Otology

Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: A comparison with age- and sex-matched controls†

  1. Jenny R. Wang BHSc,
  2. Heng W. Yuen MD,
  3. David B. Shipp MA, FAAA,
  4. Suzanne Stewart MSc, CCC-A,
  5. Vincent Y. W. Lin MD, FRCPS(c),
  6. Joseph M. Chen MD, FRCPS(c),
  7. Julian M. Nedzelski MD, FRCPS(c)*

Article first published online: 16 NOV 2010

DOI: 10.1002/lary.21060

Issue

The Laryngoscope

The Laryngoscope

Volume 120, Issue 12, pages 2478–2483, December 2010

Additional Information

How to Cite

Wang, J. R., Yuen, H. W., Shipp, D. B., Stewart, S., Lin, V. Y. W., Chen, J. M. and Nedzelski, J. M. (2010), Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: A comparison with age- and sex-matched controls. The Laryngoscope, 120: 2478–2483. doi: 10.1002/lary.21060

Author Information

  1. Department of Otolaryngology–Head and Neck Surgery, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada

*Department of Otolaryngology–Head and Neck Surgery, Sunnybrook Health Sciences Centre, 2075 Bayview Avenue, Suite M1-102, Toronto, Ontario, Canada

  1. †

    The authors have no funding, financial relationships, or conflicts of interest to disclose.

Publication History

  1. Issue published online: 24 NOV 2010
  2. Article first published online: 16 NOV 2010
  3. Manuscript Accepted: 26 APR 2010
  4. Manuscript Revised: 22 APR 2010
  5. Manuscript Received: 25 JAN 2010

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Keywords:

  • Autoimmune inner ear disease;
  • Cogan syndrome;
  • cochlear implantation;
  • sensorineural hearing loss;
  • deafness;
  • Level of Evidence: 3b

Abstract

Objectives/Hypothesis:

Evaluate the characteristics and outcomes of patients with autoimmune inner ear disease (AIED) who have undergone cochlear implantation (CI) and compare post-CI performance in AIED to matched controls.

Study Design:

Retrospective case control study.

Methods:

Study cohort was comprised of 25 adult implantees (AIED [n = 18], Cogan syndrome [n = 7]). The AIED group was defined by rapidly progressive bilateral sensorineural hearing loss leading to unusable hearing within weeks to months. Patients with Cogan syndrome, the archetypal inner ear autoimmune disease, were also examined and used for within-cohort comparison. Clinical and operative records were reviewed. Post-CI performance was assessed using open-set sentence tests. Age- and sex-matched individuals deafened by other postlingual causes were used as controls.

Results:

Of 25 patients, 24 had uneventful, full electrode insertions. One AIED patient had partial insertion due to cochlear ossification and did not achieve open-set speech perception post-CI. Mean open-set sentence scores for study patients with uneventful insertions were 92.8%, 97.3%, and 96.4% at 6 months, 1 year, and ≥2 years, respectively. Compared to matched controls, patients deafened by autoimmune causes had significantly higher post-CI performance at all postoperative test intervals (P < .05). There was no significant difference in postimplantation performance between Cogan syndrome and AIED patients.

Conclusions:

To our knowledge this was the largest study of cochlear implantation in AIED and Cogan syndrome patients. In our experience, both groups generally attained high levels of post-CI speech perception and performed above average. Cochlear ossification affecting implantation in Cogan syndrome patients was not observed in our series, contrary to some reports. Laryngoscope, 2010

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