Novel Case of Profound Hearing Loss and Cochlear Implantation From New-Generation Iron Chelation Therapy

Iron chelation plays a central role in the treatment of hemosiderosis. Historically, the chelator of choice was deferoxamine. While effective, this medication is administered subcutaneously via lengthy transfusions and exhibits a robust side effect profile, including sensorineural hearing loss (SNHL) in 25% to 30% of patients.1 Because of this, orally administered chelators deferiprone and deferasirox (DFX) have been developed. To date, there are limited data regarding the otologic side effect profile for these new-generation iron chelators. The most convincing data are derived from clinical trials of DFX in which hearing loss was reported in up to 1.1% of patients.2,3 Subsequent studies have found rates of mild hearing loss ranging up to 45% in patients who have beta thalassemia treated with DFX.4,5 Descriptions of patients with greater than mild DFX-associated hearing loss or those requiring cochlear implantation are not present in the current literature.