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Pediatrics
Diagnosis
Head and Neck
Temporal Bone and Skull Base
Large Vestibular Aqueduct (IP-II)
Large Vestibular Aqueduct (IP-II)
Caroline D. Robson, MBChB
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Large endolymphatic sac anomaly (LESA): T2 MR term
              • Enlarged vestibular aqueduct (EVA): CT term
              • Mondini anomaly (historic terminology): IP-II + LVA

            • Definitions

              • Large vestibular aqueduct (LVA): Enlarged bony vestibular aqueduct (VA) houses large endolymphatic sac (ES) & duct associated with variable cochlear malformation
              • IP-II: Incomplete partition (deficient interscalar septum) between middle & apical cochlear turns is usually seen with LVA

            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. El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
                                        2. 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
                                        3. Sone M et al: Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes. Laryngoscope. 126(6):1446-50, 2015
                                        4. 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
                                        5. 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
                                        6. 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
                                        7. Atkin JS et al: Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly. Int J Pediatr Otorhinolaryngol. 73(12):1682-5, 2009
                                        8. 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
                                        9. 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
                                        10. 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
                                        11. 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
                                        12. Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
                                        13. 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
                                        14. 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
                                        15. 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
                                        16. 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
                                        17. 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
                                        18. Miyamoto RT et al: Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope. 112(7 Pt 1):1178-82, 2002
                                        19. Naganawa S et al: Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol. 12 Suppl 3:S114-7, 2002
                                        20. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                        21. Pyle GM: Embryological development and large vestibular aqueduct syndrome. Laryngoscope. 110(11):1837-42, 2000
                                        22. 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
                                        23. 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
                                        24. Phelps PD et al: Radiological malformations of the ear in Pendred syndrome. Clin Radiol. 53(4):268-73, 1998
                                        25. 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
                                        26. Tong KA et al: Large vestibular aqueduct syndrome: a genetic disease? AJR Am J Roentgenol. 168(4):1097-101, 1997
                                        27. Okumura T et al: Magnetic resonance imaging of patients with large vestibular aqueducts. Eur Arch Otorhinolaryngol. 253(7):425-8, 1996
                                        28. 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
                                        29. Zalzal GH et al: Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg. 121(1):23-8, 1995
                                        30. Bagger-Sjoback D: Surgical anatomy of the endolymphatic sac. Am J Otol. 14(6):576-9, 1993
                                        31. Jackler RK et al: The large vestibular aqueduct syndrome. Laryngoscope. 99(12):1238-42; discussion 1242-3, 1989
                                        32. Valvassori GE et al: The large vestibular aqueduct syndrome. Laryngoscope. 88(5):723-8, 1978
                                        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

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Large endolymphatic sac anomaly (LESA): T2 MR term
                                                    • Enlarged vestibular aqueduct (EVA): CT term
                                                    • Mondini anomaly (historic terminology): IP-II + LVA

                                                  • Definitions

                                                    • Large vestibular aqueduct (LVA): Enlarged bony vestibular aqueduct (VA) houses large endolymphatic sac (ES) & duct associated with variable cochlear malformation
                                                    • IP-II: Incomplete partition (deficient interscalar septum) between middle & apical cochlear turns is usually seen with LVA

                                                  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. El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
                                                                              2. 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
                                                                              3. Sone M et al: Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes. Laryngoscope. 126(6):1446-50, 2015
                                                                              4. 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
                                                                              5. 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
                                                                              6. 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
                                                                              7. Atkin JS et al: Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly. Int J Pediatr Otorhinolaryngol. 73(12):1682-5, 2009
                                                                              8. 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
                                                                              9. 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
                                                                              10. 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
                                                                              11. 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
                                                                              12. Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
                                                                              13. 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
                                                                              14. 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
                                                                              15. 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
                                                                              16. 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
                                                                              17. 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
                                                                              18. Miyamoto RT et al: Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope. 112(7 Pt 1):1178-82, 2002
                                                                              19. Naganawa S et al: Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol. 12 Suppl 3:S114-7, 2002
                                                                              20. Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
                                                                              21. Pyle GM: Embryological development and large vestibular aqueduct syndrome. Laryngoscope. 110(11):1837-42, 2000
                                                                              22. 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
                                                                              23. 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
                                                                              24. Phelps PD et al: Radiological malformations of the ear in Pendred syndrome. Clin Radiol. 53(4):268-73, 1998
                                                                              25. 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
                                                                              26. Tong KA et al: Large vestibular aqueduct syndrome: a genetic disease? AJR Am J Roentgenol. 168(4):1097-101, 1997
                                                                              27. Okumura T et al: Magnetic resonance imaging of patients with large vestibular aqueducts. Eur Arch Otorhinolaryngol. 253(7):425-8, 1996
                                                                              28. 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
                                                                              29. Zalzal GH et al: Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg. 121(1):23-8, 1995
                                                                              30. Bagger-Sjoback D: Surgical anatomy of the endolymphatic sac. Am J Otol. 14(6):576-9, 1993
                                                                              31. Jackler RK et al: The large vestibular aqueduct syndrome. Laryngoscope. 99(12):1238-42; discussion 1242-3, 1989
                                                                              32. Valvassori GE et al: The large vestibular aqueduct syndrome. Laryngoscope. 88(5):723-8, 1978
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