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Nonsyndromic Autosomal Dominant Progressive Sensorineural Hearing Loss: Audiologic Analysis of a Pedigree Linked to DFNA2
Author(s) -
Kunst Henricus,
Marres Henri,
Huygen Patrick,
Ensink Robert,
Van Camp Guy,
Van Hauwe Peter,
Coucke Paul,
Willems Patrick,
Cremers Cor
Publication year - 1998
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1097/00005537-199801000-00014
Subject(s) - sensorineural hearing loss , audiology , hearing loss , medicine , absolute threshold of hearing
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 kHz) than at the lower ones(approximately 0 dB at 0.25 to 1 kHz). Hearing impairment at the higher frequencies may therefore have been present already at birth or in early childhood. The regression coefficient, or the ‘annual threshold increase,’ expressed in dB/y, was about 1 dB/y on average, but the higher frequencies (1 to 8 kHz) showed significantly more rapid progression than the lower frequencies (0.25 to 0.5 kHz).