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Related Experiment Videos

Usher syndrome: an otoneurologic study.

C G Möller1, W J Kimberling, S L Davenport

  • 1Boys Town National Institute for Communication Disorders in Children, Omaha, Neb.

The Laryngoscope
|January 1, 1989
PubMed
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Usher syndrome causes hearing loss and retinitis pigmentosa. This study found that hearing and balance issues stem from inner ear damage, not central nervous system problems.

Area of Science:

  • Genetics
  • Ophthalmology
  • Audiology
  • Neurology

Background:

  • Usher syndrome is an autosomal recessive disorder.
  • It is characterized by severe hearing loss (or deafness) and retinitis pigmentosa.
  • This study investigated Usher syndrome types I and II.

Purpose of the Study:

  • To characterize Usher syndrome types I and II.
  • To investigate the underlying causes of hearing loss, balance problems, and ataxia in Usher syndrome.
  • To determine if central nervous system disturbances are involved.

Main Methods:

  • Genetic studies
  • Clinical examination
  • Audiological, ophthalmologic, and otoneurological tests

Related Experiment Videos

  • Magnetic resonance imaging (MRI)

    • Main Results:

    • 16 patients diagnosed with Usher type I had profound hearing loss and absent vestibular responses.
    • 9 patients diagnosed with Usher type II had moderate to profound hearing loss and normal vestibular function.
    • MRI and otoneurological tests showed no central nervous system disturbances in any patients.
    • Ataxia in Usher type I resulted from retinitis pigmentosa and peripheral vestibular deficiency.

    Conclusions:

    • Hearing loss and balance problems in Usher syndrome are caused by inner ear damage.
    • There is no evidence of central nervous system disturbances in Usher syndrome.
    • Ataxia in Usher type I is a combined result of visual and vestibular impairment.