A patient with sickle cell anemia may develop hearing loss and deafness over time.
Features of hearing loss in sickle cell anemia:
(1) The hearing loss is sensorineural.
(2) The onset may be sudden after a sickle crisis or gradual.
(3) Some recovery of hearing can occur if mild.
(4) A patient may have mixed hearing loss if other causes of deafness are present.
(5) Significant hearing loss during childhood can impair the child's education.
(6) Hearing loss may contribute to disability.
Hearing dysfunction may be both cochlear and retro-cochlear dysfunction (Gould et al). There is no consistent audiometric pattern that is pathognomonic for sickle cell disease.
A patient with significant sickle cell disease should have audiologic testing to monitor function over time.
A cochlear implant can improve hearing.
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