The study of the Bronx Waltzer mouse strain, with the gene BV that underlies the deafness of this mouse, is being continued. As previously reported, we have located the gene to the long arm of chromosome 5 and are attempting to further define the gene. The study of a transgenic insertional mutation affecting the mouse gene mi and causing an inner ear disorder in the mouse has been continued. We have cloned the human gene that is homologous to the mouse gene mi and mapped this human gene to chromosome 3p14.1-p12.3. Strong evidence has been published from a British laboratory that a mutation of this human gene can cause Waardenburg syndrome type II. We have continued our study of the distribution of AMPA glutamate receptors in the inner ear, with the working hypothesis that such receptors may play a role in regeneration of auditory sensory cells in the inner ear. Findings have indicated that the amount of RNA messages required for the expression of AMPA receptors in the inner ear would be quite low. We have initiated a collaboration wit the LCB, NIDCD, for the use of monoclonal antibodies to define and study the distribution of proteins that are of particular importance for the auditory functions of the inner ear.
