Vestibular Function Tests Research Articles

OUTCOME MEASURES FOR CERVICOGENIC DIZZINESS: A META ANALYSIS.

Background: Cervicogenic dizziness (CGD) is a complex condition characterized by dizziness arising from cervical spine disorders. It affects approximately 40% of patients with chronic neck pain and can significantly impact daily activities, quality of life, and mental health. The involvement of cervical structure in vestibular function highlights the need for effective assessment tools. Outcome measures play a crucial role in diagnosing CGD, evaluating treatment effectiveness, and improving patient outcomes. This evolved, focusing on both subjective and objective assessments, including symptom questionnaires, functional scales, and vestibular function tests. Standardized outcome measures are essential for clinical making and guideline development. Objective: The objective of the study is to systematically evaluate and compare the reliability, validity and sensitivity data of various outcome measures of cervicogenic dizziness. Method: The systemic data were searched using PubMed, Scopus, Google scholar, Science direct, PEDro, Cochrane library to identify studies published from the year 2001 up to 2023. Study selection is reported according to PRISMA guidelines. A total of 50 review articles were collected on outcome measures for Cervicogenic Dizziness (CGD). Data extraction included Research, Evidence based study, Literature, Systematic reviews and Meta-analysis articles. Result: This meta-analysis states that outcomes measures for Cervicogenic Dizziness (CGD) show a highly significant difference. Conclusion: After analysis of various articles, it was concluded that diagnosing patients with outcome measures for Cervicogenic Dizziness shows highly significant and improving health care efficiency. This study identified reliable and valid outcome measures for assessing Cervicogenic Dizziness (CGD). Implication: This study findings have significant implication for clinical practice, research, and policy, enhancing diagnosis, treatment and patient outcomes, standardized use of validated outcome measures will inform evidence – based guidelines for Cervicogenic Dizziness.

Graviception Uncertainty, Spatial Anxiety, and Derealization in Patients with Persistent Postural-Perceptual Dizziness

Objectives: Persistent Postural-Perceptual Dizziness (PPPD) is a frequent diagnosis in patients with chronic dizziness, ineffective postural control, visual dependence, and emotional symptoms. Methods: 53 patients with PPPD (25–84 years old) and 53 adults (29–84 years old) with no vestibular disease agreed to participate in this study. Assessments included: vestibular function tests (sinusoidal yaw rotation and vestibular-evoked myogenic potentials); accuracy and precision of Subjective Visual Vertical (SVV) estimation while static and during on-axis yaw rotation; static posturography with open/closed eyes and 30° neck extension, while standing on hard/soft surface; questionnaires on symptoms of unsteadiness, spatial anxiety, dizziness-related handicap, anxiety/depression, depersonalization/derealization, and perceived stress. After preliminary bivariate analyses, analysis of covariance was performed on the measurements of postural sway, spatial anxiety, and dizziness-related handicap (p < 0.05). Results: Higher intraindividual variability (reduced precision) on SVV estimations was evident in patients with PPPD compared to adults with no vestibular disease, which was related to the length of postural sway, to velocity displacement in the sagittal plane, as well as to spatial anxiety and common mental symptoms (including depersonalization/derealization symptoms). Covariance analysis showed contribution of these factors to the dizziness-related handicap reported by the patients. Conclusions: Unprecise graviception could be a contributing factor to the postural instability and mental symptoms reported by patients with PPPD, which in turn contribute to their dizziness-related handicap.

Correlation between the etiology of severe hearing loss and endolymphatic hydrops.

This study aimed to investigate correlation between the presence of endolymphatic hydrops(EH) and factors such as causes of hearing loss, patient age, duration of deafness, and results of vestibular function tests. We retrospectively reviewed medical charts of 128 ears of cochlear implantees who were not considered relevant to Meniere's disease. When comparing group with genetic variants of GJB2, SLC26A4, LMX1A and other genetic mutation group, the proportion of vestibular EH and cochlear EH found in group with genetic variants of GJB2, SLC26A4, LMX1A was significantly higher than group with other genetic etiology (p < 0.01) or the group with all the other causes of hearing loss (p < 0.01). The rate of vestibular and cochlear EH detection was higher in younger patients (41.5% and 35.4%) than in older patients (25.4% and 20.6%). A higher ratio of vestibular and cochlear EH was observed in patients with a longer duration of deafness (37.5% and 31.3%) than those with a shorter duration of deafness (29.7% and 25.0%). The group with vestibular EH showed a higher incidence of abnormal findings in the caloric test (42.9%) than the group without vestibular EH (28.2%). Patients with genetic variants of GJB2, SLC26A4, LMX1A, younger patients, those with longer deaf durations showed a higher prevalence of vestibular and cochlear EH, implying EH appears to be formed as a developmental disorder in association with a certain set of genetic variants, rather than a phenotypic marker as a result of severe to profound hearing loss.

Assessment of hearing and balance functions in patients with acromegaly by the use of an audiovestibular test battery: a cross-sectional study.

Patients with acromegaly may have abnormalities in their hearing and balance as a result of modifications in body composition and involvement of the temporal bone. The objective of this study is to examine if there are any changes in the auditory and vestibular systems in individuals with acromegaly by using audiogram and vestibular function tests. This prospective study included 33 healthy controls and 33 acromegaly patients who were matched for age and gender distribution. A pure-tone audiometry test was conducted, including frequencies ranging from 250Hz to 8000Hz. Videonystagmography (VNG) was employed to assess nystagmus, an essential parameter used for assessing vestibular functions. The Video Head Impulse Test (v-HIT) was used to assess the vestibulo-ocular reflex (VOR). The Dizziness Handicap Inventory (DHI) was applied to evaluate the subjective complaints of the participants. The acromegaly patients had significantly elevated hearing thresholds at all frequencies (250, 500, 1000, 2000, 4000, and 6000Hz) compared to the control group (p < 0.005). The VNG tests, including gaze horizontal, gaze vertical, saccade, spontaneous nystagmus, optokinetic, smooth pursuit, and positioning tests, did not show any statistically significant difference between the two groups (p values > 0.05). The patient group demonstrated reduced VOR gains compared to the control group in the anterior and posterior channels (p < 0.005). There was no statistically significant difference between the two groups for the occurrence of aberrant eye movements (p values > 0.05). The patient group had a total DHI score of 6.6 ± 3.2, while the control group had a score of 3.2 ± 2.6 (independent samples t-test; p < 0.001). Therefore, The patient group exhibited significantly greater subjective vestibular symptoms. Patients with acromegaly experience impaired auditory function. The central vestibular system remains unaffected, while the gains of the vestibulo-ocular reflex (VOR) in the posterior and anterior semicircular canals are decreased. Additionally, these patients report experiencing subjective dizziness. Screening for hearing and balance in patients with acromegaly may improve the quality of life of patients and prevent problems related to balance disorders at an early stage.

Otolith function in Usher type II syndrome

Background Usher’s syndrome type II (USH2) is a rare genetic disorder encompassing hearing loss, vision impairment, and apparent intact vestibular function. Recent research suggests a potential involvement of the otolith vestibular receptors in USH2. Aims/Objectives Evaluate otolith dynamic function in USH2. Material and Methods Twenty-two USH2 (median age 53.9 ± 2.99) and age-matched controls underwent a complete battery vestibular testing including air conducted cervical and ocular vestibular evoked myogenic potentials (c-VEMPs and o-VEMPs). Vestibular function tests were correlated with Activities Balance Scale (ABC) and Dizziness Handicap Inventory (DHI) scores. Results Fourteen USH2 reported previous vertigo (vs none control). Among 88 ears, c-VEMPs were absent in 15 USH2 cases and 4 controls (p = 0.034), while o-VEMPs were absent in 22 USH2 cases and 12 controls (p = 0.129). There were significant differences between USH2 vs controls in right ear o-VEMP N1 latencies (median 11.60/10.40, p < 0.010), N1-P1 amplitudes (median 5.15/10.10, p < 0.003) and in o-VEMP N1-P1 asymmetry ratio (median 24.78/40.50, p < 0.014). USH2 showed a strong correlation between o-VEMP amplitude and DHI score (p = 0.003, ρ = 0.769). No association was found between vertigo and VEMPs subgroups. Conclusions and Significance Our findings suggest the presence of otolith dysfunction in USH2, which is independent from subjectively reported dizziness. Incorporating vestibular testing into USH2 evaluation and monitoring could enhance characterization of this multisensory disease.

Electroencephalography microstate alterations reflect potential double-edged cognitive adaptation in Ménière's disease.

To explore the microstate characteristics and underlying brain network activity of Ménière's disease (MD) patients based on high-density electroencephalography (EEG), elucidate the association between microstate dynamics and clinical manifestation, and explore the potential of EEG microstate features as future neurobiomarkers for MD. Thirty-two patients diagnosed with MD and 29 healthy controls (HC) matched for demographic characteristics were included in the study. Dysfunction and subjective symptom severity were assessed by neuropsychological questionnaires, pure tone audiometry, and vestibular function tests. Resting-state EEG recordings were obtained using a 256-channel EEG system, and the electric field topographies were clustered into four dominant microstate classes (A, B, C, and D). The dynamic parameters of each microstate were analyzed and utilized as input for a support vector machine (SVM) classifier to identify significant microstate signatures associated with MD. The clinical significance was further explored through Spearman correlation analysis. MD patients exhibited an increased presence of microstate class C and a decreased frequency of transitions between microstate class A and B, as well as between class A and D. The transitions from microstate class A to C were also elevated. Further analysis revealed a positive correlation between equilibrium scores and the transitions from microstate class A to C under somatosensory challenging conditions. Conversely, transitions between class A and B were negatively correlated with vertigo symptoms. No significant correlations were detected between these characteristics and auditory test results or emotional scores. Utilizing the microstate features identified via sequential backward selection, the linear SVM classifier achieved a sensitivity of 86.21% and a specificity of 90.61% in distinguishing MD patients from HC. We identified several EEG microstate characteristics in MD patients that facilitate postural control yet exacerbate subjective symptoms, and effectively discriminate MD from HC. The microstate features may offer a new approach for optimizing cognitive compensation strategies and exploring potential neurobiological markers in MD.

Hearing, balance, and imaging assessment in adolescent Menière's disease: A retrospective analysis.

To retrospectively analyze clinical features in adolescent Menière's disease (MD). The medical records of adolescents with MD (11-17 years old) from May 2014 to March 2023 in Shandong Provincial ENT Hospital were retrospectively analyzed, including clinical features, a battery of auditory and vestibular function tests, sensory organization test, and imaging assessments. Patients with recurrent vertigo of childhood (RVC) were as controls. Compared with RVC, adolescent MD showed higher pure tone average threshold (p < .001), lower speech discrimination score (p = .014), and lower otoacoustic emission pass rates (p = .005). Adolescents with MD exhibited significant reduction in equilibrium score (Conditions 1, 5, and 6; p1 = .035; p5 = .033; p6 = .003), composite sensory score (p = .014), and vestibular sensory score (p = .029). Adolescents with bilateral MD exhibited worse performance in equilibrium score and strategy score compared to adolescents with unilateral MD. For the affected ear, the more severe endolymphatic hydrops detected by gadolinium-enhanced magnetic resonance imaging, the higher the auditory brainstem response threshold (r = .850, p = .007), and the lower the otoacoustic emission pass rate (r = -.976, p < .001). Adolescent MD has similar vestibular information inputs with that of RVC, but the ability for the nerve center to use these clues to maintain balance is worse in adolescents with MD. There were potential differences in vestibular weights in adolescents with unilateral and bilateral MD, also potential effects on vision and proprioception. Level 4.

Clinical features of persistent postural-perceptual dizziness coexisting with Meniere's disease in comparison with Meniere's disease alone.

Persistent postural-perceptual dizziness (PPPD) is a chronic vestibular syndrome often triggered by acute or episodic vestibular syndromes, such as Meniere's disease (MD). According to the diagnostic criteria, PPPD may coexist with other structural diseases, and the evidence of another active illness does not necessarily exclude PPPD diagnosis. However, persistent symptoms, even those meeting the PPPD criteria even long after Meniere's attack, are often overlooked as potential PPPD precipitated by MD. Some clinicians overlook PPPD in such patients, treating them solely for MD once diagnosed. Since a treatment strategy for PPPD is completely different from that for MD, this may result in the deprivation of adequate treatments. To emphasize the importance of diagnosing PPPD coexisting with MD including not treating such patients solely for MD, and to compare the clinical features of PPPD and MD. Vestibular function tests, including canal paresis (CP)%, c- and o-vestibular myogenic potentials, vestibulo-ocular reflex-direction preponderance, and posturography and clinical symptom scales, including the Dizziness Handicap Inventory, Niigata PPPD Questionnaire, and Hospital Anxiety and Depression Scale, were compared between 105 PPPD patients with MD or other precipitants and 130 patients with MD alone. The clinical symptom scales were further compared between 23 patients with PPPD coexisting with MD and those with MD alone. The CP% was significantly higher in patients with MD than in those with PPPD. However, the total and subscores of all three clinical symptom scales were higher in patients with PPPD than in those with MD. The total score on all clinical symptom scales was higher in patients with PPPD coexisting with MD than in those with MD alone. Persistent postural-perceptual dizziness development from a precipitating MD may be associated with more severe clinical symptoms. Thus, clinical symptom scales may be useful for detecting PPPD in patients with Meniere's disease.

Impact of audiovestibular factors on prognosis in patients with sudden sensorineural hearing loss without vertigo.

This study aimed to investigate the hearing outcomes in patients with sudden sensorineural hearing loss without vertigo (SSNHLwoV). Patients with SSNHLwoV managed from December 2016 to March 2020 were prospectively enrolled in an academic tertiary referral center. Fifty-one patients with SSNHLwoV who completed high-dose steroid treatment. The hearing prognosis was analyzed using a multivariate Cox regression model. The rates of complete, partial, and no hearing recovery were 52.9%, 17.6%, and 29.4% in patients with SSNHLwoV, respectively. The video head impulse test (vHIT) of the posterior semicircular canal (PSC), high-tone hearing loss (4-8kHz) ≥ 30 dB, and average hearing threshold (0.5-1-2-4kHz) were significantly associated with incomplete recovery of hearing after treatment. In multivariate analysis, the vHIT of the PSC (hazard ratio [HR], 14.502; 95% confidence interval [CI], 1.371-153.355) and high-tone hearing loss ≥ 30 dB (HR, 9.170; 95% CI, 2.283-36.830) remained robust. Abnormal vestibular function tests were performed in 80.4% of the patients with SSNHLwoV. Abnormal vHIT of the PSC and high-tone hearing loss ≥ 30 dB were independent factors resulting in incomplete recovery of hearing in patients with SSNHLwoV. In the SSNHLwoV cohort, the caloric test was not significantly associated with hearing prognosis, and vHIT was a feasible predictor of treatment outcome.

Vestibular hair cells are more prone to damage by excessive acceleration insult in the mouse with KCNQ4 dysfunction

KCNQ4 is a voltage-gated K+ channel was reported to distribute over the basolateral surface of type 1 vestibular hair cell and/or inner surface of calyx and heminode of the vestibular nerve connected to the type 1 vestibular hair cells of the inner ear. However, the precise localization of KCNQ4 is still controversial and little is known about the vestibular phenotypes caused by KCNQ4 dysfunction or the specific role of KCNQ4 in the vestibular organs. To investigate the role of KCNQ4 in the vestibular organ, 6-g hypergravity stimulation for 24 h, which represents excessive mechanical stimulation of the sensory epithelium, was applied to p.W277S Kcnq4 transgenic mice. KCNQ4 was detected on the inner surface of calyx of the vestibular afferent in transmission electron microscope images with immunogold labelling. Vestibular function decrease was more severe in the Kcnq4p.W277S/p.W277S mice than in the Kcnq4+/+ and Kcnq4+/p.W277S mice after the stimulation. The vestibular function loss was resulted from the loss of type 1 vestibular hair cells, which was possibly caused by increased depolarization duration. Retigabine, a KCNQ activator, prevented hypergravity-induced vestibular dysfunction and hair cell loss. Patients with KCNQ4 mutations also showed abnormal clinical vestibular function tests. These findings suggest that KCNQ4 plays an essential role in calyx and afferent of type 1 vestibular hair cell preserving vestibular function against excessive mechanical stimulation.

High-Frequency Vestibular Function Is Vulnerable to Presbyvestibulopathy.

In 2019, mild vestibular function deficiency in elder populations was defined as presbyvestibulopathy (PVP) by the Classification Committee of the Bárány Society. The diagnostic criteria include tests for low-, mid-, and high-frequency vestibular function, represented by caloric testing, rotary chair testing, and head impulse testing, respectively. However, there is still a lack of large-scale reports supporting the relationship between vestibular function tests (VFTs) and aging. In this study, we evaluated whether each test is correlated with aging in the elderly population aged over 50. This study retrospectively enrolled 1043 subjects from a single university hospital database after excluding those with unilateral and bilateral vestibulopathy, central dizziness, and acute dizziness. Enrolled subjects had caloric canal paresis <20%, vHIT lateral canal gain >0.6, vHIT interaural difference <0.3, and age >50 years old. Significant negative correlations with age were identified in the vHIT (p < 0.001) and rotary chair test (RCT) 1.0 Hz gain (p = 0.030). However, the caloric test (p = 0.739 and 0.745 on the left and right sides, respectively) and RCT 0.12 Hz gain (p = 0.298) did not show a significant correlation with age. A total of 4.83% of subjects aged 70 years or older showed sub-normal vHIT gain that met the criteria of PVP, whereas only 0.50% of subjects aged 60 to 69 did. The prevalence of sub-normal caloric test results, however, was not significantly different between the two age groups (21.55% in the 60-69 age group and 26.59% in the >70 age group). The high-frequency range vestibular function seems vulnerable to aging, and this is more discernible at age >70 years. The weak correlation between age and low-frequency vestibular function tests, such as the caloric test and low-frequency rotary chair testing, suggests the need to revisit the diagnostic criteria for PVP.

Epidemiology and Comorbidities of Vestibular Disorders: Preliminary Findings of the AVOCADO Study.

Studies on incidence and prevalence of vestibular disorders tend to focus on small pockets of patients recruited from specialized clinics and often exclude measures of vestibular function. The objectives of the study were to characterize patients with common vestibular disorders, estimate the prevalence of common vestibular disorders, and ascertain whether patients with vestibular disorders experience increased risks of falls and morbidity. This retrospective cohort study includes both inpatient and outpatient routine clinical care data culled from a nationally representative, population-based sample. Patients were included if their record in the TriNetX Diamond Cohort comprised at least one vestibular function test or vestibular diagnosis. The main outcome measures were diagnosis with a vestibular disorder, a fall, or a common medical comorbidity (e.g., diabetes, cerebrovascular disease). The cohort includes n = 4,575,724 patients, of which 55% (n = 2,497,136) had a minimum of one vestibular diagnosis. Patients with vestibular diagnoses were 61.3 ± 16.6 years old (mean ± standard deviation), 67% women, 28% White race (69% unknown race), and 30% of non-Hispanic or Latino ethnicity (66% unknown ethnicity). The prevalence of vestibular disorders was estimated at 2.98% (95% confidence interval [CI]: 2.98-2.98%). Patients with vestibular diagnoses experienced a significantly greater odds of falls (odds ratio [OR] = 1.04; 95% CI: 1.02-1.05), cerebrovascular disease (OR = 1.42; 95% CI: 1.40-1.43), ischemic heart disease (OR = 1.17; 95% CI: 1.16-1.19), and diabetes (OR = 1.14; 95% CI: 1.13-1.15), among others. Vestibular disorders affect an estimated 3% of the U.S. population, after weighting. Patients with these disorders are at greater risk for many common, consequential medical conditions.

Postural motion perception during vestibular stimulation depends on the motion perception threshold in persistent postural-perceptual dizziness

Patients with persistent postural-perceptual dizziness (PPPD) perceive postural instability larger than the observed sway. It is unknown whether the concept of postural misperception prevails during vestibular stimulation and whether it may account for the unsteadiness patients complain during body movements. We tested the hypothesis of an abnormal sensory-perceptual scaling mechanism in PPPD by recording objective, perceived, and the reproduced postural sway under various standing conditions, modulating visual and proprioceptive input, by binaural galvanic vestibular stimulation (GVS). We related postural sway speed to individual vestibular motion perceptional thresholds and disease-related PPPD questionnaires in 32 patients and 28 age-matched healthy control subjects (HC). All participants showed normal vestibular function tests on quantitative testing at the time of enrollment. The perception threshold of GVS was lower in patients. Compared to HC, patients showed and perceived larger sway on the firm platform. With GVS, posturo-perceptual ratios did not show group differences. The ratio of reproduced to real postural sway showed no group differences indicating normal postural sway perception during vestibular stimulation. Noticeably, only in patients, reproduced postural instability became larger with lower individual thresholds of vestibular motion detection. We conclude that posturo-perceptual (metacognitive) scaling of postural control seems to be largely preserved in PPPD during GVS. Vestibular stimulation does not destabilize patients more than HC, even in challenging postural conditions. Low individual thresholds of vestibular motion perception seem to facilitate instability and postural misperception on solid grounds. This conclusion is important for an effective physical therapy with vestibular exercises in PPPD.

Analysis of postural stability using foam posturography in patients with persistent postural-perceptual dizziness.

Persistent postural-perceptual dizziness (PPPD) is worsened in a standing posture, or by body movement, or visual stimulation. We aimed to evaluate postural stability in PPPD patients using foam posturography and to investigate the dependence on visual and somatosensory input in the standing posture. Foam posturography was performed on 53 PPPD patients, and data from the PPPD patients were compared with the data from an age- and sex-matched healthy controls. The PPPD patients were divided into four groups based on the findings of vestibular function tests and the effect of vestibular function on posturographic data was examined. Romberg's ratios were significantly higher in PPPD patients than in controls. The median Romberg's ratios in PPPD patients with normal vestibular function were also higher than those in controls. However, foam ratio was significantly lower in PPPD patients than in controls. The median foam ratios in PPPD patients with vestibular dysfunction were also lower than those in controls. In a standing posture, PPPD patients may be more dependent on visual input and less dependent on somatosensory input than healthy subjects. Higher dependence on visual and lower dependence on somatosensory input in PPPD may be a feature unaffected by vestibular function.

A mouse model of autoimmune inner ear disease without endolymphatic hydrops

Autoimmune inner ear disease (AIED) is an organ-specific disease characterized by irreversible, prolonged, and progressive hearing and equilibrium dysfunctions. The primary symptoms of AIED include asymmetric sensorineural hearing loss accompanied by vertigo, aural fullness, and tinnitus. AIED is divided into primary and secondary types. Research has been conducted using animal models of rheumatoid arthritis (RA), a cause of secondary AIED. However, current models are insufficient to accurately analyze vestibular function, and the mechanism underlying the onset of AIED has not yet been fully elucidated. Elucidation of the mechanism of AIED onset is urgently needed to develop effective treatments. In the present study, we analyzed the pathogenesis of vertigo in autoimmune diseases using a mouse model of type II collagen-induced RA. Auditory brain stem response analysis demonstrated that the RA mouse models exhibited hearing loss, which is the primary symptom of AIED. In addition, our vestibulo-oculomotor reflex analysis, which is an excellent vestibular function test, accurately captured vertigo symptoms in the RA mouse models. Moreover, our results revealed that the cause of hearing loss and vestibular dysfunction was not endolymphatic hydrops, but rather structural destruction of the organ of Corti and the lateral semicircular canal ampulla due to an autoimmune reaction against type II collagen. Overall, we were able to establish a mouse model of AIED without endolymphatic hydrops. Our findings will help elucidate the mechanisms of hearing loss and vertigo associated with AIED and facilitate the development of new therapeutic methods.

Bone-Anchored Hearing Aid Effects on Vestibular Function: A Preliminary Report.

Cochlear receptors are sensitive to vibratory stimuli. Based on this sensibility, bone-anchored hearing aids have been introduced to correct unilateral or bilateral conductive or mixed hearing loss and unilateral deafness. The vestibular system is also sensitive to the vibratory stimulus and this type of response is used in clinics to test its functionality. Being aware of this double separated sensibility, we wondered whether bone vibration, which activates the acoustic receptors of patients with bone conduction aids, can also influence the functionality of the vestibular system. To this end, we recruited 12 patients with a bone-anchored hearing aid and evaluated their vestibular function with and without an activated vibratory acoustic device. Our results show that the vibratory stimulus delivered by the bone conduction aid also reaches and stimulates the vestibular receptors; this stimulation is evidenced by the appearance or modification of some nystagmus findings during bedside vestibular testing. Despite this, none of these patients complained of dizziness or vertigo during prosthesis use. Nystagmus that appeared or changed during acoustic vibratory stimulation through the prosthesis was almost all predominantly horizontal, unidirectional with respect to gaze or body position, inhibited by fixation, and most often consistent with vestibular function tests indicating peripheral vestibular damage. The findings of sound-evoked nystagmus seem to indicate peripheral rather than central vestibular activation. The occurrence of some predominantly horizontal and high-frequency induced nystagmus seems to attribute the response mainly to the utricle and lateral semicircular canal.

A Clinical Framework for Video Head Impulse Testing and Vestibular Evoked Myogenic Potential Assessments in Primary School-Aged Children.

This study aimed to offer normative data and age trends of an age-appropriate vestibular test protocol in a large group (n = 140) of school-aged children (6 to 13 years old) as well as to provide a practical and clinical framework for accurate performance and interpretation of vestibular test results in this specific age group. The typically developing participants (mean age of 9.51 ± 2.04 years) were recruited to provide a representative group of 20 children for each of the seven age groups that were composed of children aged from 6 to 13 years in 1-year intervals. Each age group consisted of 10 boys and 10 girls. The protocol comprises the video head impulse test, and cervical and ocular vestibular evoked myogenic potential assessments to provide a child-friendly, noninvasive, short, and portable test battery, which is equally applicable in the hospital and office-practice, and which provides information on the integrity of all five parts of the peripheral vestibular system. The study demonstrates that all included tests and methods, with an overall test duration of 25 min 12 sec ± 5 min 10 sec, were feasible to perform in primary school-aged children, taking into account some practical adaptations. Concerning the video head impulse test, no clinically relevant sex and age effects were noted. However, t tests revealed significant differences for the mean gain of the horizontal (right > left; t[139] = 14.563; p < 0.001) and posterior semicircular canals (left > right; t[139] = -4.823; p < 0.001) between both sides. For the cVEMP assessment, no laterality differences were observed for any of the parameters, but a significantly shorter N1 latencies in the youngest age categories (<8 years), compared with the oldest groups were observed [F(6,118) = 8.336; p < 0.001; partial ƞ² = 0.298]. For all oVEMP parameters, no laterality, sex, or age differences were seen. On the basis of the presented normative data, cutoff criteria were proposed with accompanying clinical recommendations to perform vestibular function testing in this target population. This is the first study in a large group of school-aged children offering normative data and age trends of an age-appropriate vestibular test protocol that evaluates the integrity of all parts of the peripheral vestibular organ. The reported normative values and clinical cutoff values will enable appropriate and age-specific interpretation of clinical and scientific results. Moreover, in combination with extensive history taking, and additional vestibular testing (e.g., rotatory chair test, caloric testing) when needed, the results of this study may support clinicians in the diagnosis of side-specific and location-specific vestibular deficits, which is required for accurate counseling and referral for further follow-up and/or intervention.

Characterization of Vestibular Phenotypes in Patients with Genetic Hearing Loss.

Background: The vestibular phenotypes of patients with genetic hearing loss are poorly understood. Methods: we performed genetic testing including exome sequencing and vestibular function tests to investigate vestibular phenotypes and functions in patients with genetic hearing loss. Results: Among 627 patients, 143 (22.8%) had vestibular symptoms. Genetic variations were confirmed in 45 (31.5%) of the 143 patients. Nineteen deafness genes were linked with vestibular symptoms; the most frequent genes in autosomal dominant and recessive individuals were COCH and SLC26A4, respectively. Vestibular symptoms were mostly of the vertigo type, recurrent, and persisted for hours in the genetically confirmed and unconfirmed groups. Decreased vestibular function in the caloric test, video head impulse test, cervical vestibular-evoked myogenic potential, and ocular vestibular-evoked myogenic potential was observed in 42.0%, 16.3%, 57.8%, and 85.0% of the patients, respectively. The caloric test revealed a significantly higher incidence of abnormal results in autosomal recessive individuals than in autosomal dominant individuals (p = 0.011). The genes, including SLC26A4, COCH, KCNQ4, MYH9, NLRP3, EYA4, MYO7A, MYO15A, and MYH9, were heterogeneously associated with abnormalities in the vestibular function test. Conclusions: In conclusion, diverse vestibular symptoms are commonly concomitant with genetic hearing loss and are easily overlooked.

Clinical, Biomarker, and Research Tests Among US Government Personnel and Their Family Members Involved in Anomalous Health Incidents

Since 2015, US government and related personnel have reported dizziness, pain, visual problems, and cognitive dysfunction after experiencing intrusive sounds and head pressure. The US government has labeled these anomalous health incidents (AHIs). To assess whether participants with AHIs differ significantly from US government control participants with respect to clinical, research, and biomarker assessments. Exploratory study conducted between June 2018 and July 2022 at the National Institutes of Health Clinical Center, involving 86 US government staff and family members with AHIs from Cuba, Austria, China, and other locations as well as 30 US government control participants. AHIs. Participants were assessed with extensive clinical, auditory, vestibular, balance, visual, neuropsychological, and blood biomarkers (glial fibrillary acidic protein and neurofilament light) testing. The patients were analyzed based on the risk characteristics of the AHI identifying concerning cases as well as geographic location. Eighty-six participants with AHIs (42 women and 44 men; mean [SD] age, 42.1 [9.1] years) and 30 vocationally matched government control participants (11 women and 19 men; mean [SD] age, 43.8 [10.1] years) were included in the analyses. Participants with AHIs were evaluated a median of 76 days (IQR, 30-537) from the most recent incident. In general, there were no significant differences between participants with AHIs and control participants in most tests of auditory, vestibular, cognitive, or visual function as well as levels of the blood biomarkers. Participants with AHIs had significantly increased fatigue, depression, posttraumatic stress, imbalance, and neurobehavioral symptoms compared with the control participants. There were no differences in these findings based on the risk characteristics of the incident or geographic location of the AHIs. Twenty-four patients (28%) with AHI presented with functional neurological disorders. In this exploratory study, there were no significant differences between individuals reporting AHIs and matched control participants with respect to most clinical, research, and biomarker measures, except for objective and self-reported measures of imbalance and symptoms of fatigue, posttraumatic stress, and depression. This study did not replicate the findings of previous studies, although differences in the populations included and the timing of assessments limit direct comparisons.

Visual and vestibular motion perception in persistent postural-perceptual dizziness (PPPD)

Persistent postural-perceptual dizziness (PPPD) is a chronic disorder of perceived unsteadiness. Symptoms can be exacerbated in visually complex stationary or moving environment. Visual dependence and increased motion sensitivity are predictors for PPPD but its pathophysiology remains unknown. We hypothesized an abnormal sensory–perceptual scaling mechanism in PPPD and tested visual- and vestibular perceptional thresholds in 32 patients and 28 age-matched healthy control subjects (HC). All participants showed normal vestibular function tests on quantitative testing. Visual motion coherence thresholds were assessed by random dot kinetomatograms. Vestibular perceptional thresholds of egomotion were assessed by binaural galvanic vestibular stimulation (GVS) and passive chair rotation around an earth-vertical axis. Chair rotation trials were contrasted with no-motion (sham) stimulus trials. Mean thresholds of visual motion perception were higher in patients compared to HC. The perception threshold of GVS was lower in patients but the threshold of correctly perceived egomotion during chair rotation did not differ. Interestingly, the number of trials with correct perception in the no-motion condition increased with the threshold of correct responses for rotatory egomotion in patients. Unlike expected, PPPD patients required more coherently moving random dots than HC to perceive visual motion. A poorer complex visual motion recognition, e.g., traffic visual stimuli, may increase anxiety and levels of uncertainty as visuomotor reactions might occur delayed. The vestibular rotatory perception threshold predicted the probability of making false assignments in the sham condition in PPPD, i.e., patients who readily recognize the correct egomotion direction are prone to perceive egomotion in the no-motion condition. As this relation was not found in healthy subjects, it may reflect an abnormal sensory–perceptual scaling feature of PPPD.