Rotational Chair Testing Research Articles

Evaluating cochlear implant outcomes in DFNA9 subjects: a comprehensive study on cerebral white matter lesions and vestibular abnormalities.

This study assessed whether the Fazekas score could account for the variability in cochlear implantation (CI) outcomes among individuals with DFNA9 and evaluated signal loss in the semicircular canals (SCCs) on magnetic resonance imaging (MRI) among individuals with DFNA9. This retrospective cross-sectional study included CI recipients with DFNA9. Pre-implantation MRI-scans were reviewed to determine the Fazekas score, localizing and grading cerebral white matter lesions (WML), and identify abnormalities in the SCCs. CI performance was assessed by evaluating phoneme scores one year post-implantation. The function of the SCCs was evaluated using rotatory chair testing with electronystagmography (ENG) and the video Head Impulse Test (vHIT). Forty-five subjects (49 ears) were enrolled. The phoneme scores significantly improved from 35% (IQR 11-50) pre-implantation to 84% (IQR 76-90) one year post-implantation. No correlation was observed between the Fazekas score and the one-year post-implantation phoneme score (rsp=0.003, p = 0.986). Signal loss in at least one SCCs was detected in 97.7% of subjects and 77.8% of ears. There was no correlation between vestibular test results and fluid signal loss in the SCCs on MRI. Most individuals with DFNA9 show improved speech recognition with CI. The observed variability in CI outcomes was not linked to the Fazekas score. Additionally, our study confirms a high prevalence of focal sclerosis in DFNA9. Recognizing the limitations of this study, further research is needed to explore the predictive role of the Fazekas score on CI outcomes and its relationship with vestibular function.

Just Keep Spinning? The Impact of Auditory and Somatosensory Cues on Rotary Chair Testing.

The purpose of this study was to determine whether providing realistic auditory or somatosensory cues to spatial location would affect measures of vestibulo-ocular reflex gain in a rotary chair testing (RCT) context. This was a fully within-subject design. Thirty young adults age 18-30 years (16 men, 14 women by self-identification) completed sinusoidal harmonic acceleration testing in a rotary chair under five different conditions, each at three rotational frequencies (0.01, 0.08, and 0.32 Hz). We recorded gain as the ratio of the amplitude of eye movement to chair movement using standard clinical procedures. The five conditions consisted of two without spatial information (silence, tasking via headphones) and three with either auditory (refrigerator sound, tasking via speaker) or somatosensory (fan) information. Two of the conditions also included mental tasking (tasking via headphones, tasking via speaker) and differed only in terms of the spatial localizability of the verbal instructions. We used linear mixed-effects modeling to compare pairs of conditions, specifically examining the effects of the availability of spatial cues in the environment. This study was preregistered on Open Science Framework (https://osf.io/2gqcf/). Results showed significant effects of frequency in all conditions (p < .05), but the only pairs of conditions that were significantly different were those including tasking in one condition but not the other (e.g., tasking via headphones vs. silence). Post hoc equivalence testing showed that the lack of significance in the other comparisons could be confirmed as not meaningfully different. These findings suggest that the presence of externally localizable sensory information, whether auditory or somatosensory, does not affect measures of gain in RCT to any relevant degree. However, these findings also contribute to the increasing body of evidence suggesting that mental engagement ("tasking") does increase gain whether or not it is provided via localizable instructions.

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.

Clinical characteristics and otolith dysfunction in presbyvestibulopathy: A retrospective cross-sectional analysis

ObjectiveThe Bárány Society recently established diagnostic criteria for presbyvestibulopathy, an age-related bilateral vestibular impairments in older individuals. Drawing upon a cross-sectional database, this study delves into the demographic and clinical features of presbyvestibulopathy patients and investigates the implications of otolith dysfunction. MethodsThe study retrospectively analyzed 1218 patients aged 60 years or older who visited the tertiary dizziness clinic in 2020, due to symptoms of dizziness or instability. By reviewing medical records, we gathered clinical information and laboratory vestibular test results, such as cervical and ocular vestibular evoked myogenic potentials, and subjective visual vertical. ResultsOut of 1218 patients aged 60 and above who reported dizziness or unsteadiness, 33 patients (2.7 %, with an average age of 74.2 ± 9.2 years) were diagnosed with presbyvestibulopathy. Deficiencies in horizontal angular vestibulo-ocular reflex were found in caloric tests (75 %), video head impulse tests (51.7 %), and rotatory chair tests (47.8 %), respectively. Otolith dysfunction was also observed, as shown by abnormal ocular and cervical vestibular evoked myogenic potentials in 62.96 % and 51.85 % of patients, and abnormal subjective visual vertical in 45.8 % of the cases. ConclusionsAmong elderly patients experiencing consistent dizziness or instability, the incidence of presbyvestibulopathy was approximately 2.7 % over one year. Alongside the abnormalities detected in the horizontal angular vestibulo-ocular reflex, significant changes were also noted in the ocular and cervical vestibular evoked myogenic potentials, as well as in the subjective visual vertical tests. As a result, it's vital to underscore the significance of both otolithic function and vestibulo-ocular reflex in the fundamental mechanisms of presbyvestibulopathy.

Comparing Video Head Impulse Testing With Rotary Chair in Pediatric Patients: A Controlled Trial.

To investigate the efficacy of video head impulse testing (VHIT) in detecting vestibular loss in pediatric patients with abnormal rotary chair testing, compared to a control group with normal rotary chair testing. Prospective, nonrandomized, controlled trial. Pediatric vestibular program at tertiary level children's hospital. Patients 3 to 18 years of age were recruited to undergo VHIT and rotary chair testing between September 2015 and November 2022. VHIT results were compared between an experimental group of 23 patients with vestibular symptoms and abnormal rotary chair testing results versus a control group of 14 patients without vestibular symptoms and with normal rotary chair testing results. The experimental group demonstrated reduced mean VHIT gain for the lateral semicircular canals (right = 0.83; left = 0.75) relative to the control group (right = 1.04, P = .005; left = 0.98, P < .001), but there was no significant difference between groups in superior and posterior canal responses. Among controls, there were no significant differences in mean gain values for each canal plane between age groups (3-7, 8-12, and 13-18 years) or between genders. A VHIT lateral canal gain value <0.85 and/or corrective saccades demonstrated 100% specificity and 100% positive predictive value for detecting vestibular loss on rotary chair, but only 69.6% sensitivity and 66.7% negative predictive value. VHIT is a useful test for detecting impairment of the lateral semicircular canals in children, though its sensitivity is limited relative to rotary chair. Its role in detecting vertical canal dysfunction in pediatric patients may be more limited and warrants further study.

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.

Dynamic change of vestibular function and the long-term prognosis of vestibular neuritis.

To comprehensively evaluate the dynamic change of vestibular function during long-term follow-up of vestibular neuritis, as well as the co-relationship with the outcomes of vestibular neuritis (VN), which provides the recommendations for vestibular function tests during the course of VN. A prospective cohort study was conducted on 16 patients with acute VN. Caloric test, vHIT, rotatory chair tests, VEMP, dizziness handicap inventory (DHI) score, and dynamic dizzy scales (VAS-DD) was first performed within 7 days of neuritis onset, which were further re-evaluated during the 6-12 months of follow-up. The dynamic changes on multiple objective vestibular examinations were analyzed during the acute and recovery stage of VN. We further evaluated the co-relationship between the vestibular dysfunction scales and the prognosis of VN. In more than 6 months of follow-up, 44% of the ultralow frequency, 94% of the low-to-mid frequency, and 44% of the high-frequency function of the horizontal semicircular canal returned to normal (p < 0.05). The change degree in symmetry of the rotatory chair test was correlated with the gain of the horizontal semicircular canal on the vHIT and the unilateral weakness (UW) value on the caloric test (p < 0.05). The change in DHI score was correlated with the phase; change in VAS-DD level correlated with the symmetry and TC of the rotatory chair test at VN recovery stage (p < 0.05). There was no significant correlation between the change in DHI score or change in VAS-DD and the degree of vestibular function recovery (p > 0.05). In general, vestibular function improved during the course of VN. The rotatory chair test can be used to evaluate the overall function of the vestibular system and the compensatory state in patients with VN.

Hypoxic acclimatization training improves the resistance to motion sickness.

Vestibular provocation is one of the main causes of flight illusions, and its occurrence is closely related to the susceptibility of motion sickness (MS). However, existing training programs have limited effect in improving the resistance to motion sickness. In this study, we investigated the effects of hypoxia acclimatization training (HAT) on the resistance to motion sickness. Healthy military college students were identified as subjects according to the criteria. MS model was induced by a rotary chair. Experimental groups included control, HAT, 3D roller training (3DRT), and combined training. The Graybiel scores were decreased in the HAT group and the 3DRT group and further decreased in the combined training group in MS induced by the rotary chair. Participants had a significant increase in blood pressure after the rotary chair test and a significant increase in the heart rate during the rotary chair test, but these changes disappeared in all three training groups. Additionally, LFn was increased, HFn was decreased, and LF/HF was increased accordingly during the rotary chair test in the control group, but the changes of these three parameters were completely opposite in the three training groups during the rotary chair test. Compared with the control group, the decreasing changes in pupillary contraction velocity (PCV) and pupillary minimum diameter (PMD) of the three training groups were smaller. In particular, the binocular PCV changes were further attenuated in the combined training group. Our research provides a possible candidate solution for training military pilots in the resistance to motion sickness.

Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients

ImportanceThe development of a vestibular implant has reached milestones and seems to be a promising therapeutic tool for bilateral vestibulopathy (BV). Given the former lack of therapeutic options for BV, the disease has received scant attention in the previous research literature. It is therefore of major importance to gain more insight into the underlying pathology of BV. Furthermore, as some research groups specifically use a combined vestibulo-cochlear implant, the size of the group of BV patients with associated hearing loss is of special interest.ObjectivesThe study aimed to determine the definite and probable etiology in bilateral vestibulopathy (BV) patients and to report on their hearing status.DesignThis study involves multicenter retrospective study design.SettingThe research setting is at tertiary referral centers.ParticipantsConsecutive BV patients diagnosed at the Antwerp University Hospital between 2004 and 2018 at the Maastricht University Medical Center between 2002 and 2015 and at the Geneva University Hospital between 2013 and 2018, who met the BV diagnostic criteria of the Bárány Society.Main outcome measuresPrimary interests were the etiology and hearing status of BV patients. Moreover, the data of vestibular tests were examined (caloric irrigation, rotatory chair tests, and video-head impulse test).ResultsThe authors identified 315 BV patients, of whom 56% were male patients. Mean age at diagnosis was 58.6 ± 15.1 (range 7–91) years. The definite cause was determined in 37% of the patients and the probable cause in 26% of the patients. No cause was identified in 37% of BV patients. The largest subgroup included patients with genetic etiology (31%), most frequently COCH mutation. Only 21% of patients (n = 61) had bilateral normal hearing. Almost half of the patients (45%, n = 134) had profound hearing loss in at least one ear.ConclusionBV is a heterogeneous condition, with over a third of cases remaining idiopathic, and nearly three-quarters affected by hearing loss. COCH mutation is the most common non-idiopathic cause of BV in our population. Only 21% of our BV patients presented with bilateral normal hearing.

Comparative utility of vestibular function tests in patients with peripheral and central vestibular dysfunction

BackgroundBithermal caloric irrigation, video head impulse test (vHIT), and rotational testing are commonly used to assess peripheral vestibular function, but the relative clinical utility of each test in differentiating patients with peripheral vestibulopathy is debated. ObjectivesTo determine whether (1) the combination of two or more vestibular tests enhances diagnostic utility over a single test; (2) abnormal test results on vestibular tests correlate with one another. MethodsRetrospective analysis of data collected from multidisciplinary vestibular clinics at two academic medical centers from 2016 to 2022. Results150 patients (54.10 ± 15.09 years, 88 females) were included. No individual test was significantly better at predicting the presence of peripheral vestibular damage (p > 0.05). vHIT test results improved significantly when combined with either the caloric test (p = 0.007) or rotary chair test (p = 0.039). Caloric and rotational testing had high sensitivity (74.65% and 76.06%, respectively) and specificity (83.54% and 78.48%, respectively). vHIT demonstrated excellent specificity (89.87%) but poor sensitivity (47.89%). Caloric, vHIT, and rotary chair tests results did not correlate with one another (p > 0.05). ConclusionsVestibular function tests have comparable diagnostic utility, yet each offers unique advantages. Caloric and rotational testing may be best suited for screening peripheral damage and vHIT may function ideally as a confirmatory test.

Application of the Barany Society's Presbyvestibulopathy Criteria in Older Adults With Chronic Dizziness.

To evaluate the older adults with chronic dizziness using the Barany Society's presbyvestibulopathy (PVP) diagnostic criteria and to assess the clinical usability of the criteria. Retrospective study. Single tertiary medical center. Among a total of 1964 patients aged 60 years or older, 55 patients who had symptoms suitable for the PVP criteria and who underwent all 3 vestibular function tests were analyzed. Of these 55 patients, 13 patients were diagnosed with PVP based on the peripheral vestibular hypofunction criteria; the remaining 42 patients were categorized as patients without PVP. In both patients with PVP and patients without PVP, postural imbalance or unsteadiness was the most frequently reported vestibular symptom. The rotatory chair test contributed the most to the quantitative diagnosis of PVP (6 of 13), followed by the caloric response test (3 of 13) and video head impulse test (vHIT; 2 of 13). When we included the presence of catch-up saccades in addition to the vHIT gain in the diagnostic criteria, 5 patients without PVP were additionally diagnosed with PVP. Furthermore, when accounting for the inclusion of catch-up saccades, patients who experienced recurrent falls demonstrated a significantly higher rate of impairments in the lateral and posterior semicircular canals according to the multivariate analysis. Considering the catch-up saccades in addition to vHIT gain seemed to contribute to the diagnosis of PVP and prediction of recurrent fall risks in older adults. Further studies are needed to solidify the diagnostic criteria for PVP.

Vestibular Preservation in Pediatric Cochlear Implantation.

Evaluate the rate of preserved vestibular function in pediatric cochlear implant surgery. Retrospective case review. Pre- and post-operative vestibular tests were compared in children who underwent cochlear implantation at a tertiary level pediatric hospital over a 4-year period. Data from 59 implanted ears in 44 children was included. Median age was 2.8 years at initial testing (range 7 months - 21 years) with 1:1 male/female ratio. Implant surgeries were 26 unilateral, 13 bilateral simultaneous, and 5 bilateral sequential. The majority were implanted with slim, non-styletted electrodes (86.4%) via a round window approach (91.5%). Normal pre-operative results were preserved post-operatively on rotary chair testing in 75% (21/28) of patients, cervical vestibular evoked myogenic potential testing in (75%) 30/40 of ears tested, ocular vestibular evoked myogenic potential testing in 85.7% (6/7) of ears tested, video head impulse testing in 100% (9/9) of ears tested, and computerized dynamic posturography in 100% (5/5) of patients tested. Overall, 62.5% of patients had no new deficits on any vestibular test performed post-operatively. Preservation rates of vestibular function following cochlear implant surgery were higher in this cohort than what has been reported in many earlier studies. Contemporary, less traumatic electrodes and insertion techniques may be a significant factor. The risk of causing a new, severe bilateral vestibular loss with long-term functional impacts appears to be low. Further study is warranted on the impacts of different cochlear implant electrode designs and insertion approaches on post-operative vestibular preservation. 4, Case Series Laryngoscope, 134:1913-1918, 2024.

The function of the vestibular organ in Hashimoto's thyroiditis.

The aim of the study was to identify the prognostic factors and the relationship between vertigo and the results of objective assessment of the vestibular organ and the levels of thyroid status in patients with Hashimoto's thyroiditis. The study population consisted of 28 women with Hashimoto's thyroiditis and coexisting chronic vertigo. In all patients, audiological assessment of hearing (tonal audiometry and impedance audiometry), Dix-Hallpike manoeuvre, caloric test, and kinetic tests (rotary chair test and swing chair test) were evaluated. Thyroid hormone levels [thyroid-stimulating hormone (TSH), free thyroxine (FT4)] and thyroid antibodies [autoantibodies against thyroid peroxidase (anti-TPO) and thyroglobulin (anti-TG)] were determined. The relationships between age, weight, height, and BMI and the results of the objective assessment of the vestibular organ were calculated. In the study group the mean age was 48 years and the mean BMI was 26.425. Normal hearing was found in 15 patients (54%). BPPV (n = 19), followed by Meniere's disease (n = 7) and vestibular neuronitis (n = 2), were the causes of chronic vertigo in this group of patients. The analysis of the objective assessment of the vestibular organ showed decreased excitability of the labyrinth in 15 patients (54%). Twenty-four patients presented with normal TSH and FT4 levels (85%). All patients presented with elevated anti-TPO and anti-TG levels. No correlation was found between age, weight, height, BMI, and the results of thyroid function tests or the assessment of the vestibular organ. We did not confirm the negative influence of thyroid levels or the increase in thyroid antibodies on the abnormal results of the rotary chair test or the caloric test.

Age-Related Differences of Vestibulo-Ocular Reflex Gains Based on Presbyvestibulopathy Objective Criteria

Background and Objectives With the growing acknowledgment of age-related vestibular impairments, consensus diagnostic criteria for presbyvestibulopathy (PVP) have been recently published. The PVP criteria retains its objectivity with video head impulse test (vHIT), rotatory chair test (RCT) and caloric test. These objective tests share testing principles, but targeted frequencies vary across the tests. The PVP criteria are applicable for subjects of age over 60. However, there are different age cut-offs for older male in different areas. The purpose of the present study was to explore whether the PVP diagnostic criteria were appropriate for early elderly (Eel) patients and to identify the most sensitive test for age-related loss of vestibular function.Subjects and Method Eligible patients, aged 60 years or older, complained of dizziness for at least 3 weeks, and have undergone at least one of the followings: vHIT, RCT, or caloric test. We selected two groups based on age: the Eel group (aged &gt;60 years but &lt;65 years) and the late elderly (Lel) group (aged ≥75 years).Results The vestibulo-ocular reflex (VOR) gains differed significantly between the Eel and Lel groups for vHIT and caloric gain test. No Eel patient met the PVP criteria but 26.1% of Lel patients met the criteria for vHIT, and the proportions of such patients in the two groups differed significantly.Conclusion Eel subjects could be considered to differ from Lel subjects. Given the significance of both averages, VOR difference and the proportional difference in terms of meeting the PVP criteria and vHIT may be optimal when evaluating age-related changes.

Patterns of vestibular function in patients with dizziness after COVID-19 vaccination; dual tertiary referral center study

Background Although dizziness is a common symptom after vaccination, the mechanism, and prognosis are not well understood. Aims/Objectives This study aimed to investigate patients with dizziness after COVID-19 vaccination by analyzing objective information. Methods A retrospective study of patients who visited the outpatient clinics of two institutes with a complaint of dizziness occurring within 72 h after a COVID-19 vaccination. Results In most cases, patients experienced only a single event of dizziness, and the subjective symptom was relieved after a few weeks. All patients decreased gain of vestibular ocular reflex (VOR). The vestibular function test results showed signs of central vestibulopathy in some cases. We separated patients into two groups; the direction-fixed nystagmus (DFN) group and the direction-changing nystagmus (DCN) group. All patients showed decreased gain on the rotational chair test (RCT). The DFN group showed an 80% decrease in video head impulse test (vHIT) gain, whereas the DCN group only showed a decrease of 25%. In RCT, 66% of the DFN group showed asymmetry compared to 20% showing asymmetry in the DCN group. Conclusion and significance The patients who suffered from dizziness after the COVID-19 vaccination exhibited decreased VOR gain and in some cases signs of central vestibulopathy.

Vestibular Dysfunction in Replication Factor C Subunit 1 (RFC1) Spectrum Disorder

More than 90% of replication factor c subunit 1 (RFC1) gene-related spectrum disorders such as cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) have bilateral vestibular dysfunction. A case with CANVAS presented in this paper showed repeat extension of AAGGG in the intron region of the RFC1 gene, and showed bilateral vestibular dysfunction in caloric test, vestibular evoked myogenic potential, video Head Impulse Test and rotary chair test. Visual enhanced vestibulo-ocular reflex tests also revealed abnormalities, suggesting the presence of combined lesions of the cerebellum and brainstem including vestibular nuclei.

Comparison of vestibular ocular reflex and gross motor development in children with semicircular canal aplasia and hypoplasia.

To examine the effect of vestibular ocular reflex on gross motor development in children with semicircular canal aplasia and hypoplasia. Case series. Children with congenital hearing loss underwent temporal bone computed tomography to assess their inner ear morphology. In this study, we examined eight children with bilateral complete aplasia of semicircular canals and seven children with bilateral hypoplasia of semicircular canals. Their vestibular ocular reflex was assessed using a damped rotational chair test. Their gross motor development was assessed by examining their ages of acquisition of head control and independent walking. All eight children with semicircular canal aplasia were diagnosed as having CHARGE syndrome and five children were found to have CHD7 mutations. Cochlear morphological examination showed that cochlear hypoplasia type III was the most common in eight of 16 ears in children with semicircular canal aplasia. In semicircular canal hypoplasia, incomplete partition type II was found in seven of 14 ears. All eight children with semicircular canal aplasia showed no response in the damped rotational chair test. Even the three children followed up were unresponsive in all tests. In the group with semicircular canal hypoplasia, three children showed normal responses, one child showed no response, and three children showed poor responses. The mean age of acquisition of head control was 9.1±6.3 months and that of independent walking was 31±8.1 months in children with semicircular canal aplasia. The mean age of acquisition of head control was 6.0±2.8 months and that of independent walking was 19.9±4.9 months in children with semicircular canal hypoplasia. We found that children with semicircular canal aplasia have severe dysfunction of the vestibular ocular reflex, and vestibular sensory cells derived from the semicircular canal may not be present. Semicircular canal hypoplasia is associated with dysfunction of the vestibular ocular reflex, but vestibular sensory cells may be present, and rotary nystagmus develops with growth similar to that observed in normal children previously reported. Semicircular canal aplasia is associated with CHARGE syndrome, and affected children tended to have a slower gross motor development. It was suggested that the combination of visual impairment and mental retardation may delay the development of central-level integrated functions and may further delay motor development.

Balance and Vestibular Deficits in Pediatric Patients with Autism Spectrum Disorder: An Underappreciated Clinical Aspect.

Children with autism spectrum disorder (ASD) not only have communication and social difficulties, but also exhibit poor balance and motor control ability, which frequently affect daily activities. Effective balance and motor control rely on the integration of somatosensory, visual, and vestibular inputs. Although reports of balance dysfunction in ASD have been documented, comprehensive studies of balance and vestibular function in children with ASD are scarce. In this study, we retrospectively reviewed 36 pediatric patients diagnosed with ASD who underwent balance/vestibular laboratory testing in our speciality clinic. Results from sensory organization test (SOT) or modified clinical test for sensory integration of balance (mCTSIB) found that out of 15 patients, 80% had abnormal findings. Of the children who successfully completed each vestibular test, abnormal responses were observed in 12 (80%) sensory organization tests, 5 (24%) vestibular evoked myogenic potential (VEMP), 22 (66%) videonystagmography (VNG), and 11 (32%) sinusoidal rotary chair tests. These results indicate that balance and vestibular testing may be of diagnostic value for clinicians and providers as an aid in early detection, intervention, and the development of appropriate management and therapies for this patient population. Increased awareness of this topic is warranted to promote better clinical management of this special group of patients and improve their quality of life.

Differences in vestibulo-ocular reflex time constants in patients with vestibular migraine versus vestibular neuritis/labyrinthitis

ObjectiveRecurrent dizziness, vertigo, and imbalance, as well as nausea or vomiting, can be induced by central or peripheral vestibular pathology. Vestibular migraine (VM) is a central pathology in which migraines reciprocally interact with vestibular nuclei. Vestibular neuritis and labyrinthitis (VN/L) are peripheral diseases involving inflammation of structures in the vestibular nerve or labyrinth. Because VM and VN/L in early stages can produce similar symptoms, diagnosis may require prolonged clinical evaluation. It has been suggested that differences in a patient's Vestibulo-Ocular Reflex (VOR) measured during the rotary chair step rotation test (RCS Test) can differentiate VM from peripheral pathologies, allowing for faster diagnosis. We sought to compare VOR time constants (VORTcs) in patients with VM versus VN/L, with the goal of exploring potential diagnostic value. Study designRetrospective chart review of patients seen at a hospital balance center between January 2010 and June 2019. SettingTertiary referral center. Patients68 patients (mean age = 58.15, 65 % female, 35 % male) were placed into two groups based on clinical diagnosis codes: 1) VM or 2) VN/L. Main outcome measure(s)Primary variable of VORTc, evoked by rotational step testing. ResultsPatients in the VM group demonstrated statistically significant elevated VOR Tcs outside the normed range of 10–19.5 compared to those in the VN/L group (p = 0.0003) while patients in the VN/L group demonstrated statistically significant shortened VOR Tcs (p = 0.0443) with the two populations having distinctly different VOR Tc profiles. ConclusionsOur results support the conclusion that elevated VOR Tcs are a sign of central vestibular pathology and highlight the benefit of utilizing rotary chair testing early in the diagnostic process as a powerful diagnostic tool when evaluating dizzy patients for central versus peripheral vestibular dysfunction. Our results suggest more particularly that elevated VOR Tcs can be utilized to identify patients with vestibular migraine, thus offering differentiation from patients with peripheral vestibular dysfunction earlier in the process of diagnosis, with an opportunity for earlier intervention.

Vestibular Imaging and Function in Patients With Inner Ear Malformation Presenting With Profound Hearing Loss.

ObjectiveVestibular impairment has been observed in patients with congenital hearing loss, but little is known about the vestibular anatomy and function of those in this group with inner ear malformations. This study aims to investigate the association between vestibulocochlear anatomy and vestibular function test results in children with inner ear malformations.Study DesignCase series with chart review.SettingPediatric patients with inner ear malformations presenting with bilateral profound hearing loss at a tertiary hospital from 1999 to 2017.MethodsEars were classified into subgroups based on anatomic abnormalities seen on computed tomography imaging. Cervical vestibular evoked myogenic potential (cVEMP), rotatory chair, and caloric test results were obtained and collated. Descriptive and inferential statistics were calculated.ResultsOf 82 ears, 29.3% had incomplete partition type II malformation, the most common type. The second-most common type was isolated vestibular organ anomaly (20.7%), which is not included in currently accepted categories. Most ears exhibited abnormal vestibular function. Abnormal vestibule volume was associated with a nonreactive cVEMP (P < .001). Radiologically abnormal lateral semicircular canals were associated with abnormal caloric and rotatory chair results (P < .001).ConclusionWith a relatively large number of cases of isolated vestibular organ anomaly not only in our study but also in previous publications, we suggest that this category be added to the subsets of inner ear malformations. Abnormal vestibule volume was significantly associated with a nonreactive cVEMP finding. The majority of patients with hearing loss secondary to inner ear malformations have abnormal vestibular function test results.