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Congenital Cholesteatoma
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Congenital cholesteatoma (CCh)
            • Synonyms

              • Primary cholesteatoma, epidermoid
            • Definitions

              • Benign mass secondary to epithelial rests of embryonal origin
              • Most commonly occurs in middle ear (ME), behind intact tympanic membrane (TM) in patient without history of surgery, chronic 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. Mierzwinski J et al: Cochlear implant and congenital cholesteatoma. J Otolaryngol Head Neck Surg. 45:8, 2016
                                          2. van Egmond SL et al: A systematic review of non-echo planar diffusion-weighted magnetic resonance imaging for detection of primary and postoperative cholesteatoma. Otolaryngol Head Neck Surg. 154(2):233-40, 2015
                                          3. Chung J et al: Congenital cholesteatoma and cochlear implantation: Implications for management. Cochlear Implants Int. 14(1):32-5, 2013
                                          4. Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
                                          5. Choi HG et al: Clinical experience of 71 cases of congenital middle ear cholesteatoma. Acta Otolaryngol. 130(1):62-7, 2010
                                          6. De Foer B et al: Diffusion-weighted magnetic resonance imaging of the temporal bone. Neuroradiology. 52(9):785-807, 2010
                                          7. De Foer B et al: Middle ear cholesteatoma: non-echo-planar diffusion-weighted MR imaging versus delayed gadolinium-enhanced T1-weighted MR imaging--value in detection. Radiology. 255(3):866-72, 2010
                                          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. 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
                                          11. Darrouzet V et al: Congenital middle ear cholesteatomas in children: our experience in 34 cases. Otolaryngol Head Neck Surg. 126(1):34-40, 2002
                                          12. El-Bitar MA et al: Bilateral occurrence of congenital middle ear cholesteatoma. Otolaryngol Head Neck Surg. 127(5):480-2, 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. Yeo SW et al: The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma. Am J Otolaryngol. 22(3):184-9, 2001
                                          18. Tos M: A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope. 110(11):1890-7, 2000
                                          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 cholesteatoma (CCh)
                                                    • Synonyms

                                                      • Primary cholesteatoma, epidermoid
                                                    • Definitions

                                                      • Benign mass secondary to epithelial rests of embryonal origin
                                                      • Most commonly occurs in middle ear (ME), behind intact tympanic membrane (TM) in patient without history of surgery, chronic 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. Mierzwinski J et al: Cochlear implant and congenital cholesteatoma. J Otolaryngol Head Neck Surg. 45:8, 2016
                                                                                  2. van Egmond SL et al: A systematic review of non-echo planar diffusion-weighted magnetic resonance imaging for detection of primary and postoperative cholesteatoma. Otolaryngol Head Neck Surg. 154(2):233-40, 2015
                                                                                  3. Chung J et al: Congenital cholesteatoma and cochlear implantation: Implications for management. Cochlear Implants Int. 14(1):32-5, 2013
                                                                                  4. Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
                                                                                  5. Choi HG et al: Clinical experience of 71 cases of congenital middle ear cholesteatoma. Acta Otolaryngol. 130(1):62-7, 2010
                                                                                  6. De Foer B et al: Diffusion-weighted magnetic resonance imaging of the temporal bone. Neuroradiology. 52(9):785-807, 2010
                                                                                  7. De Foer B et al: Middle ear cholesteatoma: non-echo-planar diffusion-weighted MR imaging versus delayed gadolinium-enhanced T1-weighted MR imaging--value in detection. Radiology. 255(3):866-72, 2010
                                                                                  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. 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
                                                                                  11. Darrouzet V et al: Congenital middle ear cholesteatomas in children: our experience in 34 cases. Otolaryngol Head Neck Surg. 126(1):34-40, 2002
                                                                                  12. El-Bitar MA et al: Bilateral occurrence of congenital middle ear cholesteatoma. Otolaryngol Head Neck Surg. 127(5):480-2, 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. Yeo SW et al: The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma. Am J Otolaryngol. 22(3):184-9, 2001
                                                                                  18. Tos M: A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope. 110(11):1890-7, 2000