title: Sensorineural hearing loss in sickle cell disease patients in Sicily.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Pediatric, Hematology-Oncology, University of Catania, Catania, Italy. psamperi@unict.it
authors.forename: P
authors.initials: P
authors.lastname: Samperi
authors.forename: G
authors.initials: G
authors.lastname: Bertuna
authors.forename: G
authors.initials: G
authors.lastname: Rossi
authors.forename: G
authors.initials: G
authors.lastname: Poli
authors.forename: A
authors.initials: A
authors.lastname: Serra
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis; D016009:Chi-Square Distribution; D002648:Child; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D015995:Prevalence; D012959:Socioeconomic Factors; D017086:beta-Thalassemia / Q000150:complications* / Q000175:diagnosis