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Congenital Middle Ear Cholesteatoma
H. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Congenital middle ear cholesteatoma (CMEC)
            • Synonyms

              • Primary cholesteatoma, epidermoid
            • Definitions

              • Cholesteatoma in middle ear (ME) behind intact TM in patient with no history of surgery, otitis media, or otorrhea

            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

                                          Selected References

                                          1. Bacciu A et al: Open vs closed type congenital cholesteatoma of the middle ear: two distinct entities or two aspects of the same phenomenon? Int J Pediatr Otorhinolaryngol. 78(12):2205-9, 2014
                                          2. Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
                                          3. Ganaha A et al: Efficacy of diffusion-weighted magnetic resonance imaging in the diagnosis of middle ear cholesteatoma. Auris Nasus Larynx. 38(3):329-34, 2011
                                          4. Park KH et al: Congenital middle ear cholesteatoma in children; retrospective review of 35 cases. J Korean Med Sci. 24(1):126-31, 2009
                                          5. De Foer B et al: The value of single-shot turbo spin-echo diffusion-weighted MR imaging in the detection of middle ear cholesteatoma. Neuroradiology. 49(10):841-8, 2007
                                          6. Isaacson G: Diagnosis of pediatric cholesteatoma. Pediatrics. 120(3):603-8, 2007
                                          7. Kutz JW Jr et al: Congenital middle ear cholesteatoma. Ear Nose Throat J. 86(11):654, 2007
                                          8. Lazard DS et al: Congenital cholesteatoma: risk factors for residual disease and retraction pockets--a report on 117 cases. Laryngoscope. 117(4):634-7, 2007
                                          9. Kojima H et al: Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol. 27(5):299-305, 2006
                                          10. Shirazi MA et al: Surgical treatment of pediatric cholesteatomas. Laryngoscope. 116(9):1603-7, 2006
                                          11. El-Bitar MA et al: Congenital middle ear cholesteatoma: need for early recognition--role of computed tomography scan. Int J Pediatr Otorhinolaryngol. 67(3):231-5, 2003
                                          12. Darrouzet V et al: Congenital middle ear cholesteatomas in children: our experience in 34 cases. Otolaryngol Head Neck Surg. 126(1):34-40, 2002
                                          13. Koltai PJ et al: The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(7):804-9, 2002
                                          14. Nelson M et al: Congenital cholesteatoma: classification, management, and outcome. Arch Otolaryngol Head Neck Surg. 128(7):810-4, 2002
                                          15. Potsic WP et al: A staging system for congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(9):1009-12, 2002
                                          16. Potsic WP et al: Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia. Otolaryngol Head Neck Surg. 126(4):409-14, 2002
                                          17. Yammine FG et al: Anterior and posterior middle ear congenital cholesteatomas in children. J Otolaryngol. 30(1):29-33, 2001
                                          18. Yeo SW et al: The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma. Am J Otolaryngol. 22(3):184-9, 2001
                                          19. Tos M: A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope. 110(11):1890-7, 2000
                                          20. De la Cruz A et al: Detection and management of childhood cholesteatoma. Pediatr Ann. 28(6):370-3, 1999
                                          21. Friedberg J: Congenital cholesteatoma. Laryngoscope. 104(3 Pt 2):1-24, 1994
                                          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

                                                      • Congenital middle ear cholesteatoma (CMEC)
                                                    • Synonyms

                                                      • Primary cholesteatoma, epidermoid
                                                    • Definitions

                                                      • Cholesteatoma in middle ear (ME) behind intact TM in patient with no history of surgery, otitis media, or otorrhea

                                                    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

                                                                                  Selected References

                                                                                  1. Bacciu A et al: Open vs closed type congenital cholesteatoma of the middle ear: two distinct entities or two aspects of the same phenomenon? Int J Pediatr Otorhinolaryngol. 78(12):2205-9, 2014
                                                                                  2. Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
                                                                                  3. Ganaha A et al: Efficacy of diffusion-weighted magnetic resonance imaging in the diagnosis of middle ear cholesteatoma. Auris Nasus Larynx. 38(3):329-34, 2011
                                                                                  4. Park KH et al: Congenital middle ear cholesteatoma in children; retrospective review of 35 cases. J Korean Med Sci. 24(1):126-31, 2009
                                                                                  5. De Foer B et al: The value of single-shot turbo spin-echo diffusion-weighted MR imaging in the detection of middle ear cholesteatoma. Neuroradiology. 49(10):841-8, 2007
                                                                                  6. Isaacson G: Diagnosis of pediatric cholesteatoma. Pediatrics. 120(3):603-8, 2007
                                                                                  7. Kutz JW Jr et al: Congenital middle ear cholesteatoma. Ear Nose Throat J. 86(11):654, 2007
                                                                                  8. Lazard DS et al: Congenital cholesteatoma: risk factors for residual disease and retraction pockets--a report on 117 cases. Laryngoscope. 117(4):634-7, 2007
                                                                                  9. Kojima H et al: Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol. 27(5):299-305, 2006
                                                                                  10. Shirazi MA et al: Surgical treatment of pediatric cholesteatomas. Laryngoscope. 116(9):1603-7, 2006
                                                                                  11. El-Bitar MA et al: Congenital middle ear cholesteatoma: need for early recognition--role of computed tomography scan. Int J Pediatr Otorhinolaryngol. 67(3):231-5, 2003
                                                                                  12. Darrouzet V et al: Congenital middle ear cholesteatomas in children: our experience in 34 cases. Otolaryngol Head Neck Surg. 126(1):34-40, 2002
                                                                                  13. Koltai PJ et al: The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(7):804-9, 2002
                                                                                  14. Nelson M et al: Congenital cholesteatoma: classification, management, and outcome. Arch Otolaryngol Head Neck Surg. 128(7):810-4, 2002
                                                                                  15. Potsic WP et al: A staging system for congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(9):1009-12, 2002
                                                                                  16. Potsic WP et al: Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia. Otolaryngol Head Neck Surg. 126(4):409-14, 2002
                                                                                  17. Yammine FG et al: Anterior and posterior middle ear congenital cholesteatomas in children. J Otolaryngol. 30(1):29-33, 2001
                                                                                  18. Yeo SW et al: The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma. Am J Otolaryngol. 22(3):184-9, 2001
                                                                                  19. Tos M: A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope. 110(11):1890-7, 2000
                                                                                  20. De la Cruz A et al: Detection and management of childhood cholesteatoma. Pediatr Ann. 28(6):370-3, 1999
                                                                                  21. Friedberg J: Congenital cholesteatoma. Laryngoscope. 104(3 Pt 2):1-24, 1994