) – A guide to Hearing Loss

This long sounding name is given to a condition in which the certain structures within the inner ear become swollen and enlarged. This can result in fluid flowing back into these structures which causes hearing loss. What is the ‘vestibular aqueduct?’ There is a bony canal (the ‘aqueduct’) that runs through the skull, connecting the cranial cavity to the inner ear (the ‘vestibule’). And within that canal is a tube called the ‘endolymphatic duct’ which runs through this aqueduct to a collection pouch called the ‘endolymphatic sac’.

This duct contains a fluid called ‘endolymph’ which travels through this duct to the sac – hence the name. This fluid normally passes from the inner ear towards this collection sac.  But if both this duct and sac become enlarged then this forces the endolymph back towards the inner ear where it affects balance and hearing.

How does this cause hearing loss?

No-one is exactly sure how this causes hearing loss but several theories have been put forward which include:

• Endolymphatic fluid may be too concentrated and damages fine hair cells within the inner ear which results in sudden hearing loss.
• A minor head injury causes changes in air pressure within the ear, causes a tear in one of the delicate membranes in the inner ear which permanently damages hearing.

Causes of large vestibular aqueduct syndrome

This is a recently discovered condition so there is much more to learn about the exact causes of it. But experts now believe that this condition actually starts in childhood when the ear structures are still developing which makes them vulnerable to disease or injury.

Other possible causes include damage caused by a head injury, increase in cerebrospinal fluid during exercise or physical exertion or changes in air pressure within the ear. At present the only way of diagnosing this condition is via a CT scan.

The most obvious symptom of large vestibular aqueduct syndrome is that of hearing loss. The extent of this loss will vary between individuals and in some cases there will be complete hearing loss (permanent deafness).

Treatment of large vestibular aqueduct syndrome

As long as there is still partial hearing then there is one form of treatment available which is a hearing aid. Any hearing lost cannot be restored but at the very least this will enable the sufferer to maintain their current level of hearing and communicate with others. However, regular hearing tests will be needed to check for signs of any further deterioration in hearing.

DEAF not DUMB

A little insight into Miss A 🙂

Well as I mentioned Miss A (3.5 years old) is Deaf and in my opinion has been since she was about 9-12 months old (after an acute illness and 2 hospitalizations) although I will never really know. We didn’t have it confirmed until she was 2. As it stands today she is profoundly Deaf in her right ear and Severely Deaf in her right. Her remaining hearing could go at anytime as she has a condition called LVAS. She wears adorable pink hearing aids with pink and purple moulds. Her first language is Auslan but she is slowly starting to speak more and more as time goes on.

Some parents find the word “DEAF” hard to accept and prefer the term “hard of hearing”. I am not really sure why but sometimes when I listen to them talk or read their stories in forums I begin to think it comes down to the same stigma Deaf people have been living with throughout history and that is the idea that “Deaf = Dumb” and they don’t want people thinking that of their child.
I am sorry but that isn’t the case one little bit. Yes my daughter is Deaf, she is the only Deaf person I am aware of within the family but that doesn’t make her dumb, it does however mean that her speech and language are delayed but that’s her only delay. She is still a regular 3 year old girl who love dolls, makeup, dressup and talking on the phone. She is not in anyway lacking in quality of life and the things she for lack of a better word “misses” due to being unable to hear she makes up for visually. Her world may differ slightly from her hearing peers but I can tell just by looking at her she doesn’t mind one bit.