Pediatric Radiology

, Volume 42, Issue 12, pp 1449–1455 | Cite as

Inner ear anomalies seen on CT images in people with Down syndrome

  • Jarunee Intrapiromkul
  • Nafi Aygun
  • David E. Tunkel
  • Marco Carone
  • David M. YousemEmail author
Original Article



Although dysplasia of inner ear structures in Down syndrome has been reported in several histopathological studies, the imaging findings have not been widely studied.


To evaluate the prevalence and clinical significance of inner ear anomalies detected on CT images in patients with Down syndrome.

Materials and 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).


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.


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.


Down syndrome Trisomy 21 Inner ear CT Temporal bone 



Conductive hearing loss


Confidence interval


Down syndrome


Internal auditory canal


Lateral semicircular canal


Mixed hearing loss


Sensorineural hearing loss


Semicircular canal dehiscence



We would like to thank Dr. Mahadevappa Mahesh for acquiring the radiation dose information.

Conflict of interest

We have no conflicts of interest to declare.


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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Jarunee Intrapiromkul
    • 1
  • Nafi Aygun
    • 1
  • David E. Tunkel
    • 2
  • Marco Carone
    • 3
  • David M. Yousem
    • 1
    Email author
  1. 1.The Russell H. Morgan Department of Radiology and Radiological SciencesThe Johns Hopkins Medical InstitutionsBaltimoreUSA
  2. 2.Department of Otolaryngology-Head and Neck SurgeryThe Johns Hopkins Medical InstitutionsBaltimoreUSA
  3. 3.Division of Biostatistics, School of Public HealthUniversity of CaliforniaBerkeleyUSA

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