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."
This grant is Dr. Zheng's fourth NIH grant for research involving auditory disease with accumulated $4 million funding.
"A clear translational goal, expertise of his research team and a very strong publication record, Dr. Zheng has potential to uncover new therapeutic approaches to treat hearing loss in Usher syndrome. I'm very grateful for the NIH for its forward-thinking commitment to Dr. Zheng and his innovative medical science research," said Dr. James Arnold, Julius McCall Professor and Chair of the Department of Otolaryngology Head and Neck Surgery at Case Western Reserve University School of Medicine.
Dr. Zheng aims to: 1) identify the mutation in and characterize a new mouse deafness model (for Usher 1 syndrome and presbycusis or age-related hearing loss) that provides an ideal window of time for evaluating drug therapy; 2) identify key molecules and mechanisms that lead to hair cell death and hearing loss in the models for the Usher 1 syndrome; 3) prove the concept that genetic hearing loss and hair cell death can be prevented by otoprotection therapy.
"We are very honored and pleased that our department has been given this significant award from the NIH," says Dr. Cliff Megerian, Richard W. and Patricia Pogue Professor of Auditory Surgery and Hearing Sciences at University Hospitals Case Medical Center. "The impact of deafness on quality of life and language development is devastating. The financial impact and individual human suffering are devastating, due to the lack of effective drug therapy," adds Dr. Megerian who has collaborated on several research projects with Dr. Zheng.
|Contact: Christina DeAngelis|
Case Western Reserve University