title: Cochlear implant and thiamine-responsive megaloblastic anemia syndrome.
abstract: Thiamine-responsive megaloblastic anemia syndrome is a rare autosomal recessive disorder defined by the occurrence of megaloblastic anemia, diabetes mellitus, and bilateral sensorineural deafness, responding in varying degrees to thiamine treatment. We report a precedence case for the treatment of deafness associated with the typical triad of thiamine-responsive megaloblastic anemia in a 4-year-old boy who showed a poor use of preoperative hearing aids but demonstrated significant improvements in hearing ability 1 year after receiving a cochlear implant.
authors.affiliation: Dr. Abdulrahman Abdullah Hagr, ORL, King Agbdulaziz University Hospital,, PO Box 245, Riyadh 11411,, Saudi Arabia, T: +966505216261, F: +966114775735 dhagr90@yahoo.com.
authors.forename: Abdulrahman Abdullah
authors.initials: AA
authors.lastname: Hagr
mesh_terms: D000749:Anemia, Megaloblastic / Q000150:complications*; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D003638:Deafness / Q000601:surgery*; D003920:Diabetes Mellitus; D006319:Hearing Loss, Sensorineural / Q000150:complications*; D006801:Humans; D007655:Ketoglutarate Dehydrogenase Complex / Q000172:deficiency*; D008297:Male; D013832:Thiamine Deficiency / Q000151:congenital; D016896:Treatment Outcome
chemical_list: D007655:Ketoglutarate Dehydrogenase Complex