Case of the Month #171: Osteogenesis Imperfecta of the Temporal Bone

  title={Case of the Month \#171: Osteogenesis Imperfecta of the Temporal Bone},
  author={Elisa Fuller and Vincent Y. W. Lin and Mary Jane Bell and Aditya Bharatha and R Yeung and Richard I. Aviv and Sean Symons},
  journal={Canadian Association of Radiologists Journal},
  pages={296 - 298}
A 35-year-old man presented to the Department of Otolaryngology with progressive bilateral hearing loss, worse on the right, over the past 12 years. Audiometry determined the hearing loss to be mixed, both conductive and sensorineural. He had no otalgia, vertigo, or otorrhea. There was no history of otologic surgery, noise exposure, or familial hearing loss. He described remote minor head injury at age 4 years that required stitches, and prior fractures, which generally occurred during hockey… Expand
Temporal Bone Histopathology Case of the Month Osteogenesis ImperfectaVA Human Temporal Bone Case Study
Osteogenesis imperfecta (OI) is defined as one of the inherited connective tissue disorders that can result in fragile bones, blue sclera, and deafness. This genetic disease affects collagen type 1Expand
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In the University of Minnesota temporal bone collection, the authors found one case that fits in the OI diagnosis, and this case represents the patients that do not fit into subtypes I–III. Expand
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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 showedExpand
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The results raise the question whether OI and OS lead to similar labyrinthine bone alterations, based on different aetiologies or whether these diseases may co-exist, OS being part of OI. Expand
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CT Grading of Otosclerosis
A recently published CT grading for otosclerosis on the basis of location of involvement yielded excellent interobserver and intraobserver agreement. Expand
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Osteogenesis imperfecta
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Type V Osteogenesis Imperfecta: A New Form of Brittle Bone Disease
  • F. Glorieux, F. Rauch, +7 authors N. Bishop
  • Medicine
  • Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
  • 2000
OI type V is a new form of autosomal dominant OI, which does not appear to be associated with collagen type I mutations, and the genetic defect underlying this disease remains to be elucidated. Expand
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The computed tomographic findings in the temporal bone of two patients with osteogenesis imperfecta tarda are described. One of the patients had bilateral facial nerve paresis, and both patientsExpand
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Histomorphometric analyses of iliac crest bone samples revealed findings similar to OI type I, with decreased cortical width and trabecular number, increased bone turnover, and preservation of the birefringent pattern of lamellar bone. Expand