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Cochlear implantation and Cogan syndrome.

Enrico Pasanisi1, Vincenzo Vincenti, Andrea Bacciu

  • 1Department of Ophthalmology and Otorhinolaryngology, University of Parma, Parma, Italy. enrico.pasanisi@unipr.it

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [And] European Academy of Otology and Neurotology
|July 10, 2003
PubMed
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Cochlear implantation in Cogan syndrome patients led to significant speech understanding improvements. Surgical challenges like cochlear ossification were managed, with no major complications observed during follow-up.

Area of Science:

  • Otolaryngology
  • Ophthalmology
  • Neuroscience

Background:

  • Cogan syndrome is a rare autoimmune disorder affecting the eyes and audiovestibular system.
  • Progressive hearing loss is a common and debilitating symptom of Cogan syndrome.
  • Cochlear implantation is a potential treatment for sensorineural hearing loss.

Purpose of the Study:

  • To assess the efficacy and safety of cochlear implantation in patients with Cogan syndrome.
  • To identify potential surgical challenges and postoperative complications associated with cochlear implantation in this population.

Main Methods:

  • A prospective cohort study involving five adult patients with Cogan syndrome and postlingual deafness.
  • Evaluation of speech perception using word and sentence recognition tests.

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  • Monitoring of surgical outcomes, including electrode insertion and postoperative complications.

    • Main Results:

    • All patients achieved useful open-set speech perception within 12 months, with high scores on word (91%) and sentence (95%) recognition.
    • Two patients required modified electrode insertion due to cochlear ossification (scala tympani).
    • Two patients experienced a recurrence of keratitis post-surgery; no other major complications were reported during 1-4 years of follow-up.

    Conclusions:

    • Cochlear implantation is effective in restoring speech understanding for Cogan syndrome patients.
    • Cochlear ossification presents a surgical challenge, necessitating technique adaptation.
    • Surgical stress may trigger relapses of Cogan syndrome's inflammatory manifestations.