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Cochlear Hypoplasia
Bernadette L. Koch, MD; Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Cochlear hypoplasia (CH)
              • Definitions

                • Cochlear underdevelopment: External dimensions < normal cochlea with internal architecture deformities
                • Term "cochlea with 1.5 turns" used to define CH (especially CH type III; cochlea with < 2 turns)
                  • Difficult to count number of turns on CT/MR in CH
                  • Avoid this term for cochlear incomplete partition type II (IP-II)

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • 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. Pamuk G et al: Radiological measurement of cochlear dimensions in cochlear hypoplasia and its effect on cochlear implant selection. J Laryngol Otol. 1-7, 2021
                                        3. 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
                                        4. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                        5. Collier TJ et al: Is alpha-synuclein loss-of-function a contributor to Parkinsonian Pathology? Evidence from Non-human Primates. Front Neurosci. 10:12, 2016
                                        6. Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-2, 2016
                                        7. Sennaroglu L: Cochlear implantation in inner ear malformations--a review article. Cochlear Implants Int. 11(1):4-41, 2010
                                        8. Teissier N et al: Computed tomography measurements of the normal and the pathologic cochlea in children. Pediatr Radiol. 40(3):275-83, 2010
                                        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

                                                      • Cochlear hypoplasia (CH)
                                                    • Definitions

                                                      • Cochlear underdevelopment: External dimensions < normal cochlea with internal architecture deformities
                                                      • Term "cochlea with 1.5 turns" used to define CH (especially CH type III; cochlea with < 2 turns)
                                                        • Difficult to count number of turns on CT/MR in CH
                                                        • Avoid this term for cochlear incomplete partition type II (IP-II)

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Staging, Grading, & Classification

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • 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. Pamuk G et al: Radiological measurement of cochlear dimensions in cochlear hypoplasia and its effect on cochlear implant selection. J Laryngol Otol. 1-7, 2021
                                                                              3. 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
                                                                              4. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                                                              5. Collier TJ et al: Is alpha-synuclein loss-of-function a contributor to Parkinsonian Pathology? Evidence from Non-human Primates. Front Neurosci. 10:12, 2016
                                                                              6. Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-2, 2016
                                                                              7. Sennaroglu L: Cochlear implantation in inner ear malformations--a review article. Cochlear Implants Int. 11(1):4-41, 2010
                                                                              8. Teissier N et al: Computed tomography measurements of the normal and the pathologic cochlea in children. Pediatr Radiol. 40(3):275-83, 2010