Sensorineural hearing loss in children with thalassemia major in Northern Greece

Abstract

Eighty eight (88) beta-thalassemic patients undergoing regular transfusion- chelation therapy with desferrioxamine (DFO) were studied for ENT problems from 1988 to 1993, as DFO has been implicated for auditory neurotoxicity. The mean age of the patients was 9.66 +/- 3.1 years, their pre-transfusion haemoglobin level was 9 +/- 2 g/dl, serum ferritin level was 2065 +/- 898 ng/ml and the daily DFO dose was 50.7 +/- 9.5 mg/kg for 5 days/week. The ENT study included, ENT examination, pure tone audiometry, speech audiometry, tympanometry, tone decay test and ABR. During this 6-year study 24/88 (27%) patients developed bilateral or ipsilateral sensorineural hearing loss in high tone frequencies, sometimes exceeding 80 dB, which was attributed to DFO toxicity. Therefore, a reduction or temporary withdrawal of DFO followed. After this intervention 12/24 patients recovered almost completely, 7/24 remained stable and 5/24 presented aggravation of their hearing loss. This study confirms the DFO induced auditory neurotoxicity and the necessity of periodical audiology control of beta-thalassemic patients for prompt diagnosis and management of this complication.