Cochlear and retinal degeneration in the Tubby mouse

Kevin K. Ohlemiller, Ruth M. Hughes, Judith Mosinger-Ogilvie, Judith D. Speck, David H. Grosof, Martin S. Silverman

Research output: Contribution to journalArticlepeer-review

72 Scopus citations

Abstract

A number of autosomal recessive syndromes feature both sensorineural hearing loss and retinal degeneration. The mouse mutant tubby also combines hearing loss with progressive retinal degeneration, and thus may constitute a useful model of one form of human sensorineural deafness/retinal dystrophic syndrome. It has not been directly demonstrated that the hearing loss in this mouse involves the cochlea, however. We have examined the cochleas of adult tubby mice using light microscopy. The tubby cochlea shows pronounced degeneration of the organ of Corti and loss of afferent neurons in the base, with relative sparing of the apex. Our findings support the tubby mouse as a model of human sensorineural deafness/retinal dystrophic syndrome. Possible human counterparts include Usher’s, Alstrom’s, and Bardet–Biedl syndromes.

Original languageEnglish
Pages (from-to)845-849
Number of pages5
JournalNeuroReport
Volume6
Issue number6
DOIs
StatePublished - Apr 1995

Keywords

  • Alstrom’s syndrome
  • Bardet–Biedl syndrome
  • Cochlear histology
  • Retinal histology
  • Tubby mouse
  • Usher’s syndrome

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