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Objective: To evaluate ocular and cervical vestibular evoked myogenic potentials (oVEMPs and cVEMPs) in patients with solely intracochlear localization of an intralabyrinthine schwannoma (ILS). Study Design: Retrospective analysis of a series of cases. Setting: Monocentric study at a tertiary referral center. Patients: Patients with intracochlear schwannoma (ICS) and VEMP measurements. Outcome Measures: Signed asymmetry ratio (AR) of cVEMPs and oVEMPs to air conducted sound with AR cut-offs considered to be asymmetrical when exceeding ±30% for cVEMPs and ±40% for oVEMPs with respect to the side affected by the tumor (reduced amplitudes on the affected side indicated by negative values, enhanced amplitudes by positive values); VEMP amplitudes and latencies; tumor localization in the cochlear turn and scala. Results: Nineteen patients with a solely intracochlear tumor (ICS patients) [10 males, 9 females, mean age 57.1 (SD: 13.4) years] were included in the study. On the affected side, cVEMPs were absent or reduced in 47% of the patients, normal in 32%, and enhanced in 21%. Ocular VEMPs on the affected side were absent or reduced in 53% of the patients, normal in 32% and enhanced in 15%. Latencies for cVEMPs and oVEMPs were not significantly different between the affected and non-affected side. In all patients with enhanced VEMPs, the tumor was located in the scala tympani and scala vestibuli. Conclusions: As a new and unexpected finding, VEMP amplitudes can be enhanced in patients with intracochlear schwannoma, mimicking the third window syndrome.

Purpose of review To examine the recent literature concerning the neural basis and clinical evidence for the response of the labyrinth to sound and vibration: vestibular-evoked myogenic potentials (VEMPs) and vibration-induced nystagmus (VIN). Recent findings There are two streams of information from each otolith - a sustained stream (afferents with regular resting activity, signalling gravity and low-frequency linear accelerations) and a transient stream (afferents with irregular resting activity) signalling onset of linear acceleration, and sound and vibration. These irregular neurons are synchronized to each cycle of the stimulus. Neurons in the transient stream are tested by presenting sounds or vibration (500 Hz) and using surface electrodes to measure myogenic potentials from muscles activated by otolithic stimuli (VEMPs). 100 Hz vibration activates irregular canal afferents and causes a stimulus-locked VIN in patients with asymmetric canal function. These new tests of the transient system have one big advantage over older tests of the sustained system - they reliably show the effect of long-term unilateral vestibular loss. Summary The new physiological and anatomical evidence shows how sound and vibration activate otolith and canal receptors and so provides the scientific foundation for VEMPs and VIN, which are important tools for diagnosing vestibular disorders. VIDEO ABSTRACT: http://links.lww.com/CONR/A47.