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Congenital Auricle Malformations
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          TERMINOLOGY

          • Synonyms

            • Congenital aural dysplasia: Congenital external ear malformation
            • Outer ear: Auricle & external auditory canal (EAC)
            • Auricle: Pinna
          • Definitions

            • Microtia: Congenital malformation with underdeveloped & misshapen auricle
            • Anotia: Absent auricle

          IMAGING

          • General Features

            • CT Findings

              • Imaging Recommendations

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Staging, Grading, & Classification

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Treatment

                            DIAGNOSTIC CHECKLIST

                            • Image Interpretation Pearls

                              Selected References

                              1. Nada A et al: Cross-sectional imaging evaluation of congenital temporal bone anomalies: what each radiologist should know. Curr Probl Diagn Radiol. 50(5):716-24, 2021
                              2. Zhang TY et al: International Consensus Recommendations on Microtia, Aural Atresia and Functional Ear Reconstruction. J Int Adv Otol. 15(2):204-208, 2019
                              3. Bartel-Friedrich S: Congenital auricular malformations: description of anomalies and syndromes. Facial Plast Surg. 31(6):567-80, 2015
                              4. Cabin JA et al: Microtia reconstruction: autologous rib and alloplast techniques. Facial Plast Surg Clin North Am. 22(4):623-38, 2014
                              5. Casale G et al: Acquired ear canal cholesteatoma in congenital aural atresia/stenosis. Otol Neurotol. 35(8):1474-9, 2014
                              6. Nadaraja GS et al: Hearing outcomes of atresia surgery versus osseointegrated bone conduction device in patients with congenital aural atresia: a systematic review. Otol Neurotol. 34(8):1394-9, 2013
                              7. Som PH et al: Head & Neck Imaging. 5th ed. St. Louis: Mosby, 2011
                              8. 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
                              9. Weerda H: Classification of congenital deformities of the auricle. Facial Plast Surg. 5(5):385-8, 1988
                              Related Anatomy
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Imaging

                                  • Top Differential Diagnoses

                                    • Pathology

                                      TERMINOLOGY

                                      • Synonyms

                                        • Congenital aural dysplasia: Congenital external ear malformation
                                        • Outer ear: Auricle & external auditory canal (EAC)
                                        • Auricle: Pinna
                                      • Definitions

                                        • Microtia: Congenital malformation with underdeveloped & misshapen auricle
                                        • Anotia: Absent auricle

                                      IMAGING

                                      • General Features

                                        • CT Findings

                                          • Imaging Recommendations

                                            DIFFERENTIAL DIAGNOSIS

                                              PATHOLOGY

                                              • General Features

                                                • Staging, Grading, & Classification

                                                  CLINICAL ISSUES

                                                  • Presentation

                                                    • Demographics

                                                      • Treatment

                                                        DIAGNOSTIC CHECKLIST

                                                        • Image Interpretation Pearls

                                                          Selected References

                                                          1. Nada A et al: Cross-sectional imaging evaluation of congenital temporal bone anomalies: what each radiologist should know. Curr Probl Diagn Radiol. 50(5):716-24, 2021
                                                          2. Zhang TY et al: International Consensus Recommendations on Microtia, Aural Atresia and Functional Ear Reconstruction. J Int Adv Otol. 15(2):204-208, 2019
                                                          3. Bartel-Friedrich S: Congenital auricular malformations: description of anomalies and syndromes. Facial Plast Surg. 31(6):567-80, 2015
                                                          4. Cabin JA et al: Microtia reconstruction: autologous rib and alloplast techniques. Facial Plast Surg Clin North Am. 22(4):623-38, 2014
                                                          5. Casale G et al: Acquired ear canal cholesteatoma in congenital aural atresia/stenosis. Otol Neurotol. 35(8):1474-9, 2014
                                                          6. Nadaraja GS et al: Hearing outcomes of atresia surgery versus osseointegrated bone conduction device in patients with congenital aural atresia: a systematic review. Otol Neurotol. 34(8):1394-9, 2013
                                                          7. Som PH et al: Head & Neck Imaging. 5th ed. St. Louis: Mosby, 2011
                                                          8. 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
                                                          9. Weerda H: Classification of congenital deformities of the auricle. Facial Plast Surg. 5(5):385-8, 1988