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Otosclerosis
Troy A. Hutchins, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Fenestral otosclerosis (FOto)
                • Cochlear otosclerosis (COto)
              • Synonyms

                • Otospongiosis, fenestral otospongiosis, cochlear otospongiosis
              • Definitions

                • Pathologic appearance of lytic spongy bone foci in bony labyrinth of unknown cause
                  • Starts perifenestral (FOto), progresses to surround cochlea (FOto + COto)
                • Fissula ante fenestram: Cleft of fibrocartilaginous tissue between inner & middle ears just anterior to oval window
                  • Also called "cochlear cleft"

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            • Reporting Tips

                                              Selected References

                                              1. Mukaida T et al: Magnetic Resonance Imaging Evaluation of Endolymphatic Hydrops in Cases With Otosclerosis. Otol Neurotol. 36(7):1146-50, 2015
                                              2. Whetstone J et al: Surgical and clinical confirmation of temporal bone CT findings in patients with otosclerosis with failed stapes surgery. AJNR Am J Neuroradiol. 35(6):1195-201, 2014
                                              3. Ukkola-Pons E et al: Oval window niche height: quantitative evaluation with CT before stapes surgery for otosclerosis. AJNR Am J Neuroradiol. 34(5):1082-5, 2013
                                              4. Lee TC et al: CT grading of otosclerosis. AJNR Am J Neuroradiol. 30(7):1435-9, 2009
                                              5. Moser T et al: The hypodense focus in the petrous apex: a potential pitfall on multidetector CT imaging of the temporal bone. AJNR Am J Neuroradiol. 29(1):35-9, 2008
                                              6. Marshall AH et al: Cochlear implantation in cochlear otosclerosis. Laryngoscope. 115(10):1728-33, 2005
                                              7. Chadwell JB et al: The cochlear cleft. AJNR Am J Neuroradiol. 25(1):21-4, 2004
                                              8. Pekkola J et al: Localized pericochlear hypoattenuating foci at temporal-bone thin-section CT in pediatric patients: nonpathologic differential diagnostic entity? Radiology. 230(1):88-92, 2004
                                              9. Rondini-Gilli E et al: [Otosclerosis surgical techniques and results in 150 patients] Ann Otolaryngol Chir Cervicofac. 119(4):227-33, 2002
                                              10. Stimmer H et al: Magnetic resonance imaging and high-resolution computed tomography in the otospongiotic phase of otosclerosis. ORL J Otorhinolaryngol Relat Spec. 64(6):451-3, 2002
                                              11. Chole RA et al: Pathophysiology of otosclerosis. Otol Neurotol. 22(2):249-57, 2001
                                              12. Ruckenstein MJ et al: Management of far advanced otosclerosis in the era of cochlear implantation. Otol Neurotol. 22(4):471-4, 2001
                                              13. Shin YJ et al: Correlations between computed tomography findings and family history in otosclerotic patients. Otol Neurotol. 22(4):461-4, 2001
                                              14. Shin YJ et al: Sensorineural hearing loss and otosclerosis: a clinical and radiologic survey of 437 cases. Acta Otolaryngol. 121(2):200-4, 2001
                                              15. Veillon F et al: Imaging of the windows of the temporal bone. Semin Ultrasound CT MR. 22(3):271-80, 2001
                                              16. Niyazov D et al: Fenestration surgery for otosclerosis: CT findings of an old surgical procedure. AJNR Am J Neuroradiol. 21(9):1670-2, 2000
                                              17. Ziyeh S et al: MRI-visible pericochlear lesions in osteogenesis imperfecta type I. Eur Radiol. 10(10):1675-7, 2000
                                              18. Ziyeh S et al: MRI of active otosclerosis. Neuroradiology. 39(6):453-7, 1997
                                              19. Miura M et al: Computed tomographic image analysis of ears with otosclerosis. ORL J Otorhinolaryngol Relat Spec. 58(4):200-3, 1996
                                              20. Valvassori GE: Imaging of otosclerosis. Otolaryngol Clin North Am. 26(3):359-71, 1993
                                              21. Wilbrand HF: Radioanatomy of cochlear and stapedial otosclerosis. Scand Audiol Suppl. 30:181-3, 1988
                                              22. Mafee MF et al: Use of CT in stapedial otosclerosis. Radiology. 156(3):709-14, 1985
                                              23. Swartz JD et al: Fenestral and cochlear otosclerosis: computed tomographic evaluation. Am J Otol. 6(6):476-81, 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

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Fenestral otosclerosis (FOto)
                                                            • Cochlear otosclerosis (COto)
                                                          • Synonyms

                                                            • Otospongiosis, fenestral otospongiosis, cochlear otospongiosis
                                                          • Definitions

                                                            • Pathologic appearance of lytic spongy bone foci in bony labyrinth of unknown cause
                                                              • Starts perifenestral (FOto), progresses to surround cochlea (FOto + COto)
                                                            • Fissula ante fenestram: Cleft of fibrocartilaginous tissue between inner & middle ears just anterior to oval window
                                                              • Also called "cochlear cleft"

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Consider

                                                                                      • Image Interpretation Pearls

                                                                                        • Reporting Tips

                                                                                          Selected References

                                                                                          1. Mukaida T et al: Magnetic Resonance Imaging Evaluation of Endolymphatic Hydrops in Cases With Otosclerosis. Otol Neurotol. 36(7):1146-50, 2015
                                                                                          2. Whetstone J et al: Surgical and clinical confirmation of temporal bone CT findings in patients with otosclerosis with failed stapes surgery. AJNR Am J Neuroradiol. 35(6):1195-201, 2014
                                                                                          3. Ukkola-Pons E et al: Oval window niche height: quantitative evaluation with CT before stapes surgery for otosclerosis. AJNR Am J Neuroradiol. 34(5):1082-5, 2013
                                                                                          4. Lee TC et al: CT grading of otosclerosis. AJNR Am J Neuroradiol. 30(7):1435-9, 2009
                                                                                          5. Moser T et al: The hypodense focus in the petrous apex: a potential pitfall on multidetector CT imaging of the temporal bone. AJNR Am J Neuroradiol. 29(1):35-9, 2008
                                                                                          6. Marshall AH et al: Cochlear implantation in cochlear otosclerosis. Laryngoscope. 115(10):1728-33, 2005
                                                                                          7. Chadwell JB et al: The cochlear cleft. AJNR Am J Neuroradiol. 25(1):21-4, 2004
                                                                                          8. Pekkola J et al: Localized pericochlear hypoattenuating foci at temporal-bone thin-section CT in pediatric patients: nonpathologic differential diagnostic entity? Radiology. 230(1):88-92, 2004
                                                                                          9. Rondini-Gilli E et al: [Otosclerosis surgical techniques and results in 150 patients] Ann Otolaryngol Chir Cervicofac. 119(4):227-33, 2002
                                                                                          10. Stimmer H et al: Magnetic resonance imaging and high-resolution computed tomography in the otospongiotic phase of otosclerosis. ORL J Otorhinolaryngol Relat Spec. 64(6):451-3, 2002
                                                                                          11. Chole RA et al: Pathophysiology of otosclerosis. Otol Neurotol. 22(2):249-57, 2001
                                                                                          12. Ruckenstein MJ et al: Management of far advanced otosclerosis in the era of cochlear implantation. Otol Neurotol. 22(4):471-4, 2001
                                                                                          13. Shin YJ et al: Correlations between computed tomography findings and family history in otosclerotic patients. Otol Neurotol. 22(4):461-4, 2001
                                                                                          14. Shin YJ et al: Sensorineural hearing loss and otosclerosis: a clinical and radiologic survey of 437 cases. Acta Otolaryngol. 121(2):200-4, 2001
                                                                                          15. Veillon F et al: Imaging of the windows of the temporal bone. Semin Ultrasound CT MR. 22(3):271-80, 2001
                                                                                          16. Niyazov D et al: Fenestration surgery for otosclerosis: CT findings of an old surgical procedure. AJNR Am J Neuroradiol. 21(9):1670-2, 2000
                                                                                          17. Ziyeh S et al: MRI-visible pericochlear lesions in osteogenesis imperfecta type I. Eur Radiol. 10(10):1675-7, 2000
                                                                                          18. Ziyeh S et al: MRI of active otosclerosis. Neuroradiology. 39(6):453-7, 1997
                                                                                          19. Miura M et al: Computed tomographic image analysis of ears with otosclerosis. ORL J Otorhinolaryngol Relat Spec. 58(4):200-3, 1996
                                                                                          20. Valvassori GE: Imaging of otosclerosis. Otolaryngol Clin North Am. 26(3):359-71, 1993
                                                                                          21. Wilbrand HF: Radioanatomy of cochlear and stapedial otosclerosis. Scand Audiol Suppl. 30:181-3, 1988
                                                                                          22. Mafee MF et al: Use of CT in stapedial otosclerosis. Radiology. 156(3):709-14, 1985
                                                                                          23. Swartz JD et al: Fenestral and cochlear otosclerosis: computed tomographic evaluation. Am J Otol. 6(6):476-81, 1985