Subjective visual horizontal correlates better with ocular than with cervical vestibular evoked myogenic potentials.

OBJECTIVE: To examine the relationship between widely used otolith function tests: the Subjective Visual Horizontal (SVH) and Vestibular Evoked Myogenic Potentials (VEMP). METHODS: A retrospective analysis was performed on 301 patients who underwent SVH, ocular and cervical VEMP (oVEMP and cVEMP) tests on the same day. Correlations between the mean SVH tilt and amplitude asymmetry ratios for bone-conducted (BC) oVEMP and air-conducted (AC) cVEMP were examined. Diagnoses included vestibular neuritis, stroke, vestibular migraine, Meniere's disease, sudden sensorineural hearing loss (SSNHL) and vestibular schwannoma. RESULTS: SVH results were concordant with the oVEMP in 64% of cases and the cVEMP in 51%. Across all patients, SVH demonstrated a significant moderate correlation with BC oVEMP amplitude asymmetry ratios (r = 0.55, p < 0.001) and a weak correlation with AC cVEMP amplitude asymmetry ratios (r = 0.35, p < 0.001). A stronger correlation between SVH and oVEMPs was observed in patients with vestibular neuritis (r = 0.67, p < 0.001) and SSNHL (r = 0.76, p = 0.001). CONCLUSIONS: SVH correlates better with oVEMP than cVEMP symmetry. SIGNIFICANCE: This finding reinforces the hypothesis of a common utricular origin for both SVH and oVEMPs which is distinct from the saccular origin of cVEMPs.

Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects.

PURPOSE: Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. METHODS: Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. RESULTS: The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. CONCLUSIONS: In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

The human vestibulo-ocular reflex and compensatory saccades in schwannoma patients before and after vestibular nerve section.

OBJECTIVE: To examine the vestibulo-ocular reflex (VOR) and compensatory-saccades before and after complete unilateral vestibular deafferentation (UVD). METHODS: Forty patients were studied before and after surgery for vestibular or facial schwannoma using the video head-impulse test (vHIT) and multivariable regression. RESULTS: Prior to UVD (median(IQR), 14(58.4) days), the average VOR-gain towards the lesioned-ear was lower than in normal for all semicircular canals (lateral, anterior, posterior: 0.69, 0.72, 0.49). One-week after UVD (5(3.0) days) VOR gains were further reduced (0.22, 0.37, 0.27), however, within one-year after UVD (171(125.0) days) the lesioned-ear VOR gains had slightly increased (+0.08, +0.11, +0.03), maximally for the anterior-canal. After UVD, the VOR gain asymmetry (gain towards minus away from intact-ear) was lower for the intact posterior-canal plane (0.56, 0.56, 0.22). For the lesioned canals, the frequency and amplitude of the first compensatory-saccade increased from 61-93% and 1.9-3.6° pre-surgery, to 98-99% and to 3.1-5.9° one-week post-surgery and remained unchanged over one-year; second saccade frequency and amplitude decreased over the same timespan. CONCLUSIONS: After UVD the high-acceleration VOR for the intact posterior-canal plane is more symmetrical than the other canals. First compensatory-saccades adapt within one week and subsequently change only marginally. SIGNIFICANCE: Saccade compensation from surgical UVD is near complete by one-week.

Impact of Cochlear Implantation on Canal and Otolith Function.

OBJECTIVE: To quantify the impact of cochlear implantation (CI) on all five vestibular end-organs and on subjective ratings of post-CI dizziness. METHODS: Seventy-two patients undergoing unilateral CI were recruited for the study. All participants completed pre- and post-CI three-dimensional video head-impulse tests (3D vHITs) to assess semicircular-canal (SC) function, air- and bone-conducted (AC and BC) cervical and ocular vestibular-evoked myogenic potentials (cVEMPs and oVEMPs) to assess otolith-function and the dizziness handicap inventory (DHI) to measure self-perceived disability. RESULTS: Nineteen percent of patients reported new or worsened dizziness postsurgery. Post-CI abnormalities (new lesions and significant deteriorations) were seen in the AC cVEMP (48%), AC oVEMP (34%), BC cVEMP (10%), and BC oVEMP (7%); and lateral (L) (17%), posterior (P) (10%), and anterior (A) (13%) SC vHITs. CI surgery was more likely to affect the AC cVEMP compared with the other tests (χ2 test, p < 0.05). Fifty percent of patients reported no dizziness pre- and postsurgery. In the implanted ear, normal pre-CI vHIT gain was preserved in lateral semicircular canal (LSC) (69%), anterior semicircular canal (ASC) (74%), and posterior semicircular canal (PSC) (67%), and normal reflex amplitudes were found in AC cVEMP (25%), AC oVEMP (20%), BC cVEMP (59%), and BC oVEMP (74%). Statistically significant decreases were observed in LSC vHIT gain, AC cVEMP amplitude, and AC oVEMP amplitude postsurgery (p < 0.05). There was a significant moderate positive correlation between change in DHI scores and the summed vestibular deficit postsurgery (r(51) = 0.38, p < 0.05). CONCLUSION: CI can impact tests that assess all five vestibular end-organs and subjective ratings of dizziness. These results support pre and post-surgical vestibular testing and assist preoperative counseling and candidate selection.

A Window Into the Whole Story: Temporal Bone Plasmacytoma Presenting With a Mobile Third Window.

A 63-year-old man presented with imbalance when coughing due to a respiratory tract infection. He had a history of multiple myeloma with a plasmacytoma of the left temporal bone. Examination revealed a positive leftward head impulse test, no spontaneous nystagmus, left-beating positional nystagmus, and left-beating Valsalva-induced nystagmus. Videonystagmography, audiology, and comprehensive vestibular function tests revealed a subtotal left peripheral audio-vestibular loss. Temporal bone computed tomography showed an unchanged bony erosion of the left labyrinth from 2 years prior. Vertigo subsided after treatment of the respiratory tract infection. Although no tumor progression was evident, coughing had triggered a preexisting third mobile window to declare itself. Laryngoscope, 131:E966-E969, 2021.

Vestibular evoked myogenic potentials in practice: Methods, pitfalls and clinical applications.

Vestibular evoked myogenic potentials (VEMPs) are a useful and increasingly popular component of the neuro-otology test battery. These otolith-dependent reflexes are produced by stimulating the ears with air-conducted sound or skull vibration and recorded from surface electrodes placed over the neck (cervical VEMPs) and eye muscles (ocular VEMPs). VEMP abnormalities have been reported in various diseases of the ear and vestibular system, and VEMPs have a clear role in the diagnosis of superior semicircular canal dehiscence. However there is significant variability in the methods used to stimulate the otoliths and record the reflexes. This review discusses VEMP methodology and provides a detailed theoretical background for the techniques that are typically used. The review also outlines the common pitfalls in VEMP recording and the clinical applications of VEMPs.

Otolith Function Testing.

Two technically simple tests, vestibular evoked myogenic potential (VEMP) and subjective visual vertical/horizontal (SVV/H) test, have the potential to transform otolith function testing from the research laboratory to the outpatient clinic. Cervical- and ocular-VEMPs are short latency surface potentials produced through the activation of saccular and utricular afferents by sound and vibration. They are tests of dynamic otolith function. The SVV/H test in peripheral lesions probes static asymmetries in utricular function and represents a perceptual error in perceived gravitational vertical/horizontal. VEMPs and SVV/H enable the characterization of patterns and severity of otolith dysfunction in common vestibular disorders. Combined with tests of semicircular canal function, they provide a useful tool for eliciting diagnostic profiles in vestibular neuritis and Ménière's disease. VEMPs are valuable in the pre-surgical confirmation of superior semicircular canal dehiscence and in some cases, may alert the clinician to the presence of a vestibular schwannoma in patients with symmetrical hearing.

Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes).

The triad of central nervous system symptoms, visual disturbance and hearing impairment is an oft-encountered clinical scenario. A number of immune-mediated diseases should be considered among the differential diagnoses including: Susac syndrome, Cogan syndrome or Vogt-Koyanagi-Harada disease; demyelinating conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder; systemic diseases such as systemic lupus erythematosus, Sjögren syndrome or Behcet disease and granulomatous diseases such as sarcoidosis. In this article, we coin the term 'BEE syndromes' to draw attention to the various immune-mediated diseases that affect the brain, eye and ear. We present common disease manifestations and identify key clinical and investigation features.

Acute unilateral peripheral vestibulopathy in neurosyphilis.

INTRODUCTION: Neurosyphilis producing basal meningitis presenting as sequential transient cranial nerve palsies was well recognized before the antibiotic era. OBJECTIVE: To report two patients presenting with acute unilateral peripheral vestibulopathy due to syphilitic basal meningitis. RESULTS: In Case 1 basal meningitis occurred early in the secondary phase of the infection, in Case 2 in the late latent phase. The diagnosis was not made immediately in either case; in Case 1 after previous presentation with increasing hearing loss and then with facial palsy and then a subsequent presentation with optic neuritis; in Case 2 after investigation for possible lymphoma. CONCLUSION: Syphilitic basal meningitis in either the secondary or in the latent phase can present as acute unilateral peripheral vestibulopathy with transient involvement of the facial or auditory nerve.

Vertigo with sudden hearing loss: audio-vestibular characteristics.

Acute vertigo with sudden sensorineural hearing loss (SSNHL) is a rare clinical emergency. Here, we report the audio-vestibular test profiles of 27 subjects who presented with these symptoms. The vestibular test battery consisted of a three-dimensional video head impulse test (vHIT) of semicircular canal function and recording ocular and cervical vestibular-evoked myogenic potentials (oVEMP, cVEMP) to test otolith dysfunction. Unlike vestibular neuritis, where the horizontal and anterior canals with utricular function are more frequently impaired, 74 % of subjects with vertigo and SSNHL demonstrated impairment of the posterior canal gain (0.45 ± 0.20). Only 41 % showed impairment of the horizontal canal gains (0.78 ± 0.27) and 30 % of the anterior canal gains (0.79 ± 0.26), while 38 % of oVEMPs [asymmetry ratio (AR) = 41.0 ± 41.3 %] and 33 % of cVEMPs (AR = 47.3 ± 41.2 %) were significantly asymmetrical. Twenty-three subjects were diagnosed with labyrinthitis/labyrinthine infarction in the absence of evidence for an underlying pathology. Four subjects had a definitive diagnosis [Ramsay Hunt Syndrome, vestibular schwannoma, anterior inferior cerebellar artery (AICA) infarction, and traction injury]. Ischemia involving the common-cochlear or vestibulo-cochlear branches of the labyrinthine artery could be the simplest explanation for vertigo with SSNHL. Audio-vestibular tests did not provide easy separation between ischaemic and non-ischaemic causes of vertigo with SSNHL.

Prevalence of vestibular dysfunction in patients with vestibular schwannoma using video head-impulses and vestibular-evoked potentials.

We sought to investigate the utility of new non-invasive tests of semicircular-canal and otolith function that are usable in the neuro-otology office practice in patients with vestibular schwannoma. Fifty patients with vestibular schwannoma were assessed using a 5-item battery consisting of air-conducted cervical- and bone conducted ocular-vestibular-evoked myogenic potentials (AC cVEMPs and BC oVEMPs) and video head impulse testing (vHIT) in all three canal planes. VEMP asymmetry ratios, latencies, and vHIT gains were used to determine the test sensitivity, relationship with tumour size and the pattern of vestibular nerve involvement. The percentage of abnormalities for each of the five tests for the entire sample ranged between 36.2-61.7%. In 58.3 % of patients, test abnormalities were referable to both superior and inferior vestibular nerve divisions. Selective inferior nerve dysfunction was identified in 10.4% and superior nerve dysfunction in 12.5%. The remaining 18.8% of patients demonstrated a normal test profile. The sensitivity of the 5-item battery increased with tumour size and all patients with medium to large (>14 mm) schwannoma had at least two abnormal vestibular test result. Our results indicate that dysfunction of the superior and inferior vestibular nerve evolves in parallel for most patients with schwannoma. Unexplained vHIT and VEMP asymmetry should alert otologists and neurologists to undertake imaging in patients presenting with non-specific disequilibrium or vertigo.

Lhermitte-Duclos disease presenting with atypical positional nystagmus.

We describe a patient with dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) who presented with an acute onset of positional disequilibrium. Video-oculography in the right Hallpike position revealed rightward torsional down-beat nystagmus, initially thought to be right anterior canal benign positional vertigo. However, the presence of spontaneous nystagmus, the persistent character of the positional nystagmus and the absence of fatigability indicated central positional nystagmus, attributable to his right-sided Lhermitte-Duclos disease. These findings emphasise the need for clinicians to reconsider a central cause before diagnosing the rare anterior canal benign positioning vertigo variant.

Causes and characteristics of horizontal positional nystagmus.

Direction changing horizontal positional nystagmus can be observed in a variety of central and peripheral vestibular disorders. We tested sixty subjects with horizontal positional nystagmus and vertigo on the Epley Omniax(®) rotator. Monocular video recordings were performed with the right or left ear down, in the supine and prone positions. Nystagmus slow-phase velocity (SPV) was plotted as a function of time. Thirty-one subjects diagnosed with horizontal canalolithiasis had paroxysmal horizontal geotropic nystagmus with the affected ear down (onset 0.8 ± 1 s, range 0-4.9 s, duration 11.7-47.9 s, peak SPV 79 ± 67°/s). The SPV peaked at 5-20 s and declined to 0 by 60 s; at 40 s from onset, the average SPV was 1.8 % of the peak. Nine subjects diagnosed with cupulolithiasis had persistent apogeotropic horizontal nystagmus (onset 0.7 ± 1.4 s, range 0-4.3 s). Peak SPV was 54.2 ± 31.8°/s and 26.6 ± 12.2°/s with unaffected and affected ears down, respectively. At 40 s, the average SPV had decayed to only 81 % (unaffected ear down) and 65 % (affected ear down) of the peak. Twenty subjects were diagnosed with disorders other than benign positional vertigo (BPV) [vestibular migraine (VM), Ménière's Disease, vestibular schwannoma, unilateral or bilateral peripheral vestibular loss]. Subjects with VM (n = 13) had persistent geotropic or apogeotropic horizontal nystagmus. On average, at 40 s from nystagmus onset, the SPV was 61 % of the peak. Two patients with Ménière's Disease had persistent apogeotropic horizontal nystagmus; the peak SPV at 40 s ranged between 28.6 and 49.5 % of the peak. Symptomatic horizontal positional nystagmus can be observed in canalolithiasis, cupulolithiasis and diverse central and peripheral vestibulopathies; its temporal and intensity profile could be helpful in the separation of these entities.

Asymmetry of balance responses to monaural galvanic vestibular stimulation in subjects with vestibular schwannoma.

OBJECTIVE: We investigated the potential of galvanic vestibular stimulation (GVS) to quantify lateralised asymmetry of the vestibulospinal pathways by measuring balance responses to monaural GVS in 10 subjects with vestibular schwannoma and 22 healthy control subjects. METHODS: Subjects standing without vision were stimulated with 3 s, 1 mA direct current stimuli delivered monaurally. The mean magnitude and direction of the evoked balance responses in the horizontal plane were measured from ground-reaction forces and from displacement and velocity of the trunk. Vestibular-evoked myogenic potentials (VEMPs) to 500 Hz air and bone-conducted tones were also recorded. RESULTS: In healthy subjects, the magnitudes of the force, velocity and displacement responses were not significantly different for left compared to right ear stimulation. Their individual asymmetry ratios were always < 30%. Subjects with vestibular schwannoma had significantly smaller force, velocity and displacement responses to stimulation of the affected compared with non-affected ear. Their mean asymmetry ratios were significantly elevated for all three measures (41.2 ± 10.3%, 40.3 ± 15.1% and 21.9 ± 14.6%). CONCLUSIONS: Asymmetry ratios of balance responses to monaural GVS provide a quantitative and clinically applicable lateralising test of the vestibulospinal pathways. SIGNIFICANCE: This method offers a more clinically relevant measure of standing balance than existing vestibular function tests which assess only vestibuloocular and vestibulocollic pathways.

Vestibular schwannoma mimicking horizontal cupulolithiasis.

Positional vertigo and nystagmus can be due to canalithiasis, cupulolithiasis and less commonly, an underlying peripheral or central vestibular disorder. We present a patient with vestibular schwannoma who initially sought treatment for positioning vertigo. Video-oculography on the roll-test revealed direction-changing horizontal apogeotropic nystagmus, consistent with horizontal cupulolithiasis. However, further audio-vestibular investigations and imaging confirmed a right vestibulopathy attributable to a schwannoma of the right vestibular nerve. This case report suggests that vestibular schwannoma should be considered as another potential mimicker of horizontal cupulolithiasis.

Ocular versus cervical VEMPs in the diagnosis of superior semicircular canal dehiscence syndrome.

OBJECTIVES: To determine whether cervical vestibular evoked myogenic potential (cVEMP) thresholds or ocular VEMP (oVEMP) amplitudes are more sensitive and specific in the diagnosis of superior semicircular canal dehiscence syndrome (SCDS). STUDY DESIGN: Prospective case-control study. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Twenty-nine patients with SCDS (mean age 48 yr; range, 31-66 yr) and 25 age-matched controls (mean age 48 yr; range, 30-66 yr). INTERVENTION(S): cVEMP and oVEMP in response to air-conducted sound. All patients underwent surgery for repair of SCDS. MAIN OUTCOME MEASURE(S): cVEMP thresholds; oVEMP n10 and peak-to-peak amplitudes. RESULTS: cVEMP threshold results showed sensitivity and specificity ranging from 80% to 100% for the diagnosis of SCDS. In contrast, oVEMP amplitudes demonstrated sensitivity and specificity greater than 90%. CONCLUSION: oVEMP amplitudes are superior to cVEMP thresholds in the diagnosis of SCDS.

Waiting for the evidence: VEMP testing and the ability to differentiate utricular versus saccular function.

The advent of cervical vestibular evoked myogenic potentials (CVEMPs) marked a milestone in clinical vestibular testing because they provided a simple means of assessing human otolith function. The availability of air-conducted (AC) sound and bone-conducted vibration (BCV) to evoke CVEMPs and development of a new technique of recording ocular vestibular-evoked myogenic potentials (OVEMPs) have increased the complexity of this simple test, yet extended its diagnostic capabilities. Here we highlight the evidence-based assumptions that guide interpretation of AC sound- and BCV-evoked VEMPs and the gaps in VEMP research thus far.

Vestibular-evoked myogenic potential thresholds normalize on plugging superior canal dehiscence.

BACKGROUND: Diagnosis of the superior canal dehiscence syndrome (SCDS) relies on symptoms such as sound- or pressure-induced vertigo or oscillopsia, demonstration of sound or pressure-evoked vertical/torsional eye movements, and the presence of a defect in the bony roof overlying the superior semicircular canal. Lowered thresholds for eliciting vestibular-evoked myogenic potentials (VEMPs) provide additional conformation. OBJECTIVE: To examine VEMP characteristics before and after canal plugging for SCDS. METHODS: VEMPs evoked by air- and bone-conducted tones were measured from the sternocleidomastoid muscles (cVEMP) and periocular sites (oVEMP) of 20 normal volunteers, 10 newly diagnosed subjects with SCDS, and 12 subjects who underwent successful superior canal plugging. RESULTS: In all SCDS ears, thresholds for evoking VEMP using air-conducted tones were pathologically lowered, with average values of 83.85 +/- 1.40 dB sound pressure level (SPL) for cVEMP and 85.38 +/- 1.32 dB SPL for oVEMP, 20 to 30 dB below those of controls. Successful canal plugging resulted in normal reflex thresholds. For bone vibration, average thresholds in SCDS ears were 114.62 +/- 1.54 dB FL (force level) for cVEMP and 116.0 +/- 1.52 dB FL for oVEMP, 10 to 20 dB below controls, yet three SCDS ears had normal thresholds. CONCLUSIONS: Ocular and cervical vestibular-evoked myogenic potentials evoked by air-conducted sound are equally useful in the diagnosis and follow-up of superior canal dehiscence syndrome. Stimulus thresholds are consistently lowered upon presentation and normalize after corrective surgery. Thresholds for bone vibration, in contrast, have a lower diagnostic yield.