The emergence and prevalence of hearing loss in children with homozygous sickle cell disease

Many children with sickle cell disease (SCD) experience the effect of cochlear insult due to hemolytic anemia and vaso-occlusion that accompanies the disease. As a result, hearing sensitivity eventually decreases. The purpose of this study was to investigate the emergence of hearing loss and the prevalence of such in children with homozygous SCD. A large pediatric database was utilized to gather audiometric outcomes in children with homozygous SCD. Children were identified by primary diagnosis ICD codes. Demographic and audiometric data was drawn to determine presence, type, degree, and laterality of hearing loss. Hearing loss was defined as an elevated audiometric threshold >15 dB HL for pure tone air conduction audiometry or >20 dB HL minimal response level for sound-field testing. One hundred and twenty-eight children were identified. The prevalence of hearing loss ranged from 28.8% to 50.8% depending on the calculation method (i.e., individual vs. ear specific prevalence and any elevated threshold vs. a three-frequency pure tone average). Conductive hearing loss and bilateral loss were most prevalent. The degree of hearing loss ranged from slight to profound. The mean age of identification of sensorineural hearing loss was 9.6 years. The prevalence of hearing loss in children with homozygous SCD is higher than in normal children. Regular hearing assessments of children with SCD is warranted and should be advocated in early infancy and as part of their ongoing care.