Teaching Video NeuroImages: Intralabyrinthine schwannoma masquerading as Ménière disease

A 56-year-old woman presented with recurrent spontaneous vertigo, fluctuating tinnitus, and ear fullness. Examination showed spontaneous nystagmus beating leftward and counter-clockwise without fixation, which changed into right-beating during rightward gaze and after hyperventilation (video 1). She also showed positive head impulse tests for all semicircular canals on the right side (figure, A), right caloric paresis of 94%, and right sensorineural hearing loss from schwannoma restricted to right labyrinth (figure, B). Intralabyrinthine schwannoma is a rare and underrecognized cause of recurrent audiovestibulopathy, masquerading as Ménière disease.¹ High-resolution MRIs allow antemortem diagnosis,² and labyrinthectomy may be attempted when refractory with medication.¹

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Figure Patient findings

(A) Video head-impulse tests are positive to the right. AC = anterior canal; HC = horizontal canal; PC = posterior canal. (B) MRIs show a loss of T2 signal intensity at the basal cochlea (dashed lines and arrow) and vestibule (arrowhead) in the right ear (B.a) and abnormal enhancements in the corresponding areas (B.b).

This study followed the tenets of the Declaration of Helsinki and was performed according to the guidelines of the institutional review board of Seoul National University Bundang Hospital (1902/523-109).

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• © 2020 American Academy of Neurology