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

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 det...

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Bibliographic Details
Published in:	American journal of neuroradiology : AJNR 2004-06, Vol.25 (6), p.1106-1109
Main Authors:	Alkadhi, Hatem, Rissmann, Diana, Kollias, Spyros S
Format:	Article
Language:	eng
Subjects:	Adult Biological and medical sciences Bone Diseases - diagnosis Female Head and Neck Hearing Loss - diagnosis Humans Investigative techniques, diagnostic techniques (general aspects) Magnetic Resonance Imaging Medical sciences Nervous system Osteogenesis Imperfecta - diagnosis Radiodiagnosis. Nmr imagery. Nmr spectrometry Scleral Diseases - diagnosis Syndrome Temporal Bone Tomography, X-Ray Computed
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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.
ISSN:	0195-6108 1936-959X