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Head and Neck
Diagnosis
Temporal Bone
Inner Ear
Congenital Lesions
Semicircular Canal Hypoplasia-Aplasia
Semicircular Canal Hypoplasia-Aplasia
Surjith Vattoth, MD, FRCR; William T. O'Brien, Sr., DO, FAOCR; Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Semicircular canal (SCC)-vestibule malformation

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            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. D'Arco F et al: Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide. Eur J Radiol. 123:108803, 2020
                                3. Verheij E et al: Anatomic malformations of the middle and inner ear in 22q11.2 deletion syndrome: case series and literature review. AJNR Am J Neuroradiol. 39(5):928-34, 2018
                                4. Green GE et al: CHD7 mutations and CHARGE syndrome in semicircular canal dysplasia. Otol Neurotol. 35(8):1466-70, 2014
                                5. Saliba I et al: Down syndrome: an electrophysiological and radiological profile. Laryngoscope. 124(4):E141-7, 2014
                                6. Breheret R et al: Bilateral semicircular canal aplasia. Eur Ann Otorhinolaryngol Head Neck Dis. 130(4):225-8, 2013
                                7. Elmaleh-Bergès M et al: Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations. AJNR Am J Neuroradiol. 34(6):1257-63, 2013
                                8. Intrapiromkul J et al: Inner ear anomalies seen on CT images in people with Down syndrome. Pediatr Radiol. 42(12):1449-55, 2012
                                9. Joshi VM et al: CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss. Radiographics. 32(3):683-98, 2012
                                10. Blaser S et al: Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope. 116(12):2113-9, 2006
                                11. Jongmans MC et al: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet. 43(4):306-14, 2006
                                12. Koch B et al: Partial absence of the posterior semicircular canal in Alagille syndrome: CT findings. Pediatr Radiol. 36(9):977-9, 2006
                                13. Morimoto AK et al: Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. AJNR Am J Neuroradiol. 27(8):1663-71, 2006
                                14. Purcell D et al: Establishment of normative cochlear and vestibular measurements to aid in the diagnosis of inner ear malformations. Otolaryngol Head Neck Surg. 128(1):78-87, 2003
                                15. Satar B et al: Congenital aplasia of the semicircular canals. Otol Neurotol. 24(3):437-46, 2003
                                16. Yu KK et al: Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases. Otolaryngol Head Neck Surg. 129(6):637-46, 2003
                                17. Lemmerling MM et al: Unilateral semicircular canal aplasia in Goldenhar's syndrome. AJNR Am J Neuroradiol. 21(7):1334-6, 2000
                                18. Mizuno M et al: Labyrinthine anomalies with normal cochlear function. ORL J Otorhinolaryngol Relat Spec. 54(5):278-81, 1992
                                19. Parnes LS et al: Bilateral semicircular canal aplasia with near-normal cochlear development. Two case reports. Ann Otol Rhinol Laryngol. 99(12):957-9, 1990
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Top Differential Diagnoses

                                      • Diagnostic Checklist

                                        TERMINOLOGY

                                        • Synonyms

                                          • Semicircular canal (SCC)-vestibule malformation

                                        IMAGING

                                        • General Features

                                          • CT Findings

                                            • MR Findings

                                              • Imaging Recommendations

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    • Gross Pathologic & Surgical Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          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. D'Arco F et al: Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide. Eur J Radiol. 123:108803, 2020
                                                              3. Verheij E et al: Anatomic malformations of the middle and inner ear in 22q11.2 deletion syndrome: case series and literature review. AJNR Am J Neuroradiol. 39(5):928-34, 2018
                                                              4. Green GE et al: CHD7 mutations and CHARGE syndrome in semicircular canal dysplasia. Otol Neurotol. 35(8):1466-70, 2014
                                                              5. Saliba I et al: Down syndrome: an electrophysiological and radiological profile. Laryngoscope. 124(4):E141-7, 2014
                                                              6. Breheret R et al: Bilateral semicircular canal aplasia. Eur Ann Otorhinolaryngol Head Neck Dis. 130(4):225-8, 2013
                                                              7. Elmaleh-Bergès M et al: Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations. AJNR Am J Neuroradiol. 34(6):1257-63, 2013
                                                              8. Intrapiromkul J et al: Inner ear anomalies seen on CT images in people with Down syndrome. Pediatr Radiol. 42(12):1449-55, 2012
                                                              9. Joshi VM et al: CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss. Radiographics. 32(3):683-98, 2012
                                                              10. Blaser S et al: Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope. 116(12):2113-9, 2006
                                                              11. Jongmans MC et al: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet. 43(4):306-14, 2006
                                                              12. Koch B et al: Partial absence of the posterior semicircular canal in Alagille syndrome: CT findings. Pediatr Radiol. 36(9):977-9, 2006
                                                              13. Morimoto AK et al: Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. AJNR Am J Neuroradiol. 27(8):1663-71, 2006
                                                              14. Purcell D et al: Establishment of normative cochlear and vestibular measurements to aid in the diagnosis of inner ear malformations. Otolaryngol Head Neck Surg. 128(1):78-87, 2003
                                                              15. Satar B et al: Congenital aplasia of the semicircular canals. Otol Neurotol. 24(3):437-46, 2003
                                                              16. Yu KK et al: Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases. Otolaryngol Head Neck Surg. 129(6):637-46, 2003
                                                              17. Lemmerling MM et al: Unilateral semicircular canal aplasia in Goldenhar's syndrome. AJNR Am J Neuroradiol. 21(7):1334-6, 2000
                                                              18. Mizuno M et al: Labyrinthine anomalies with normal cochlear function. ORL J Otorhinolaryngol Relat Spec. 54(5):278-81, 1992
                                                              19. Parnes LS et al: Bilateral semicircular canal aplasia with near-normal cochlear development. Two case reports. Ann Otol Rhinol Laryngol. 99(12):957-9, 1990