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Related Experiment Video

Updated: May 29, 2026

Cochlear Implantation in the Guinea Pig
09:56

Cochlear Implantation in the Guinea Pig

Published on: June 15, 2018

Cochlear implantation in Pendred syndrome.

Georgios Kontorinis1, Thomas Lenarz, Anke Lesinski-Schiedat

  • 1Otorhinolaryngology Department, Hanover Medical University, Hanover, Germany. Kontorinis.Georgios@mh-hannover.de

Cochlear Implants International
|September 16, 2011
PubMed
Summary

Cochlear implantation offers satisfactory hearing rehabilitation for Pendred syndrome patients, despite inner ear malformations complicating surgery. Surgeons should anticipate challenges for optimal outcomes in these complex cases.

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Area of Science:

  • Otolaryngology
  • Genetics
  • Audiology

Background:

  • Pendred syndrome presents unique challenges for cochlear implantation due to goiter, severe hearing loss, and inner ear dysplasias.
  • Previous evaluations of hearing outcomes in Pendred syndrome cochlear implant recipients are limited.

Purpose of the Study:

  • To investigate anatomical, audiological, and surgical factors influencing hearing outcomes in Pendred syndrome patients receiving cochlear implants.
  • To assess the efficacy and challenges of cochlear implantation in this specific patient population.

Main Methods:

  • A retrospective study was conducted at a tertiary referral cochlear implant center.
  • Data from Pendred syndrome patients who underwent cochlear implantation between 1999 and 2008 were analyzed.

Related Experiment Videos

Last Updated: May 29, 2026

Cochlear Implantation in the Guinea Pig
09:56

Cochlear Implantation in the Guinea Pig

Published on: June 15, 2018

Main Results:

  • Five Pendred syndrome patients (four unilateral, one bilateral) received cochlear implants with an average follow-up of 4.8 years.
  • Enlarged vestibular aqueduct and Mondini dysplasia were common, prolonging surgery but not affecting electrode insertion.
  • Cerebrospinal fluid gusher was the most frequent intraoperative complication; all patients achieved satisfactory hearing rehabilitation.

Conclusions:

  • Cochlear implantation is effective for hearing rehabilitation in Pendred syndrome.
  • Inner ear malformations can lead to surgical difficulties and extended operating times.
  • Preexisting hearing experience is a positive prognostic factor for successful outcomes in Pendred syndrome patients.