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Congenital External and Middle Ear Malformation
Caroline D. Robson, MBChB; H. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Congenital external & middle ear malformation (CEMEM)
            • Synonyms

              • Congenital aural atresia or dysplasia
            • Definitions

              • Anotia: Absent auricle
              • Microtia: Small, malformed auricle
              • External ear malformation [auricle & external auditory canal (EAC)]
                • EAC atresia: Absent EAC, tympanic plate (TP), & tympanic membrane (TM)
                • EAC stenosis: Narrow EAC, TP hypoplasia, TM present
                • EAC duplication: Duplication of part or all of EAC
              • Middle ear cavity (MEC) malformation: Aplasia or hypoplasia MEC + ossicular anomaly

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Reporting Tips

                                        Selected References

                                        1. Bartel-Friedrich S: Congenital auricular malformations: description of anomalies and syndromes. Facial Plast Surg. 31(6):567-80, 2015
                                        2. Jacob R et al: High-resolution CT findings in children with a normal pinna or grade I microtia and unilateral mild stenosis of the external auditory canal. AJNR Am J Neuroradiol. 36(1):176-80, 2015
                                        3. Zhao S et al: An imaging study of the facial nerve canal in congenital aural atresia. Ear Nose Throat J. 94(10-11):E6-13, 2015
                                        4. Casale G et al: Acquired ear canal cholesteatoma in congenital aural atresia/stenosis. Otol Neurotol. 35(8):1474-9, 2014
                                        5. Mukherjee S et al: The "boomerang" malleus-incus complex in congenital aural atresia. AJNR Am J Neuroradiol. 35(11):2181-5, 2014
                                        6. El-Begermy MA et al: Congenital auditory meatal atresia: a numerical review. Eur Arch Otorhinolaryngol. 266(4):501-6, 2009
                                        7. Kösling S et al: Congenital malformations of the external and middle ear. Eur J Radiol. 69(2):269-79, 2009
                                        8. Shonka DC Jr et al: The Jahrsdoerfer grading scale in surgery to repair congenital aural atresia. Arch Otolaryngol Head Neck Surg. 134(8):873-7, 2008
                                        9. Yu Z et al: Facial nerve course in congenital aural atresia--identified by preoperative CT scanning and surgical findings. Acta Otolaryngol. 128(12):1375-80, 2008
                                        10. Chang SO et al: Long term results of postoperative canal stenosis in congenital aural atresia surgery. Acta Otolaryngol Suppl. (558):15-21, 2007
                                        11. Yamane H et al: Disregard of cholesteatoma in congenital aural stenosis. Acta Otolaryngol. 127(2):221-4, 2007
                                        12. Blevins NH et al: External auditory canal duplication anomalies associated with congenital aural atresia. J Laryngol Otol. 117(1):32-8, 2003
                                        13. Klingebiel R et al: Multislice computed tomographic imaging in temporal bone dysplasia. Otol Neurotol. 23(5):715-22, 2002
                                        14. Calzolari F et al: Clinical and radiological evaluation in children with microtia. Br J Audiol. 33(5):303-12, 1999
                                        15. Declau F et al: Diagnosis and management strategies in congenital atresia of the external auditory canal. Study Group on Otological Malformations and Hearing Impairment. Br J Audiol. 33(5):313-27, 1999
                                        16. Selesnick S et al: Surgical treatment of acquired external auditory canal atresia. Am J Otol. 19(2):123-30, 1998
                                        17. Mayer TE et al: High-resolution CT of the temporal bone in dysplasia of the auricle and external auditory canal. AJNR Am J Neuroradiol. 18(1):53-65, 1997
                                        18. Chandrasekhar SS et al: Surgery of congenital aural atresia. Am J Otol. 16(6):713-7, 1995
                                        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 external & middle ear malformation (CEMEM)
                                                  • Synonyms

                                                    • Congenital aural atresia or dysplasia
                                                  • Definitions

                                                    • Anotia: Absent auricle
                                                    • Microtia: Small, malformed auricle
                                                    • External ear malformation [auricle & external auditory canal (EAC)]
                                                      • EAC atresia: Absent EAC, tympanic plate (TP), & tympanic membrane (TM)
                                                      • EAC stenosis: Narrow EAC, TP hypoplasia, TM present
                                                      • EAC duplication: Duplication of part or all of EAC
                                                    • Middle ear cavity (MEC) malformation: Aplasia or hypoplasia MEC + ossicular anomaly

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Staging, Grading, & Classification

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Bartel-Friedrich S: Congenital auricular malformations: description of anomalies and syndromes. Facial Plast Surg. 31(6):567-80, 2015
                                                                              2. Jacob R et al: High-resolution CT findings in children with a normal pinna or grade I microtia and unilateral mild stenosis of the external auditory canal. AJNR Am J Neuroradiol. 36(1):176-80, 2015
                                                                              3. Zhao S et al: An imaging study of the facial nerve canal in congenital aural atresia. Ear Nose Throat J. 94(10-11):E6-13, 2015
                                                                              4. Casale G et al: Acquired ear canal cholesteatoma in congenital aural atresia/stenosis. Otol Neurotol. 35(8):1474-9, 2014
                                                                              5. Mukherjee S et al: The "boomerang" malleus-incus complex in congenital aural atresia. AJNR Am J Neuroradiol. 35(11):2181-5, 2014
                                                                              6. El-Begermy MA et al: Congenital auditory meatal atresia: a numerical review. Eur Arch Otorhinolaryngol. 266(4):501-6, 2009
                                                                              7. Kösling S et al: Congenital malformations of the external and middle ear. Eur J Radiol. 69(2):269-79, 2009
                                                                              8. Shonka DC Jr et al: The Jahrsdoerfer grading scale in surgery to repair congenital aural atresia. Arch Otolaryngol Head Neck Surg. 134(8):873-7, 2008
                                                                              9. Yu Z et al: Facial nerve course in congenital aural atresia--identified by preoperative CT scanning and surgical findings. Acta Otolaryngol. 128(12):1375-80, 2008
                                                                              10. Chang SO et al: Long term results of postoperative canal stenosis in congenital aural atresia surgery. Acta Otolaryngol Suppl. (558):15-21, 2007
                                                                              11. Yamane H et al: Disregard of cholesteatoma in congenital aural stenosis. Acta Otolaryngol. 127(2):221-4, 2007
                                                                              12. Blevins NH et al: External auditory canal duplication anomalies associated with congenital aural atresia. J Laryngol Otol. 117(1):32-8, 2003
                                                                              13. Klingebiel R et al: Multislice computed tomographic imaging in temporal bone dysplasia. Otol Neurotol. 23(5):715-22, 2002
                                                                              14. Calzolari F et al: Clinical and radiological evaluation in children with microtia. Br J Audiol. 33(5):303-12, 1999
                                                                              15. Declau F et al: Diagnosis and management strategies in congenital atresia of the external auditory canal. Study Group on Otological Malformations and Hearing Impairment. Br J Audiol. 33(5):313-27, 1999
                                                                              16. Selesnick S et al: Surgical treatment of acquired external auditory canal atresia. Am J Otol. 19(2):123-30, 1998
                                                                              17. Mayer TE et al: High-resolution CT of the temporal bone in dysplasia of the auricle and external auditory canal. AJNR Am J Neuroradiol. 18(1):53-65, 1997
                                                                              18. Chandrasekhar SS et al: Surgery of congenital aural atresia. Am J Otol. 16(6):713-7, 1995