Altered Cochlear Fibrocytes in a Mouse Model of DFN3 Nonsyndromic Deafness

Science  27 Aug 1999:
Vol. 285, Issue 5432, pp. 1408-1411
DOI: 10.1126/science.285.5432.1408

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DFN3, an X chromosome-linked nonsyndromic mixed deafness, is caused by mutations in the BRN-4 gene, which encodes a POU transcription factor. Brn-4-deficient mice were created and found to exhibit profound deafness. No gross morphological changes were observed in the conductive ossicles or cochlea, although there was a dramatic reduction in endocochlear potential. Electron microscopy revealed severe ultrastructural alterations in cochlear spiral ligament fibrocytes. The findings suggest that these fibrocytes, which are mesenchymal in origin and for which a role in potassium ion homeostasis has been postulated, may play a critical role in auditory function.

  • * These authors contributed equally to this work.

  • To whom correspondence should be addressed. E-mail: tnoda{at}

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