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Bionic Ear Restores Hearing to Von Hippel-Lindau Disease Patients

Scientists have for the first time shown that a bionic ear may help restore hearing in patients with von Hippel-Lindau disease.

Researchers at Georgetown University Medical Center and the National Institutes of Health (NIH) say that their work offers new hope for patients with the rare disorder, which can produce non-malignant tumors in ears, as well as in the eyes, brain, and kidneys.

The researchers say that they could achieve this advancement because such tumors do not affect the cochlear nerve necessary for receipt of sound in the brain.

The device used in the surgery is known as a cochlear implant, which stimulates the cochlear nerve with electrical impulses, and is predominately used to treat the deaf.

Based on our understanding of how these tumors affect the inner ear, we felt that a cochlear implant could work, and it did, said the studys lead author, Dr. H. Jeffrey Kim, Assistant Professor in the Department of Otolaryngology.

He further said that the quality of life of the patient has significantly improved two years after the surgery.

Dr. Kim says that about 30 per cent of the patients with von Hippel-Lindau disease develop tumors in their ears, and the only option to help control such tumors is repeated surgery, which is often unsuccessful.

Such tumors occur in the endolymphatic sac, part of the inner ear labyrinth of fluid passages. They are benign, but are invasive, and can cause hemorrhages that lead to tinnitus, vertigo, and hearing loss.

Dr. Kims study also sheds light on other ear problems, including Mnire's Disease, a disorder of the inner ear that can affect hearing and balance due to pressure in the same endolymphatic sacs.

This is a much more common condition, so we hope that what we learn from von Hippel-Lindau disease may help in the treatment of hearing problems that affect many of us, he said.


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