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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:1106-1109, June-July 2004
© 2004 American Society of Neuroradiology

Case Report
HEAD AND NECK

Osteogenesis Imperfecta of the Temporal Bone: CT and MR Imaging in Van der Hoeve-de Kleyn Syndrome

Hatem Alkadhi^a, Diana Rissmann^a and Spyros S. Kollias^a

^a From the Institute of Neuroradiology, University Hospital Zurich, Zurich, Switzerland

Address correspondence to: Spyros S. Kollias, MD, Institute of Neuroradiology, University Hospital Zurich, Frauenklinikstrasse 10, CH-8091 Zurich, Switzerland

Summary: We report the progressive otic capsule demineralization around the membranous labyrinth and facial nerve in an adult patient with osteogenesis imperfecta tarda. Whereas the initial CT scan showed bandlike, undermineralized pericochlear areas, 2 years later, repeat CT performed because of hearing deterioration showed progression of these findings to the promontory, the round window niche, and the labyrinthine and tympanic segments of the facial nerve canal. MR imaging demonstrated enhancement of the abnormal otic capsule and of the intratemporal and canalicular facial nerve. The differential diagnosis of osteogenesis imperfecta tarda affecting the temporal bone includes otosclerosis, Paget disease, otosyphilis, and Camurati-Engelmann disease.