Sensorineural hearing loss in sickle cell disease patients in Sicily

Department of Pediatric, Hematology-Oncology, University of Catania, Catania, Italy.
Minerva pediatrica (Impact Factor: 0.43). 11/2005; 57(5):285-8.
Source: PubMed


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.
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.
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.
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.

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    ABSTRACT: Sickle cell disease (SCD) is associated with sensorineural hearing loss (SNHL). Although the hearing loss is usually mild, some develop severe-to-profound hearing loss, in whom cochlear implants (CI) may be an option. We present the cases of two children with SCD who developed bilateral severe-to-profound SNHL and underwent cochlear implantation. One patient became profoundly deaf after an acute episode of dizziness. Imaging indicated bilateral cochlear ossification, making subsequent cochlear implant surgery challenging. The second patient developed bilateral severe-to-profound SNHL following acute vaso-occlusive crises. She went on to have uncomplicated cochlear implant surgery. These cases illustrate the variable manner in which children with SCD may develop SNHL, and the difficulties associated with managing such cases. We recommend that children with SCD should undergo regular audiological assessment. Furthermore, clinicians should be aware of the risk of cochlear fibrosis and ossification and ensure prompt assessment following an acute vaso-occlusive crisis or unexplained vestibulocochlear event. © 2015 Japan Pediatric Society.
    Pediatrics International 02/2015; 57(1):174-6. DOI:10.1111/ped.12413 · 0.73 Impact Factor