Cochlear Implantation in Alport Syndrome: A Novel Case Series

Evan Moon ¹, Laurel Bond ², Steven A Gordon ²

⁰University of Cincinnati College of Medicine.

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology +

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October 31, 2025

View abstract on PubMed

Summary

Cochlear implantation (CI) successfully restored hearing in three patients with Alport syndrome, demonstrating significant improvements in speech perception and hearing thresholds. These findings suggest CI is a viable option for Alport syndrome-related hearing loss.

Area Of Science

Otolaryngology
Genetics
Audiology

Background

Alport syndrome is a genetic disorder affecting collagen, often leading to progressive sensorineural hearing loss.
Hearing loss in Alport syndrome can significantly impact quality of life and communication.

Purpose Of The Study

To evaluate the clinical outcomes of cochlear implantation (CI) in familial patients diagnosed with Alport syndrome.
To assess the efficacy of CI in addressing progressive sensorineural hearing loss associated with Alport syndrome.

Main Methods

A case series involving three familial patients (father and two daughters) with Alport syndrome who underwent cochlear implantation.
Preoperative and postoperative audiological assessments, including pure-tone average (PTA), consonant-nucleus-consonant (CNC) scores, and Arizona Bioindustry Association (AzBio) scores, were utilized.

Main Results

Patients demonstrated significant improvements in PTA, CNC, and AzBio scores post-implantation.
One patient achieved 92% CNC and 87% AzBio scores 10 years after CI, while another showed 84% CNC and 62% AzBio scores at 3 months post-CI.
The study highlights successful unilateral CI outcomes without complications in this patient cohort.

Conclusions

Unilateral cochlear implantation is a safe and effective treatment for hearing loss in Alport syndrome.
Further research into the efficacy of CI for individuals with Alport syndrome and other collagen-related disorders is warranted.

Keywords:

Alport syndrome Cochlear implant Sensorineural hearing loss Syndromic deafness