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Related Experiment Videos

Usher syndrome and cochlear implantation.

Natalie Loundon1, Sandrine Marlin, Denise Busquet

  • 1Service d'Otorhinolaryngologie et de chirurgie cervico-faciale, Hôpital d'enfants Armand-Trousseau, Paris, France. loundon.moatti@trs.ap-hop-paris.fr

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [And] European Academy of Otology and Neurotology
|March 7, 2003
PubMed
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The earliest sign of Usher syndrome is delayed walking, and electroretinograms are crucial for diagnosis. Early cochlear implantation in children with Usher syndrome improves speech and hearing outcomes.

Area of Science:

  • Otolaryngology
  • Genetics
  • Audiology

Background:

  • Usher syndrome is a genetic disorder causing hearing loss and vision impairment.
  • Cochlear implantation is a treatment option for severe to profound hearing loss.

Purpose of the Study:

  • To evaluate diagnostic symptoms and rehabilitation outcomes of cochlear implantation in Usher syndrome patients.
  • To identify factors influencing speech and hearing development post-implantation.

Main Methods:

  • Retrospective cohort study of 13 Usher syndrome patients undergoing cochlear implantation.
  • Evaluated diagnostic signs, audiological tests (electroretinogram), vestibular function, and logopedic outcomes.
  • Follow-up averaged 52 months.

Main Results:

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  • Delayed walking (mean 20 months) was the most frequent initial sign of Usher syndrome.
  • Electroretinogram was abnormal in all cases; vestibular function was impaired in most.
  • Good speech perception was achieved in most patients, with best results in those implanted before age 9.

Conclusions:

  • Late walking is an early indicator of Usher syndrome, necessitating electroretinogram screening.
  • Early cochlear implantation optimizes speech therapy and rehabilitation outcomes.
  • Diagnosis and intervention timing are critical for Usher syndrome management.