Histopathology of the Human Inner Ear in Alström's Syndrome.
Document Type
Article
Publication Date
2015
JAX Source
Audiol Neurotol 2015 20:267-272.
Volume
20
Issue
4
First Page
267
Last Page
272
ISSN
1421-9700
PMID
26111748
Grant
HD036878
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. © 2015 S. Karger AG, Basel. Audiol Neurotol 2015 20:267-272.
Recommended Citation
Nadol J,
Marshall JD,
Bronson R.
Histopathology of the Human Inner Ear in Alström's Syndrome. Audiol Neurotol 2015 20:267-272.