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Updated: Dec 29, 2025

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

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

Published on: January 9, 2019

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

Summary

This summary is machine-generated.

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 syndrome cochlear implant deafness

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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.