Clarification of the mechanism of the cochlear nerve degeneration in an animal model of deafness

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DFN3, an X-linked nonsyndromic mixed deafness is caused by mutations in BRN gene, which encodes a POU transcription factor gene. By gene targeting technology Brn-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 drastic reduction in endocochlear potential (EP). Electron microscopy revealed severe ultrastructural alterations in cochlear spiral ligament fibrocytes. These findings suggest that these fibrocytes, which are mesenchymal in origin and have been postulated to function in K^+ homeostasis, may play a critical role in auditory function and show a major cause of the hereditary deafness.