Fabry disease (FD) is a lysosomal storage disorder resulting from deficient activity of the enzyme alpha-galactosidase A. The vestibulocochlear system is also affected, with tinnitus and hearing loss being common complaints. Enzyme replacement therapy (ERT) appears to stabilise or improve the function of various affected organs. The aim of this study is to assess the hearing profile of patients with FD, identify possible prognostic factors and analyse the effect of ERT on hearing function.