Researchers cure mouse deafness with gene therapy

Researchers have for the first time used gene therapy to restore normal hearing to mice that were born deaf.  The findings could lead to a successful therapy for people with congenital hearing loss.

Congenital hearing loss or deafness from birth is one of the most common sensory deficits. It is often the result of genetic defects that affect the proper functioning of hair cells inside the inner ear. The hair cells, which move in response to sound vibration, transmit auditory signals to the brain. Experts believe there may be as many as 100 genes whose mutation can cause some form of hearing loss.

Researchers created a mouse model of congenital deafness in humans by breeding rodents that lacked the gene that makes vesicular glutamate transporter-3, or VGLUT3 for short - an essential protein that enables hair cells in the ear to receive and send auditory signals. People with a defective copy of the VGLUT-3 gene suffer diminished hearing over time. Because the experimental mice didn’t have the gene at all, their deafness was profound.

 Lawrence Lustig, an otolaryngologist or hearing specialist at the University of California San Francisco, says researchers used a virus that...

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