Vestibular Neuritis with a Peripheral Skew Deviation

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Identifier Vestibular_neuritis_with_skewdeviation
Title Vestibular Neuritis with a Peripheral Skew Deviation
Subject Abnormal VOR; Abnormal Alignment; Eighth Nerve; Skew Deviation; Lateropulsion; Jerk Nystagmus; Vestibular Nystagmus; Acute Vestibular Syndrome
Creator Daniel R. Gold, DO, Departments of Neurology, Ophthalmology, Neurosurgery, Otolaryngology - Head & Neck Surgery, Emergency Medicine, and Medicine, The Johns Hopkins School of Medicine
Description This is a 55-year-old hypertensive man who developed acute onset continuous vertigo and presented to the Emergency Department (ED) after several hours of symptoms. He was noted to have spontaneous nystagmus and had a normal brain MRI within the first 24 hours. The first portion of the video was recorded during his hospitalization, and if his head was in any position other than left ear down, he experienced severe vertigo and nausea. Nystagmus was left-beating (LB) with a torsional component (top poles beating toward left ear), the LB lessened in right gaze, remained LB in vertical gaze, and increased in left gaze (in accordance with Alexander's law). Nystagmus also increased in intensity with fixation removed, which combined with his unidirectional and mixed horizontal-torsional nystagmus, were features suggestive of a peripheral vestibular localization (but could still be seen with a central disorder). The patient was extremely symptomatic but allowed the examiner to perform one single head impulse test (HIT), which was abnormal to the right side (i.e., when the head was moved quickly to the right, the eyes initially moved with the head to the right due to a deficient vestibulo-ocular reflex involving the right horizontal canal, and this was followed by a catch-up saccade back to the left to the examiner's nose). The patient also experienced binocular vertical diplopia, and a right hypotropia was apparent in primary gaze with cover-uncover testing, which was consistent with a skew deviation. Finally, while there was no clear ocular lateropulsion to the right while upright, rightward horizontal gaze deviation was noted on the MRI. However, this finding does not predict a central localization.(1) Examination at 1 week showed much improved LB nystagmus, although the right hypotropia persisted. This was measured as 4 prism diopters, and was constant in right, left, up, down gaze and with right and left head tilt. Fundus photos showed a mild ocular counterroll with top poles toward the right ear, which paired with his skew deviation (right hypotropia) suggested a partial ocular tilt reaction (in the absence of a clear head tilt) from utriculo-ocular pathway (or graviceptive-ocular motor pathway [mediating inputs from vertical semicircular canals and the utricle]) involvement. His HIT to the right remained abnormal and video HIT demonstrated low gains and overt saccades in the planes of the right horizontal and anterior canals, both of which are innervated by the superior division of the vestibular nerve. The utricle is also innervated by the superior division of the vestibular nerve, and while a skew is possible in a peripheral vestibular disorder, this is rare, and a peripheral skew deviation tends to be small and transient. Although right-sided vestibular neuritis with a ‘peripheral' skew deviation was suspected as the cause, given the possibility of a false-negative MRI as an inpatient (MRI was done within 24 hours of symptom onset, where the false negative rate with posterior fossa vestibular strokes in the first 24-48 hours approaches ~20%(2) a second MRI with and without contrast was ordered. This was normal, and the diagnosis of right vestibular neuritis was confirmed. The patient improved significantly over the following months. Of note, patients with vestibular neuritis tend to have more intense nystagmus and vertigo with the bad (affected) ear down, which is why the patient maintained a left ear down position throughout most of his hospitalization.
References 1. Kattah JC, Pula J, Newman-Toker DE. Ocular lateropulsion as a central oculomotor sign in acute vestibular syndrome is not posturally dependent. Ann N Y Acad Sci 2011;1233:249-255. 2. Saber Tehrani AS, Kattah JC, Mantokoudis G, et al. Small strokes causing severe vertigo: frequency of false-negative MRIs and nonlacunar mechanisms. Neurology 2014;83:169-173.
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 2018-12
Type Image/MovingImage
Format video/mp4
Relation is Part of NOVEL: Neuro-ophthalmology Virtual Education Library Examination Collection
Rights Management Copyright 2018. For further information regarding the rights to this collection, please visit:
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E, SLC, UT 84112-5890
Collection Neuro-ophthalmology Virtual Education Library: NOVEL
Language eng
ARK ark:/87278/s6ht70fx
Setname ehsl_novel_gold
Date Created 2018-12-19
Date Modified 2021-06-28
ID 1390070
Reference URL
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