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You are here : 3-RX.com > Home > Ear / Nose / Throat - Genetics -

Genetic disease carries risk of sudden deafness

Ear / Nose / Throat • • GeneticsJul 04, 07

People diagnosed with a genetic disease called von Hippel-Lindau disease or VHL may suddenly experience hearing loss caused by a tumor, researchers advise in this week’s Journal of the American Medical Association.

VHL disease is characterized by the development of tumors in blood vessels in the retina of the eye and in the brain. Lesions and cysts can also develop in other parts of the body.

Tumors of the inner ear occur sporadically in the general population but may be particularly associated with VHL. These tumors are associated with significant impairment of hearing and balance, including sudden irreversible hearing loss, the new study confirms.

Dr. John A. Butman, of the National Institutes Health, Bethesda, Maryland, and colleagues studied 35 VHL patients with inner ear tumors, three of whom had tumors in both ears. The found that hearing loss was the most common symptom associated with inner ear tumors.

In 7 patients, the tumor had invaded the inner ear. In the other 31 ears, hearing loss developed gradually in 13 and suddenly in 14, while 4 had normal hearing.

Butman and colleagues say “early surgical intervention may be warranted” in people with inner ear tumors. Surgery for these tumors has been shown to safely and effectively alleviate symptoms while preserving hearing, they note.

SOURCE: Journal of the American Medical Association, July 3, 2007.

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