link
Bookmarks
Otosclerosis
Bronwyn E. Hamilton, MD; Troy A. Hutchins, MD
To access 4,300 diagnoses written by the world's leading experts in radiology.Try it free - 15 days
0
18
5
7

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
                • Cochlear cleft is fatty marrow due to incomplete ossification that parallels cochlea rather than localizing to fissula antefenestram
                  • May be present in children & adults, should be differentiated from otosclerosis

              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. Fujima N et al: Utility of deep learning for the diagnosis of otosclerosis on temporal bone CT. Eur Radiol. 31(7):5206-11, 2021
                                              2. Akazawa Y et al: Measurement of stapes footplate thickness in otosclerosis by ultra-high-resolution computed tomography. Acta Otolaryngol. 140(11):899-903, 2020
                                              3. Kösling S et al: Imaging of otosclerosis. Rofo. 192(8):745-53, 2020
                                              4. Maxwell AK et al: Sensitivity of high-resolution computed tomography in otosclerosis patients undergoing primary stapedotomy. Ann Otol Rhinol Laryngol. 129(9):918-23, 2020
                                              5. Maxwell AK et al: Failure to close the gap: concomitant superior canal dehiscence in otosclerosis patients. Laryngoscope. 130(4):1023-7, 2020
                                              6. McClellan J et al: Stapes surgery outcomes in patients with concurrent otosclerosis and superior semicircular canal dehiscence. Otol Neurotol. 41(7):912-5, 2020
                                              7. Pucetaite M et al: The cochlear cleft: CT correlation with histopathology. Otol Neurotol. 41(6):745-9, 2020
                                              8. Purohit B et al: Role of MRI as first-line modality in the detection of previously undiagnosed otosclerosis: a single tertiary institute experience. Insights Imaging. 11(1):71, 2020
                                              9. Andreu-Arasa VC et al: Otosclerosis and dysplasias of the temporal bone. Neuroimaging Clin N Am. 29(1):29-47, 2019
                                              10. Bae YJ et al: "Third window" and "single window" effects impede surgical success: analysis of retrofenestral otosclerosis involving the internal auditory canal or round window. J Clin Med. 8(8), 2019
                                              11. Brown LA et al: Diagnostic protocol for detecting otosclerosis on high-resolution temporal bone CT. Ann Otol Rhinol Laryngol. 128(11):1054-60, 2019
                                              12. Nguyen T et al: Conductive hearing loss with a "dry middle ear cleft"-a comprehensive pictorial review with CT. Eur J Radiol. 110:74-80, 2019
                                              13. Shim YJ et al: Involvement of the internal auditory canal in subjects with cochlear otosclerosis: a less acknowledged third window that affects surgical outcome. Otol Neurotol. 40(3):e186-90, 2019
                                              14. Yagi C et al: Otosclerosis: anatomical distribution of otosclerotic loci analyzed by high-resolution computed tomography. Eur Arch Otorhinolaryngol. 276(5):1335-40, 2019
                                              15. Berrettini S et al: 3D fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging at different stages of otosclerosis. Eur Arch Otorhinolaryngol. 275(11):2643-52, 2018
                                              16. Puac P et al: Cavitary plaques in otospongiosis: CT findings and clinical implications. AJNR Am J Neuroradiol. 39(6):1135-9, 2018
                                              17. Quesnel AM et al: Otosclerosis: temporal bone pathology. Otolaryngol Clin North Am. 51(2):291-303, 2018
                                              18. Sanghan N et al: Retrospective review of otic capsule contour and thickness in patients with otosclerosis and individuals with normal hearing on CT. AJNR Am J Neuroradiol. 39(12):2350-5, 2018
                                              19. Wolfovitz A et al: Impact of imaging in management of otosclerosis. Otolaryngol Clin North Am. 51(2):343-55, 2018
                                              20. Dudau C et al: Diagnostic efficacy and therapeutic impact of computed tomography in the evaluation of clinically suspected otosclerosis. Eur Radiol. 27(3):1195-201, 2017
                                              21. Yamashita K et al: Additive value of "otosclerosis-weighted" images for the CT diagnosis of fenestral otosclerosis. Acta Radiol. 58(10):1215-21, 2017
                                              22. Anand V et al: Obliquity of the stapes in otosclerosis: a new radiological sign. Int Arch Otorhinolaryngol. 20(2):94-8, 2016
                                              23. Atan D et al: Relation of otosclerosis and osteoporosis: a bone mineral density study. Auris Nasus Larynx. 43(4):400-3, 2016
                                              24. Mukaida T et al: Magnetic resonance imaging evaluation of endolymphatic hydrops in cases with otosclerosis. Otol Neurotol. 36(7):1146-50, 2015
                                              25. 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
                                              26. 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
                                              27. Lee TC et al: CT grading of otosclerosis. AJNR Am J Neuroradiol. 30(7):1435-9, 2009
                                              28. 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
                                              29. Marshall AH et al: Cochlear implantation in cochlear otosclerosis. Laryngoscope. 115(10):1728-33, 2005
                                              30. Chadwell JB et al: The cochlear cleft. AJNR Am J Neuroradiol. 25(1):21-4, 2004
                                              31. 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
                                              32. Rondini-Gilli E et al: [Otosclerosis surgical techniques and results in 150 patients] Ann Otolaryngol Chir Cervicofac. 119(4):227-33, 2002
                                              33. 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
                                              34. Chole RA et al: Pathophysiology of otosclerosis. Otol Neurotol. 22(2):249-57, 2001
                                              35. Ruckenstein MJ et al: Management of far advanced otosclerosis in the era of cochlear implantation. Otol Neurotol. 22(4):471-4, 2001
                                              36. Shin YJ et al: Correlations between computed tomography findings and family history in otosclerotic patients. Otol Neurotol. 22(4):461-4, 2001
                                              37. Shin YJ et al: Sensorineural hearing loss and otosclerosis: a clinical and radiologic survey of 437 cases. Acta Otolaryngol. 121(2):200-4, 2001
                                              38. Veillon F et al: Imaging of the windows of the temporal bone. Semin Ultrasound CT MR. 22(3):271-80, 2001
                                              39. Niyazov D et al: Fenestration surgery for otosclerosis: CT findings of an old surgical procedure. AJNR Am J Neuroradiol. 21(9):1670-2, 2000
                                              40. Ziyeh S et al: MRI-visible pericochlear lesions in osteogenesis imperfecta type I. Eur Radiol. 10(10):1675-7, 2000
                                              41. Ziyeh S et al: MRI of active otosclerosis. Neuroradiology. 39(6):453-7, 1997
                                              42. Miura M et al: Computed tomographic image analysis of ears with otosclerosis. ORL J Otorhinolaryngol Relat Spec. 58(4):200-3, 1996
                                              43. Valvassori GE: Imaging of otosclerosis. Otolaryngol Clin North Am. 26(3):359-71, 1993
                                              44. Wilbrand HF: Radioanatomy of cochlear and stapedial otosclerosis. Scand Audiol Suppl. 30:181-3, 1988
                                              45. Mafee MF et al: Use of CT in stapedial otosclerosis. Radiology. 156(3):709-14, 1985
                                              46. Swartz JD et al: Fenestral and cochlear otosclerosis: computed tomographic evaluation. Am J Otol. 6(6):476-81, 1985
                                              Related Anatomy
                                              Loading...
                                              Related Differential Diagnoses
                                              Loading...
                                              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
                                                            • Cochlear cleft is fatty marrow due to incomplete ossification that parallels cochlea rather than localizing to fissula antefenestram
                                                              • May be present in children & adults, should be differentiated from otosclerosis

                                                          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. Fujima N et al: Utility of deep learning for the diagnosis of otosclerosis on temporal bone CT. Eur Radiol. 31(7):5206-11, 2021
                                                                                          2. Akazawa Y et al: Measurement of stapes footplate thickness in otosclerosis by ultra-high-resolution computed tomography. Acta Otolaryngol. 140(11):899-903, 2020
                                                                                          3. Kösling S et al: Imaging of otosclerosis. Rofo. 192(8):745-53, 2020
                                                                                          4. Maxwell AK et al: Sensitivity of high-resolution computed tomography in otosclerosis patients undergoing primary stapedotomy. Ann Otol Rhinol Laryngol. 129(9):918-23, 2020
                                                                                          5. Maxwell AK et al: Failure to close the gap: concomitant superior canal dehiscence in otosclerosis patients. Laryngoscope. 130(4):1023-7, 2020
                                                                                          6. McClellan J et al: Stapes surgery outcomes in patients with concurrent otosclerosis and superior semicircular canal dehiscence. Otol Neurotol. 41(7):912-5, 2020
                                                                                          7. Pucetaite M et al: The cochlear cleft: CT correlation with histopathology. Otol Neurotol. 41(6):745-9, 2020
                                                                                          8. Purohit B et al: Role of MRI as first-line modality in the detection of previously undiagnosed otosclerosis: a single tertiary institute experience. Insights Imaging. 11(1):71, 2020
                                                                                          9. Andreu-Arasa VC et al: Otosclerosis and dysplasias of the temporal bone. Neuroimaging Clin N Am. 29(1):29-47, 2019
                                                                                          10. Bae YJ et al: "Third window" and "single window" effects impede surgical success: analysis of retrofenestral otosclerosis involving the internal auditory canal or round window. J Clin Med. 8(8), 2019
                                                                                          11. Brown LA et al: Diagnostic protocol for detecting otosclerosis on high-resolution temporal bone CT. Ann Otol Rhinol Laryngol. 128(11):1054-60, 2019
                                                                                          12. Nguyen T et al: Conductive hearing loss with a "dry middle ear cleft"-a comprehensive pictorial review with CT. Eur J Radiol. 110:74-80, 2019
                                                                                          13. Shim YJ et al: Involvement of the internal auditory canal in subjects with cochlear otosclerosis: a less acknowledged third window that affects surgical outcome. Otol Neurotol. 40(3):e186-90, 2019
                                                                                          14. Yagi C et al: Otosclerosis: anatomical distribution of otosclerotic loci analyzed by high-resolution computed tomography. Eur Arch Otorhinolaryngol. 276(5):1335-40, 2019
                                                                                          15. Berrettini S et al: 3D fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging at different stages of otosclerosis. Eur Arch Otorhinolaryngol. 275(11):2643-52, 2018
                                                                                          16. Puac P et al: Cavitary plaques in otospongiosis: CT findings and clinical implications. AJNR Am J Neuroradiol. 39(6):1135-9, 2018
                                                                                          17. Quesnel AM et al: Otosclerosis: temporal bone pathology. Otolaryngol Clin North Am. 51(2):291-303, 2018
                                                                                          18. Sanghan N et al: Retrospective review of otic capsule contour and thickness in patients with otosclerosis and individuals with normal hearing on CT. AJNR Am J Neuroradiol. 39(12):2350-5, 2018
                                                                                          19. Wolfovitz A et al: Impact of imaging in management of otosclerosis. Otolaryngol Clin North Am. 51(2):343-55, 2018
                                                                                          20. Dudau C et al: Diagnostic efficacy and therapeutic impact of computed tomography in the evaluation of clinically suspected otosclerosis. Eur Radiol. 27(3):1195-201, 2017
                                                                                          21. Yamashita K et al: Additive value of "otosclerosis-weighted" images for the CT diagnosis of fenestral otosclerosis. Acta Radiol. 58(10):1215-21, 2017
                                                                                          22. Anand V et al: Obliquity of the stapes in otosclerosis: a new radiological sign. Int Arch Otorhinolaryngol. 20(2):94-8, 2016
                                                                                          23. Atan D et al: Relation of otosclerosis and osteoporosis: a bone mineral density study. Auris Nasus Larynx. 43(4):400-3, 2016
                                                                                          24. Mukaida T et al: Magnetic resonance imaging evaluation of endolymphatic hydrops in cases with otosclerosis. Otol Neurotol. 36(7):1146-50, 2015
                                                                                          25. 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
                                                                                          26. 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
                                                                                          27. Lee TC et al: CT grading of otosclerosis. AJNR Am J Neuroradiol. 30(7):1435-9, 2009
                                                                                          28. 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
                                                                                          29. Marshall AH et al: Cochlear implantation in cochlear otosclerosis. Laryngoscope. 115(10):1728-33, 2005
                                                                                          30. Chadwell JB et al: The cochlear cleft. AJNR Am J Neuroradiol. 25(1):21-4, 2004
                                                                                          31. 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
                                                                                          32. Rondini-Gilli E et al: [Otosclerosis surgical techniques and results in 150 patients] Ann Otolaryngol Chir Cervicofac. 119(4):227-33, 2002
                                                                                          33. 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
                                                                                          34. Chole RA et al: Pathophysiology of otosclerosis. Otol Neurotol. 22(2):249-57, 2001
                                                                                          35. Ruckenstein MJ et al: Management of far advanced otosclerosis in the era of cochlear implantation. Otol Neurotol. 22(4):471-4, 2001
                                                                                          36. Shin YJ et al: Correlations between computed tomography findings and family history in otosclerotic patients. Otol Neurotol. 22(4):461-4, 2001
                                                                                          37. Shin YJ et al: Sensorineural hearing loss and otosclerosis: a clinical and radiologic survey of 437 cases. Acta Otolaryngol. 121(2):200-4, 2001
                                                                                          38. Veillon F et al: Imaging of the windows of the temporal bone. Semin Ultrasound CT MR. 22(3):271-80, 2001
                                                                                          39. Niyazov D et al: Fenestration surgery for otosclerosis: CT findings of an old surgical procedure. AJNR Am J Neuroradiol. 21(9):1670-2, 2000
                                                                                          40. Ziyeh S et al: MRI-visible pericochlear lesions in osteogenesis imperfecta type I. Eur Radiol. 10(10):1675-7, 2000
                                                                                          41. Ziyeh S et al: MRI of active otosclerosis. Neuroradiology. 39(6):453-7, 1997
                                                                                          42. Miura M et al: Computed tomographic image analysis of ears with otosclerosis. ORL J Otorhinolaryngol Relat Spec. 58(4):200-3, 1996
                                                                                          43. Valvassori GE: Imaging of otosclerosis. Otolaryngol Clin North Am. 26(3):359-71, 1993
                                                                                          44. Wilbrand HF: Radioanatomy of cochlear and stapedial otosclerosis. Scand Audiol Suppl. 30:181-3, 1988
                                                                                          45. Mafee MF et al: Use of CT in stapedial otosclerosis. Radiology. 156(3):709-14, 1985
                                                                                          46. Swartz JD et al: Fenestral and cochlear otosclerosis: computed tomographic evaluation. Am J Otol. 6(6):476-81, 1985