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Article

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

Nederlands tijdschrift voor geneeskunde - 19 Nov 2005

Cremers C W R, Kemperman M H, Bom S J H, Huygen P L M, Verhagen W I M, Kremer J M J

Abstract excerpt

DFNA9 is an autosomal dominant genetic inner-ear hearing impairment that starts to show itself in the 3rd and 4th decades of life. This hearing impairment may be of a different degree of severity in each ear. Progression of hearing loss is about 3 dB/year. In about one in three patients severe vestibular symptoms similar to those in Ménière's disease are present as a result of a progressive impairment of the...

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Source

Identifiers

PMID: 16355574

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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[From gene to disease; a progressive cochlear-vestibular dysfunction with onset in middle-age (DFNA9)].PMID 16355574Read ↗

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

Mutations in a novel cochlear gene cause DFNA9, a human nonsyndromic deafness with vestibular dysfunctionSemantic proximity Read ↗

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

[From gene to disease; DFNA8/12, an autosomal dominant inherited bowl-shaped sensorineural hearing impairment].Semantic proximity Read ↗

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

On the pathophysiology of DFNA9: Effect of pathogenic variants in the COCH gene on inner ear functioning in human and transgenic mice.Semantic 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 ↗

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

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

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

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