Related Experiment Video
Updated: May 25, 2026
Enhanced Cochlear Coverage and Hearing Preservation in High-Frequency Hearing Loss via Electric Acoustic Stimulation with Longer Electrode
Published on: October 11, 2024
Improved hearing with cochlear implantation in Fabry's disease
David J Clinkard1, Hosam Amoodi, Vincent Y W Lin
1Sunnybrook Health Sciences Center, Toronto, Ontario, Canada. David.Clinkard@sunnybrook.ca
Objective:
To describe our center's experience with cochlear implantation in patients suffering from Fabry's disease, an inherited mutation resulting in an alpha-galactosidase A enzyme deficiency.
Clinical Presentation:
Case report of two patients aged 49 and 59 at implant, with genetically confirmed Fabry's disease and progressive hearing loss.
Intervention:
Surgical implantation of Clarion (Advanced Bionics) and Nucleus Freedom (Cochlear) cochlear implants.
Conclusion:
Cochlear implantation improves hearing discrimination by 60 points on the HINT scale. This suggests that cochlear implantation is a safe and effective intervention that improves hearing discrimination in patients suffering from Fabry's disease.
