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October 1972

The Etiology of Deafness in Alport's Syndrome

Author Affiliations

From the Department of Neurology, Harvard Medical School, and the Section of Neurology, Peter Bent Bringham Hospital, Boston. Dr. Myers is currently at the University of Rochester School of Medicine and Dentistry, Rochester, NY.

Arch Otolaryngol. 1972;96(4):333-340. doi:10.1001/archotol.1972.00770090509007

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.

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