Carriers of the Usher syndrome type IB: Is audiometric identification possible?

M. Wagenaar, A. F.M. Snik, W. J. Kimberling, C. W.R.J. Cremers

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


Some studies in the past have shown that carriers of genes for recessive deafness cannot be identified by standard audiometry. However, remarkable results with regard to the identification of heterozygotes have been reported using Bekesy audiometry and Audioscan audiometry. In the present study, nine obligate carriers from five families with the Usher syndrome type IB were examined. Methods that reflect the function of the cochlea, including pure-tone audiometry, Audioscan audiometry, and otoacoustic emission measurements were used to detect (subtle) audiometric manifestations of heterozygosity. Abnormalities in hearing sensitivity were found in some obligate carriers but to the same extent in some of the controls. No statistically significant differences were found in the presence of audiometric abnormalities between carriers and controls. Additional audiologic measurements indicated that if hearing loss was present in Usher type IB carriers, it was presumably of cochlear origin. It is concluded that carriers of the Usher syndrome type IB cannot be identified properly via standard audiometric methods.

Original languageEnglish (US)
Pages (from-to)853-858
Number of pages6
JournalAmerican Journal of Otology
Issue number6
StatePublished - Nov 1996


  • Audiometric identification
  • Usher syndrome type IB

ASJC Scopus subject areas

  • Otorhinolaryngology

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