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Ménière disease caused by an anomalous vein of the vestibular aqueduct.

The precise cause of Ménière disease remains unclear. Multiple causes have been proposed with most experimental evidence pointing to impaired fluid resorption by the endolymphatic duct and sac as the final common pathway in development of hydrops. We report a unique case of Ménière disease secondary to compression of the endolymphatic duct and sac by an anomalous vein of the vestibular aqueduct. The resulting mechanical obstruction led to excessive accumulation of endolymph with clinical signs of Ménière disease. We review the literature and discuss proposed pathogenesis of disease. The finding of this anomalous vein provides further evidence that anatomical obstruction of the endolymphatic duct and sac may lead to Ménière-like symptoms. This unique example of an anatomical variant offers additional insight into the pathophysiology of endolymphatic hydrops.