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Hereditary deafness affects 1 in 2000 children and many of the mutated genes responsible have been identified. In her Perspective, Karen Steel provides us with an update on the latest findings in deafness research including results in this issue ( Minowa et al.). The Minowa study shows that disruption of the cation balance in endolymph (the fluid in the cochlea of the inner ear) results in impaired hearing in a mouse model of human deafness in which the transcription factor Pou3f4 is inactivated. In these mice the fibrocyte cells of the spiral ligament (part of the cochlea), which are responsible for recycling Kions back into the endolymph, were found to be abnormal.
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Karen P. Steel (1999) studied this question.
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