Improved hearing with cochlear implantation in Fabry's disease

Abstract

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.