Historical descriptions of nystagmus and abnormal involuntary eye movements in various ancient cultures.

Original texts and expert translations from various ancient cultures covering a time span from the 2nd millennium BC to the ninth century AD were searched for historical observations of involuntary eye movements. Abnormal, spontaneous eye movements are an easily recognisable neuro-ophthalmological symptom that can be both congenital and acquired. Ocular oscillations termed 'hippos' by Hippocrates (460-370BC) and Galenos (129-216AD) are synonymous with nystagmus, a term first introduced in the eighteenth century. The original description of hippos suggests an innate onset, which retrospectively can be related to either congenital (infantile) nystagmus or continuous involuntary eye movements of the blind. Other descriptions of abnormal involuntary eye movements with different beating directions, possibly associated with vertigo, seizures or ear symptoms and their impact on patients' quality of life (e.g. oscillopsia, blurred vision) are preserved in many fragmentary ancient documents including papyrus scrolls and stone tablets from Egypt, Mesopotamia, India, China, Greece, Rome and the Middle East. Although the sparse original descriptions of the direction and type of eye movements may inspire daring medical interpretations, caution is required when attempting to assign them to distinct nystagmus forms according to our current clinical classification of ocular motor disorders.

Differential effects of gender and age on dynamic subjective visual vertical.

In a retrospective study, the data of direction-dependent deviations in dynamic subjective visual vertical (SVV) testing were analysed in 1811 dizzy patients (174 benign paroxysmal positional vertigo, 99 unilateral vestibulopathy, 67 bilateral vestibulopathy, 151 Menière's disease, 375 vestibular migraine, 82 cerebellar disorder, 522 functional dizziness, 341 unclear diagnosis) and in 59 healthy controls. Major findings were (i) a significant gender difference with higher directional deviations in females over the entire range of age, (ii) a significant increase of directional deviations with increasing age for both genders and in all disease subgroups as well as in healthy controls, and (iii) a lack of significant difference of directional deviations between all tested diseases. Thus, the data allow no recommendation for performing additional angular deviation analysis in dynamic SVV testing as part of routine clinical management of dizzy patients. However, as shown in earlier longitudinal studies, it still appears reasonable that dynamic SVV in acute rather than chronic vestibular disorders may provide a useful instrument for the monitoring of acute unilateral vestibular tonus imbalances in the course of the disease.

The Semont-Plus Maneuver or the Epley Maneuver in Posterior Canal Benign Paroxysmal Positional Vertigo: A Randomized Clinical Study.

Importance: Questions remain concerning treatment efficacy for the common condition of benign paroxysmal positional vertigo (BPPV). Objective: To compare the effectiveness of the Semont-plus maneuver (SM-plus) and the Epley maneuver (EM) for treatment of posterior canal benign paroxysmal positional vertigo (pcBPPV) canalolithiasis. Design, Setting, and Participants: This prospective randomized clinical trial was performed at 3 national referral centers (in Munich, Germany; Siena, Italy; and Bruges, Belgium) over 2 years, with a follow-up to 4 weeks after the initial examination. Recruitment took place from June 1, 2020, until March 10, 2022. Patients were selected randomly during routine outpatient care after being referred to 1 of the 3 centers. Two hundred fifty-three patients were assessed for eligibility. After consideration of the exclusion criteria as well as informed consent, 56 patients were excluded and 2 declined to participate, with 195 participants included in the final analysis. The analysis was prespecified and per-protocol. Interventions: After being randomized to the SM-plus or the EM group, patients received 1 initial maneuver from a physician, then subsequently performed self-maneuvers at home 3 times in the morning, 3 times at noon, and 3 times in the evening. Main Outcome and Measures: Patients had to document whether they could provoke positional vertigo every morning. The primary end point was the number of days until no positional vertigo could be induced on 3 consecutive mornings. The secondary end point was the effect of the single maneuver performed by the physician. Results: Of the 195 participants included in the analysis, the mean (SD) age was 62.6 (13.9) years, and 125 (64.1%) were women. The mean (SD) time until no positional vertigo attacks could be induced in the SM-plus group was 2.0 (1.6) days (median, 1 [range, 1-8] day; 95% CI, 1.64-2.28 days); in the EM group, 3.3 (3.6) days (median, 2 [range, 1-20] days; 95% CI, 2.62-4.06 days) (P = .01; α = .05, 2-tailed Mann-Whitney test). For the secondary end point (effect of a single maneuver), no significant difference was detected (67 of 98 [68.4%] vs 61 of 97 [62.9%]; P = .42; α = .05). No serious adverse event was detected with both maneuvers. Nineteen patients (19.6%) in the EM group and 24 (24.5%) in the SM-plus group experienced relevant nausea. Conclusions and Relevance: The SM-plus self-maneuver is superior to the EM self-maneuver in terms of the number of days until recovery in pcBPPV. Trial Registration: ClinicalTrials.gov Identifier: NCT05853328.

Vestibular paroxysmia entails vestibular nerve function, microstructure and endolymphatic space changes linked to root-entry zone neurovascular compression.

Combining magnetic resonance imaging (MRI) sequences that permit the determination of vestibular nerve angulation (NA = change of nerve caliber or direction), structural nerve integrity via diffusion tensor imaging (DTI), and exclusion of endolymphatic hydrops (ELH) via delayed gadolinium-enhanced MRI of the inner ear (iMRI) could increase the diagnostic accuracy in patients with vestibular paroxysmia (VP). Thirty-six participants were examined, 18 with VP (52.6 ± 18.1 years) and 18 age-matched with normal vestibulocochlear testing (NP 50.3 ± 16.5 years). This study investigated whether (i) NA, (ii) DTI changes, or (iii) ELH occur in VP, and (iv) to what extent said parameters relate. Methods included vestibulocochlear testing and MRI data analyses for neurovascular compression (NVC) and NA verification, DTI and ELS quantification. As a result, (i) NA increased NVC specificity. (ii) DTI structural integrity was reduced on the side affected by VP (p < 0.05). (iii) 61.1% VP showed mild ELH and higher asymmetry indices than NP (p > 0.05). (iv) "Disease duration" and "total number of attacks" correlated with the decreased structural integrity of the affected nerve in DTI (p < 0.001). NVC distance within the nerve's root-entry zone correlated with nerve function (Roh = 0.72, p < 0.001), nerve integrity loss (Roh = - 0.638, p < 0.001), and ELS volume (Roh = - 0.604, p < 0.001) in VP. In conclusion, this study is the first to link eighth cranial nerve function, microstructure, and ELS changes in VP to clinical features and increased vulnerability of NVC in the root-entry zone. Combined MRI with NVC or NA verification, DTI and ELS quantification increased the diagnostic accuracy at group-level but did not suffice to diagnose VP on a single-subject level due to individual variability and lack of diagnostic specificity.

Endolymphatic space is age-dependent.

Knowledge of the physiological endolymphatic space (ELS) is necessary to estimate endolymphatic hydrops (ELH) in patients with vestibulocochlear syndromes. Therefore, the current study investigated age-dependent changes in the ELS of participants with normal vestibulocochlear testing. Sixty-four ears of 32 participants with normal vestibulocochlear testing aged between 21 and 75 years (45.8 ± 17.2 years, 20 females, 30 right-handed, two left-handed) were examined by intravenous delayed gadolinium-enhanced magnetic resonance imaging of the inner ear (iMRI). Clinical diagnostics included neuro-otological assessment, video-oculography during caloric stimulation, and head-impulse test. iMRI data analysis provided semi-quantitative visual grading and automatic algorithmic quantitative segmentation of ELS volume (3D, mm3) using a deep learning-based segmentation of the inner ear's total fluid space (TFS) and volumetric local thresholding, as described earlier. As a result, following a 4-point ordinal scale, a mild ELH (grade 1) was found in 21/64 (32.8%) ears uni- or bilaterally in either cochlear, vestibulum, or both. Age and ELS were found to be positively correlated for the inner ear (r(64) = 0.33, p < 0.01), and vestibulum (r(64) = 0.25, p < 0.05). For the cochlea, the values correlated positively without reaching significance (r(64) = 0.21). In conclusion, age-dependent increases of the ELS should be considered when evaluating potential ELH in single subjects and statistical group comparisons.

Vestibular syndromes after COVID-19 vaccination: A prospective cohort study.

BACKGROUND AND PURPOSE: Dizziness and vertigo are common symptoms after COVID-19-vaccination. We aimed to prospectively evaluate objective central or peripheral vestibular function in patients with dizziness, vertigo, and postural symptoms that started or worsened after COVID-19-vaccination. METHODS: Of 4137 patients who presented between January 2021 and April 2022 at the German Center for Vertigo and Balance Disorders, Ludwig Maximilian University of Munich, we identified 72 patients (mean age = 47 years) with enduring vestibular symptoms following COVID-19 vaccination. All underwent medical history-taking, and neurological and neuro-otological workup with bithermal caloric test, video head-impulse test, orthoptics, and audiometry. Diagnoses were based on international criteria. The distribution of diagnoses was compared to a cohort of 39,964 patients seen before the COVID-19 pandemic. RESULTS: Symptom onset was within the first 4 weeks postvaccination. The most prevalent diagnoses were somatoform vestibular disorders (34.7%), vestibular migraine (19.4%), and overlap syndromes of both (18.1%). These disorders were significantly overrepresented compared to the prepandemic control cohort. Thirty-six percent of patients with somatoform complaints reported a positive history of depressive or anxiety disorders. Nine patients presented with benign paroxysmal positional vertigo, three with acute unilateral vestibulopathy, and seven with different entities (vestibular paroxysmia, Ménière disease, polyneuropathy, ocular muscular paresis). Causally related central vestibular deficits were lacking. Novel peripheral vestibular deficits were found in four patients. CONCLUSIONS: Newly induced persistent vestibular deficits following COVID-19 vaccination were rare. The predominant causes of prolonged vestibular complaints were somatoform vestibular disorders and vestibular migraine, possibly triggered or aggravated by stress-related circumstances due to the COVID-19 pandemic or vaccination. An increase of other central or peripheral vestibular syndromes after COVID-19 vaccination was not observed.

Intravenous Delayed Gadolinium-Enhanced MR Imaging of the Endolymphatic Space: A Methodological Comparative Study.

In-vivo non-invasive verification of endolymphatic hydrops (ELH) by means of intravenous delayed gadolinium (Gd) enhanced magnetic resonance imaging of the inner ear (iMRI) is rapidly developing into a standard clinical tool to investigate peripheral vestibulo-cochlear syndromes. In this context, methodological comparative studies providing standardization and comparability between labs seem even more important, but so far very few are available. One hundred eight participants [75 patients with Meniere's disease (MD; 55.2 ± 14.9 years) and 33 vestibular healthy controls (HC; 46.4 ± 15.6 years)] were examined. The aim was to understand (i) how variations in acquisition protocols influence endolymphatic space (ELS) MR-signals; (ii) how ELS quantification methods correlate to each other or clinical data; and finally, (iii) how ELS extent influences MR-signals. Diagnostics included neuro-otological assessment, video-oculography during caloric stimulation, head-impulse test, audiometry, and iMRI. Data analysis provided semi-quantitative (SQ) visual grading and automatic algorithmic quantitative segmentation of ELS area [2D, mm2] and volume [3D, mm3] using deep learning-based segmentation and volumetric local thresholding. Within the range of 0.1-0.2 mmol/kg Gd dosage and a 4 h ± 30 min time delay, SQ grading and 2D- or 3D-quantifications were independent of signal intensity (SI) and signal-to-noise ratio (SNR; FWE corrected, p < 0.05). The ELS quantification methods used were highly reproducible across raters or thresholds and correlated strongly (0.3-0.8). However, 3D-quantifications showed the least variability. Asymmetry indices and normalized ELH proved the most useful for predicting quantitative clinical data. ELH size influenced SI (cochlear basal turn p < 0.001), but not SNR. SI could not predict the presence of ELH. In conclusion, (1) Gd dosage of 0.1-0.2 mmol/kg after 4 h ± 30 min time delay suffices for ELS quantification. (2) A consensus is needed on a clinical SQ grading classification including a standardized level of evaluation reconstructed to anatomical fixpoints. (3) 3D-quantification methods of the ELS are best suited for correlations with clinical variables and should include both ears and ELS values reported relative or normalized to size. (4) The presence of ELH increases signal intensity in the basal cochlear turn weakly, but cannot predict the presence of ELH.

Endolymphatic Hydrops in Patients With Vestibular Migraine and Concurrent Meniere's Disease.

Objective: Intravenous contrast agent enhanced, high-resolution magnetic resonance imaging of the inner ear (iMRI) confirmed that patients with Menière's disease (MD) and vestibular migraine (VM) could present with endolymphatic hydrops (EH). The present study aimed to investigate EH characteristics and their interrelation to neurotologic testing in patients with VM, MD, or VM with concurrent MD (VM-MD). Methods: Sixty-two patients (45 females, aged 23-81 years) with definite or probable VM (n = 25, 19 definite), MD (n = 29, 17 definite), or showing characteristics of both diseases (n = 8) were included in this study. Diagnostic workup included neurotologic assessments including video-oculography (VOG) during caloric stimulation and head-impulse test (HIT), ocular and cervical vestibular evoked myogenic potentials (o/cVEMP), pure tone audiometry (PTA), as well as iMRI. EH's degree was assessed visually and via volumetric quantification using a probabilistic atlas-based segmentation of the bony labyrinth and volumetric local thresholding (VOLT). Results: Although a relevant number of VM patients reported varying auditory symptoms (13 of 25, 52.0%), EH in VM was only observed twice. In contrast, EH in VM-MD was prevalent (2/8, 25%) and in MD frequent [23/29, 79.3%; χ2(2) = 29.1, p < 0.001, φ = 0.7]. Location and laterality of EH and neurophysiological testing classifications were highly associated (Fisher exact test, p < 0.005). In MD, visual semi-quantitative grading and volumetric quantification correlated highly to each other (r S = 0.8, p < 0.005, two-sided) and to side differences in VOG during caloric irrigation (vestibular EH ipsilateral: r S = 0.6, p < 0.05, two-sided). In VM, correlations were less pronounced. VM-MD assumed an intermediate position between VM and MD. Conclusion: Cochlear and vestibular hydrops can occur in MD and VM patients with auditory symptoms; this suggests inner ear damage irrespective of the diagnosis of MD or VM. The EH grades often correlated with auditory symptoms such as hearing impairment and tinnitus. Further research is required to uncover whether migraine is one causative factor of EH or whether EH in VM patients with auditory symptoms suggests an additional pathology due to MD.