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.