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Article

Hereditary cochleovestibular dysfunction due to a COCH gene mutation (DFNA9): a follow-up study of a family.

Clinical otolaryngology and allied sciences - 1 Dec 2001

Verhagen W I, Bom S J, Fransen E, Van Camp G, Huygen P L, Theunissen E J, Cremers C W

Abstract excerpt

Cochleovestibular impairment was evaluated, in relation to age, in a longitudinal follow-up study on a Dutch family with a DFNA9 trait caused by a Pro51Ser mutation in the COCH gene on chromosome 14q12-q13. Fourteen cases were genotyped. The onset age of progressive impairment reported by the mutation carriers was between age 35 and 45 years. Pure-tone thresholds deteriorated by about 2-7 dB per year (mean 3.8 dB...

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Source

Identifiers

PMID: 11843927
DOI: 10.1046/j.1365-2273.2001.00505.x

Genes

COCH

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Hereditary cochleovestibular dysfunction due to a COCH gene mutation (DFNA9): a follow-up study of a family.PMID 11843927Read ↗

Vestibular deterioration precedes hearing deterioration in the P51S COCH mutation (DFNA9): an analysis in 74 mutation carriers.Semantic proximity Read ↗

A Pro51Ser mutation in the COCH gene is associated with late onset autosomal dominant progressive sensorineural hearing loss with vestibular defectsSemantic proximity Read ↗

Hereditary Otovestibular Dysfunction and M??ni??re's Disease in a Large Belgian Family Is Caused by a Missense Mutation in the COCH GeneSemantic proximity Read ↗

Audiometric, vestibular, and genetic aspects of a DFNA9 family with a G88E COCH mutation.Semantic proximity Read ↗

Cross-sectional analysis of hearing threshold in relation to age in a large family with cochleovestibular impairment thoroughly genotyped for DFNA9/COCH.Semantic proximity Read ↗

[From gene to disease; a progressive cochlear-vestibular dysfunction with onset in middle-age (DFNA9)].Semantic proximity Read ↗

Bi-allelic inactivating variants in the COCH gene cause autosomal recessive prelingual hearing impairment.Semantic proximity Read ↗

High prevalence of symptoms of Menière's disease in three families with a mutation in the COCH gene.Semantic proximity Read ↗

Genotype-Phenotype Correlations of Pathogenic COCH Variants in DFNA9: A HuGE Systematic Review and Audiometric Meta-Analysis.Semantic proximity Read ↗

Clinical characterization of a novel COCH mutation G87V in a Chinese DFNA9 family.Semantic proximity Read ↗

A novel mutation in COCH-implications for genotype-phenotype correlations in DFNA9 hearing loss.Semantic proximity Read ↗

Clinical characteristics of a Dutch DFNA9 family with a novel COCH mutation, G87W.Semantic proximity Read ↗

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