Pediatric Radiology

, Volume 21, Issue 8, pp 580–583

The temporal bone in the Johanson-Blizzard syndrome

A CT study


  • J. Braun
    • Department of Diagnostic RadiologyRambam Medical Center
  • A. Lerner
    • Department of PediatricsLady Davis Carmel Hospital
  • R. Gershoni-Baruch
    • Department of GeneticsRambam Medical Center

DOI: 10.1007/BF02012603

Cite this article as:
Braun, J., Lerner, A. & Gershoni-Baruch, R. Pediatr Radiol (1991) 21: 580. doi:10.1007/BF02012603


The findings of thin-section high-resolution CT of the temporal bone in two children with Johanson-Blizzard syndrome are reported. In both cases an anomaly of the inner ear was found, consisting of bilateral cystic dilatation of the cochlea and the vestibulum. The possible etiologic basis for the hearing loss in this syndrome is discussed and the literature is reviewed.

Copyright information

© Springer-Verlag 1991