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MINERVA PEDIATRICA

A Journal on Pediatrics, Neonatology, Adolescent Medicine,
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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.


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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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