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Cystic Cochleovestibular Malformation
Surjith Vattoth, MD, FRCR; Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Cystic cochleovestibular malformation (CCVM)
              • Synonyms

                • Cystic cochleovestibular anomaly
                • Cochlear incomplete partition type I (IP-I)
              • Definitions

                • Cochlea lacks interscalar septum & modiolus (IP-I) + dilated vestibule & lateral (horizontal) semicircular canal (SCC)

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        • Reporting Tips

                                          Selected References

                                          1. Ohta S et al: Hearing crosstalk: the molecular conversation orchestrating inner ear dorsoventral patterning. Wiley Interdiscip Rev Dev Biol. 7(1), 2018
                                          2. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                          3. Banumathy N et al: Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly. J Otol. 11(1):38-41, 2016
                                          4. Wani NA et al: Recurrent meningitis in an adult secondary to an inner ear malformation: imaging demonstration. Ear Nose Throat J. 91(4):E23-6, 2012
                                          5. Sennaroglu L et al: Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 27(5):615-23, 2006
                                          6. Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
                                          7. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                          8. 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

                                                      • Abbreviations

                                                        • Cystic cochleovestibular malformation (CCVM)
                                                      • Synonyms

                                                        • Cystic cochleovestibular anomaly
                                                        • Cochlear incomplete partition type I (IP-I)
                                                      • Definitions

                                                        • Cochlea lacks interscalar septum & modiolus (IP-I) + dilated vestibule & lateral (horizontal) semicircular canal (SCC)

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              • Image Interpretation Pearls

                                                                                • Reporting Tips

                                                                                  Selected References

                                                                                  1. Ohta S et al: Hearing crosstalk: the molecular conversation orchestrating inner ear dorsoventral patterning. Wiley Interdiscip Rev Dev Biol. 7(1), 2018
                                                                                  2. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                                                                  3. Banumathy N et al: Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly. J Otol. 11(1):38-41, 2016
                                                                                  4. Wani NA et al: Recurrent meningitis in an adult secondary to an inner ear malformation: imaging demonstration. Ear Nose Throat J. 91(4):E23-6, 2012
                                                                                  5. Sennaroglu L et al: Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 27(5):615-23, 2006
                                                                                  6. Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
                                                                                  7. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                                                                  8. 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