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Syndrome

Enlarged

Aqueduct

Vestibular

Made By: Akeyia Watts

What is the cause? EVA has many causes, but the most well-known in "EVA and hearing loss is mutations to a gene known as SLC26A4 (also referred to as the PDS gene) on chromosome 7. Scientists believe that other, currently unknown genetic or environmental factors also may result in EVA."

What is it? "Large vestibular aqueduct, also known as enlarged vestibular aqueduct syndrome(EVAS), is a syndromic form of hearing loss, caused by enlargement of the vestibular aqueduct, in the inner ear. It is one of the most common inner ear deformities which results in hearing loss during childhood."

Is it common/rare? Can it be cured with medication or surgery? EVAS is considered to be rare. In the past, "medical and surgical treatments have not reversed" it. Currently, to manage it, the best way is "prevention, primarily by protecting the head from traumas that will worsen the progression of hearing loss and vestibular symptoms." With greater hearing losses, hearing aids can be used, but their often unsuccessful. "For some, cochlear implantation has significantly improved hearing. In some people with related vestibular symptoms, treatment may include vestibular rehabilitation therapy; however, as is also common for patients experiencing an active phase of Meniere’s disease, EVAS may not respond well to vestibular rehabilitation."

What are the resuts? "Hearing loss; distorted or fluctuating hearing; Tinnitus (ringing, roaring, buzzing, whooshing, or other noises in the ear); Sensitivity to loud noises or environments; Sudden loud sounds may increase symptoms of vertigo, dizziness, or imbalance."

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