Abstract
We present the first-published cochlear implant in an adolescent with sickle cell anemia (SCA) and bilateral profound sensorineural hearing loss (SNHL). A 15-year-old Saudi girl, previously diagnosed with SCA, developed gradual bilateral permanent profound SNHL over 18 months and underwent a successful cochlear implantation. SNHL associated with sickle cell crises is a well-known phenomenon. Regular hearing assessments are recommended for sickle cell patients, and early referrals for cochlear implantations are advocated for those with permanent profound SNHL.