Large endolymphatic sac. A congenital deformity of the inner ear shown by magnetic resonance imaging.

@article{Phelps1997LargeES,
  title={Large endolymphatic sac. A congenital deformity of the inner ear shown by magnetic resonance imaging.},
  author={Peter David Phelps and C F Mahoney and L. M. Luxon},
  journal={The Journal of laryngology and otology},
  year={1997},
  volume={111 8},
  pages={
          754-6
        }
}
Fluctuant and progressive hearing impairment in a patient with a wide vestibular aqueduct has been called the 'large vestibular aqueduct syndrome'. Recently reports of magnetic resonance imaging (MRI) studies describe enlargement of the endolymphatic sac and duct in patients shown to have large vestibular aqueducts by computed tomography (CT). A patient with progressive deafness was shown to have borderline or slightly enlarged vestibular aqueducts by re-formatted sagittal CT. However, MRI in… 
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Two Cases of Large Vestibular Aqueduct Syndrome
TLDR
Two cases of large vestibular aqueduct syndrome are reported, a rare congenital malformation of the inner ear originally described by Valvassori and Clemis in 1978, and both patients showed severe bilateral combined hearing loss on pure tone audiogram.
Large vestibular aqueduct syndrome: audiological, radiological, clinical, and genetic features.
Original contributions Large vestibular aqueduct syndrome: audiological, radiological, clinical, and genetic features
TLDR
The data underscore the frequent role of the large vestibular aqueduct syndrome in the pathogenesis of sensorineural hearing loss and the overall wide variability in its audiological features.
Estimation of the Endolymphatic Sac and Vestibular Aqueduct Using Magnetic Resonance Imaging
Objective To evaluate the diagnostic accuracy of magnetic resonance imaging for assessment of the endolymphatic sac and vestibular aqueduct.
Lateral Semicircular Canal and Vertigo in Patients With Large Vestibular Aqueduct Syndrome
  • I. Ishida, M. Sugiura, +4 authors T. Yoshino
  • Medicine
    Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
  • 2006
TLDR
The results reveal that the morphology of semicircular canals is clinically associated with vertigo and patients with LVAS may have disturbed morphogenesis of both membranous and bony labyrinths.
MR imaging of the cochlear modiolus: area measurement in healthy subjects and in patients with a large endolymphatic duct and sac.
TLDR
Findings in this study confirm that a large endolymphatic duct and sac is frequently associated with modiolar deficiency, but theModiolar area is normal in some cases, and does not support the recently proposed hypothesis that hearing loss with a largeendolymphic duct andsac is caused by the transmission of subarachnoid pressure forces into the labyrinth through a deficient modiolus.
Imaging and anatomy for cochlear implants.
  • A. Fishman
  • Medicine
    Otolaryngologic clinics of North America
  • 2012
Vestibular Evoked Myogenic Potential (VEMP) in children with Enlarged Vestibular Aqueduct (EVA)

References

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TLDR
The large vestibular aqueduct syndrome describes an abnormally large endolymphatic duct and sac with associated sensorineural hearing loss and magnetic resonance imaging of two patients diagnosed with this syndrome is reported.
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TLDR
The significance of a diagnosis of large vestibular aqueduct syndrome, in the presence of serviceable hearing, is that it identifies patients who should refrain from activities that increase intracranial pressure to prevent hearing loss.
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TLDR
Clinical observations indicate that the hearing loss in children with an isolated enlargement of the vestibular aqueduct is acquired during childhood.
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TLDR
Characteristic temporal bone findings include cochlear hypoplasia, dilation of the vestibular aqueduct, bulbous internal auditory canals, deep posterior fossae, and acutely-angled promontories.
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