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
Video 1
Video-oculography (SLMED; SLVNG, Seoul, South Korea) of the patient. The patient shows spontaneous nystagmus beating leftward without visual fixation, which changes into right-beating during rightward gaze and after hyperventilation.Download Supplementary Video 1 via http://dx.doi.org/10.1212/009095_Video_1
(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).
Study funding
This study was supported by the Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education, Science and Technology (no. NRF-2016R1D1A1B04935568).
Disclosure
S. Lee, H. Kim, J. Choi, J. Song, and B. Choi report no disclosures relevant to the manuscript. J. Kim serves as an associate editor of Frontiers in Neuro-otology and on the editorial boards of the Journal of Clinical Neurology, Frontiers in Neuro-ophthalmology, Journal of Neuro-ophthalmology, Journal of Vestibular Research, Journal of Neurology, and Medicine. Go to Neurology.org/N for full disclosures.
Appendix Authors
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Teaching slides links.lww.com/WNL/B57
- © 2020 American Academy of Neurology
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