Genetic basis of early hearing loss uncovered

How genetic mutation causes people to lose their hearing in their early twenties,says scientists.

Written by PTI | Melbourne | Published: June 17, 2013 9:29:37 am

Australian scientists have claimed to have discovered how a genetic mutation causes people to lose their hearing in their early twenties.

According to Monash university statement,scientists from its university and University of Melbourne found the link. The study,now published in the American Journal of Pathology,has been led by Phillip Bird and Dion Kaiserman from Monash University’s School of Biomedical Sciences.

It builds understanding of a genetic mutation discovered in 2010.

The mutation induces malfunction of an inhibitor,SERPINB6,which protects inner ear cells from destruction. “We cannot yet identify how SERPINB6 mutation accelerates hearing loss,but affected people can lose their hearing in their twenties,rather than their sixties,” Bird said.

Using animal models to mimic the progression of SERPINB6-accelerated hearing loss in humans,Justin Tan from Melbourne University’s Department of Otolaryngology found that specialised hearing cells in the inner ear were affected. The sensory cells that detect sound and neighbouring fibrocytes that support them were destroyed. Bird said that the destruction of fibrocytes was uncommon and significant. “We are finally beginning to understand the role of these proteins in protecting our hearing,” he said.

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