Cochlear Implantation in a Patient With Sickle Cell Disease With Early Cochlear Sclerosis.

OBJECTIVE: We report a case of bilateral sudden sensorineural hearing loss (SNHL) and early cochlear sclerosis in a patient with sickle cell disease.

METHODS: A 19-year-old female presented with sequential bilateral sudden SNHL and early cochlear sclerosis. Cochlear implantation was performed.

RESULTS: Early cochlear fibrosis in the hook region and basal turn was encountered within a few months of deafness. Implantation required serial dilation using various insertion guides. Postsurgical telemetry readings revealed 19 electrodes (7 paired basal electrodes, 5 single apical electrodes) in a good working order with low impedances in bilateral ears. Activation of the processors successfully provided access to the speech frequency range in both the ears.

CONCLUSION: This is the first case of intraoperative documentation of rapid cochlear sclerosis in a patient with SNHL caused by sickle cell disease. Early cochlear implantation should be considered in these patients, and otolaryngologists should be aware of the possibility of rapid cochlear sclerosis without ossification in these patients.