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Audiol Neurootol. 2015;20(4):267-72. doi: 10.1159/000381935. Epub 2015 Jun 24.

Histopathology of the human inner ear in Alström's syndrome.

Author information

1
Otopathology Laboratory, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Mass., USA.

Abstract

Alström's syndrome is an autosomal recessive syndromic genetic disorder caused by mutations in the ALMS1 gene. Sensorineural hearing loss occurs in greater than 85% of patients. Histopathology of the inner ear abnormalities in the human has not previously been fully described. Histopathology of the inner ear in Alström's syndrome is presented in 2 genetically confirmed cases. The predominant histopathologic correlates of the sensorineural loss were degeneration of the organ of Corti, both inner and outer hair cells, degeneration of spiral ganglion cells, and atrophy of the stria vascularis and spiral ligament.

PMID:
26111748
PMCID:
PMC4540704
DOI:
10.1159/000381935
[Indexed for MEDLINE]
Free PMC Article

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