Distortion product emissions in normal-hearing and low-frequency hearing loss carriers of genes for Waardenburg's syndrome

Ann Otol Rhinol Laryngol. 1997 Mar;106(3):220-5. doi: 10.1177/000348949710600307.

Abstract

Eight patients with Waardenburg's syndrome (WS) with normal hearing and 3 additional patients exhibiting a low-frequency hearing loss were tested for the level of the acoustic distortion product 2f1-f2 by means of the Otodynamics Distortion Product Analyser (ILO92). Wide notches in distortion product otoacoustic emissions (DPOAEs) between 1,000 and 3,000 Hz were found in 7 (12 ears, 87.5%) examined patients with normal audiograms, which was a significantly higher rate than that found in the control group (10%). The 3 patients with low-frequency hearing loss a gave a consistent pattern in audiometric configuration shown by both pure tone audiograms and DPOAEs. It is concluded from these initial results that DPOAEs may be a useful approach to identifying subclinical pathologic aberrations in the inner ear in WS patients, and may be a predictor of low-frequency sensorineural hearing loss.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Audiometry / instrumentation
  • Child
  • Child, Preschool
  • Female
  • Hearing / genetics*
  • Hearing Loss, Sensorineural / genetics*
  • Heterozygote
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Waardenburg Syndrome / genetics
  • Waardenburg Syndrome / physiopathology*