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Summary: Background: Despite numerous studies, the true scenario of hearing loss in beta‐thalassaemia remains rather nebulous. Materials and methods: Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non‐transfusion‐dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected. Results: Patient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non‐transfusion‐dependent vs. 87/310 transfusion‐dependent patients; p = 0.16). Sensorineural‐notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non‐significantly lower compared to chelation‐treated patients while it was significantly higher compared to controls (p = 0.003). Conclusions: Sensorineural hypoacusis rate is high in beta‐thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural‐notch in beta‐thalassaemia appears questionable. [ABSTRACT FROM AUTHOR]