UNASSIGNED: 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.
UNASSIGNED: Evaluate otolith dynamic function in USH2.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

UNASSIGNED: Patients with superior canal dehiscence syndrome (SCDS) can present with a plethora of auditory and/or vestibular symptoms associated with a bony defect of the superior semicircular canal. While surgical repair is a reasonable option for patients with significant localizing symptoms, the degree of clinical improvement will vary among patients and poses challenges in outcome prediction. This study aims to assess the relationship between preoperative and postoperative symptoms and identify predictors of symptom persistence following repair.
UNASSIGNED: Retrospective chart review.
UNASSIGNED: Tertiary neurotology single-institution care center.
UNASSIGNED: The primary outcome was to determine the proportion of resolved and persistent primary (most bothersome) and non-primary audiologic and vestibular symptoms following SCD repair. Secondary outcomes included comparison of patient, operative and radiologic characteristics between patients with resolved vs. persistent symptoms. Standardized patient questionnaires including 11 auditory and 8 vestibular symptoms were administered to patients at their preoperative and follow-up visits. Patient pre- vs. postoperative survey results, demographic and clinical characteristics, operative characteristics, audiometric data and cervical vestibular evoked myogenic potential (cVEMP) thresholds were compared via univariate χ2 and multivariate binary logistic regression analyses between those patients reporting full postoperative resolution of symptoms and persistence of one or more symptoms. Radiologic computed tomography (CT) measurements of superior canal dehiscence (SCD) defect size, location, and laterality were also compared between these two groups.
UNASSIGNED: Of 126 patients (132 ears) included in our study, 119 patients (90.2%) reported postoperative resolution (n = 82, 62.1%) or improvement (n = 37, 28.0%) of primary (most bothersome) symptoms, while 13 patients (9.8%) reported persistence of primary symptoms. The median (interquartile range) and range between surgery and questionnaire completion were 9 (4-28), 1-124 months, respectively. Analyzing all symptoms (primary and non-primary) 69 (52.3%) and 68 (51.1%) patients reported complete postoperative auditory and vestibular symptom resolution, respectively. The most likely persistent symptoms included imbalance (33/65/67, 50.8%), positional dizziness (7/20, 35.0%) and oscillopsia (44/15, 26.7%). Factors associated with persistent auditory symptoms included history of seizures (0% vs. 7.6%, p = 0.023), auditory chief complaint (50.0% vs. 70.5%), higher PTA (mean 19.6 vs. 25.1 dB, p = 0.043) and higher cervical vestibular evoked myogenic potential (cVEMP) thresholds at 1000 Hz (mean 66.5 vs. 71.4, p = 0.033). A migraine diagnosis (14.0% vs. 41.9% p < 0.010), bilateral radiologic SCD (17.5% vs. 38.1%, p = 0.034) and revision cases (0.0% vs. 14.0%, p = 0.002) were associated with persistent vestibular symptoms. Neither SCD defect size nor location were significantly associated with symptom persistence (P > 0.05).
UNASSIGNED: Surgical repair for SCDS offers meaningful reduction in the majority of auditory and vestibular symptoms. However, the persistence of certain, mostly non-primary, symptoms and the identification of potential associated factors including migraines, PTA thresholds, cVEMP threshold, bilateral SCD, and revision cases emphasize the importance of individualized patient counseling and management strategies.

UNASSIGNED: To investigate whether multi-frequency Vestibular Evoked Myogenic Potential (VEMP) testing at 500, 750, 1,000, and 2,000 Hz, would improve the detection of present dynamic otolith responses in patients with bilateral vestibulopathy (BV).
UNASSIGNED: Prospective study in a tertiary referral center. BV patients underwent multi-frequency VEMP testing. Cervical VEMPs and ocular VEMPs were recorded with the Neuro-Audio system (v2010, Neurosoft, Ivanovo, Russia). The stimuli included air-conducted tone bursts of 500, 750, 1,000, and 2,000 Hz, at a stimulation rate of 13 Hz. Outcome measures included the percentage of present and absent VEMP responses, and VEMP thresholds. Outcomes were compared between frequencies and type of VEMPs (cVEMPs, oVEMPs). VEMP outcomes obtained with the 500 Hz stimulus, were also compared to normative values obtained in healthy subjects.
UNASSIGNED: Forty-nine BV patients completed VEMP testing: 47 patients completed cVEMP testing and 48 patients completed oVEMP testing. Six to 15 % more present VEMP responses were obtained with multifrequency testing, compared to only testing at 500 Hz. The 2,000 Hz stimulus elicited significantly fewer present cVEMP responses (right and left ears) and oVEMP responses (right ears) compared to the other frequencies (p ≤ 0.044). Using multi-frequency testing, 78% of BV patients demonstrated at least one present VEMP response in at least one ear. In 46% a present VEMP response was found bilaterally. BV patients demonstrated a significantly higher percentage of absent VEMP responses and significantly higher VEMP thresholds than healthy subjects, when corrected for age (p ≤ 0.002). Based on these results, a pragmatic VEMP testing paradigm is proposed, taking into account multi-frequency VEMP testing.
UNASSIGNED: Multi-frequency VEMP testing improves the detection rate of present otolith responses in BV patients. Therefore, multi-frequency VEMPs should be considered when evaluation of (residual) otolith function is indicated.

UNASSIGNED: Occupational workers are increasingly aware of the risk of noise overexposure to the auditory system but lack awareness about potential risks to the vestibular system. The purpose of this study was to investigate changes in vestibular end organ function in a known at-risk noise-exposed population, firefighters compared to age- and sex-matched controls using electrophysiologic measures of cervical vestibular evoked myogenic potentials (cVEMP).
UNASSIGNED: A cross-sectional observational study compared cVEMP response characteristics in 38 noise-exposed firefighters. Firefighters were grouped by years of exposure in the fire service. The cVEMP responses were compared within firefighter groups and between firefighters and age- and sex-matched controls. Dependent variables included the response characteristics of amplitude, latency and threshold.
UNASSIGNED: cVEMP response amplitudes were significantly decreased in firefighters compared to their age- and sex-matched controls. Threshold of the cVEMP responses were significantly higher in firefighters compared to controls and firefighters had a higher incidence of absent cVEMP responses compared to controls. Response amplitudes decreased with increasing years in the fire-service at an increased rate compared to their age- and sex-matched controls. Latency of the cVEMP response was not significantly different in firefighters compared to controls. These findings are consistent with both animal and human studies suggesting noise-induced changes in the sacculocollic pathway.
UNASSIGNED: In the absence of any reported vestibular symptoms or auditory indicators of noise-induced hearing loss, these early effects on the vestibular system point to a potential hidden vestibular loss.

UNASSIGNED: Cervical vestibular evoked myogenic potentials (cVEMPs) provide an objective measure of the integrity of the sacculo-collic pathway leading to their widespread use as a clinical tool in the diagnostic vestibular test battery. Though the application of cVEMPs in preclinical models to assess vestibular function, as performed in relevant clinical populations, remains limited. The present study aimed to establish a rodent model of cVEMP with standardized methods and protocols, examine the neural basis of the responses, and characterize and validate important features for interpretation and assessment of vestibular function.
UNASSIGNED: We compared air-conducted sound (ACS)-evoked VEMPs from the sternocleidomastoid muscles in naïve Brown Norway rats. A custom setup facilitated repeatable and reliable measurements which were carried out at multiple intensities with ACS between 1 and 16 kHz and over 7 days. The myogenic potentials were identified by the presence of a positive (P1)-negative (N1) waveform at 3-5 ms from the stimulus onset. Threshold, amplitude, and latency were compared with intensity- and frequency-matched responses within and between animals.
UNASSIGNED: cVEMP responses were repeatedly evoked with stimulus intensities between 50-100 dB SPL with excellent test-retest reliability and across multiple measurements over 7 days for all frequencies tested. Suprathreshold, cVEMP responses at 90 dB SPL for 6-10 kHz stimuli demonstrated significantly larger amplitudes (p < 0.01) and shorter latencies (p < 0.001) compared to cVEMP responses for 1-4 kHz stimuli. Latency of cVEMP showed sex-dependent variability, but no significant differences in threshold or amplitude between males and females was observed.
UNASSIGNED: The results provide a replicable and reliable setup, test protocol, and comprehensive characterization of cVEMP responses in a preclinical model which can be used in future studies to elucidate pathophysiological characteristics of vestibular dysfunctions or test efficacy of therapeutics.

BACKGROUND: Balance weaknesses related to mobility and fall risk in patients with rheumatic diseases are well-known. Vestibular dysfunction could negatively contribute to the balance ability of this patient population. This study aims to investigate the effects of Rheumatoid Arthritis (RA) and Ankylosing Spondylitis (AS), among the most common rheumatic diseases, on postural balance related to vestibular function.
METHODS: Seventy-eight participants were grouped as RA (n=34, 43%), AS (n=24, 30.7%), and the control group consisted of healthy individuals (n=20, 25.6%). Cervical Vestibular Evoked Myogenic Potentials (cVEMP) test, which assesses the vestibular function objectively, Dizziness Handicap Inventory (DHI), which evaluates vertigo subjectively, and Berg Balance Scale (BBS) were performed.
RESULTS: Different degrees of VEMP latency prolongations were found in the AS and RA groups. Right, and left ear N1 latencies were significantly longer in the AS group than in RA and control. Right ear P1 latency prolongation was statistically significant in the RA group. Amplitude asymmetry ratio (AAR) was found to be considerably higher in the RA and AS groups than in the control group (p<0.05). The mean BBS score in the AS group was below the fall risk score of 45. A negative statistically significant effect was observed between latency prolongation and BBS in AS groups.
CONCLUSIONS: The abnormal VEMP findings in individuals with RA and AS shows inner ear vestibular system dysfunction. This vestibular impairment strictly contributes to their postural imbalance and requires a focused vestibular rehabilitation program for balance treatment.

OBJECTIVE: The Cervical Vestibular Evoked Myogenic Potential (cVEMP) is often employed in routine clinical practice as part of the vestibular test battery assessments. However, there is currently no well-established paediatric cVEMP normative data in Singapore. Additionally, limited literature has examined the relationship between neck length and cVEMP parameters. The main aims of this study are to 1) establish cVEMP normative data from Singaporean school-aged children aged 6-12 years old, and 2) examine if there is a significant correlation between neck length and cVEMP parameters.
METHODS: 31 healthy children participated in this study. Every participant was screened to ensure that they had normal auditory and vestibular profiles before completing the cVEMP procedure, which involved 500 Hz tone burst through insertphones and head elevation from supine position as the method of neck contraction.
RESULTS: The response rate in 62 ears was 98.4% at 95 dBnHL and 100% at 100 dBnHL. The mean P1 and N1 latencies were 13.96 ± 1.17 m s and 21.50 ± 1.66 m s, with a mean corrected P1-N1 amplitude of 0.88 ± 0.34, and mean asymmetry ratio of 13 ± 10%. Median threshold was 80 dBnHL. Significant positive correlation between neck length and both P1, N1 latencies, and significant negative correlation between neck length and corrected P1-N1 amplitude were observed.
CONCLUSIONS: cVEMP paediatric normative data has been established for Singaporean school-aged children. The study also confirmed that neck length did have a significant influence on both latencies and corrected amplitude.

Angular acceleration stimulation of a semicircular canal causes an increased firing rate in primary canal afferent neurons that result in nystagmus in healthy adult animals. However, increased firing rate in canal afferent neurons can also be caused by sound or vibration in patients after a semicircular canal dehiscence, and so these unusual stimuli will also cause nystagmus. The recent data and model by Iversen and Rabbitt show that sound or vibration may increase firing rate either by neural activation locked to the individual cycles of the stimulus or by slow changes in firing rate due to fluid pumping (\"acoustic streaming\"), which causes cupula deflection. Both mechanisms will act to increase the primary afferent firing rate and so trigger nystagmus. The primary afferent data in guinea pigs indicate that in some situations, these two mechanisms may oppose each other. This review has shown how these three clinical phenomena-skull vibration-induced nystagmus, enhanced vestibular evoked myogenic potentials, and the Tullio phenomenon-have a common tie: they are caused by the new response of semicircular canal afferent neurons to sound and vibration after a semicircular canal dehiscence.

The video head impulse test (vHIT) and cervical and ocular vestibular evoked myogenic potentials (cVEMP and oVEMP) are new methods for measuring peripheral vestibular function. The objectives of this study were to compare these tests and the traditionally used caloric test in patients with small and medium-sized untreated vestibular schwannoma (VS) and to measure the correlation between the tests\' results and tumor volume.
National cross-sectional study.
Tertiary university clinic.
Prevalence of abnormal cVEMP, oVEMP, caloric test, and 6-canal vHIT results on the tumor side and the nontumor side were compared and related to tumor volume with regression analyses in 137 consecutive VS patients assigned to a wait-and-scan protocol in the period 2017 to 2019.
The sensitivity of 6-canal vHIT, caloric test, cVEMP, and oVEMP to detect vestibulopathy in VS patients was 51%, 47%, 39%, and 25%, respectively. Normal tests were found in 21% of the patients. The results of vHIT and caloric test were related to tumor volume, but this was not found for cVEMP and oVEMP.
The caloric test and 6-canal vHIT showed the highest sensitivity in detecting vestibulopathy in untreated VS patients. vHIT, and particularly the posterior canal, was limited with a high prevalence of abnormal results on the nontumor side. A combination of cVEMP and caloric test was favorable in terms of a relatively high sensitivity and low prevalence of abnormal results on the nontumor side. Larger tumors had a higher rate of pathology on caloric testing and vHIT.

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.
CONCLUSIONS: This finding reinforces the hypothesis of a common utricular origin for both SVH and oVEMPs which is distinct from the saccular origin of cVEMPs.