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Otol Neurotol. 2003 Sep;24(5):743-8.

Progressive late-onset sensorineural hearing loss and vestibular impairment with vertigo (DFNA9/COCH): longitudinal analyses in a belgian family.

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  • 1Department of Otorhinolaryngology and Head and Neck Surgery, University Hospitals Leuven, Belgium.

Abstract

OBJECTIVE:

To evaluate audiometric and vestibular signs and symptoms in a new DFNA9 family.

SETTING:

Tertiary referral centers.

METHODS:

A multigeneration Belgian family with late-onset progressive sensorineural hearing loss and concomitant ves-tibular impairment with an autosomal dominant pattern of inheritance underwent clinical and genetic evaluation. Medical history was recorded. Blood samples were taken for genetic linkage and mutation analyses. Pure-tone audiometry, speech audiometry and vestibular examinations were performed. Onset and progression in hearing impairment were evaluated with linear regression analysis of longitudinal threshold-on-age data.

RESULTS:

Linkage to DFNA9 was confirmed and mutation analysis revealed a P51S mutation in the COCH gene. Several patients had a Ménière's-like presentation. All patients developed late-onset progressive sensorineural hearing loss eventually leading to severe deafness and vestibular failure.

PMID:
14501450
[PubMed - indexed for MEDLINE]
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