BACKGROUND Hearing disorders are reported in thalassemia patients treated with deferoxamine. This study aimed to assess hearing loss in Iranian thalassemia major patients treated with deferoxamine. METHODS This review article was designed based on PRISMA guidelines. To review the literature, two researchers studied national and international databases including Iranmedex, Magiran, Medlib, SID, Scopus, PubMed, Science Direct, Web of Science and Google Scholar without time limit until May 2017. Cochran's Q test and I2 index were used to assess the heterogeneity of the studies. The data were analyzed using Comprehensive Meta-Analysis software version 2 and p<0.05 was considered significant. RESULTS A total of 17 articles involving 1,835 Iranian thalassemia major patients treated with deferoxamine were included in the meta-analysis. The overall prevalence of hearing loss was estimated 27.3% (95% confidence intervals (CI): 19-37.6). The prevalence of sensorineural, conductive and mixed hearing loss was estimated 10.6% (95% CI: 5.7-18.8), 14.6% (95% CI: 10.5-20.6) and 9.1% (95% CI: 5.6-14.6), respectively. No significant differences were noted regarding the relationship hearing loss and mean serum ferritin (P=0.29) and average daily deferoxamine (P=0.30). Meta-regression model showed an increased significance in the prevalence of hearing loss based on the year of studies (p<0.0001). CONCLUSIONS There is a high prevalence of hearing loss in Iranian thalassemia major patients treated with deferoxamine. Therefore, periodic hearing assessments and regular check-ups after the initiation of chelation therapy are necessary.