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Hearing loss in myotonic dystrophy.

R B Wright1, R H Glantz, J Butcher

  • 1Department of Neurology, Rush Medical College, Chicago, IL.

Annals of Neurology
|February 1, 1988
PubMed
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Routine hearing tests revealed moderate to severe hearing loss in most patients with myotonic dystrophy. This sensorineural hearing loss was treatable in some cases, but no single cause was identified.

Area of Science:

  • Neurology
  • Audiology
  • Genetics

Background:

  • Myotonic dystrophy is a multisystem disorder with variable clinical manifestations.
  • Hearing impairment is a potential complication of myotonic dystrophy, but its prevalence and characteristics are not fully understood.

Purpose of the Study:

  • To investigate the prevalence and nature of hearing loss in patients with myotonic dystrophy.
  • To determine if hearing loss in this population is treatable.

Main Methods:

  • Audiometric screening was performed on 25 patients with myotonic dystrophy.
  • Further audiological and etiological investigations were conducted for patients with identified hearing loss.

Main Results:

  • Seventeen out of 25 patients (68%) exhibited moderate to severe hearing loss.

Related Experiment Videos

  • The hearing loss was predominantly sensorineural in type.
  • No single underlying pathophysiological process was identified for the hearing loss.
  • Hearing loss was treatable in some affected individuals.

    • Conclusions:

    • Hearing loss is a common and significant issue in patients with myotonic dystrophy.
    • Sensorineural hearing loss in myotonic dystrophy may have diverse etiologies.
    • Early identification through audiometric screening and subsequent management can improve outcomes for some patients.