Histopathologic Features of the Inner Ear Associated With Kearns-Sayre Syndrome
- 1 December 1976
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA Otolaryngology–Head & Neck Surgery
- Vol. 102 (12) , 747-752
- https://doi.org/10.1001/archotol.1976.00780170065011
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)This publication has 7 references indexed in Scilit:
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