Cochlear Implantation in Alport Syndrome: A Novel Case Series.

OBJECTIVE

To evaluate the clinical outcomes of cochlear implantation (CI) in 3 familial patients with Alport syndrome.

PATIENTS: Three familial patients-a father and 2 daughters-diagnosed with Alport syndrome who underwent CI due to progressive sensorineural hearing loss.

INTERVENTION: CI was performed between 1999 and 2024 at a tertiary academic medical center. Patient 1 received a left Cochlear Americas CI 24 Nucleus, patient 2 a right Cochlear Nucleus CI24RE with Contour Advance, and patient 3 a right Cochlear Americas CI 622.

MAIN OUTCOME MEASURES: Preoperative and postoperative consonant-nucleus-consonant (CNC) and Arizona Bioindustry Association (AzBio) scores, as well as pure-tone average (PTA).

RESULTS

Postoperative PTA was 30 dB for patient 1, 25 dB for patient 2, and 30 dB for patient 3. Patient 1 had limited pre-implantation records, but 16 years post-implantation, he had a CNC of 64% and AzBio of 47%. Patient 2's right-sided preoperative CNC and AzBio were both 0% with a PTA of 110 dB. At 10 years postop, her CNC improved to 92% and AzBio to 87%. Patient 3's preoperative CNC was 56% in each ear and her AzBio was 60% in the bilateral condition with a PTA of 96 dB. At 3 months post-CI, her CNC improved to 84% and AzBio was 62% for the CI ear only.

CONCLUSIONS

This novel case series highlights successful unilateral CI in Alport syndrome-related hearing loss without complications. These findings support further research on CI efficacy in individuals with Alport syndrome and other collagen-related disorders.