TY - JOUR T1 - Osteogenesis Imperfecta of the Temporal Bone: CT and MR Imaging in Van der Hoeve-de Kleyn Syndrome JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. SP - 1106 LP - 1109 VL - 25 IS - 6 AU - Hatem Alkadhi AU - Diana Rissmann AU - Spyros S. Kollias Y1 - 2004/06/01 UR - http://www.ajnr.org/content/25/6/1106.abstract N2 - 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. ER -