title: Inner ear anomalies seen on CT images in people with Down syndrome.
abstract: BACKGROUND
  Although dysplasia of inner ear structures in Down syndrome has been reported in several histopathological studies, the imaging findings have not been widely studied.
  
  
  OBJECTIVE
  To evaluate the prevalence and clinical significance of inner ear anomalies detected on CT images in patients with Down syndrome.
  
  
  METHODS
  The temporal bone CT images of patients with Down syndrome were assessed for inner ear anomalies; clinical notes and audiograms were reviewed for hearing loss. Logistic regression models were employed to identify which CT findings were associated with sensorineural hearing loss (SNHL).
  
  
  RESULTS
  Inner ear anomalies were observed in 74.5% (38/51) of patients. Malformed bone islands of lateral semicircular canal (LSCC), narrow internal auditory canals (IACs), cochlear nerve canal stenoses, semicircular canal dehiscence (SCCD), and enlarged vestibular aqueducts were detected in 52.5% (53/101), 24.5% (25/102), 21.4% (21/98), 8.8% (9/102) and 2% (2/101) of patients' ears, respectively. IAC stenosis had the highest odds ratio (OR = 5.37, 95% CI: 1.0-28.9, P = 0.05) for SNHL.
  
  
  CONCLUSIONS
  Inner ear anomalies occurred in 74.5% of our population, with malformed (<3 mm) bone island of LSCC being the most common (52.5%) anomaly. Narrow IAC was seen in 24.5% of patients with Down syndrome and in 57.1% of ears with SNHL. High-resolution CT is a valuable for assessing the cause of hearing loss in people with Down syndrome.
authors.affiliation: The Russell H. Morgan Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, 600 N. Wolfe St., Phipps B100F, Baltimore, MD 21287, USA.
authors.forename: Jarunee
authors.initials: J
authors.lastname: Intrapiromkul
authors.forename: Nafi
authors.initials: N
authors.lastname: Aygun
authors.forename: David E
authors.initials: DE
authors.lastname: Tunkel
authors.forename: Marco
authors.initials: M
authors.lastname: Carone
authors.forename: David M
authors.initials: DM
authors.lastname: Yousem
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D015897:Comorbidity; D004314:Down Syndrome / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005260:Female; D006314:Hearing Loss, Conductive / Q000000981:diagnostic imaging / Q000453:epidemiology; D006319:Hearing Loss, Sensorineural / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D006801:Humans; D008297:Male; D008396:Maryland / Q000453:epidemiology; D015995:Prevalence; D015203:Reproducibility of Results; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D014057:Tomography, X-Ray Computed / Q000379:methods*; D055815:Young Adult