Machine learning models help differentiate between causes of recurrent spontaneous vertigo.

BACKGROUND: Vestibular migraine (VM) and Menière's disease (MD) are two common causes of recurrent spontaneous vertigo. Using history, video-nystagmography and audiovestibular tests, we developed machine learning models to separate these two disorders. METHODS: We recruited patients with VM or MD from a neurology outpatient facility. One hundred features from six "feature subsets": history, acute video-nystagmography and four laboratory tests (video head impulse test, vestibular-evoked myogenic potentials, caloric testing and audiogram) were used. We applied ten machine learning algorithms to develop classification models. Modelling was performed using three "tiers" of data availability to simulate three clinical settings. "Tier 1" used all available data to simulate the neuro-otology clinic, "Tier 2" used only history, audiogram and caloric test data, representing the general neurology clinic, and "Tier 3" used history alone as occurs in primary care. Model performance was evaluated using tenfold cross-validation. RESULTS: Data from 160 patients with VM and 114 with MD were used for model development. All models effectively separated the two disorders for all three tiers, with accuracies of 85.77-97.81%. The best performing algorithms (AdaBoost and Random Forest) yielded accuracies of 97.81% (95% CI 95.24-99.60), 94.53% (91.09-99.52%) and 92.34% (92.28-96.76%) for tiers 1, 2 and 3. The best feature subset combination was history, acute video-nystagmography, video head impulse test and caloric testing, and the best single feature subset was history. CONCLUSIONS: Machine learning models can accurately differentiate between VM and MD and are promising tools to assist diagnosis by medical practitioners with diverse levels of expertise and resources.

Post Cochlear Implantation Vertigo: Ictal Nystagmus and Audiovestibular Test Characteristics.

OBJECTIVE: To investigate ictal nystagmus and audiovestibular characteristics in episodic spontaneous vertigo after cochlear implantation (CI). STUDY DESIGN: Retrospective and prospective case series. PATIENTS: Twenty-one CI patients with episodic spontaneous vertigo after implantation were recruited. INTERVENTIONS: Patient-initiated home video-oculography recordings were performed during one or more attacks of vertigo, using miniature portable home video-glasses. To assess canal and otolith function, video head-impulse tests (vHITs) and vestibular-evoked myogenic potential tests were conducted. MAIN OUTCOME MEASURES: Nystagmus slow-phase velocities (SPVs), the presence of horizontal direction-changing nystagmus, and post-CI audiovestibular tests. RESULTS: Main final diagnoses were post-CI secondary endolymphatic hydrops (48%) and exacerbation of existing Ménière's disease (29%). Symptomatic patients demonstrated high-velocity horizontal ictal-nystagmus (SPV, 44.2°/s and 68.2°/s in post-CI secondary endolymphatic hydrop and Ménière's disease). Direction-changing nystagmus was observed in 80 and 75%. Two were diagnosed with presumed autoimmune inner ear disease (SPV, 6.6°/s and 172.9°/s). One patient was diagnosed with probable vestibular migraine (15.1°/s).VHIT gains were 0.80 ± 0.20 (lateral), 0.70 ± 0.17 (anterior), and 0.62 ± 0.27 (posterior) in the implanted ear, with abnormal values in 33, 35, and 35% of each canal. Bone-conducted cervical and ocular vestibular-evoked myogenic potentials were asymmetric in 52 and 29% of patients (all lateralized to the implanted ear) with mean asymmetry ratios of 51.2 and 35.7%. Reversible reduction in vHIT gain was recorded in three acutely symptomatic patients. CONCLUSION: High-velocity, direction-changing nystagmus time-locked with vertigo attacks may be observed in post-CI implant vertigo and may indicate endolymphatic hydrops. Fluctuating vHIT gain may be an additional marker of a recurrent peripheral vestibulopathy.

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.

Modeling the effect of gravity on periodic alternating nystagmus.

Periodic alternating nystagmus (PAN) is a rare oscillatory ocular motor disorder. The effects of gravity on the dynamic behavior of PAN can be studied by monitoring the nystagmus while changing head orientation. Previous studies of patients with PAN reached different conclusions about the effect of changing the orientation of the head relative to gravity on the ongoing PAN, either no effect or a damping of the nystagmus within several minutes. What neuronal circuits could account for the difference in the effects of gravity among PAN patients? We modeled how the brain resolves the tilt-translation ambiguity in normal individuals and added an unstable, oscillatory vestibular system generating PAN. PAN was suppressed in our patient in ear-down positions, in a similar pattern to that of a previously reported patient. This effect was simulated by reducing the gain of the projection of the "rotation feedback" loop to the velocity-storage integrator to approximately 5% of its normal value. With normal "rotation feedback" PAN is expected to dissipate quickly as soon as the head is rotated away from upright position. Moreover, by disconnecting the rotation feedback completely (gain = zero) the model simulated PAN that was reported to be unaffected by gravity. Thus, understanding the effect of this single parameter, the gain of the rotation feedback, can explain the observed variability among our own and previous studies.

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.

A Portrait of Menière's Disease Using Contemporary Hearing and Balance Tests.

OBJECTIVE: Menière's disease (MD) is characterized by recurrent vertigo and fluctuating aural symptoms. Diagnosis is straightforward in typical presentations, but a proportion of patients present with atypical symptoms. Our aim is to profile the array of symptoms patients may initially present with and to analyze the vestibular and audiological test results of patients with a diagnosis of MD. DESIGN: A retrospective study of patient files. SETTING: A tertiary, neuro-otology clinic Royal Prince Alfred Hospital, Sydney, Australia. METHOD: We identified 375 patients. Their history, examination, vestibular-evoked myogenic potentials (VEMP), video head-impulse test, canal-paresis on caloric testing, subjective visual horizontal (SVH), electrocochleography, ictal nystagmus, and audiometry were assessed. RESULTS: Atypical presenting symptoms were disequilibrium (n = 49), imbalance (n = 13), drop-attacks (n = 12), rocking vertigo (n = 2), and unexplained vomiting (n = 3), nonspontaneous vestibular symptoms in 21.6%, fluctuation of aural symptoms only (46%), and headaches (31.2%). Low velocity, interictal spontaneous-nystagmus in 13.3% and persistent positional-nystagmus in 12.5%. Nystagmus recorded ictally in 90 patients was mostly horizontal (93%) and of high velocity (48 ±â€Š34°/s). Testing yielded abnormal caloric responses in 69.6% and abnormal video head impulse test 12.7%. Air-conducted cervical VEMPs were abnormal in 32.2% (mean asymmetry ratio [AR] 30.2 ±â€Š46.5%) and bone-conducted ocular VEMPs abnormal in 8.8% (AR 11.2 ±â€Š26.8%). Abnormal interictal SVH was in 30.6%, (ipsiversive n = 46 and contraversive n = 19). Mean pure-tone averages 50 dB ±â€Š23.5 and 20 dB ±â€Š13 for affected and unaffected ears. CONCLUSION: Menière's disease has a distinctive history, but atypical presentations with normal vestibular function and hearing are a diagnostic challenge delaying treatment initiation.

Capturing vertigo in the emergency room: three tools to double the rate of diagnosis.

OBJECTIVE: Many patients attending the emergency room (ER) with vertigo, leave without a diagnosis. We assessed whether the three tools could improve ER diagnosis of vertigo. METHODS: A prospective observational study was undertaken on 539 patients presenting to ER with vertigo. We used three tools: a structured-history and examination, nystagmus video-oculography (VOG) in all patients, additional video head-impulse testing (vHIT) for acute-vestibular-syndrome (AVS). RESULTS: In the intervention-group (n = 424), case-history classified AVS in 34.9%, episodic spontaneous-vertigo (ESV 32.1%), and episodic positional-vertigo (EPV 22.6%). In AVS, we employed "Quantitative-HINTS plus" (Head-Impulse, Nystagmus and Test-of-Skew quantified by vHIT and VOG, audiometry) to identify vestibular-neuritis (VN) and stroke (41.2 and 31.1%). vHIT gain ≤ 0.72, catch-up saccade amplitude > 1.4○, saccade-frequency > 154%, and unidirectional horizontal-nystagmus, separated stroke from VN with 93.1% sensitivity and 88.5% specificity. In ESV, 66.2 and 14% were diagnosed with vestibular migraine and Meniere's Disease by using history and audiometry. Horizontal-nystagmus velocity was lower in migraine 0.4 ± 1.6○/s than Meniere's 5.7 ± 5.5○/s (p < 0.01). In EPV, benign positional vertigo (BPV) was identified in 82.3% using VOG. Paroxysmal positional-nystagmus lasting < 60 s separated BPV from non-BPV with 90% sensitivity and 100% specificity. In the control group of ER patients undergoing management-as-usual (n = 115), diagnoses included BPV (38.3%) and non-specific vertigo (41.7%). Unblinded assessors reached a final diagnosis in 90.6 and 30.4% of the intervention and control groups. Blinded assessors provided with the data gathered from each group reached a diagnosis in 86.3 and 41.1%. CONCLUSION: Three tools: a structured-assessment, vHIT and VOG doubled the rate of diagnosis in the ER.

Clinical, oculographic and vestibular test characteristics of Ménière's disease.

Seventy Ménière's disease (MD) patients with spontaneous vertigo (100%), unilateral aural fullness (57.1%), tinnitus (78.6%), and subjective hearing loss (75.7%) self-recorded nystagmus during their episodes of vertigo using portable video oculography goggles. All demonstrated ictal spontaneous nystagmus, horizontal in 94.3% (n = 66) and vertical in 5.7% (n = 4), with a mean slow-phase velocity (SPV) of 42.8 ± 31.1°/s (range 5.3-160.1). Direction reversal of spontaneous horizontal nystagmus was captured in 58.6%, within the same episode in 34.3%, and over different days in 24.3%. In 18.6%, we observed ipsiversive then contraversive nystagmus, and in 12.9% contraversive to ipsiversive direction reversal. Ictal nystagmus SPV (42.8 ± 31.1°/s) was significantly faster than interictal (1.4 ± 3.1°/s, p < 0.001, CI 34.277-48.776). Compared to age-matched healthy controls, interictal video head impulse test gains in MD ears were significantly lower, cumulative and first saccade (S1) amplitudes were significantly larger, and S1 peak velocities were significantly faster (p = 0.038/0.019/0.008/ < 0.001, CI 0.002-0.071/0.130-1.444/0.138-0.909/14.614-41.506). Audiometry showed asymmetrically increased thresholds in 100% of MD ears (n = 70). Significant caloric, air-conducted (AC) cervical vestibular-evoked myogenic potential (VEMP), and AC ocular VEMP asymmetries were found in 61.4, 37.9, and 44.4% of patients (MD ear reduced). Transtympanic electrocochleography tested in 36 ears (23 patients) showed 81.8% of MD ears had a positive result for hydrops (either a summating potential at 1/2 kHz < - 6 µV, or an SP/AP ratio > 40%). Using ictal nystagmus findings of SPV > 12°/s, and a caloric canal paresis > 25%, we correctly separated a diagnosis MD from Vestibular Migraine with a sensitivity and specificity of 95.7% and 85.1% (CI 0.89-0.97).

Clinical, oculographic, and vestibular test characteristics of vestibular migraine.

BACKGROUND: We characterise the history, vestibular tests, ictal and interictal nystagmus in vestibular migraine. METHOD: We present our observations on 101 adult-patients presenting to an outpatient facility with recurrent spontaneous and/or positional vertigo whose final diagnosis was vestibular migraine (n = 27) or probable vestibular migraine (n = 74). Ictal and interictal video-oculography, caloric and video head impulse tests, vestibular-evoked myogenic potentials and audiometry were performed. RESULTS: Common presenting symptoms were headache (81.2%), spinning vertigo (72.3%), Mal de Débarquement (58.4%), and motion sensitivity (30.7%). With fixation denied, ictal and interictal spontaneous nystagmus was observed in 71.3 and 14.9%, and purely positional nystagmus in 25.8 and 55.4%. Spontaneous ictal nystagmus was horizontal in 49.5%, and vertical in 21.8%. Ictal spontaneous and positional nystagmus velocities were 5.3 ± 9.0°/s (range 0.0-57.4), and 10.4 ± 5.8°/s (0.0-99.9). Interictal spontaneous and positional nystagmus velocities were <3°/s in 91.8 and 23.3%. Nystagmus velocities were significantly higher when ictal (p < 0.001/confidence interval: 2.908‒6.733, p < 0.001/confidence interval: 5.308‒10.085). Normal lateral video head impulse test gains were found in 97.8% (mean gain 0.95 ± 0.12) and symmetric caloric results in 84.2% (mean canal paresis 7.0 ± 23.3%). Air- and bone-conducted cervical-vestibular-evoked myogenic potential amplitudes were symmetric in 88.4 and 93.4% (mean corrected amplitude 1.6 ± 0.7, 1.6 ± 0.8) with mean asymmetry ratios of 13.0 and 9.0%. Air- and bone-conducted ocular-vestibular-evoked myogenic potentials were symmetric in 67.7 and 97.2% (mean amplitude 9.2 ± 6.4 and 20.3 ± 12.8 µV) with mean asymmetry ratios of 15.7 and 9.9%. Audiometry was age consistent and symmetric in 85.5%. CONCLUSION: Vestibular migraine is characterised by low velocity ictal spontaneous nystagmus, which can be horizontal, vertical, or torsional, and normal audiovestibular test results.

Modeling the interaction among three cerebellar disorders of eye movements: periodic alternating, gaze-evoked and rebound nystagmus.

A woman, age 44, with a positive anti-YO paraneoplastic cerebellar syndrome and normal imaging developed an ocular motor disorder including periodic alternating nystagmus (PAN), gaze-evoked nystagmus (GEN) and rebound nystagmus (RN). During fixation there was typical PAN but changes in gaze position evoked complex, time-varying oscillations of GEN and RN. To unravel the pathophysiology of this unusual pattern of nystagmus, we developed a mathematical model of normal function of the circuits mediating the vestibular-ocular reflex and gaze-holding including their adaptive mechanisms. Simulations showed that all the findings of our patient could be explained by two, small, isolated changes in cerebellar circuits: reducing the time constant of the gaze-holding integrator, producing GEN and RN, and increasing the gain of the vestibular velocity-storage positive feedback loop, producing PAN. We conclude that the gaze- and time-varying pattern of nystagmus in our patient can be accounted for by superposition of one model that produces typical PAN and another model that produces typical GEN and RN, without requiring a new oscillator in the gaze-holding system or a more complex, nonlinear interaction between the two models. This analysis suggest a strategy for uncovering gaze-evoked and rebound nystagmus in the setting of a time-varying nystagmus such as PAN. Our results are also consistent with current ideas of compartmentalization of cerebellar functions for the control of the vestibular velocity-storage mechanism (nodulus and ventral uvula) and for holding horizontal gaze steady (the flocculus and tonsil).

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.

Bone-Conducted oVEMP Latency Delays Assist in the Differential Diagnosis of Large Air-Conducted oVEMP Amplitudes.

Background: A sensitive test for Superior Semicircular Canal Dehiscence (SCD) is the air-conducted, ocular vestibular evoked myogenic potential (AC oVEMP). However, not all patients with large AC oVEMPs have SCD. This retrospective study sought to identify alternate diagnoses also producing enlarged AC oVEMPs and investigated bone-conducted (BC) oVEMP outcome measures that would help differentiate between these, and cases of SCD. Methods: We reviewed the clinical records and BC oVEMP results of 65 patients (86 ears) presenting with dizziness or balance problems who underwent CT imaging to investigate enlarged 105 dB nHL click AC oVEMP amplitudes. All patients were tested with BC oVEMPs using two different stimuli (1 ms square-wave pulse and 8 ms 125 Hz sine wave). Logistic regression and odds ratios were used to determine the efficacy of BC oVEMP amplitudes and latencies in differentiating between enlarged AC oVEMP amplitudes due to dehiscence from those with an alternate diagnosis. Results: Fifty-three ears (61.6%) with enlarged AC oVEMP amplitudes were identified as having frank dehiscence on imaging; 33 (38.4%) had alternate diagnoses that included thinning of the bone covering (near dehiscence, n = 13), vestibular migraine (n = 12 ears of 10 patients), enlarged vestibular aqueduct syndrome (n = 2) and other causes of recurrent episodic vertigo (n = 6). BC oVEMP amplitudes of dehiscent and non-dehiscent ears were not significantly different (p > 0.05); distributions of both groups overlapped with the range of healthy controls. There were significant differences in BC oVEMP latencies between dehiscent and non-dehiscent ears for both stimuli (p < 0.001). A prolonged n1 125 Hz latency (>11.5 ms) was the best predictor of dehiscence (odd ratio = 27.8; 95% CI:7.0-111.4); abnormal n1 latencies were identified in 79.2% of ears with dehiscence compared with 9.1% of ears without dehiscence. Conclusions: A two-step protocol of click AC oVEMP amplitudes and 125 Hz BC oVEMP latency measures optimizes the specificity of VEMP testing in SCD.

Nystagmus goggles: how to use them, what you find and what it means.

A fundamental characteristic of peripheral vestibular nystagmus, in particular horizontal nystagmus, is that it is suppressed by visual fixation. This means that a patient with a vertigo attack of peripheral vestibular origin might have no obvious spontaneous nystagmus on clinical examination. Goggles that reduce or remove visual fixation allow the cliniican to observe nystagmus in this situation. Nystagmus goggles are essential for any clinician dealing with dizzy patients. Here, we discuss why this is so and how easy it is to acquire and use them.

The neuro-otology of Susac syndrome.

OBJECTIVE: We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. METHODS: The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vestibular function tests, treatment and outcomes. RESULTS: Our findings demonstrate that 29 of our 30 patients with Susac syndrome developed neuro-otological symptoms such as hearing loss, disequilibrium, tinnitus or vertigo during their disease course. Hearing loss was the most common neuro-otological symptom occurring in 93% of patients. A rising configuration of low-frequency greater than the high-frequency sensorineural hearing loss was the most characteristic finding on audiological testing (37% of reviewed audiograms). Disproportionately poor speech discrimination was identified in 20% of cases, and one case demonstrated a retrocochlear pattern on electrophysiological testing. Four patients required hearing aids and a further two patients required a cochlear implant due to severe hearing loss. Two out of two treated patients had improvements in hearing after the prompt administration of corticosteroids, indicating the potential for recoverable hearing loss if relapses are treated early. Effects on vestibular function were variable in ten patients who were tested, with most showing preservation of function despite significant hearing loss. CONCLUSIONS: Neuro-otological symptoms in Susac syndrome are almost universal. In the correct clinical context, a rising configuration of low to high-frequency sensorineural hearing loss should prompt consideration of Susac syndrome. Treatment of inner ear symptoms in Susac syndrome requires further research as early immunotherapy may be beneficial.

Contralesional subjective visual horizontal predicts endolymphatic hydrops.

Background: The subjective visual horizontal (SVH) is a test of utricular function that assesses conjugate ocular torsion which is a component of the ocular tilt reaction (OTR). In unilateral destructive peripheral vestibular lesions, the OTR and so the SVH tilt is usually ipsiversive.Aims/objective: Our study aimed to profile the causes of a contraversive SVH tilt in patients with a confirmed unilateral peripheral vestibular deficit.Materials and methods: The clinical records, nystagmus and vestibular investigation characteristics of 52 patients with a unilateral canal paresis (CP) on caloric of ≥30%, a contraversive SVH tilt of ≥4 degrees and at least one pure tone audiometry were retrospectively analysed.Results: The most common diagnosis of patients (n = 39) with a contraversive SVH and ipsilesional CP was endolymphatic hydrops: 35 (67.3%) had Meniere's disease (MD) and 4 (7.7%) had delayed endolymphatic hydrops (DEH). The remaining 13 (25%) of cases had other peripheral aetiologies or an unknown diagnosis. 16 (30.8%) patients had ictal spontaneous nystagmus at the time of SVH or caloric testing.Conclusions and significance: A contraversive SVH with unilateral CP suggests endolymphatic hydrops.

Video Head Impulse Testing: From Bench to Bedside.

Over 30 years ago, the head impulse test (HIT) was measured with search coil recordings and it provided robust evidence for a new test of vestibular function that could detect impairment of a single semicircular canal, that is, the lateral canal. Over the next two decades, the diagnostic spectrum of HIT was expanded to the testing of vertical canals, differentiation of central from peripheral vestibulopathy, and incorporation of visual interaction-the suppressed head impulse. However, HIT measurement was limited to very few specialized laboratories that were able to maintain the time-consuming and expensive operation of the scleral search coil system, which is the gold standard in eye movement recording. The video HIT (vHIT) was validated for the first time over 10 years ago, against the search coils, and its introduction into dizzy clinics worldwide has revolutionized the practice of neuro-otology. Here we review the basic physiology, practical aspects, and clinical application of the vHIT.