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Protein tied to usher syndrome may be hearing's 'Missing Link'

A protein associated with a disorder that causes deafness and blindness in people may be a key to unraveling one of the foremost mysteries of how we hear, says a study in the June 28 issue of the Journal of Neuroscience. Scientists with the National Institute on Deafness and Other Communication Disorders (NIDCD), one of the National Institutes of Health (NIH), and the University of Sussex, Brighton, United Kingdom, have identified protocadherin-15 as a likely player in the moment-of-truth reaction in which sound is converted into electrical signals. (Protocadherin-15 is a protein made by a gene that causes one form of type 1 Usher syndrome, the most common cause of deaf-blindness in humans.) The findings will not only provide insight into how hearing takes place at the molecular level, but also may help us figure out why some people temporarily lose their hearing after being exposed to loud noise, only to regain it a day or two later.

"These findings offer a more precise picture of the complicated processes involved with our sense of hearing," says Elias A. Zerhouni, M.D., director of the NIH. "With roughly 15 percent of American adults reporting some degree of hearing loss, it is increasingly vital that we continue making inroads into our understanding of these processes, helping us seek new and better treatments, and opening the doors to better hearing health for Americans."

Tapping Your Inner "Tip Link"

Researchers have long known that hair cells, small sensory cells in the inner ear, convert sound energy into electrical signals that travel to the brain, a process called mechanotransduction. However, the closer one zooms in on the structures involved, the murkier our understanding becomes. When fluid in the inner ear is set into motion by vibrations emanating from the bones of the middle ear, the rippling effect causes bristly structures atop the hair cells to bump up against an overlying membrane and to deflect. Like
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Contact: Jennifer Wenger
jwenger@mail.nih.gov
301-496-7243
NIH/National Institute on Deafness and Other Communication Disorders
27-Jun-2006


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