Spontaneous plugging of the horizontal semicircular canal with reversible canal dysfunction and recovery of vestibular evoked myogenic potentials

Objective: To evaluate the clinical pathophysiology of oculomotor changes in a patient presenting with a spontaneous semicircular horizontal canal plug. Patient: A 42-year-old man with acute spontaneous vertigo with spinning and persistent left-horizontal nystagmus, intensity but not direction dependent on head orientation with respect to gravity, indicating a benign paroxysmal positional vertigo due to otoconia causing a plug in the horizontal semicircular canal. Intervention: Electrophysiological and video-oculographic testing; vestibular rehabilitation. Main Outcome Measures: Cervical and ocular vestibular evoked myogenic potentials (VEMPs); video head impulse testing. Results: The video head-impulse test revealed an eye velocity cutoff at 80°/s in the time interval from 40 to 90 ms after initiation of head impulses to the right. This normalized within 2 days after liberatory maneuvers, documenting for the first time a reversible deficiency of the cupular-endolymph highfrequency system dynamics. Cervical and ocular vestibular myogenic potentials were absent during stimulation of the affected side before the liberatory maneuvers but normalized within 30 to 80 days. Conclusion: This case is special in 4 respects: 1) nystagmus intensity, but not direction, was dependent on head orientation with respect to gravity, indicating a horizontal canal plug; 2) VEMPs were asymmetrical before liberatory maneuvers; 3) VEMPs recovered after Day 30; and 4) video head-impulse test asymmetry recovered. These observations challenge the common belief that VEMPs are evoked by otolith stimulation only. Instead, the assumption of a reversible canal dysfunction by a plug offers a more plausible explanation for all effects.