title: Distortion product emissions in normal-hearing and low-frequency hearing loss carriers of genes for Waardenburg's syndrome.
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.
authors.affiliation: Centre for Audiology, Education of the Deaf & Speech Pathology, University of Manchester, England.
authors.forename: X Z
authors.initials: XZ
authors.lastname: Liu
authors.forename: V E
authors.initials: VE
authors.lastname: Newton
mesh_terms: D000293:Adolescent; D000328:Adult; D001299:Audiometry / Q000295:instrumentation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006309:Hearing / Q000235:genetics*; D006319:Hearing Loss, Sensorineural / Q000235:genetics*; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D014849:Waardenburg Syndrome / Q000235:genetics / Q000503:physiopathology*