PMID: 9432071Jan 31, 1998Paper

Nonsyndromic autosomal dominant progressive sensorineural hearing loss: audiologic analysis of a pedigree linked to DFNA2

The Laryngoscope
H KunstC Cremers

Abstract

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).

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Citations

Mar 13, 2002·The Laryngoscope·Yuya TamagawaKeiichi Ichimura
Apr 13, 2000·Proceedings of the National Academy of Sciences of the United States of America·T KharkovetsT J Jentsch
Feb 9, 2005·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Vedat TopsakalCor W R J Cremers
Jul 13, 2000·British Journal of Audiology·P Van HauweG Van Camp
Feb 13, 2007·Brain Research·Sonia M S Rocha-SanchezKirk W Beisel
Jun 12, 2013·Journal of Cellular and Molecular Medicine·Yanhong GaoLiping Nie
Mar 28, 2002·The Annals of Otology, Rhinology, and Laryngology·Els M R De LeenheerCor W Cremers
May 19, 2011·The Annals of Otology, Rhinology, and Laryngology·Anne-Martine R de HeerCor W R J Cremers

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