Background: Beta-thalassemia is a common inherited congenital disorder of hemoglobin production, resulting in hemolytic anemia and multiorgan involvement. Each year, nearly 60,000 beta-thalassemia children are born worldwide, while carriers are estimated to be around 90 million people (1.5% of the global population). In the last half century, the life expectancy of beta-thalassemia patients has strikingly increased mostly due to regular blood transfusions and chelation treatments. The improved survival, however, has allowed for the emergence of comorbidities, such as hearing loss, with a non-negligible impact on the patient's quality of life. Beta-thalassemia major patients may have audiological problems, this could be related to chronic anemia, iron overload, extramedullary hematopoiesis, and adverse reactions of iron chelating agents that are used for their management. Therefore, it is important to routinely assess audiological functions in those patients, especially children and adolescents.

Aim: This review aimed to highlight the acquired knowledge regarding hearing impairment in beta-thalassemia major.

Conclusion: The cause of hearing loss in patients with beta-thalassemia major may result from dose-related ototoxicity of the iron chelators, so it is important to do routine audiological assessment, careful adjustment of the dose, and/or a reasoned pharmacological shift.