CLEVELAND April 13, 2009 Qing Yin Zheng, M.D., assistant professor of Otolaryngology-Head and Neck Surgery and Genetics at the Case Western Reserve University School of Medicine, and member of the Case Comprehensive Cancer Center, has been awarded a five year $1.66 million grant from the National Institutes of Health (NIH) Research Project Grant Program (R01) to explore the impact of several molecular pathways on inner ear dysfunction in Ushers syndrome.
Usher Syndrome (USH) is a clinically and genetically heterogeneous disorder characterized by congenital deafness and retinitis pigmentosa. It is the most common cause of deafness accompanied by blindness. *There are about 10-15,000 people with Usher syndrome in the United States today and it accounts for 6 percent of the congenitally deaf population and more than 50 percent of the deaf- blind population. While there is currently no medical treatment for Usher syndrome, cochlear implants have been found to be very affective in patients with severe or profound hearing loss.
"Many USH patients benefit from cochlear implants, but preservation and a minimal density of spiral ganglion cells are required for an effective implant," said Zheng.
Spiral ganglion cells are the cells that provide the sense of hearing by sending sound waves from the cochlea to the brain. Dr. Zheng believes residual hair cells in the cochlea could promote the survival of the spiral ganglion neurons by release of neurotrophic substances or growth factors, and as a result he is particularly interested in the discovery of therapeutic targets that prevent hair cell death, as this is the key to helping Usher 1 patients to respond to cochlear implants and other treatment options successfully.
"Hair cell replacement or regeneration therapies have not really solved the deafness problem of ear diseases," said Zheng. "An alternative to replacing lost hair cells is to prevent their loss in the first place."
|Contact: Christina DeAngelis|
Case Western Reserve University