Clinical and radiologic findings of inner ear involvement in sickle cell disease

BACKGROUND AND PURPOSE: SCD has been reported to involve the inner ear and result in LH and LO. Our purpose was to examine the prevalence of inner ear involvement and to assess the relationship between clinical and imaging findings in patients with SCD. MATERIALS AND METHODS: Review of our institution's imaging data base for patients with SCD who underwent imaging of the brain or head and neck region or both by CT, MR, or both from 2004 to 2008 was performed. Presenting symptoms, type of SCD, sex, imaging studies performed, and imaging findings were documented. RESULTS: Among 89 patients with SCD identified (41 males, 48 females), 17 patients (14 males, 3 females; 10-48 years old) underwent imaging evaluation for inner ear complaints, including SNHL, dizziness, vertigo, and tinnitus. LH was identified in 3 patients (3 males) and LO was identified in another 3 patients (2 males, 1 female). All patients with LH had sickle-hemoglobin C disease, whereas those with LO consisted of 2 patients homozygous for HbS (2) and 1 with HbS/β-thalassemia. Patients with LH presented with vestibular symptoms (2 vertigo, 1 dizziness), whereas patients with LO presented with SNHL. LH was seen in the basal turn of cochlea and vestibule, whereas LO involved the lateral semicircular canal. CONCLUSIONS: LH and LO were found in approximately one-third of patients with SCD with inner ear symptoms and preferentially affected males.