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Labyrinthine Aplasia
Bernadette L. Koch, MDSurjith Vattoth, MD, FRCRLuke L. Linscott, MD
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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, and 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. 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. Siu JM et al: Efficacy of a selective imaging paradigm prior to pediatric cochlear implantation. Laryngoscope. 129(11):2627-33, 2019
                                            4. Eisenberg LS et al: Early communication development of children with auditory brainstem implants. J Deaf Stud Deaf Educ. 23(3):249-60, 2018
                                            5. Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                            6. 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
                                            7. Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int.17(1):3-20, 2015
                                            8. 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
                                            9. Young JY et al: Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation. Radiographics. 34(5):E133-49, 2014
                                            10. Clemmens CS et al: Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg. 149(2):318-25, 2013
                                            11. 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
                                            12. 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
                                            13. Bosley TM et al: The clinical spectrum of homozygous HOXA1 mutations. Am J Med Genet A. 146A(10):1235-40, 2008
                                            14. 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
                                            15. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                            16. 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, and 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. 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. Siu JM et al: Efficacy of a selective imaging paradigm prior to pediatric cochlear implantation. Laryngoscope. 129(11):2627-33, 2019
                                                                                      4. Eisenberg LS et al: Early communication development of children with auditory brainstem implants. J Deaf Stud Deaf Educ. 23(3):249-60, 2018
                                                                                      5. Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                                                                      6. 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
                                                                                      7. Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int.17(1):3-20, 2015
                                                                                      8. 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
                                                                                      9. Young JY et al: Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation. Radiographics. 34(5):E133-49, 2014
                                                                                      10. Clemmens CS et al: Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg. 149(2):318-25, 2013
                                                                                      11. 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
                                                                                      12. 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
                                                                                      13. Bosley TM et al: The clinical spectrum of homozygous HOXA1 mutations. Am J Med Genet A. 146A(10):1235-40, 2008
                                                                                      14. 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
                                                                                      15. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                                                                      16. Hersh JH et al: Michel's anomaly, type I microtia and microdontia. Ear Nose Throat J. 70(3):155-7, 1991