SENSORINEURAL HEARING-LOSS IN CHILDREN WITH THALASSEMIA MAJOR IN NORTHERN GREECE

Eighty eight (88) beta-thalassemic patients undergoing regular transfu sion- chelation therapy with desferrioxamine (DFO) were studied for EN T 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, th eir 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 t one audiometry, speech audiometry, tympanometry, tone decay test and A BR. During this 6-year study 24/88 (27%) patients developed bilateral or ipsilateral sensorineural hearing loss in high tone frequencies, so metimes exceeding 80 dB, which was attributed to DFO toxicity. Therefo re, a reduction or temporary withdrawal of DFO followed. After this in tervention 12/24 patients recovered almost completely, 7/24 remained s table 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 d iagnosis and management of this complication.