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CPA-IAC Superficial Siderosis
Bronwyn E. Hamilton, MD; H. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        TERMINOLOGY

        • Abbreviations

          • Cerebellopontine angle (CPA)
          • Internal auditory canal (IAC)
          • Superficial siderosis (SS)
        • Synonyms

          • Siderosis, CNS siderosis
        • Definitions

          • Recurrent subarachnoid hemorrhage (SAH) causes hemosiderin deposition on surface of brain, brainstem, and cranial nerve leptomeninges

        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

                                    • Reporting Tips

                                      Selected References

                                      1. Albayram MS et al: Frequency, extent, and correlates of superficial siderosis and ependymal siderosis in premature infants with germinal matrix hemorrhage: an SWI study. AJNR Am J Neuroradiol. 41(2):331-7, 2020
                                      2. Friedauer L et al: Spinal dural leaks in patients with infratentorial superficial siderosis of the central nervous system-refinement of a diagnostic algorithm. Eur J Neurol. ePub, 2020
                                      3. Alshehabi M et al: Cochlear implantation in the presence of superficial siderosis. Clin Otolaryngol. 44(6):1166-9, 2019
                                      4. Wagner F et al: Superficial siderosis of the central nervous system: neurotological findings related to magnetic resonance imaging. Otol Neurotol. 40(1):31-7, 2019
                                      5. Beitzke M et al: Acute diffusion-weighted imaging lesions in cerebral amyloid angiopathy-related convexal subarachnoid hemorrhage. J Cereb Blood Flow Metab. 38(2):225-9, 2018
                                      6. Takeda T et al: Vestibular dysfunction in patients with superficial siderosis of the central nervous system. Otol Neurotol. 39(6):e468-74, 2018
                                      7. Wipplinger C et al: Superficial siderosis of central nervous system as primary clinical manifestation secondary to intradural thoracic disk herniation. World Neurosurg. 119:40-4, 2018
                                      8. Pichler M et al: Prevalence and natural history of superficial siderosis: a population-based study. Stroke. 48(12):3210-14, 2017
                                      9. Lauer A et al: Microbleeds on MRI are associated with microinfarcts on autopsy in cerebral amyloid angiopathy. Neurology. 87(14):1488-92, 2016
                                      10. Charidimou A et al: Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. Brain. 138(Pt 8):2126-39, 2015
                                      11. Kishimoto I et al: Clinical features of rapidly progressive bilateral sensorineural hearing loss. Acta Otolaryngol. 134(1):58-65, 2014
                                      12. Tyler GK et al: Systematic review of outcome of cochlear implantation in superficial siderosis. Otol Neurotol. 33(6):976-82, 2012
                                      13. Nadol JB Jr et al: Temporal bone histopathology in a case of sensorineural hearing loss caused by superficial siderosis of the central nervous system and treated by cochlear implantation. Otol Neurotol. 32(5):748-55, 2011
                                      14. Linn J et al: Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. Neurology. 74(17):1346-50, 2010
                                      15. Koeppen AH et al: The pathology of superficial siderosis of the central nervous system. Acta Neuropathol. 116(4):371-82, 2008
                                      16. Kumar N: Superficial siderosis: associations and therapeutic implications. Arch Neurol. 64(4):491-6, 2007
                                      17. Kumar N et al: Superficial siderosis. Neurology. 66(8):1144-52, 2006
                                      18. Leussink VI et al: Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches. Acta Neurol Scand. 107(1):54-61, 2003
                                      19. Dhooge IJ et al: Cochlear implantation in a patient with superficial siderosis of the central nervous system. Otol Neurotol. 23(4):468-72, 2002
                                      20. Hsu WC et al: Superficial siderosis of the CNS associated with multiple cavernous malformations. AJNR Am J Neuroradiol. 20(7):1245-8, 1999
                                      21. Weller M et al: Elevated CSF lactoferrin in superficial siderosis of the central nervous system. J Neurol. 246(10):943-5, 1999
                                      22. Lemmerling M et al: Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss. Neuroradiology. 40(5):312-4, 1998
                                      23. Castelli ML et al: Superficial siderosis of the central nervous system: an underestimated cause of hearing loss. J Laryngol Otol. 111(1):60-2, 1997
                                      24. Maurizi CP: Superficial siderosis of the brain: roles for cerebrospinal fluid circulation, iron and the hydroxyl radical. Med Hypotheses. 47(4):261-4, 1996
                                      25. Offenbacher H et al: Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology. 38 Suppl 1:S51-6, 1996
                                      26. Bracchi M et al: Superficial siderosis of the CNS: MR diagnosis and clinical findings. AJNR Am J Neuroradiol. 14(1):227-36, 1993
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Pathology

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Cerebellopontine angle (CPA)
                                              • Internal auditory canal (IAC)
                                              • Superficial siderosis (SS)
                                            • Synonyms

                                              • Siderosis, CNS siderosis
                                            • Definitions

                                              • Recurrent subarachnoid hemorrhage (SAH) causes hemosiderin deposition on surface of brain, brainstem, and cranial nerve leptomeninges

                                            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

                                                                        • Reporting Tips

                                                                          Selected References

                                                                          1. Albayram MS et al: Frequency, extent, and correlates of superficial siderosis and ependymal siderosis in premature infants with germinal matrix hemorrhage: an SWI study. AJNR Am J Neuroradiol. 41(2):331-7, 2020
                                                                          2. Friedauer L et al: Spinal dural leaks in patients with infratentorial superficial siderosis of the central nervous system-refinement of a diagnostic algorithm. Eur J Neurol. ePub, 2020
                                                                          3. Alshehabi M et al: Cochlear implantation in the presence of superficial siderosis. Clin Otolaryngol. 44(6):1166-9, 2019
                                                                          4. Wagner F et al: Superficial siderosis of the central nervous system: neurotological findings related to magnetic resonance imaging. Otol Neurotol. 40(1):31-7, 2019
                                                                          5. Beitzke M et al: Acute diffusion-weighted imaging lesions in cerebral amyloid angiopathy-related convexal subarachnoid hemorrhage. J Cereb Blood Flow Metab. 38(2):225-9, 2018
                                                                          6. Takeda T et al: Vestibular dysfunction in patients with superficial siderosis of the central nervous system. Otol Neurotol. 39(6):e468-74, 2018
                                                                          7. Wipplinger C et al: Superficial siderosis of central nervous system as primary clinical manifestation secondary to intradural thoracic disk herniation. World Neurosurg. 119:40-4, 2018
                                                                          8. Pichler M et al: Prevalence and natural history of superficial siderosis: a population-based study. Stroke. 48(12):3210-14, 2017
                                                                          9. Lauer A et al: Microbleeds on MRI are associated with microinfarcts on autopsy in cerebral amyloid angiopathy. Neurology. 87(14):1488-92, 2016
                                                                          10. Charidimou A et al: Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. Brain. 138(Pt 8):2126-39, 2015
                                                                          11. Kishimoto I et al: Clinical features of rapidly progressive bilateral sensorineural hearing loss. Acta Otolaryngol. 134(1):58-65, 2014
                                                                          12. Tyler GK et al: Systematic review of outcome of cochlear implantation in superficial siderosis. Otol Neurotol. 33(6):976-82, 2012
                                                                          13. Nadol JB Jr et al: Temporal bone histopathology in a case of sensorineural hearing loss caused by superficial siderosis of the central nervous system and treated by cochlear implantation. Otol Neurotol. 32(5):748-55, 2011
                                                                          14. Linn J et al: Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. Neurology. 74(17):1346-50, 2010
                                                                          15. Koeppen AH et al: The pathology of superficial siderosis of the central nervous system. Acta Neuropathol. 116(4):371-82, 2008
                                                                          16. Kumar N: Superficial siderosis: associations and therapeutic implications. Arch Neurol. 64(4):491-6, 2007
                                                                          17. Kumar N et al: Superficial siderosis. Neurology. 66(8):1144-52, 2006
                                                                          18. Leussink VI et al: Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches. Acta Neurol Scand. 107(1):54-61, 2003
                                                                          19. Dhooge IJ et al: Cochlear implantation in a patient with superficial siderosis of the central nervous system. Otol Neurotol. 23(4):468-72, 2002
                                                                          20. Hsu WC et al: Superficial siderosis of the CNS associated with multiple cavernous malformations. AJNR Am J Neuroradiol. 20(7):1245-8, 1999
                                                                          21. Weller M et al: Elevated CSF lactoferrin in superficial siderosis of the central nervous system. J Neurol. 246(10):943-5, 1999
                                                                          22. Lemmerling M et al: Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss. Neuroradiology. 40(5):312-4, 1998
                                                                          23. Castelli ML et al: Superficial siderosis of the central nervous system: an underestimated cause of hearing loss. J Laryngol Otol. 111(1):60-2, 1997
                                                                          24. Maurizi CP: Superficial siderosis of the brain: roles for cerebrospinal fluid circulation, iron and the hydroxyl radical. Med Hypotheses. 47(4):261-4, 1996
                                                                          25. Offenbacher H et al: Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology. 38 Suppl 1:S51-6, 1996
                                                                          26. Bracchi M et al: Superficial siderosis of the CNS: MR diagnosis and clinical findings. AJNR Am J Neuroradiol. 14(1):227-36, 1993