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Related Experiment Video

Updated: Dec 29, 2025

Cochlear Implant Surgery and Electrically-evoked Auditory Brainstem Response Recordings in C57BL/6 Mice
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Cochlear Implants in Alström Syndrome.

Flavia Gheller1, Samanta Gallo1, Patrizia Trevisi1

  • 1Department of Neurosciences, ENT Clinic, Padova University Hospital, Padua, Italy.

The Annals of Otology, Rhinology, and Laryngology
|February 6, 2020
PubMed
Summary

Cochlear implants (CI) offer significant hearing rehabilitation for individuals with Alström syndrome (AS), a rare genetic disorder. This case report details the first AS patient to receive bilateral CI, demonstrating excellent outcomes and no complications.

Keywords:
Alström syndromecochlear implantdeafness

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Area of Science:

  • Otolaryngology
  • Genetics
  • Audiology

Background:

  • Alström syndrome (AS) is a rare genetic disorder with progressive hearing loss.
  • Hearing rehabilitation options, particularly cochlear implants (CI), are not well-documented in AS patients.
  • Potential comorbidities in AS may raise concerns about surgical interventions like CI.

Observation:

  • This report details the first case of a 22-year-old woman with genetically confirmed AS undergoing sequential bilateral CI (Bi-CI) rehabilitation.
  • The patient's audiological profile and outcomes pre- and post-implantation are described.

Findings:

  • The patient achieved excellent functional results with bilateral CI.
  • The CI enabled communicative, social, and academic achievements comparable to peers.
  • No complications were observed following the sequential bilateral CI procedure.

Implications:

  • Alström syndrome is not an absolute contraindication for cochlear implantation.
  • Adequate cognitive function and life expectancy in AS patients indicate the need for timely hearing rehabilitation with CI.
  • This case contributes to developing tailored audiological treatments for AS patients with specific needs.