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Head and Neck
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Temporal Bone
Inner Ear
Congenital Lesions
Semicircular Canal-Vestibule Globular Anomaly
Semicircular Canal-Vestibule Globular Anomaly
Bernadette L. Koch, MD; Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Persistent semicircular canal (SCC) anlage anomaly

            • Definitions

              • Dilatation of SCC and globular vestibule
              • Small or absent bone island between vestibule and affected SCC

            IMAGING

            • General Features

              • CT Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      CLINICAL ISSUES

                      • Presentation

                        DIAGNOSTIC CHECKLIST

                        • Consider

                          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. Tahir E et al: Inner-ear malformations as a cause of single-sided deafness. J Laryngol Otol. 134(6):509-18, 2020
                          4. Intrapiromkul J et al: Inner ear anomalies seen on CT images in people with Down syndrome. Pediatr Radiol. 42(12):1449-55, 2012
                          5. Lan MY et al: Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing loss. Eur Arch Otorhinolaryngol. 266(9):1361-4, 2009
                          6. Zhou G et al: Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect. Otol Neurotol. 30(2):184-9, 2009
                          7. Dallan I et al: Bilateral, isolated, lateral semicircular canal malformation without hearing loss. J Laryngol Otol. 122(8):858-60, 2008
                          8. Blaser S et al: Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope. 116(12):2113-9, 2006
                          9. Quintero-Rivera F et al: Intracranial anomalies detected by imaging studies in 30 patients with Apert syndrome. Am J Med Genet A. 140(12):1337-8, 2006
                          10. Matsunaga T et al: Familial lateral semicircular canal malformation with external and middle ear abnormalities. Am J Med Genet A. 116A(4):360-7, 2003
                          11. 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
                          12. Johnson J et al: Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope. 110(10 Pt 1):1673-9, 2000
                          Related Anatomy
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                          Related Differential Diagnoses
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                          References
                          Tables

                          Tables

                          KEY FACTS

                          • Terminology

                            • Imaging

                              • Top Differential Diagnoses

                                • Clinical Issues

                                  • Diagnostic Checklist

                                    TERMINOLOGY

                                    • Synonyms

                                      • Persistent semicircular canal (SCC) anlage anomaly

                                    • Definitions

                                      • Dilatation of SCC and globular vestibule
                                      • Small or absent bone island between vestibule and affected SCC

                                    IMAGING

                                    • General Features

                                      • CT Findings

                                        • Imaging Recommendations

                                          DIFFERENTIAL DIAGNOSIS

                                            PATHOLOGY

                                            • General Features

                                              CLINICAL ISSUES

                                              • Presentation

                                                DIAGNOSTIC CHECKLIST

                                                • Consider

                                                  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. Tahir E et al: Inner-ear malformations as a cause of single-sided deafness. J Laryngol Otol. 134(6):509-18, 2020
                                                  4. Intrapiromkul J et al: Inner ear anomalies seen on CT images in people with Down syndrome. Pediatr Radiol. 42(12):1449-55, 2012
                                                  5. Lan MY et al: Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing loss. Eur Arch Otorhinolaryngol. 266(9):1361-4, 2009
                                                  6. Zhou G et al: Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect. Otol Neurotol. 30(2):184-9, 2009
                                                  7. Dallan I et al: Bilateral, isolated, lateral semicircular canal malformation without hearing loss. J Laryngol Otol. 122(8):858-60, 2008
                                                  8. Blaser S et al: Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope. 116(12):2113-9, 2006
                                                  9. Quintero-Rivera F et al: Intracranial anomalies detected by imaging studies in 30 patients with Apert syndrome. Am J Med Genet A. 140(12):1337-8, 2006
                                                  10. Matsunaga T et al: Familial lateral semicircular canal malformation with external and middle ear abnormalities. Am J Med Genet A. 116A(4):360-7, 2003
                                                  11. 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
                                                  12. Johnson J et al: Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope. 110(10 Pt 1):1673-9, 2000