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Cochlear function in facioscapulohumeral muscular dystrophy.

Dimitrios G Balatsouras1, Stavros Korres, Panayota Manta

  • 1Department of Otolaryngology-Head and Neck Surgery, Tzanion General Hospital, Piraeus, Greece. balats@panafonet.gr

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [And] European Academy of Otology and Neurotology
|November 16, 2006
PubMed
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Facioscapulohumeral muscular dystrophy (FSHD) patients often have subclinical cochlear damage, even with normal hearing. Otoacoustic emissions reveal reduced cochlear function in FSHD, suggesting their utility in clinical assessment.

Area of Science:

  • Otoacoustic Emissions
  • Auditory Neuroscience
  • Neuromuscular Disorders

Background:

  • Facioscapulohumeral muscular dystrophy (FSHD) is frequently linked to high-frequency hearing loss.
  • Early detection of cochlear involvement in FSHD is crucial for management.

Purpose of the Study:

  • To investigate subclinical cochlear dysfunction in FSHD patients with normal audiometric thresholds using otoacoustic emissions.
  • To assess the utility of otoacoustic emissions in identifying early cochlear changes in FSHD.

Main Methods:

  • A prospective, randomized clinical trial was conducted with 24 FSHD patients and 40 healthy controls.
  • Transiently evoked otoacoustic emissions (TEOAEs) were measured, analyzing reproducibility and response levels.
  • Audiometric pure-tone thresholds were normal to near-normal in all participants.

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Main Results:

  • FSHD patients exhibited significantly diminished TEOAE reproducibility and response scores compared to controls.
  • Subclinical cochlear involvement was detected in most FSHD patients, despite normal audiograms.
  • Scores were reduced across octave bands from 1 to 5 kHz.

Conclusions:

  • Subclinical cochlear involvement is prevalent in FSHD, even with normal hearing.
  • Otoacoustic emissions are sensitive to early cochlear damage in FSHD.
  • TEOAEs may serve as a valuable tool for clinical evaluation and monitoring of FSHD patients.