Animal models of pigment and hearing abnormalities in man.

The inherited syndrome of irregular depigmentation of the skin, hair, iris and ocular fundus with associated congenital defects of the inner ear resulting in hearing loss or deafness occurs in several different species including man. The "deaf white cat" provides a useful model of this syndrome. There are several genetic mechanisms involved. The deafness is sometimes progressive in the newborn period. The pathology suggests involvement of the neural crest.