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Intralabyrinthine Schwannoma
Karen L. Salzman, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Intralabyrinthine schwannoma (ILS)
            • Synonyms

              • Inner ear schwannoma
            • Definitions

              • ILS: Benign tumor arising from Schwann cells within structures of membranous labyrinth
                • Includes schwannomas of cochlea, vestibule, or both as well as tumors of inner ear extending to internal auditory canal (IAC) or middle ear

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Totten DJ et al: Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas. Am J Otolaryngol. 42(4):102984, 2021
                                        2. Bagattini M et al: Histopathologic evaluation of intralabyrinthine schwannoma. Audiol Neurootol. 1-8, 2020
                                        3. Cho SJ et al: Diagnostic assessment of magnetic resonance imaging for patients with intralabyrinthine schwannoma: a systematic review. J Neuroradiol. ePub, 2020
                                        4. Choudhury B et al: Intralabyrinthine schwannomas: disease presentation, tumor management, and hearing rehabilitation. J Neurol Surg B Skull Base. 80(2):196-202, 2019
                                        5. Bae YJ et al: Differentiation between intralabyrinthine schwannoma and contrast-enhancing labyrinthitis on MRI: quantitative analysis of signal intensity Characteristics. Otol Neurotol. 39(8):1045-52, 2018
                                        6. Marinelli JP et al: Incidence of intralabyrinthine schwannoma: a population-based study within the United States. Otol Neurotol. 39(9):1191-4, 2018
                                        7. Frisch CD et al: Intralabyrinthine schwannomas. Otolaryngol Clin North Am. 48(3):423-41, 2015
                                        8. Gosselin É et al: Meta-analysis on the clinical outcomes in patients with intralabyrinthine schwannomas: conservative management vs. microsurgery. Eur Arch Otorhinolaryngol. 273(6):1357-67, 2015
                                        9. Lee JJ et al: Recovery of hearing after surgical removal of intralabyrinthine schwannoma. Laryngoscope. 125(8):1968-71, 2015
                                        10. Slattery EL et al: Intralabyrinthine schwannomas mimic cochleovestibular disease: symptoms from tumor mass effect in the labyrinth. Otol Neurotol. 36(1):167-71, 2015
                                        11. Dubernard X et al: Clinical presentation of intralabyrinthine schwannomas: a multicenter study of 110 cases. Otol Neurotol. 35(9):1641-9, 2014
                                        12. Peng R et al: Intensity of gadolinium enhancement on MRI is useful in differentiation of intracochlear inflammation from tumor. Otol Neurotol. 35(5):905-10, 2014
                                        13. Schutt CA et al: Cochlear implantation after resection of an intralabyrinthine schwannoma. Am J Otolaryngol. 35(2):257-60, 2014
                                        14. Van Abel KM et al: Primary inner ear schwannomas: a case series and systematic review of the literature. Laryngoscope. 123(8):1957-66, 2013
                                        15. Salzman KL et al: Intralabyrinthine schwannomas: imaging diagnosis and classification. AJNR Am J Neuroradiol. 33(1):104-9, 2012
                                        16. Dubrulle F et al: Differential diagnosis and prognosis of T1-weighted post-gadolinium intralabyrinthine hyperintensities. Eur Radiol. 20(11):2628-36, 2010
                                        17. Iseri M et al: Hearing loss owing to intralabyrinthine schwannoma responsive to intratympanic steroid treatment. J Otolaryngol Head Neck Surg. 38(3):E95-7, 2009
                                        18. Shin YR et al: Intralabyrinthine schwannoma involving the cochlea, vestibule, and internal auditory canal: 'canalolabyrinthine schwannoma'. Eur Arch Otorhinolaryngol. 266(1):143-5, 2009
                                        19. Jia H et al: Intralabyrinthine schwannomas: symptoms and managements. Auris Nasus Larynx. 35(1):131-6, 2008
                                        20. Tieleman A et al: Imaging of intralabyrinthine schwannomas: a retrospective study of 52 cases with emphasis on lesion growth. AJNR Am J Neuroradiol. 29(5):898-905, 2008
                                        21. Di Lella F et al: Management of intralabyrinthine schwannomas. Auris Nasus Larynx. 34(4):459-63, 2007
                                        22. Machner B et al: Intralabyrinthine schwannoma affecting the low but not high frequency function of the vestibulo-ocular reflex: implications for the clinical diagnosis of chronic peripheral vestibular deficits. J Neurol Neurosurg Psychiatry. 78(7):772-4, 2007
                                        23. Kennedy RJ et al: Intralabyrinthine schwannomas: diagnosis, management, and a new classification system. Otol Neurotol. 25(2):160-7, 2004
                                        24. Falcioni M et al: Inner ear extension of vestibular schwannomas. Laryngoscope. 113(9):1605-8, 2003
                                        25. Jackson LE et al: Intralabyrinthine schwannoma: subtle differentiating symptomatology. Otolaryngol Head Neck Surg. 129(4):439-40, 2003
                                        26. Neff BA et al: Intralabyrinthine schwannomas. Otol Neurotol. 24(2):299-307, 2003
                                        27. Hegarty JL et al: The value of enhanced magnetic resonance imaging in the evaluation of endocochlear disease. Laryngoscope. 112(1):8-17, 2002
                                        28. Green JD Jr et al: Diagnosis and management of intralabyrinthine schwannomas. Laryngoscope. 109(10):1626-31, 1999
                                        29. Deux JF et al: Slow-growing labyrinthine masses: contribution of MRI to diagnosis, follow-up and treatment. Neuroradiology. 40(10):684-9, 1998
                                        30. Mafee MF: MR imaging of intralabyrinthine schwannoma, labyrinthitis, and other labyrinthine pathology. Otolaryngol Clin North Am. 28(3):407-30, 1995
                                        31. Saeed SR et al: Intralabyrinthine schwannoma shown by magnetic resonance imaging. Neuroradiology. 36(1):63-4, 1994
                                        32. Sataloff RT et al: Intralabyrinthine schwannoma. Am J Otol. 9(4):323-6, 1988
                                        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

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Intralabyrinthine schwannoma (ILS)
                                                  • Synonyms

                                                    • Inner ear schwannoma
                                                  • Definitions

                                                    • ILS: Benign tumor arising from Schwann cells within structures of membranous labyrinth
                                                      • Includes schwannomas of cochlea, vestibule, or both as well as tumors of inner ear extending to internal auditory canal (IAC) or middle ear

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Totten DJ et al: Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas. Am J Otolaryngol. 42(4):102984, 2021
                                                                              2. Bagattini M et al: Histopathologic evaluation of intralabyrinthine schwannoma. Audiol Neurootol. 1-8, 2020
                                                                              3. Cho SJ et al: Diagnostic assessment of magnetic resonance imaging for patients with intralabyrinthine schwannoma: a systematic review. J Neuroradiol. ePub, 2020
                                                                              4. Choudhury B et al: Intralabyrinthine schwannomas: disease presentation, tumor management, and hearing rehabilitation. J Neurol Surg B Skull Base. 80(2):196-202, 2019
                                                                              5. Bae YJ et al: Differentiation between intralabyrinthine schwannoma and contrast-enhancing labyrinthitis on MRI: quantitative analysis of signal intensity Characteristics. Otol Neurotol. 39(8):1045-52, 2018
                                                                              6. Marinelli JP et al: Incidence of intralabyrinthine schwannoma: a population-based study within the United States. Otol Neurotol. 39(9):1191-4, 2018
                                                                              7. Frisch CD et al: Intralabyrinthine schwannomas. Otolaryngol Clin North Am. 48(3):423-41, 2015
                                                                              8. Gosselin É et al: Meta-analysis on the clinical outcomes in patients with intralabyrinthine schwannomas: conservative management vs. microsurgery. Eur Arch Otorhinolaryngol. 273(6):1357-67, 2015
                                                                              9. Lee JJ et al: Recovery of hearing after surgical removal of intralabyrinthine schwannoma. Laryngoscope. 125(8):1968-71, 2015
                                                                              10. Slattery EL et al: Intralabyrinthine schwannomas mimic cochleovestibular disease: symptoms from tumor mass effect in the labyrinth. Otol Neurotol. 36(1):167-71, 2015
                                                                              11. Dubernard X et al: Clinical presentation of intralabyrinthine schwannomas: a multicenter study of 110 cases. Otol Neurotol. 35(9):1641-9, 2014
                                                                              12. Peng R et al: Intensity of gadolinium enhancement on MRI is useful in differentiation of intracochlear inflammation from tumor. Otol Neurotol. 35(5):905-10, 2014
                                                                              13. Schutt CA et al: Cochlear implantation after resection of an intralabyrinthine schwannoma. Am J Otolaryngol. 35(2):257-60, 2014
                                                                              14. Van Abel KM et al: Primary inner ear schwannomas: a case series and systematic review of the literature. Laryngoscope. 123(8):1957-66, 2013
                                                                              15. Salzman KL et al: Intralabyrinthine schwannomas: imaging diagnosis and classification. AJNR Am J Neuroradiol. 33(1):104-9, 2012
                                                                              16. Dubrulle F et al: Differential diagnosis and prognosis of T1-weighted post-gadolinium intralabyrinthine hyperintensities. Eur Radiol. 20(11):2628-36, 2010
                                                                              17. Iseri M et al: Hearing loss owing to intralabyrinthine schwannoma responsive to intratympanic steroid treatment. J Otolaryngol Head Neck Surg. 38(3):E95-7, 2009
                                                                              18. Shin YR et al: Intralabyrinthine schwannoma involving the cochlea, vestibule, and internal auditory canal: 'canalolabyrinthine schwannoma'. Eur Arch Otorhinolaryngol. 266(1):143-5, 2009
                                                                              19. Jia H et al: Intralabyrinthine schwannomas: symptoms and managements. Auris Nasus Larynx. 35(1):131-6, 2008
                                                                              20. Tieleman A et al: Imaging of intralabyrinthine schwannomas: a retrospective study of 52 cases with emphasis on lesion growth. AJNR Am J Neuroradiol. 29(5):898-905, 2008
                                                                              21. Di Lella F et al: Management of intralabyrinthine schwannomas. Auris Nasus Larynx. 34(4):459-63, 2007
                                                                              22. Machner B et al: Intralabyrinthine schwannoma affecting the low but not high frequency function of the vestibulo-ocular reflex: implications for the clinical diagnosis of chronic peripheral vestibular deficits. J Neurol Neurosurg Psychiatry. 78(7):772-4, 2007
                                                                              23. Kennedy RJ et al: Intralabyrinthine schwannomas: diagnosis, management, and a new classification system. Otol Neurotol. 25(2):160-7, 2004
                                                                              24. Falcioni M et al: Inner ear extension of vestibular schwannomas. Laryngoscope. 113(9):1605-8, 2003
                                                                              25. Jackson LE et al: Intralabyrinthine schwannoma: subtle differentiating symptomatology. Otolaryngol Head Neck Surg. 129(4):439-40, 2003
                                                                              26. Neff BA et al: Intralabyrinthine schwannomas. Otol Neurotol. 24(2):299-307, 2003
                                                                              27. Hegarty JL et al: The value of enhanced magnetic resonance imaging in the evaluation of endocochlear disease. Laryngoscope. 112(1):8-17, 2002
                                                                              28. Green JD Jr et al: Diagnosis and management of intralabyrinthine schwannomas. Laryngoscope. 109(10):1626-31, 1999
                                                                              29. Deux JF et al: Slow-growing labyrinthine masses: contribution of MRI to diagnosis, follow-up and treatment. Neuroradiology. 40(10):684-9, 1998
                                                                              30. Mafee MF: MR imaging of intralabyrinthine schwannoma, labyrinthitis, and other labyrinthine pathology. Otolaryngol Clin North Am. 28(3):407-30, 1995
                                                                              31. Saeed SR et al: Intralabyrinthine schwannoma shown by magnetic resonance imaging. Neuroradiology. 36(1):63-4, 1994
                                                                              32. Sataloff RT et al: Intralabyrinthine schwannoma. Am J Otol. 9(4):323-6, 1988