Autosomal Dominant Non-Syndromic Hearing Loss

Late onset progressive deafness is the most common neurological disability of the elderly. Although hearing loss of greater than 25 decibels is present in only 1% of young adults between the ages of 18-24 years of age, this increases to 10% in persons between 55-64 years of age and approximately 50% in octogenarians.

The relative contribution of heredity to age –related hearing impairment is not known, however the majority of inherited late-onset deafness is autosomal dominant and non-syndromic (Van Camp et al., 1997). Over forty genes associated with autosomal dominant non-snydromic hearing loss have been localized and of these fifteen have been cloned. Although the function of many of these genes in the inner ear is unclear, an understanding of the biology of hearing and deafness at a molecular level is beginning to emerge. We propose to continue our work localizing and cloning genes that cause autosomal dominant non-syndromic hearing loss. Additionally, we will initiate functional studies on two of the genes cloned during the last grating period.

Molecular Otolaryngology Research Laboratories