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Cochlear Incomplete Partition Type II (IP-II)
Bernadette L. Koch, MD; Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Cochlear incomplete partition type II (IP-II)
            • Synonyms

              • Mondini anomaly (historic terminology): IP-II + large vestibular aqueduct (LVA)
            • Definitions

              • IP-II: Incomplete partition due to deficient interscalar septum (ISS) between middle & apical cochlear turns

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        CLINICAL ISSUES

                        • Presentation

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Image Interpretation Pearls

                                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. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                4. Leung KJ et al: Correlation of CT, MR, and histopathology in incomplete partition-II cochlear anomaly. Otol Neurotol. 37(5):434-7, 2016
                                5. Emmrich JV et al: Dilated dysplastic vestibule: a new computed tomographic finding in patients with large vestibular aqueduct syndrome. J Comput Assist Tomogr. 35(6):674-8, 2011
                                6. Griffith AJ et al: Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models. Hear Res. 281(1-2):11-7, 2011
                                7. King KA et al: SLC26A4 genotype, but not cochlear radiologic structure, is correlated with hearing loss in ears with an enlarged vestibular aqueduct. Laryngoscope. 120(2):384-9, 2010
                                8. Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
                                9. Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
                                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

                                          TERMINOLOGY

                                          • Abbreviations

                                            • Cochlear incomplete partition type II (IP-II)
                                          • Synonyms

                                            • Mondini anomaly (historic terminology): IP-II + large vestibular aqueduct (LVA)
                                          • Definitions

                                            • IP-II: Incomplete partition due to deficient interscalar septum (ISS) between middle & apical cochlear turns

                                          IMAGING

                                          • General Features

                                            • CT Findings

                                              • MR Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            DIAGNOSTIC CHECKLIST

                                                            • Image Interpretation Pearls

                                                              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. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                                              4. Leung KJ et al: Correlation of CT, MR, and histopathology in incomplete partition-II cochlear anomaly. Otol Neurotol. 37(5):434-7, 2016
                                                              5. Emmrich JV et al: Dilated dysplastic vestibule: a new computed tomographic finding in patients with large vestibular aqueduct syndrome. J Comput Assist Tomogr. 35(6):674-8, 2011
                                                              6. Griffith AJ et al: Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models. Hear Res. 281(1-2):11-7, 2011
                                                              7. King KA et al: SLC26A4 genotype, but not cochlear radiologic structure, is correlated with hearing loss in ears with an enlarged vestibular aqueduct. Laryngoscope. 120(2):384-9, 2010
                                                              8. Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
                                                              9. Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004