The Etiology of Deafness in Alport's Syndrome

The cause of deafness in two patients with Alport's syndrome was evaluated by the use of serially sectioned temporal bones. Both had severe deafness of similar degree. Histologically, one showed a normal inner ear while the other had a marked reduction in spiral ganglion cochlear neurons. The reported otopathology is reviewed. It is concluded that Alport's syndrome is a heterogeneous group of similar diseases.