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- Title
Histopathology of the Human Inner Ear in Alström's Syndrome.
- Authors
Nadol Jr, Joseph B.; Marshall, Jan D.; Bronson, Roderick T.
- 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
- Subjects
ALSTROM syndrome; NEUROLOGICAL disorders; EAR abnormalities; GENETIC disorders; DEAFNESS; INNER ear
- Publication
Audiology & Neurotology, 2015, Vol 20, Issue 4, p267
- ISSN
1420-3030
- Publication type
Article
- DOI
10.1159/000381935