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Related Experiment Videos

Cochlear implantation in osteogenesis imperfecta

J Szilvássy1, J Jóri, J Czigner

  • 1Dept. Otolaryngology, Albert Szent-Gyorgyi Medical University Szeged, Hungary.

Acta Oto-Rhino-Laryngologica Belgica
|November 12, 1998
PubMed
Summary

A cochlear implant improved hearing in a patient with osteogenesis imperfecta, but caused facial twitching. Adjusting electrode programming resolved this side effect, preserving hearing benefits.

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Area of Science:

  • Otolaryngology
  • Neurosurgery
  • Genetics

Background:

  • Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by brittle bones.
  • Profound deafness can be associated with OI, sometimes necessitating cochlear implantation.
  • Cochlear implants aim to restore auditory function by stimulating the auditory nerve.

Observation:

  • A 50-year-old Hungarian woman with profound deafness and OI received a Nucleus 22 channel intracochlear implant.
  • Successful electrode insertion yielded a 70 dB hearing improvement across key frequencies (250-2000 Hz).
  • Facial twitching, indicative of non-acoustic nerve excitation, occurred upon activation of specific electrodes (9-13).

Findings:

  • Deactivating the problematic electrodes eliminated the facial twitching.

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  • Auditory performance was maintained after inactivating the electrodes causing non-acoustic nerve excitation.
  • This suggests abnormal bone tissue in OI may increase current spread, risking nerve excitation.

    • Implications:

    • Cochlear implantation is feasible in patients with osteogenesis imperfecta.
    • Non-acoustic nerve excitation is a potential complication but manageable through programming.
    • Careful electrode selection and programming can optimize outcomes for OI patients with cochlear implants.