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Cochlear Implants International
An Interdisciplinary Journal for Implantable Hearing Devices
Volume 12, 2011 - Issue 4
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Case reports

Improved hearing with cochlear implantation in Fabry's disease

David J ClinkardDepartment of Otolaryngology/Head & Neck SurgerySunnybrook Health Sciences Center, Toronto, Ontario, CanadaCorrespondence[email protected]
,
Hosam AmoodiDepartment of Otolaryngology/Head & Neck SurgerySunnybrook Health Sciences Center, Toronto, Ontario, Canada
,
Vincent Y W LinDepartment of Otolaryngology/Head & Neck SurgerySunnybrook Health Sciences Center, Toronto, Ontario, Canada
,
Joseph M ChenDepartment of Otolaryngology/Head & Neck SurgerySunnybrook Health Sciences Center, Toronto, Ontario, Canada
&
Julian M NedzelskiDepartment of Otolaryngology/Head & Neck SurgerySunnybrook Health Sciences Center, Toronto, Ontario, Canada
Pages 238-240 | Published online: 18 Jul 2013
 
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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.

Acknowledgements

No funding was received for this work.

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