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Pediatrics
Diagnosis
Pediatric Neuroradiology
Head and Neck
Temporal Bone and Skull Base
Large Vestibular Aqueduct
Large Vestibular Aqueduct
Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Large vestibular aqueduct (LVA)

              • Synonyms

                • Large endolymphatic sac anomaly (LESA): T2 MR term
                • Enlarged vestibular aqueduct (EVA): CT term
                • Mondini deformity (historic terminology): LVA + minimally dilated vestibule + incomplete partition type II (IP-II)

              • Definitions

                • LVA: Enlarged bony vestibular aqueduct (VA) houses large endolymphatic sac (ES) & duct
                  • Associated with variable cochlear malformation
                •  IP-II: Deficient modiolus & interscalar septum (ISS) between middle & apical cochlear turns; often seen with LVA
                • IP-II: Normal cochlear external dimensions
                  • Not correct to call IP-II as cochlea with 1.5 turns
                  • Term cochlea with 1.5 turns should be used only for cochlear hypoplasia (CH) (especially CH-III)

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        • Reporting Tips

                                          Selected References

                                          1. Booth TN et al: Evaluation of the normal cochlear second interscalar ridge angle and depth on 3D T2-weighted images: a tool for the diagnosis of scala communis and incomplete partition type II. AJNR Am J Neuroradiol. 39(5):923-7, 2018
                                          2. Reinshagen KL et al: Measurement for detection of incomplete partition type II anomalies on MR imaging. AJNR Am J Neuroradiol. 38(10):2003-7, 2017
                                          3. Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                          4. El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
                                          5. Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-20, 2016
                                          6. Ito T et al: Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4-insufficient mouse model of large vestibular aqueduct syndrome. Neuroscience. 310:188-97, 2015
                                          7. Sone M et al: Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes. Laryngoscope. 126(6):1446-50, 2015
                                          8. Chattaraj P et al: Use of SLC26A4 mutation testing for unilateral enlargement of the vestibular aqueduct. JAMA Otolaryngol Head Neck Surg. 139(9):907-13, 2013
                                          9. Emmrich JV et al: Dilated dysplastic vestibule: a new computed tomographic finding in patients with large vestibular aqueduct syndrome. J Comput Assist Tomogr. 35(6):674-8, 2011
                                          10. Griffith AJ et al: Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models. Hear Res. 281(1-2):11-7, 2011
                                          11. Atkin JS et al: Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly. Int J Pediatr Otorhinolaryngol. 73(12):1682-5, 2009
                                          12. Madeo AC et al: Evaluation of the thyroid in patients with hearing loss and enlarged vestibular aqueducts. Arch Otolaryngol Head Neck Surg. 135(7):670-6, 2009
                                          13. Oh SH et al: Can magnetic resonance imaging provide clues to the inner ear functional status of enlarged vestibular aqueduct subjects with PDS mutation? Otol Neurotol. 29(5):593-600, 2008
                                          14. Ozgen B et al: Comparison of 45 degrees oblique reformats with axial reformats in CT evaluation of the vestibular aqueduct. AJNR Am J Neuroradiol. 29(1):30-4, 2008
                                          15. Boston M et al: The large vestibular aqueduct: a new definition based on audiologic and computed tomography correlation. Otolaryngol Head Neck Surg. 136(6):972-7, 2007
                                          16. Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
                                          17. Vijayasekaran S et al: When is the vestibular aqueduct enlarged? A statistical analysis of the normative distribution of vestibular aqueduct size. AJNR Am J Neuroradiol. 28(6):1133-8, 2007
                                          18. Yang T et al: Transcriptional control of SLC26A4 is involved in Pendred syndrome and nonsyndromic enlargement of vestibular aqueduct (DFNB4). Am J Hum Genet. 2007 Jun;80(6):1055-63. Epub 2007 Apr 23. Erratum in: Am J Hum Genet. 81(3):634, 2007
                                          19. Albert S et al: SLC26A4 gene is frequently involved in nonsyndromic hearing impairment with enlarged vestibular aqueduct in Caucasian populations. Eur J Hum Genet. 14(6):773-9, 2006
                                          20. Yang WY et al: [Clinical analysis of 95 patients with large vestibular aqueduct syndrome] Zhonghua Er Bi Yan Hou Ke Za Zhi. 38(3):191-4, 2003
                                          21. Berrettini S et al: Distal renal tubular acidosis associated with isolated large vestibular aqueduct and sensorineural hearing loss. Ann Otol Rhinol Laryngol. 111(5 Pt 1):385-91, 2002
                                          22. Miyamoto RT et al: Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope. 112(7 Pt 1):1178-82, 2002
                                          23. Naganawa S et al: Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol. 12 Suppl 3:S114-7, 2002
                                          24. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                          25. Pyle GM: Embryological development and large vestibular aqueduct syndrome. Laryngoscope. 110(11):1837-42, 2000
                                          26. Davidson HC et al: MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol. 20(8):1435-41, 1999
                                          27. Naganawa S et al: MR imaging of the cochlear modiolus: area measurement in healthy subjects and in patients with a large endolymphatic duct and sac. Radiology. 213(3):819-23, 1999
                                          28. Phelps PD et al: Radiological malformations of the ear in Pendred syndrome. Clin Radiol. 53(4):268-73, 1998
                                          29. Dahlen RT et al: Overlapping thin-section fast spin-echo MR of the large vestibular aqueduct syndrome. AJNR Am J Neuroradiol. 18(1):67-75, 1997
                                          30. Tong KA et al: Large vestibular aqueduct syndrome: a genetic disease? AJR Am J Roentgenol. 168(4):1097-101, 1997
                                          31. Okumura T et al: Magnetic resonance imaging of patients with large vestibular aqueducts. Eur Arch Otorhinolaryngol. 253(7):425-8, 1996
                                          32. Harnsberger HR et al: Advanced techniques in magnetic resonance imaging in the evaluation of the large endolymphatic duct and sac syndrome. Laryngoscope. 105(10):1037-42, 1995
                                          33. Zalzal GH et al: Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg. 121(1):23-8, 1995
                                          34. Bagger-Sjoback D: Surgical anatomy of the endolymphatic sac. Am J Otol. 14(6):576-9, 1993
                                          35. Jackler RK et al: The large vestibular aqueduct syndrome. Laryngoscope. 99(12):1238-42; discussion 1242-3, 1989
                                          36. Valvassori GE et al: The large vestibular aqueduct syndrome. Laryngoscope. 88(5):723-8, 1978
                                          Related Anatomy
                                          Loading...
                                          Related Differential Diagnoses
                                          Loading...
                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Large vestibular aqueduct (LVA)

                                                      • Synonyms

                                                        • Large endolymphatic sac anomaly (LESA): T2 MR term
                                                        • Enlarged vestibular aqueduct (EVA): CT term
                                                        • Mondini deformity (historic terminology): LVA + minimally dilated vestibule + incomplete partition type II (IP-II)

                                                      • Definitions

                                                        • LVA: Enlarged bony vestibular aqueduct (VA) houses large endolymphatic sac (ES) & duct
                                                          • Associated with variable cochlear malformation
                                                        •  IP-II: Deficient modiolus & interscalar septum (ISS) between middle & apical cochlear turns; often seen with LVA
                                                        • IP-II: Normal cochlear external dimensions
                                                          • Not correct to call IP-II as cochlea with 1.5 turns
                                                          • Term cochlea with 1.5 turns should be used only for cochlear hypoplasia (CH) (especially CH-III)

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              • Image Interpretation Pearls

                                                                                • Reporting Tips

                                                                                  Selected References

                                                                                  1. Booth TN et al: Evaluation of the normal cochlear second interscalar ridge angle and depth on 3D T2-weighted images: a tool for the diagnosis of scala communis and incomplete partition type II. AJNR Am J Neuroradiol. 39(5):923-7, 2018
                                                                                  2. Reinshagen KL et al: Measurement for detection of incomplete partition type II anomalies on MR imaging. AJNR Am J Neuroradiol. 38(10):2003-7, 2017
                                                                                  3. Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                                                                  4. El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
                                                                                  5. Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-20, 2016
                                                                                  6. Ito T et al: Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4-insufficient mouse model of large vestibular aqueduct syndrome. Neuroscience. 310:188-97, 2015
                                                                                  7. Sone M et al: Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes. Laryngoscope. 126(6):1446-50, 2015
                                                                                  8. Chattaraj P et al: Use of SLC26A4 mutation testing for unilateral enlargement of the vestibular aqueduct. JAMA Otolaryngol Head Neck Surg. 139(9):907-13, 2013
                                                                                  9. Emmrich JV et al: Dilated dysplastic vestibule: a new computed tomographic finding in patients with large vestibular aqueduct syndrome. J Comput Assist Tomogr. 35(6):674-8, 2011
                                                                                  10. Griffith AJ et al: Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models. Hear Res. 281(1-2):11-7, 2011
                                                                                  11. Atkin JS et al: Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly. Int J Pediatr Otorhinolaryngol. 73(12):1682-5, 2009
                                                                                  12. Madeo AC et al: Evaluation of the thyroid in patients with hearing loss and enlarged vestibular aqueducts. Arch Otolaryngol Head Neck Surg. 135(7):670-6, 2009
                                                                                  13. Oh SH et al: Can magnetic resonance imaging provide clues to the inner ear functional status of enlarged vestibular aqueduct subjects with PDS mutation? Otol Neurotol. 29(5):593-600, 2008
                                                                                  14. Ozgen B et al: Comparison of 45 degrees oblique reformats with axial reformats in CT evaluation of the vestibular aqueduct. AJNR Am J Neuroradiol. 29(1):30-4, 2008
                                                                                  15. Boston M et al: The large vestibular aqueduct: a new definition based on audiologic and computed tomography correlation. Otolaryngol Head Neck Surg. 136(6):972-7, 2007
                                                                                  16. Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
                                                                                  17. Vijayasekaran S et al: When is the vestibular aqueduct enlarged? A statistical analysis of the normative distribution of vestibular aqueduct size. AJNR Am J Neuroradiol. 28(6):1133-8, 2007
                                                                                  18. Yang T et al: Transcriptional control of SLC26A4 is involved in Pendred syndrome and nonsyndromic enlargement of vestibular aqueduct (DFNB4). Am J Hum Genet. 2007 Jun;80(6):1055-63. Epub 2007 Apr 23. Erratum in: Am J Hum Genet. 81(3):634, 2007
                                                                                  19. Albert S et al: SLC26A4 gene is frequently involved in nonsyndromic hearing impairment with enlarged vestibular aqueduct in Caucasian populations. Eur J Hum Genet. 14(6):773-9, 2006
                                                                                  20. Yang WY et al: [Clinical analysis of 95 patients with large vestibular aqueduct syndrome] Zhonghua Er Bi Yan Hou Ke Za Zhi. 38(3):191-4, 2003
                                                                                  21. Berrettini S et al: Distal renal tubular acidosis associated with isolated large vestibular aqueduct and sensorineural hearing loss. Ann Otol Rhinol Laryngol. 111(5 Pt 1):385-91, 2002
                                                                                  22. Miyamoto RT et al: Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope. 112(7 Pt 1):1178-82, 2002
                                                                                  23. Naganawa S et al: Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol. 12 Suppl 3:S114-7, 2002
                                                                                  24. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                                                                  25. Pyle GM: Embryological development and large vestibular aqueduct syndrome. Laryngoscope. 110(11):1837-42, 2000
                                                                                  26. Davidson HC et al: MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol. 20(8):1435-41, 1999
                                                                                  27. Naganawa S et al: MR imaging of the cochlear modiolus: area measurement in healthy subjects and in patients with a large endolymphatic duct and sac. Radiology. 213(3):819-23, 1999
                                                                                  28. Phelps PD et al: Radiological malformations of the ear in Pendred syndrome. Clin Radiol. 53(4):268-73, 1998
                                                                                  29. Dahlen RT et al: Overlapping thin-section fast spin-echo MR of the large vestibular aqueduct syndrome. AJNR Am J Neuroradiol. 18(1):67-75, 1997
                                                                                  30. Tong KA et al: Large vestibular aqueduct syndrome: a genetic disease? AJR Am J Roentgenol. 168(4):1097-101, 1997
                                                                                  31. Okumura T et al: Magnetic resonance imaging of patients with large vestibular aqueducts. Eur Arch Otorhinolaryngol. 253(7):425-8, 1996
                                                                                  32. Harnsberger HR et al: Advanced techniques in magnetic resonance imaging in the evaluation of the large endolymphatic duct and sac syndrome. Laryngoscope. 105(10):1037-42, 1995
                                                                                  33. Zalzal GH et al: Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg. 121(1):23-8, 1995
                                                                                  34. Bagger-Sjoback D: Surgical anatomy of the endolymphatic sac. Am J Otol. 14(6):576-9, 1993
                                                                                  35. Jackler RK et al: The large vestibular aqueduct syndrome. Laryngoscope. 99(12):1238-42; discussion 1242-3, 1989
                                                                                  36. Valvassori GE et al: The large vestibular aqueduct syndrome. Laryngoscope. 88(5):723-8, 1978