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Temporal Bone Osteogenesis Imperfecta
Troy A. Hutchins, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Osteogenesis imperfecta (OI)
            • Synonyms

              • OI tarda = OI type I = Van der Hoeve-de Kleyn syndrome
            • Definitions

              • Inherited connective disorder characterized by bone fragility and fractures

            IMAGING

            • General Features

              • Imaging Recommendations

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Vincent R et al: Stapedotomy in osteogenesis imperfecta: a prospective study of 32 consecutive cases. Otol Neurotol. 35(10):1785-9, 2014
                                          2. Swinnen FK et al: Temporal bone imaging in osteogenesis imperfecta patients with hearing loss. Laryngoscope. 123(8):1988-95, 2013
                                          3. Fuller E et al: Case of the month #171: osteogenesis imperfecta of the temporal bone. Can Assoc Radiol J. 62(4):296-8, 2011
                                          4. Pillion JP et al: Hearing loss in osteogenesis imperfecta: characteristics and treatment considerations. Genet Res Int. 2011:983942, 2011
                                          5. Byers PH et al: Genetic evaluation of suspected osteogenesis imperfecta (OI). Genet Med. 8(6):383-8, 2006
                                          6. Alkadhi H et al: Osteogenesis imperfecta of the temporal bone: CT and MR imaging in Van der Hoeve-de Kleyn syndrome. AJNR Am J Neuroradiol. 25(6):1106-9, 2004
                                          7. Berger G et al: Histopathology of the temporal bone in osteogenesis imperfecta congenita: a report of 5 cases. Laryngoscope. 95(2):193-9, 1985
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Osteogenesis imperfecta (OI)
                                                    • Synonyms

                                                      • OI tarda = OI type I = Van der Hoeve-de Kleyn syndrome
                                                    • Definitions

                                                      • Inherited connective disorder characterized by bone fragility and fractures

                                                    IMAGING

                                                    • General Features

                                                      • Imaging Recommendations

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Vincent R et al: Stapedotomy in osteogenesis imperfecta: a prospective study of 32 consecutive cases. Otol Neurotol. 35(10):1785-9, 2014
                                                                                  2. Swinnen FK et al: Temporal bone imaging in osteogenesis imperfecta patients with hearing loss. Laryngoscope. 123(8):1988-95, 2013
                                                                                  3. Fuller E et al: Case of the month #171: osteogenesis imperfecta of the temporal bone. Can Assoc Radiol J. 62(4):296-8, 2011
                                                                                  4. Pillion JP et al: Hearing loss in osteogenesis imperfecta: characteristics and treatment considerations. Genet Res Int. 2011:983942, 2011
                                                                                  5. Byers PH et al: Genetic evaluation of suspected osteogenesis imperfecta (OI). Genet Med. 8(6):383-8, 2006
                                                                                  6. Alkadhi H et al: Osteogenesis imperfecta of the temporal bone: CT and MR imaging in Van der Hoeve-de Kleyn syndrome. AJNR Am J Neuroradiol. 25(6):1106-9, 2004
                                                                                  7. Berger G et al: Histopathology of the temporal bone in osteogenesis imperfecta congenita: a report of 5 cases. Laryngoscope. 95(2):193-9, 1985