A Review of Progressive Phenotypes in Nonsyndromic Autosomal Dominant Hearing Impairment

RONALD JE PENNINGS Department of Otorhinolaryngology University Medical Centre St Radboud Nijmegen The NetherlandsCorrespondence[email protected]

PATRICK LM HUYGEN Department of Otorhinolaryngology University Medical Centre St Radboud Nijmegen The Netherlands

GUY VAN CAMP Department of Medical Genetics Universitaire Instellingen Antwerpen Antwerp Belgium

COR WRJ CREMERS Department of Otorhinolaryngology University Medical Centre St Radboud Nijmegen The Netherlands

Abstract

This review focuses on the following progressive nonsyndromic autosomal dominant (DFNA) trait loci: DFNA1, DFNA2, DFNA4, DFNA5, DFNA6/14(/38), DFNA7, DFNA9, DFNA10, DFNA15, DFNA16, DFNA17, DFNA20/26 and DFNA21. It describes age-related pure tone thresholds and speech discrimination scores related to age and the level of hearing impairment. Vestibular findings, as far as previous described, are included as well.

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A Review of Progressive Phenotypes in Nonsyndromic Autosomal Dominant Hearing Impairment

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A Review of Progressive Phenotypes in Nonsyndromic Autosomal Dominant Hearing Impairment

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