Sensorineural hearing loss in sickle cell disease patients in Sicily.

ABSTRACT

AIM: Sickle cell disease has been frequently associated with sensorineuronal hearing loss. Several studies have demonstrated a significant prevalence rate of sensorineuronal hearing loss in black patients reporting a range of 11-41%, while few data are reported for white people with Hb S/beta thalassemia. In this paper we evaluated the prevalence of sensorineuronal hearing loss in a Sicilian population affected by sickle cell disease. METHODS: Otologic and audiologic examinations were performed in 50 patients with S-beta thalassemia (37 with the beta(s)beta(0th) and 13 with the beta(s)beta(+th)) and 23 patients with sickle cell anemia (beta(s)beta(s)) observed at the Department of Pediatric Hematology and Oncology, University of Catania. RESULTS: A sensorial hearing loss of more than 25 dB was recorded in 24% of subjects with Hb S/beta0-thalassemia, in 23% of subjects with Hb S/beta+-thalassemia and in 30% of subjects with sickle cell anemia. We found an increase in the frequency of hearing loss with increasing age. CONCLUSIONS: Our data showed that sensorineuronal hearing loss is a common complication in white patients with sickle cell anemia, and in patients with severe forms of Hb S/beta-thalassemias.