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Labyrinthine Aplasia
Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Complete labyrinthine aplasia (CLA)
                • Old synonym: Michel anomaly
              • Definitions

                • Absent cochlea, vestibule, & semicircular canals (SCCs)

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          • Reporting Tips

                                            Selected References

                                            1. Palomeque Vera JM et al: Effectiveness of cochlear implant in inner ear bone malformations with anterior labyrinth involvement. Int J Pediatr Otorhinolaryngol. 79(3):369-73, 2015
                                            2. Sennaroglu L: Histopathology of inner ear malformations: Do we have enough evidence to explain pathophysiology? Cochlear Implants Int. ePub, 2015
                                            3. Marcus S et al: Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss. Int J Pediatr Otorhinolaryngol. 78(2):268-71, 2014
                                            4. Young JY et al: Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation. Radiographics. 34(5):E133-49, 2014
                                            5. Clemmens CS et al: Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg. 149(2):318-25, 2013
                                            6. Higley MJ et al: Bilateral complete labyrinthine aplasia with bilateral internal carotid artery aplasia, developmental delay, and gaze abnormalities: a presumptive case of a rare HOXA1 mutation syndrome. AJNR Am J Neuroradiol. 32(2):E23-5, 2011
                                            7. Ozgen B et al: Complete labyrinthine aplasia: clinical and radiologic findings with review of the literature. AJNR Am J Neuroradiol. 30(4):774-80, 2009
                                            8. Bosley TM et al: The clinical spectrum of homozygous HOXA1 mutations. Am J Med Genet A. 146A(10):1235-40, 2008
                                            9. Tekin M et al: Homozygous mutations in fibroblast growth factor 3 are associated with a new form of syndromic deafness characterized by inner ear agenesis, microtia, and microdontia. Am J Hum Genet. 80(2):338-44, 2007
                                            10. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                            11. Hersh JH et al: Michel's anomaly, type I microtia and microdontia. Ear Nose Throat J. 70(3):155-7, 1991
                                            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

                                                          • Complete labyrinthine aplasia (CLA)
                                                          • Old synonym: Michel anomaly
                                                        • Definitions

                                                          • Absent cochlea, vestibule, & semicircular canals (SCCs)

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  • Image Interpretation Pearls

                                                                                    • Reporting Tips

                                                                                      Selected References

                                                                                      1. Palomeque Vera JM et al: Effectiveness of cochlear implant in inner ear bone malformations with anterior labyrinth involvement. Int J Pediatr Otorhinolaryngol. 79(3):369-73, 2015
                                                                                      2. Sennaroglu L: Histopathology of inner ear malformations: Do we have enough evidence to explain pathophysiology? Cochlear Implants Int. ePub, 2015
                                                                                      3. Marcus S et al: Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss. Int J Pediatr Otorhinolaryngol. 78(2):268-71, 2014
                                                                                      4. Young JY et al: Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation. Radiographics. 34(5):E133-49, 2014
                                                                                      5. Clemmens CS et al: Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg. 149(2):318-25, 2013
                                                                                      6. Higley MJ et al: Bilateral complete labyrinthine aplasia with bilateral internal carotid artery aplasia, developmental delay, and gaze abnormalities: a presumptive case of a rare HOXA1 mutation syndrome. AJNR Am J Neuroradiol. 32(2):E23-5, 2011
                                                                                      7. Ozgen B et al: Complete labyrinthine aplasia: clinical and radiologic findings with review of the literature. AJNR Am J Neuroradiol. 30(4):774-80, 2009
                                                                                      8. Bosley TM et al: The clinical spectrum of homozygous HOXA1 mutations. Am J Med Genet A. 146A(10):1235-40, 2008
                                                                                      9. Tekin M et al: Homozygous mutations in fibroblast growth factor 3 are associated with a new form of syndromic deafness characterized by inner ear agenesis, microtia, and microdontia. Am J Hum Genet. 80(2):338-44, 2007
                                                                                      10. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                                                                      11. Hersh JH et al: Michel's anomaly, type I microtia and microdontia. Ear Nose Throat J. 70(3):155-7, 1991