COCHLEAR AND RETINAL DEGENERATION IN THE TUBBY MOUSE

A number of autosomal recessive syndromes feature both sensorineural h earing loss and retinal degeneration. The mouse mutant tubby also comb ines hearing loss with progressive retinal degeneration, and thus may constitute a useful model of one form of human sensorineural deafness/ retinal dystrophic syndrome. It has not been directly demonstrated tha t the hearing loss in this mouse involves the cochlea, however. We hav e examined the cochleas of adult tubby mice using light microscopy. Th e tubby cochlea shows pronounced degeneration of the organ of Corti an d loss of afferent neurons in the base, with relative sparing of the a pex. Our findings support the tubby mouse as a model of human sensorin eural deafness/retinal dystrophic syndrome. Possible human counterpart s include Usher's, Alstrom's, and Bardet-Biedl syndromes.