title: 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.
  
  
  METHODS
  Case report of two patients aged 49 and  59 at implant, with genetically confirmed Fabry's disease and progressive hearing loss.
  
  
  METHODS
  Surgical implantation of Clarion (Advanced Bionics) and Nucleus Freedom (Cochlear) cochlear implants.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Sunnybrook Health Sciences Center, Toronto, Ontario, Canada. David.Clinkard@sunnybrook.ca
authors.forename: David J
authors.initials: DJ
authors.lastname: Clinkard
authors.forename: Hosam
authors.initials: H
authors.lastname: Amoodi
authors.forename: Vincent Y W
authors.initials: VY
authors.lastname: Lin
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Chen
authors.forename: Julian M
authors.initials: JM
authors.lastname: Nedzelski
mesh_terms: D019929:Cochlear Implantation; D003054:Cochlear Implants; D000795:Fabry Disease / Q000150:complications*; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011474:Prosthesis Design