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Cochlear Aplasia
Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Absent cochlea
              • Definitions

                • Absent cochlea; vestibule, semicircular canals (SCC), & internal auditory canal (IAC) present in some form

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. Sennaro─člu L et al: Long-term results of ABI in children with severe inner ear malformations. Otol Neurotol. ePub, 2016
                                    2. Kontorinis G et al: Aplasia of the cochlea: radiologic assessment and options for hearing rehabilitation. Otol Neurotol. 34(7):1253-60, 2013
                                    3. Jeong SW et al: Cochlear implantation in children with cochlear aplasia. Acta Otolaryngol. 132(9):910-5, 2012
                                    4. Sennaroglu L et al: Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve. Otol Neurotol. 30(6):708-15, 2009
                                    5. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                    6. Jackler RK et al: Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope. 97(3 Pt 2 Suppl 40):2-14, 1987
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Absent cochlea
                                                • Definitions

                                                  • Absent cochlea; vestibule, semicircular canals (SCC), & internal auditory canal (IAC) present in some form

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. Sennaro─člu L et al: Long-term results of ABI in children with severe inner ear malformations. Otol Neurotol. ePub, 2016
                                                                      2. Kontorinis G et al: Aplasia of the cochlea: radiologic assessment and options for hearing rehabilitation. Otol Neurotol. 34(7):1253-60, 2013
                                                                      3. Jeong SW et al: Cochlear implantation in children with cochlear aplasia. Acta Otolaryngol. 132(9):910-5, 2012
                                                                      4. Sennaroglu L et al: Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve. Otol Neurotol. 30(6):708-15, 2009
                                                                      5. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                                                      6. Jackler RK et al: Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope. 97(3 Pt 2 Suppl 40):2-14, 1987