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
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
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
Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-20, 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
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)
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
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
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
Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
El-Badry MM et al: Evaluation of the radiological criteria to diagnose large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol. 81:84-91, 2016
Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-20, 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
STATdx includes over 200,000 searchable images, including x-ray, CT, MR, and ultrasound images. To access all images, please log in or subscribe.