Histopathology of the human inner ear in Alström's syndrome

Joseph B. Nadol, Jan D. Marshall, Roderick T. Bronson

Research output: Contribution to journalArticlepeer-review

9 Scopus citations


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.

Original languageEnglish
Pages (from-to)267-272
Number of pages6
JournalAudiology and Neuro-Otology
Issue number4
StatePublished - 20 Aug 2015
Externally publishedYes


  • ALMS1
  • Alström's syndrome
  • Histopathology
  • Human
  • Inner ear


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