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AJNR - American Journal of Neuroradiology

Published ahead of print on October 22, 2008
doi: 10.3174/ajnr.A1283

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American Journal of Neuroradiology 30:434-436, February 2009
© 2009 American Society of Neuroradiology

Case Report
HEAD & NECK

Dilation of the Subarachnoid Spaces Surrounding the Cranial Nerves with Petrous Apex Cephaloceles in Usher Syndrome

T.A. Stark^a, A.M. McKinney^a, C.S. Palmer^a, R.H. Maisel^a and C.L. Truwit^a

^a From the Department of Radiology, University of Minnesota Medical School & Hennepin County Medical Center, Minneapolis, Minn

Please address correspondence to Alexander McKinney, MD, Hennepin County Medical Center, Department of Radiology, 701 Park Ave South, Minneapolis, MN 55415; e-mail: mckinrad{at}umn.edu

SUMMARY: Limited studies of brain MR imaging findings in Usher syndrome have reported atrophy with enlarged subarachnoid spaces. However, the specific appearance of the subarachnoid spaces surrounding the cranial nerves has not yet been described. Herein we describe the skull base MR imaging findings in an adult with Usher syndrome. Multiple cranial nerve exits were enlarged to the point of causing cephaloceles with bony remodeling. A combination of uncommon findings in this rare disorder raises the question of an etiologic association.