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.
Myers GJ, Tyler HR. The Etiology of Deafness in Alport's Syndrome. Arch Otolaryngol. 1972;96(4):333–340. doi:10.1001/archotol.1972.00770090509007
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