ABSTRACT
Background: Thalassemia is a common genetic hematological disorder worldwide. It is also common in North India includingJammu region. These patients need lifelong repeated blood transfusions and iron chelation therapy for their survival. Chelationtherapy is known to be associated with various complications including sensorineural hearing loss (SNHL). Till now, no dataare available regarding SNHL in pediatric thalassemia major patients in Jammu region. Hence, we planned a study to assessthe prevalence of hearing loss in children with thalassemia major in the age group of 10–20 years.Methods: All the children with beta-thalassemia major in the age group of 10–20 years registered with Thalassemia Day CareCenter, Department of Pediatrics, SMGS Hospital, Government Medical College, Jammu, were enrolled in this cross-sectionalstudy. Hearing was assessed by pure tone audiometry. Clinical and demographic data of these patients were recorded onpretested pro forma and analyzed.Results: A total number of 34 children with thalassemia in the age group of 10–20 years were enrolled in this study whichcomprised 18 males and 16 females. Out of these 34 patients, 5 (14.7%) were found to have SNHL and 1 (2.9%) had conductivehearing loss. Four of the five patients in SNHL group had low- as well as high-frequency mild hearing loss (25–40 db) whileone patient had high-frequency mild hearing loss at 4000 HZ. Four out of these five patients had unilateral hearing loss on theleft side while one had bilateral SNHL. Two out of five patients in the SNHL group were taking chelation therapy in the form ofcombination of deferiprone and deferasirox at the dose of 75–100 mg/kg/day and 30–40 mg/kg/day, respectively, for more than5 years. The other three patients were taking only deferasirox at the dose of 30–40 mg/kg/day for more than 5 years.Conclusions: Regular blood transfusions and chelation therapy are essential for long-term survival of thalassemia majorpatients but are also associated with complications like SNHL.