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Minerva Pediatrica 2005 October;57(5):285-8

language: English

Sensorineural hearing loss in sickle cell disease patients in Sicily

Samperi P., Bertuna G., Rossi G., Poli G., Serra A.


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/ß 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-ß thalassemia (37 with the ßsß°th and 13 with the ßsß+th) and 23 patients with sickle cell anemia (ßsßs) 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/ß°-thalassemia, in 23% of subjects with Hb S/ß+-thalassemia and in 30% of subjects with sickle cell anemia. We found an increase in the frequency of hearing loss with increasing age.
Conclusion. 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/ß-thalassemias.

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