Editor's Choice Academic Journal Main Category: Hearing / Deafness Also Included In: Genetics Article Date: 05 Feb 2013 - 11:00 PST

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The animal study, which is still in its early stages, could eventually develop into new treatments for Usher syndrome, a congenital hearing disorder which usually goes hand-in-hand with blindness as well.

When the scientists injected the profoundly deaf mice with the genetic patch, they developed into partially hearing mice with no balance problems.

The authors explained that deafness is the most common sensory disorder. Approximately 1 in every 1,000 newborns is born with a hearing impairment. Congenital deafness is often the result of the improper development or degeneration of cochlear hair cells which form the tiny hairs in the ear that detect sound.

In one type of Usher syndrome - known as Type 1 Usher Syndrome - which French settlers brought to the USA hundreds of years ago, there is a problem with a protein called harmonin. Harmonin is required in order to form the cochlear hair cells. The same problem also causes gradual loss of vision.

In type 1 Usher syndrome, there is a mutation in the USH1C gene. This gene controls the production of harmonin. When USH1C has a fault (mutation), it produces truncated forms of harmonin.

They injected the mice, which had been genetically engineered to have Usher syndrome, with the genetic patch. Initially, they all grew up with no balance problems and reasonable hearing. This went on for a couple of months; their hearing was near-normal in the lower frequencies.

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Tiny Genetic Patch Stops Deafness

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