Hagr AA1.
Author information
1Dr. Abdulrahman Abdullah Hagr, ORL, King Agbdulaziz University Hospital,, PO Box 245, Riyadh 11411,, Saudi Arabia, T: +966505216261, F: +966114775735 dhagr90@yahoo.com.
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.