The c.260G>A variant in COCH is a missense variant predicted to cause substitution of glycine by glutamate at amino acid 87 (p.Gly87Glu). This variant is absent from gnomAD v4.1.0 (PM2_Supporting). The amino acid residue is well conserved and the REVEL computational prediction analysis tool produced a score of 0.885, which is above the threshold necessary to apply PP3 (PP3). Three different missense variants, c.259G>T; p.Gly87Trp [PMID:16835921, ClinVar Variation ID: 1297642], c.260G>C; p.Gly87Ala [ClinVar Variation ID: 517362, with variant segregation in 3 affected family members per lab communication], and c.260G>T; p.Gly87Val [PMIDs:23767834, 23993205], have been classified as pathogenic or likely pathogenic for autosomal dominant, adult-onset hearing loss with vestibular symptoms (PM5_Strong). The p.Gly87Glu variant was detected via a hearing loss gene panel for a patient with a 3-generation history of progressive adult-onset hearing loss in the 5th or 6th decade with vestibular symptoms. In summary, this variant has been classified as likely pathogenic for autosomal dominant, adult-onset, nonsyndromic, sensorineural genetic hearing loss based on the ACMG/AMP criteria applied, as specified by the ClinGen Hearing Loss VCEP: PM5_Strong, PM2_Supporting, PP3 (ClinGen Hearing Loss VCEP specifications version 1; 3/12/2025).