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.
They say this advance offers new hope for individuals with the rare disorder, which can produce non-malignant tumors in ears, as well as in the eyes, brain, and kidneys.
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.
"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 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. Two years after the surgery, the implant has significantly improved the quality of life of the patient, he said.
Based on this successful surgery, which was published as a case report in the May issue of the journal Otology & Neurology , patients with von Hippel-Lindau disease with hearing loss may be now be candidates for a cochlear implant, Kim said. 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.