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Genetic Cause For Type Of Deafness Identified; Discovery Could Lead To New Therapies For Progressive Hearing Loss

Posted on 06 September 2009 - 13:34 by Alfie

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Story Summary: Genetic Cause For Type Of Deafness Identified; Discovery Could Lead To New Therapies For Progressive Hearing Loss A team led by scientists from The Scripps Research Institute has discovered a genetic cause of progressive hearing loss. The findings will help scientists better understand the nature of age-related decline in hearing and may lead to new therapies to prevent or treat the condition. In this new research, we have linked a previously uncharacterized gene to deafness, first in mice and then in humans. Tracking Down a New GeneIn the new study, members of the Mueller lab used a technique called forward genetics in their quest to better understand the genetic basis of hearing and hearing loss. In this case, the scientists were able to generate a new mouse line with hearing impairment that they called samba and then clone the gene responsible, Loxhd1, which had never before been associated with deficits in hearing. When the mice inherited two copies of the mutated gene, they were profoundly deaf shortly after birth. Normally, hair cells or stereocilia in the inner ear respond to fluid motion or fluid pressure changes caused by sound waves that enter the outer ear, travel down the ear canal into the middle ear, then strike the eardrum, which vibrates and moves a set of delicate bones that communicate with the inner ear. There, the movement of the stereocilia transmits signals to sensory neurons, sending signals to the brain and eventually resulting in hearing. The scientists found that mutations in the Loxhd1 gene did not appear to affect the initial development of the stereocilia. Clues to Age-Related DeafnessThis is the third hearing-related gene that the Mueller lab has discovered, and one he is particularly excited about. Since this mutation can lead to progressive hearing loss, it provides us with more information on the genetic underpinnings of this condition and gives us clues as to how it might be corrected. In Other News . . . Or view hourly updated newsfeeds in your RSS reader:FeedbackTell us what you think of the new ScienceDaily — we welcome both positive and negative comments. Have any problems using the site?…Read the Full Story

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