Researchers at Georgetown University Medical Center and the National Institutes of Health (NIH) have, for the first time, used a “bionic” ear to restore hearing in a patient with von Hippel-Lindau disease.

The disease, caused by inheritance of a mutated tumor suppressor gene, occurs in 1 out of 36,000 live births, and about 30 percent of these patients develop tumors in their ears − often in both. To date, the only option to help control these tumors is repeated surgery, which is often not successful, he said. Loss of hearing is sudden, and hearing aids don’t help, Kim said.



Based on this successful surgery, patients with von Hippel-Lindau disease with hearing loss may be now be candidates for a cochlear implant, said the study’s lead author, H. Jeffrey Kim, M.D., an assistant professor in the Department of Otolaryngology − Head and Neck Surgery, and a part-time investigator at the NIH, where the surgery was performed.

The advance was possible, researchers say, because their years of research into the disease showed that these tumors do not affect the cochlear nerve necessary for receipt of sound in the brain. The device they used is known as a cochlear implant, which stimulates the cochlear nerve with electrical impulses. It is predominately used to treat the deaf.

Two years after the surgery, the implant has significantly improved the quality of life of the patient, he said.

Kim’s research also sheds light on other ear problems, including Ménière'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.