Sickle cell anemia and hearing.

The present investigation examined the effects of sickle cell anemia on threshold hearing. The study included 43 homozygous sickle cell anemia patients, ages 7-18 years, and 23 age-matched controls with documented normal hemoglobin. Both the study and control groups received otologic and audiologic examinations. Bilaterally normal hearing was found in 88% of the sickle cell subjects. Unilateral or bilateral mild high frequency sensorineural hearing loss was demonstrated in 12% (5 of 43). The control subjects revealed no hearing loss. The sickle cell group, therefore, exhibited a higher than usual incidence of hearing loss. Further, three of the five subjects with hearing loss evidenced central nervous system involvement, resulting in a probability of < 0.025 that this relation might occur by chance. Periodic hearing evaluations of children with sickle cell anemia should be performed to identify those with auditory complications and those who possibly may have CNS involvement.