Is hearing loss due to mutations in the Connexin 26 gene progressive?

Deepika Gopalarao, William J. Kimberling, Walt Jesteadt, Philip M. Kelley, Kathryn L. Beauchaine, Edward S. Cohn

Research output: Contribution to journalArticlepeer-review

18 Scopus citations


Serial audiograms were analysed for seven subjects, who were homozygous for the 35delG GJB2 mutation. The criterion for determining progression of hearing loss was at least a 1-dB loss in air conduction pure-tone average-3 (ACPTA-3) or ACPTA-4 per year for 2 to 10 years, with a minimum change of 10 dB ACPTA 3 or 4. Bilateral progression of hearing loss was found in 43% (3/7) of the subjects. A meta-analysis of seven studies with non-overlapping data sets and similar ascertainment criteria indicated that 19% of DFNB1 subjects with GJB2 mutations have progressive hearing loss. These data suggest that it may be incorrect to assume that congenital hearing loss due to this mutation is stable. We recommend rigorous audiologic surveillance for individuals with DFNB1.

Original languageEnglish (US)
Pages (from-to)11-20
Number of pages10
JournalInternational Journal of Audiology
Issue number1
StatePublished - Jan 2008


  • 35delG
  • DFNB1
  • GJB2
  • Non-syndromic recessive hearing loss
  • Progression of hearing loss

ASJC Scopus subject areas

  • Language and Linguistics
  • Linguistics and Language
  • Speech and Hearing

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