Richard Hallworth, Ph.D.
Department of Otolaryngology-Head and Neck Surgery
University of Texas Health Science Center at San Antonio
San Antonio TX 78284
Sensorineural hearing loss is the most intractable of hearing loss types because its fundamental cause is hair cell death. Spectacular advances have recently been made in our understanding of hair cells physiology and molecular biology that offer new hope of alleviating sensorineural hearing loss. In this talk, I will highlight four areas of very promising research. First, about 40 genes for hereditary hearing loss have now been described, 10 of these in 1998-1999 alone. I will discuss four of these genes and show how identification of their gene products have taught us much about progressive sensorineural hearing loss and, by inference, about traumatic hearing loss. Second, a new method of introducing genes selectively into hair cells by viral transfection has been described. The method could be used to "repair" defective genes in hereditary hearing loss or to turn off the response to acoustic trauma. Third, I will discuss the promotion of hair cell regeneration by targeted blockade of the gene p27Kip1. Finally, I will describe new work that shows that the ototoxicant quinine has effects on hair cells as well as the central nervous system.