Protein involved in human hearing discovered

28 June 2006

Scientists say the protein associated with a disorder that causes deafness and blindness in people might help them determine how we hear.

Researchers at the University of Sussex, together with colleagues at the US National Institute on Deafness and Other Communication Disorders (NIDCD), have identified protocadherin-15 as a likely player in the way 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 deafness and blindness in humans.

The scientists say their findings might also help determine why some people temporarily lose their hearing after being exposed to loud noise, only to regain it a day or two later.

"Maybe there is a genetic propensity for tough ears or gentle ears," suggests Thomas Friedman, co-author of the study.

The research team hope that if protocadherin-15 proves as important as they suspect, someday scientists may devise a genetic test to see whether certain people produce a less stable form of this protein and thus have "gentle" ears prone to hearing loss.

And in the future drugs may prevent the degradation of protocadherin-15 and thereby help to preserve hearing as people age. And gene therapy might one day enable those with Usher syndrome to hear.

The research is detailed in the June 28 issue of the Journal of Neuroscience.