Evaluation of hearing and inner ear functions of beta-thalassemia major patients receiving regular chelation therapy with desferrioxamine

Abstract

OBJECTIVE: In this study, the hearing functions and inner ear conditions of beta (b)-thalassemia major patients who were treated with desferrioxamine were evaluated. METHODS: This study was conducted with 69 pediatric patients aged seven years and older (Thalassemia group; 42 males, 27 females; mean age 11.0±4.1 year; range, 7 to 15 year), diagnosed with b-thalassemia major, treated with regular iron-chelating agent desferrioxamine for at least three years, and followed in the pediatric hematology and oncology clinic and 67 children (Control group; 36 male, 31 female; mean 12.4±6.8 year; range, 5 to 17 year) without hearing loss and chronic ear disease between January 2017 and January 2020. The patient and control groups were examined by an otolaryngologist. Pure-tone audiometry and distortion product otoacoustic emission tests were performed on the patient and control groups, and the results were compared. RESULTS: No significant difference was found in terms of age and sex in the thalassemia and control groups. In the comparison of hearing thresholds between groups with pure-tone audiometry, the hearing thresholds of the thalassemia group were higher than the control group at a frequency of 8000 Hz in the right and left ears, and the difference was statistically significant. In the comparison of the signal-to-noise ratios between the groups with the distortion product otoacoustic emission test, the decibel values in the right ear at frequencies of 1000 Hz and 6000 Hz were lower in the thalassemia group than in the control group, and the difference was statistically significant. CONCLUSION: In the study, the inner ear functions were found to be affected in patients treated with desferrioxamine. Even if b-thalassemia major patients do not clinically describe hearing loss, we believe their hearing thresholds will increase starting from high frequencies with repetitive desferrioxamine treatment, and they may experience a hearing loss.