title: Clinical and radiologic findings of inner ear involvement in sickle cell disease.
abstract: OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Department of Radiology, Boston University School of Medicine, Boston, MA, USA. naoko.saito.ns@gmail.com
authors.forename: N
authors.initials: N
authors.lastname: Saito
authors.forename: M
authors.initials: M
authors.lastname: Watanabe
authors.forename: J
authors.initials: J
authors.lastname: Liao
authors.forename: E N
authors.initials: EN
authors.lastname: Flower
authors.forename: R N
authors.initials: RN
authors.lastname: Nadgir
authors.forename: M H
authors.initials: MH
authors.lastname: Steinberg
authors.forename: O
authors.initials: O
authors.lastname: Sakai
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis*; D002648:Child; D005260:Female; D006801:Humans; D007759:Labyrinth Diseases / Q000175:diagnosis* / Q000209:etiology*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D055815:Young Adult