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Related Experiment Videos

Progressive hearing loss in Usher's syndrome.

S Karjalainen1, L Pakarinen, M Teräsvirta

  • 1Department of Otolaryngology, Kuopio University Central Hospital, Finland.

The Annals of Otology, Rhinology, and Laryngology
|November 1, 1989
PubMed
Summary

Usher syndrome can cause progressive hearing loss, impacting auditory thresholds and speech clarity. However, most patients retain speech communication abilities despite hearing impairment.

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Area of Science:

  • Ophthalmology
  • Audiology
  • Genetics

Background:

  • Usher syndrome is a genetic disorder characterized by vision and hearing loss.
  • The progressive nature of hearing loss in Usher syndrome requires further investigation.

Purpose of the Study:

  • To audiologicaly assess hearing loss in patients with Usher syndrome.
  • To evaluate the impact of hearing loss on speech communication in these patients.

Main Methods:

  • Audiological evaluation including hearing threshold and speech discrimination tests.
  • Clinical assessment of speech communication abilities.

Main Results:

  • Progressive hearing loss was confirmed in 8 out of 18 Usher syndrome patients.

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  • Despite significant hearing impairments, only one patient had speech communication difficulties.

    • Conclusions:

    • Hearing impairment in Usher syndrome may be predominantly progressive.
    • Most individuals with Usher syndrome maintain speech communication skills despite hearing loss.