Article
Nonsyndromic autosomal dominant progressive sensorineural hearing loss: audiologic analysis of a pedigree linked to DFNA2.
The Laryngoscope - 1 Jan 1998
Kunst H, Marres H, Huygen P, Ensink R, Van Camp G, Van Hauwe P, Coucke P, Willems P, Cremers C
Abstract excerpt
An analysis was performed of the regression of the individual hearing threshold on age in the affected persons in a six-generation Dutch family with nonsyndromic autosomal dominant sensorineural hearing loss, which showed linkage to the DFNA2(1p34) region, similar to at least four previously reported nonrelated families. The offset threshold was significantly higher at the high frequencies (around 30 dB at 2 to 8...
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