Enlarged Vestibular Aqueduct Syndrome (EVAS) – Information, Symptoms and Treatment Options

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What is Enlarged Vestibular Aqueduct Syndrome?                         

Enlarged vestibule aqueduct syndrome is one of the most common inner ear defects that results in the childhood. This is a condition which is present from birth. However, hearing loss is usually not present from birth and develops later over the years.

Vestibular aqueduct is basically a bony canal which goes from inner ear’s endolymphatic space to deep inside the skull. It is covered by the temporal bone, which also contains cochlea and vestibular apparatus of the inner ear. Together, these tiny organs of the inner ear help our brain make sense of the balance information and the sound that we hear.

Vestibular aqueduct houses endolymphatic duct, which is a tube containing endolymph. Endolymph is a fluid that resides in the semicircular canals, which are responsible for initiating balance information signals to the brain. Endolymph fluid is ion rich.

When vestibular aqueduct becomes larger than normal, it causes endolymphatic duct to expand as well. As a result, endolymphatic sac is also enlarged. This whole process results in imbalance of endolymph which produces symptoms associated with this condition. This condition is called enlarged vestibular aqueduct syndrome (EVAS).

Causes of Enlarged Vestibular Aqueduct Syndrome?

EVAS is thought to be caused by the genetic and environmental condition. Since this is a condition or deformity present from birth, improper development of vestibular aqueduct during fetal development is thought to be responsible for its development.

Certain genetic tests in the past have revealed that EVAS development is sometimes associated with mutation of SLC26A4 gene. This same gene is also responsible for causing Pendred syndrome.

Development of EVAS is also associated with another syndrome called branchio-otorenal.

Symptoms of Enlarged Vestibular Aqueduct Syndrome?

Listed below are the common symptoms associated with EVAS.

  • Vertigo
  • Dizziness
  • Instability
  • Imbalance
  • Rocking sensation
  • Vomiting
  • Nausea
  • Clumsiness
  • Abnormal movements

Treatment for Enlarged Vestibular Aqueduct Syndrome

Unfortunately, there are no treatments available to cure EVAS. Medication and surgical operation in the past have been unsuccessful in restoring vestibular and hearing loses. In the case of EVAS, doctors focus more on the prevention and will advise their patients to avoid head injuries.

Since EVAS mostly affects children, parents often have to make very difficult decision. Doctors advise minimal physical activities and that too with proper protection in place. Children are advised not to take part in their normal childhood activities. Parents have to decide if they want their kids to enjoy the childhood activities or prevent hearing loss in the future. Decision like this can be really difficult to make.

Hearing aids can be helpful for patients experiencing hearing loss. Patients suffering from EVA, however, may have significant hearing loss after some years. When hearing loss is significant, hearing aids might not help as much. When there is major hearing loss, cochlear implants can be really helpful. Studies have shown that cochlear implants greatly increased the hearing ability of patients suffering from EVAS.

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2 Responses

  1. art
    | Reply

    As an experienced educator and one with LVAS, cochlear implants are NOT an “excellent” choice for children with this syndrome, without further indepth tests. Its known now that the electrodes further disrupts more of the delicate vestibular areas from the trauma of it’s implantation. It destroys the membranes within the cochlea. If the child does not have other malformations or vestibular issues (which are usy an occurence 60% of the time later) then a CI may benefit. Best is trying with aids first til a child is able to communicate their needs. Drs are performing on babies as its much easier (cannot express their discomfort). I am one to learn too late, the horrible impact of a CI when I was performing, talking well with an aid. I am now totally deaf with further painful vestibular balance issues like menieres disease because the Dr. Did not do his job of evaluating me further. My loss not his- he earned the CI company 60k w/ another 50k towards himself and hospital. He has since disregard my new symptoms now. They have fine print forms to waiver all errors they may have not done beforehand. Pls. Do not put your child with lvas through this invasive surgery without further tests and other options first. Its electronic beeping abnormal sounds for voices VS normal sounds/tonation through an aid using residual hearing.

    • Steve
      | Reply

      Art, how old are you? How long did you have Hearing Aides prior to this surgery? What Country do you reside in and what Country & City was the CI surgery performed in??? Respectfully, Steve

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