For First Time, Cochlear Implant Restores Hearing To Patient With Rare Genetic DisorderJune 08, 2007
Washington, D.C.--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.
These 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. Kim and his colleagues have been following a population of patients with the disorder and are national leaders in characterizing the disorder's effect on the ears. They have published a series of findings in such journals as the New England Journal of Medicine (NEJM) and the Journal of the American Medical Association (JAMA).
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.
Georgetown University Medical Center
Related Cochlear Implant Current Events and Cochlear Implant News Articles
New computer program predicts cochlear implant success in hearing-impaired children
A new computer program that analyzes functional brain MRIs of hearing impaired children can predict whether they will develop effective language skills within two years of cochlear implant surgery, according to a study in the journal Brain and Behavior.
Early evidence suggests hybrid cochlear implants may benefit millions with common form of hearing loss
People with a common form of hearing loss not helped by hearing aids achieved significant and sometimes profound improvements in their hearing and understanding of speech with hybrid cochlear implant devices, according to a new multicenter study led by specialists at NYU Langone Medical Center.
Experts map surgical approaches for auditory brainstem implantation
A technique called auditory brainstem implantation can restore hearing for patients who can't benefit from cochlear implants.
Study: Infants Benefit from Implants with More Frequency Sounds
A new study from a UT Dallas researcher demonstrates the importance of considering developmental differences when creating programs for cochlear implants in infants.
Design prototype chip makes possible a fully implantable cochlear implant
Researchers from Massachusetts Eye and Ear, Harvard Medical School, and Massachusetts Institute of Technology (MIT) have designed a prototype system-on-chip (SoC) that could make possible a fully implanted cochlear implant.
Cochlear implants -- with no exterior hardware
Cochlear implants - medical devices that electrically stimulate the auditory nerve - have granted at least limited hearing to hundreds of thousands of people worldwide who otherwise would be totally deaf.
New strategy lets cochlear implant users hear music
For many, music is a universal language that unites people when words cannot. But for those who use cochlear implants - technology that allows deaf and hard of hearing people to comprehend speech - hearing music remains extremely challenging.
New technology will improve neuron activation induced by cochlear implants
Cochlear implants, electrical prosthetic devices that stimulate inner ear neurons of individuals who have lost their cochlear sensory cells, restore usable hearing to deaf patients.
Mom's sensitivity helps language development in children with hearing loss
University of Miami (UM) Psychologist Alexandra L. Quittner leads one of the largest, most nationally representative studies of the effects of parenting on very young, deaf children who have received cochlear implants.
Noisy classroom simulation aids comprehension in hearing-impaired children
Children with hearing loss struggle to hear in noisy school classrooms, even with the help of hearing aids and other devices to amplify their teacher's voice.
More Cochlear Implant Current Events and Cochlear Implant News Articles