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
El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
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
Sone M et al: Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes. Laryngoscope. 126(6):1446-50, 2015
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
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
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
Atkin JS et al: Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly. Int J Pediatr Otorhinolaryngol. 73(12):1682-5, 2009
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
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
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
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
Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
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
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
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
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
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
Miyamoto RT et al: Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope. 112(7 Pt 1):1178-82, 2002
Naganawa S et al: Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol. 12 Suppl 3:S114-7, 2002
Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
Pyle GM: Embryological development and large vestibular aqueduct syndrome. Laryngoscope. 110(11):1837-42, 2000
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
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
Phelps PD et al: Radiological malformations of the ear in Pendred syndrome. Clin Radiol. 53(4):268-73, 1998
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
Tong KA et al: Large vestibular aqueduct syndrome: a genetic disease? AJR Am J Roentgenol. 168(4):1097-101, 1997
Okumura T et al: Magnetic resonance imaging of patients with large vestibular aqueducts. Eur Arch Otorhinolaryngol. 253(7):425-8, 1996
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
Zalzal GH et al: Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg. 121(1):23-8, 1995
Bagger-Sjoback D: Surgical anatomy of the endolymphatic sac. Am J Otol. 14(6):576-9, 1993
Jackler RK et al: The large vestibular aqueduct syndrome. Laryngoscope. 99(12):1238-42; discussion 1242-3, 1989
Valvassori GE et al: The large vestibular aqueduct syndrome. Laryngoscope. 88(5):723-8, 1978
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Large endolymphatic sac anomaly (LESA): T2 MR term
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
El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
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
Sone M et al: Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes. Laryngoscope. 126(6):1446-50, 2015
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
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
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
Atkin JS et al: Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly. Int J Pediatr Otorhinolaryngol. 73(12):1682-5, 2009
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
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
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
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
Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
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
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
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
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
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
Miyamoto RT et al: Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope. 112(7 Pt 1):1178-82, 2002
Naganawa S et al: Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol. 12 Suppl 3:S114-7, 2002
Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
Pyle GM: Embryological development and large vestibular aqueduct syndrome. Laryngoscope. 110(11):1837-42, 2000
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
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
Phelps PD et al: Radiological malformations of the ear in Pendred syndrome. Clin Radiol. 53(4):268-73, 1998
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
Tong KA et al: Large vestibular aqueduct syndrome: a genetic disease? AJR Am J Roentgenol. 168(4):1097-101, 1997
Okumura T et al: Magnetic resonance imaging of patients with large vestibular aqueducts. Eur Arch Otorhinolaryngol. 253(7):425-8, 1996
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
Zalzal GH et al: Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg. 121(1):23-8, 1995
Bagger-Sjoback D: Surgical anatomy of the endolymphatic sac. Am J Otol. 14(6):576-9, 1993
Jackler RK et al: The large vestibular aqueduct syndrome. Laryngoscope. 99(12):1238-42; discussion 1242-3, 1989
Valvassori GE et al: The large vestibular aqueduct syndrome. Laryngoscope. 88(5):723-8, 1978
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