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John R. Lindsay, MD; Raul Hinojosa, MD

Arch Otolaryngol. 1976;102(12):747-752.

Abstract
• We describe the histopathologic features of the inner ear in a 19-year-old girl with bilateral total deafness associated with Kearns-Sayre syndrome.

The inner ear shows advanced degree of cochleosaccular degeneration, with almost complete absence of the organ of Corti in all turns. The spiral ganglion shows a reduction of about 60% to 70% of cells, with almost complete degeneration of nerve fibers in the bony spiral lamina.

PAS-positive material was found accumulated in globules between the collapsed Reissner membrane and remains of marginal cells of the stria and in the degenerated sensory cell area of the saccular macula.

(Arch Otolaryngol 102:747-752, 1976)

Author Affiliations
From the Department of Surgery, Section of Otolaryngology, Pritzker School of Medicine, University of Chicago.

Footnotes
Accepted for publication July 12, 1976.

Reprint requests to Department of Surgery, University of Chicago, 950 E 59th St, Chicago, IL 60637 (Dr Lindsay).

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