Citation, DOI & article data
Citation:
Weerakkody Y, Deng F, El-Feky M, et al. Enlarged vestibular aqueduct. Reference article, Radiopaedia.org (Accessed on 02 Feb 2023) https://doi.org/10.53347/rID-8567
Enlarged vestibular aqueduct, also known as large vestibular aqueduct or large endolymphatic duct and sac syndrome or anomaly, is the most common inner ear malformation associated with sensorineural hearing loss.
The syndrome manifests as sensorineural or mixed hearing loss. Hearing may be normal at birth but typically manifests as fluctuating and/or progressive hearing loss in childhood 4. Hearing loss may be sudden onset, either spontaneously or after minor head trauma 6.
Associations
Associated anomalies are the norm, occurring in ~85% of patients 6:
CT/MRI
The classic (Valvassori) criterion for enlarged vestibular aqueduct is a diameter >1.5 mm at the midpoint 1. The measurement is made halfway between the crus and the aperture on an axial view (i.e. AP dimension). This was originally established on polytomography and subsequently ported to CT as well as MRI 4.
The newer (Cincinnati) criteria for enlarged vestibular aqueduct are midpoint width ≥1.0 mm or opercular width ≥2.0 mm as measured in the axial plane 3,5. When measured in the Pöschl plane, a vestibular aqueduct midpoint width of 0.9 mm is the upper limit of normal 7.
History and etymology
The clinical syndrome and imaging diagnosis was first described by Valvassori and Clemis in 1978 based on hypocycloidal polytomography 1.
- 1. Valvassori G & Clemis J. The Large Vestibular Aqueduct Syndrome. Laryngoscope. 1978;88(5):723-8. doi:10.1002/lary.1978.88.5.723 - Pubmed
- 2. Goldfeld M, Glaser B, Nassir E et-al. CT of the ear in Pendred syndrome. Radiology. 2005;235 (2): 537-40. doi:10.1148/radiol.2352031583 - Pubmed citation
- 3. Boston M, Halsted M, Meinzen-Derr J et al. The Large Vestibular Aqueduct: A New Definition Based on Audiologic and Computed Tomography Correlation. Otolaryngol Head Neck Surg. 2007;136(6):972-7. doi:10.1016/j.otohns.2006.12.011 - Pubmed
- 4. Naganawa S, Koshikawa T, Iwayama E et al. MR Imaging of the Enlarged Endolymphatic Duct and Sac Syndrome by Use of a 3D Fast Asymmetric Spin-Echo Sequence: Volume and Signal-Intensity Measurement of the Endolymphatic Duct and Sac and Area Measurement of the Cochlear Modiolus. AJNR Am J Neuroradiol. 2000;21(9):1664-9. PMC8174870 - Pubmed
- 5. Vijayasekaran S, Halsted M, Boston M et al. When is the Vestibular Aqueduct Enlarged? A Statistical Analysis of the Normative Distribution of Vestibular Aqueduct Size. AJNR Am J Neuroradiol. 2007;28(6):1133-8. doi:10.3174/ajnr.A0495 - Pubmed
- 6. Joshi V, Navlekar S, Kishore G, Reddy K, Kumar E. CT and MR Imaging of the Inner Ear and Brain in Children with Congenital Sensorineural Hearing Loss. Radiographics. 2012;32(3):683-98. doi:10.1148/rg.323115073 - Pubmed
- 7. Juliano A, Ting E, Mingkwansook V, Hamberg L, Curtin H. Vestibular Aqueduct Measurements in the 45° Oblique (Pöschl) Plane. AJNR Am J Neuroradiol. 2016;37(7):1331-7. doi:10.3174/ajnr.A4735 - Pubmed
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