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Cystic Cochleovestibular Malformation (IP-I)
Bernadette L. Koch, MD; Surjith Vattoth, MD, FRCR; Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • 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. O'Brien WT , Sr et al: Nonsyndromic congenital causes of sensorineural hearing loss in children: an illustrative review. AJR Am J Roentgenol. 1-8, 2021
                                        2. D'Arco F et al: The link between inner ear malformations and the rest of the body: what we know so far about genetic, imaging and histology. Neuroradiology. 62(5):539-44, 2020
                                        3. Ohta S et al: Hearing crosstalk: the molecular conversation orchestrating inner ear dorsoventral patterning. Wiley Interdiscip Rev Dev Biol. 7(1), 2018
                                        4. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                        5. Banumathy N et al: Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly. J Otol. 11(1):38-41, 2016
                                        6. 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
                                        7. Sennaroglu L et al: Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 27(5):615-23, 2006
                                        8. Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
                                        9. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                        10. 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

                                              • 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. O'Brien WT , Sr et al: Nonsyndromic congenital causes of sensorineural hearing loss in children: an illustrative review. AJR Am J Roentgenol. 1-8, 2021
                                                                              2. D'Arco F et al: The link between inner ear malformations and the rest of the body: what we know so far about genetic, imaging and histology. Neuroradiology. 62(5):539-44, 2020
                                                                              3. Ohta S et al: Hearing crosstalk: the molecular conversation orchestrating inner ear dorsoventral patterning. Wiley Interdiscip Rev Dev Biol. 7(1), 2018
                                                                              4. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                                                              5. Banumathy N et al: Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly. J Otol. 11(1):38-41, 2016
                                                                              6. 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
                                                                              7. Sennaroglu L et al: Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 27(5):615-23, 2006
                                                                              8. Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
                                                                              9. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                                                              10. 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