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Middle Ear Schwannoma
Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR; Bronwyn E. Hamilton, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Facial nerve schwannoma, tympanic branch nerve schwannoma, chorda tympani schwannoma
          • Definitions

            • Primary schwannoma: Tumor primary to middle ear (ME)
              • Tympanic segment CNVII > > tympanic branch (CNIX branch or Jacobson nerve), chorda tympani nerve
            • Secondary schwannoma: Arises outside ME
              • Large CNIX-XI jugular foramen schwannoma eroding into ME
              • Translabyrinthine CNVIII schwannoma
                • Cerebellopontine angle-internal auditory canal (CPA-IAC) → inner ear → ME
              • Primary inner ear schwannoma → ME

          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

                                      • Reporting Tips

                                        Selected References

                                        1. Furukawa T et al: Facial nerve and chorda tympani schwannomas: case series, and advantages of using non-rigid registration of post-enhanced 3D-T1 turbo field echo and CT images (TURFECT) in their diagnosis and surgical treatment. Auris Nasus Larynx. 47(3):383-90, 2020
                                        2. Gao W et al: Facial nerve meningioma: a case mimicking facial nerve schwannoma. Ear Nose Throat J. 145561320962582, 2020
                                        3. Loos E et al: Intratemporal facial nerve schwannomas: multicenter experience of 80 cases. Eur Arch Otorhinolaryngol. 277(8):2209-17, 2020
                                        4. Kang WS et al: Surgical outcomes of intratemporal facial nerve schwannomas according to facial nerve manipulation. J Int Adv Otol. 15(3):415-9, 2019
                                        5. Quesnel AM et al: Evaluation and management of facial nerve schwannoma. Otolaryngol Clin North Am. 51(6):1179-92, 2018
                                        6. Simone M et al: Intraparotid facial nerve schwannoma: two case reports and a review of the literature. Acta Otorhinolaryngol Ital. 38(1):73-7, 2018
                                        7. Carlson ML et al: Facial nerve schwannomas: review of 80 cases over 25 years at Mayo Clinic. Mayo Clin Proc. 91(11):1563-76, 2016
                                        8. Lahlou G et al: Intratemporal facial nerve schwannoma: clinical presentation and management. Eur Arch Otorhinolaryngol. 273(11):3497-504, 2016
                                        9. Shi W et al: Fractionated stereotactic radiotherapy for facial nerve schwannomas. J Neurol Surg B Skull Base. 77(1):75-80, 2016
                                        10. McRackan TR et al: Primary tumors of the facial nerve. Otolaryngol Clin North Am. 48(3):491-500, 2015
                                        11. Mohamed A et al: Large middle ear schwannoma of the Jacobson's nerve with intracranial extension. Auris Nasus Larynx. 41(5):491-5, 2014
                                        12. Ross L et al: Atypical tumors of the facial nerve: case series and review of the literature. Neurosurg Focus. 34(3):E2, 2013
                                        13. Van Abel KM et al: Primary inner ear schwannomas: a case series and systematic review of the literature. Laryngoscope. 123(8):1957-66, 2013
                                        14. Salzman KL et al: Intralabyrinthine schwannomas: imaging diagnosis and classification. AJNR Am J Neuroradiol. 33(1):104-9, 2012
                                        15. Fabiano AJ et al: Diagnosis of facial nerve schwannoma by magnetic resonance imaging enhancement of the geniculate ganglion. Arch Neurol. 67(1):112-3, 2010
                                        16. Huoh KC et al: Chorda tympani neuroma. Otol Neurotol. 31(7):1172-3, 2010
                                        17. Roig O R JL et al: Middle ear schwannoma. Braz J Otorhinolaryngol. 76(5):673, 2010
                                        18. Thompson AL et al: Magnetic resonance imaging of facial nerve schwannoma. Laryngoscope. 119(12):2428-36, 2009
                                        19. Kim CW et al: Primary middle ear schwannoma. Am J Otolaryngol. 28(5):342-6, 2007
                                        20. Sone M et al: Middle ear schwannoma extending from the cerebellopontine angle in a patient with neurofibromatosis type 2. Otolaryngol Head Neck Surg. 137(3):511-2, 2007
                                        21. Vasama JP et al: Jacobson's nerve schwannoma. Otol Neurotol. 28(1):141, 2007
                                        22. Wiggins RH 3rd et al: The many faces of facial nerve schwannoma. AJNR Am J Neuroradiol. 27(3):694-9, 2006
                                        23. Hopkins C et al: Chorda tympani neuroma masquerading as cholesteatoma. J Laryngol Otol. 117(12):987-8, 2003
                                        24. Swartz JD: Imaging diagnosis of middle ear lesions. Curr Probl Diagn Radiol. 31(1):4-21, 2002
                                        25. Biggs ND et al: Schwannoma of the chorda tympani. J Laryngol Otol. 115(1):50-2, 2001
                                        26. Kesser BW et al: Jacobson's nerve schwannoma: a rare middle ear mass. Ann Otol Rhinol Laryngol. 110(11):1030-4, 2001
                                        27. Aydin K et al: Jacobson's nerve schwannoma presenting as middle ear mass. AJNR Am J Neuroradiol. 21(7):1331-3, 2000
                                        28. Browning ST et al: Schwannoma of the chorda tympani nerve. J Laryngol Otol. 114(1):81-2, 2000
                                        29. Magliulo G et al: Chorda tympani neuroma: diagnosis and management. Am J Otolaryngol. 21(1):65-8, 2000
                                        30. Lopes Filho O et al: Neuroma of the chorda tympani nerve. Ear Nose Throat J. 72(11):730-2, 1993
                                        31. Wiet RJ et al: Neurilemmoma of the chorda tympani nerve. Otolaryngol Head Neck Surg. 93(1):119-21, 1985
                                        32. Babin RW et al: Traumatic neuromas of the facial nerve. Arch Otolaryngol. 107(1):55-8, 1981
                                        33. Pou JW et al: Neuroma of the chorda tympani nerve. Laryngoscope. 84(7):1170-4, 1974
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Facial nerve schwannoma, tympanic branch nerve schwannoma, chorda tympani schwannoma
                                                • Definitions

                                                  • Primary schwannoma: Tumor primary to middle ear (ME)
                                                    • Tympanic segment CNVII > > tympanic branch (CNIX branch or Jacobson nerve), chorda tympani nerve
                                                  • Secondary schwannoma: Arises outside ME
                                                    • Large CNIX-XI jugular foramen schwannoma eroding into ME
                                                    • Translabyrinthine CNVIII schwannoma
                                                      • Cerebellopontine angle-internal auditory canal (CPA-IAC) → inner ear → ME
                                                    • Primary inner ear schwannoma → ME

                                                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

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Furukawa T et al: Facial nerve and chorda tympani schwannomas: case series, and advantages of using non-rigid registration of post-enhanced 3D-T1 turbo field echo and CT images (TURFECT) in their diagnosis and surgical treatment. Auris Nasus Larynx. 47(3):383-90, 2020
                                                                              2. Gao W et al: Facial nerve meningioma: a case mimicking facial nerve schwannoma. Ear Nose Throat J. 145561320962582, 2020
                                                                              3. Loos E et al: Intratemporal facial nerve schwannomas: multicenter experience of 80 cases. Eur Arch Otorhinolaryngol. 277(8):2209-17, 2020
                                                                              4. Kang WS et al: Surgical outcomes of intratemporal facial nerve schwannomas according to facial nerve manipulation. J Int Adv Otol. 15(3):415-9, 2019
                                                                              5. Quesnel AM et al: Evaluation and management of facial nerve schwannoma. Otolaryngol Clin North Am. 51(6):1179-92, 2018
                                                                              6. Simone M et al: Intraparotid facial nerve schwannoma: two case reports and a review of the literature. Acta Otorhinolaryngol Ital. 38(1):73-7, 2018
                                                                              7. Carlson ML et al: Facial nerve schwannomas: review of 80 cases over 25 years at Mayo Clinic. Mayo Clin Proc. 91(11):1563-76, 2016
                                                                              8. Lahlou G et al: Intratemporal facial nerve schwannoma: clinical presentation and management. Eur Arch Otorhinolaryngol. 273(11):3497-504, 2016
                                                                              9. Shi W et al: Fractionated stereotactic radiotherapy for facial nerve schwannomas. J Neurol Surg B Skull Base. 77(1):75-80, 2016
                                                                              10. McRackan TR et al: Primary tumors of the facial nerve. Otolaryngol Clin North Am. 48(3):491-500, 2015
                                                                              11. Mohamed A et al: Large middle ear schwannoma of the Jacobson's nerve with intracranial extension. Auris Nasus Larynx. 41(5):491-5, 2014
                                                                              12. Ross L et al: Atypical tumors of the facial nerve: case series and review of the literature. Neurosurg Focus. 34(3):E2, 2013
                                                                              13. Van Abel KM et al: Primary inner ear schwannomas: a case series and systematic review of the literature. Laryngoscope. 123(8):1957-66, 2013
                                                                              14. Salzman KL et al: Intralabyrinthine schwannomas: imaging diagnosis and classification. AJNR Am J Neuroradiol. 33(1):104-9, 2012
                                                                              15. Fabiano AJ et al: Diagnosis of facial nerve schwannoma by magnetic resonance imaging enhancement of the geniculate ganglion. Arch Neurol. 67(1):112-3, 2010
                                                                              16. Huoh KC et al: Chorda tympani neuroma. Otol Neurotol. 31(7):1172-3, 2010
                                                                              17. Roig O R JL et al: Middle ear schwannoma. Braz J Otorhinolaryngol. 76(5):673, 2010
                                                                              18. Thompson AL et al: Magnetic resonance imaging of facial nerve schwannoma. Laryngoscope. 119(12):2428-36, 2009
                                                                              19. Kim CW et al: Primary middle ear schwannoma. Am J Otolaryngol. 28(5):342-6, 2007
                                                                              20. Sone M et al: Middle ear schwannoma extending from the cerebellopontine angle in a patient with neurofibromatosis type 2. Otolaryngol Head Neck Surg. 137(3):511-2, 2007
                                                                              21. Vasama JP et al: Jacobson's nerve schwannoma. Otol Neurotol. 28(1):141, 2007
                                                                              22. Wiggins RH 3rd et al: The many faces of facial nerve schwannoma. AJNR Am J Neuroradiol. 27(3):694-9, 2006
                                                                              23. Hopkins C et al: Chorda tympani neuroma masquerading as cholesteatoma. J Laryngol Otol. 117(12):987-8, 2003
                                                                              24. Swartz JD: Imaging diagnosis of middle ear lesions. Curr Probl Diagn Radiol. 31(1):4-21, 2002
                                                                              25. Biggs ND et al: Schwannoma of the chorda tympani. J Laryngol Otol. 115(1):50-2, 2001
                                                                              26. Kesser BW et al: Jacobson's nerve schwannoma: a rare middle ear mass. Ann Otol Rhinol Laryngol. 110(11):1030-4, 2001
                                                                              27. Aydin K et al: Jacobson's nerve schwannoma presenting as middle ear mass. AJNR Am J Neuroradiol. 21(7):1331-3, 2000
                                                                              28. Browning ST et al: Schwannoma of the chorda tympani nerve. J Laryngol Otol. 114(1):81-2, 2000
                                                                              29. Magliulo G et al: Chorda tympani neuroma: diagnosis and management. Am J Otolaryngol. 21(1):65-8, 2000
                                                                              30. Lopes Filho O et al: Neuroma of the chorda tympani nerve. Ear Nose Throat J. 72(11):730-2, 1993
                                                                              31. Wiet RJ et al: Neurilemmoma of the chorda tympani nerve. Otolaryngol Head Neck Surg. 93(1):119-21, 1985
                                                                              32. Babin RW et al: Traumatic neuromas of the facial nerve. Arch Otolaryngol. 107(1):55-8, 1981
                                                                              33. Pou JW et al: Neuroma of the chorda tympani nerve. Laryngoscope. 84(7):1170-4, 1974