Nav: Home

For first time, cochlear implant restores hearing to patient with rare genetic disorder

June 07, 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.

-end-

About Georgetown University Medical Center

Georgetown University Medical Center is an internationally recognized academic medical center with a three-part mission of research, teaching and patient care (through our partnership with MedStar Health). Our mission is carried out with a strong emphasis on public service and a dedication to the Catholic, Jesuit principle of cura personalis -- or "care of the whole person." The Medical Center includes the School of Medicine and the School of Nursing and Health Studies, both nationally ranked, the world-renowned Lombardi Comprehensive Cancer Center and the Biomedical Graduate Research Organization (BGRO).

Georgetown University Medical Center
Researchers listen to zebrafish to understand human hearing loss
Can a fish with a malformed jaw tell us something about hearing loss in mice and humans?
Postmenopausal hormone therapy associated with higher risk of hearing loss
Use of postmenopausal hormone therapy was associated with higher risk of hearing loss, and the risk tended to increase with longer duration of use.
Few researchers consider hearing loss in healthcare communication: Study
Of the 67 papers reviewed, only 16 (23.9 percent) included any mention of hearing loss.
Few studies consider hearing loss when assessing communication with physicians
Doctors believe that communication with those under their care is important, but most studies of communication between physicians and older adults do not mention that hearing loss may affect this interaction.
Study shows hearing tests miss common form of hearing loss
Traditional clinical hearing tests often fail to diagnose patients with a common form of inner ear damage that might otherwise be detected by more challenging behavioral tests, according to the findings of a University at Buffalo-led study published in the journal Frontiers in Neuroscience.
Drug treatment could combat hearing loss
Researchers at MIT and Brigham and Women's Hospital have discovered a combination of drugs that induces supporting cells in the ear to differentiate into hair cells, offering a potential new way to treat hearing loss.
Second cause of hidden hearing loss identified
Some people can pass a hearing test but have trouble understanding speech in a noisy environment.
U study: Law aiding infants at risk for hearing loss
A Utah law has led to increased early identification of infants with hearing loss due to a congenital infection, according to a new study by University of Utah and Utah Department of Health researchers.
MED-EL convenes global hearing researchers for age-related hearing loss workshop
Leading scientists and hearing experts from around the world will gather for a scientific workshop sponsored by hearing implant leader MED-EL.
Iron deficiency anemia associated with hearing loss
In a study published online by JAMA Otolaryngology-Head & Neck Surgery, Kathleen M.

Best Science Podcasts 2017

We have hand picked the best science podcasts for 2017. Sit back and enjoy new science podcasts updated daily from your favorite science news services and scientists.

Now Playing: Radiolab

Truth Trolls
Today, a third story of folks relentlessly searching for the truth. But this time, the truth seekers are an unlikely bunch... internet trolls.


Now Playing: TED Radio Hour

Rethinking School
For most of modern history, humans have placed smaller humans in institutions called schools. But what parts of this model still work? And what must change? This hour, TED speakers rethink education.TED speakers include teacher Tyler DeWitt, social entrepreneur Sal Khan, international education expert Andreas Schleicher, and educator Linda Cliatt-Wayman.