Pediatric Radiology

, Volume 36, Issue 9, pp 977–979 | Cite as

Partial absence of the posterior semicircular canal in Alagille syndrome: CT findings

  • Bernadette KochEmail author
  • Amy Goold
  • John Egelhoff
  • Corning Benton
Case Report


We report a case of bilateral partial absence of the posterior semicircular canals (with normal lateral semicircular canals) imaged with CT in a patient with Alagille syndrome. Similar histologic findings have been reported in the pathology literature. This association has been previously reported only for Waardenburg syndrome in the imaging literature. We review the imaging findings and embryology of the semicircular canals, and suggest that this abnormality is specific to patients with Alagille or Waardenburg syndrome.


Congenital anomaly Temporal bone Alagille syndrome 


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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Bernadette Koch
    • 1
    Email author
  • Amy Goold
    • 2
  • John Egelhoff
    • 1
  • Corning Benton
    • 3
  1. 1.Department of RadiologyCincinnati Children’s Hospital Medical CenterCincinnatiUSA
  2. 2.Family PracticeTripler Army Medical CenterHonoluluUSA
  3. 3.Department of RadiologyCincinnati Children’s Hospital Medical CenterCincinnatiUSA

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