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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
Cochlear Implants International
|January 19, 2012
Summary
Cochlear implantation offers a safe and effective solution for hearing loss in individuals with Fabry
Area of Science:
- Otolaryngology
- Genetics
- Neurology
Background:
- Fabry disease is a rare genetic disorder caused by alpha-galactosidase A enzyme deficiency.
- Progressive hearing loss is a common symptom in patients with Fabry disease.
Observation:
- This study reports on two patients with genetically confirmed Fabry disease and progressive hearing loss.
- Both patients underwent cochlear implantation surgery.
Findings:
- Cochlear implantation significantly improved hearing discrimination by 60 points on the Hearing In Noise Test (HINT) scale.
- The intervention was well-tolerated by both patients.
Implications:
- Cochlear implantation is a viable treatment option for hearing loss associated with Fabry disease.
- Further research is warranted to explore long-term outcomes and expand treatment guidelines.
