Hearing loss in children with sickle cell disease: A prospective French cohort study

Background Sickle cell disease (SCD) is the most common genetic disease in France. In developing countries, it is associated with a high incidence of hearing loss. The aim of this study was to determine the prevalence of hearing loss in French children with SCD in order to determine if they need a close audiological follow-up. Methods Results We performed a single-center prospective cross-sectional study of children with SCD. The children, without specific hearing symptom, underwent an ear, nose and throat examination with a hearing assessment between 2015 and 2016. Eighty-nine children were included, aged from 5 to 19 years, with 73% of SS or S beta(0) genotype and 27% of SC or S beta(+) genotype. Ten children (11.2%) had hearing thresholds higher than 20 dB in at least one ear: one child with subnormal hearing, six otitis media with effusion (OME), and three sensorineural hearing loss. Late age at diagnosis of SCD, a high platelet count and a low hematocrit level were significantly associated with OME; moreover, children with OME had more severe clinical and biological characteristics than children with normal hearing. Furthermore, 12.4% of the children complained of tinnitus. The rate of sudden hearing loss was 2.2%. Finally, 7.1% of patients with normal hearing showed a speech discrimination disorder. Conclusions Several causes were identified for hearing loss in children with SCD. They therefore need a close audiological follow-up in order to avoid complications due to curable phenomena and to enable appropriate management for progressive complications.