Otolithic functions in patients with residual dizziness after successful repositioning manoeuvres for unilateral posterior canal BPPV.

OBJECTIVE: To evaluate otolithic functions in patients with residual dizziness after successful canalith repositioning procedures (CRPs) for unilateral posterior canal benign paroxysmal positional vertigo (BPPV), and to investigate possible risk factors. METHODS: This case-control observational study included healthy controls and patients with residual dizziness after improvement following CRP for BPPV. All participants were subjected to full history taking, otoscopy, audiological basic evaluation, Dix-Hallpike test to search for posterior canal BPPV, residual dizziness screening, and vestibular evoked myogenic potential (VEMP) testing. Between-group differences were assessed and possible factors associated with residual dizziness were identified by univariate analysis. RESULTS: A total of 50 patients with residual dizziness (mean age, 56.53 ± 7.46 years [29 female: 21 male]) and 50 healthy controls (mean age, 58.13 ± 7.57 years [20 female: 30 male]) were included. A significant difference in VEMP latencies was found between the patient and control group (delayed in the patient group), with no significant between-group difference in amplitude in both ears. Aging, female sex, long duration of BPPV, number of CRPs, cervical VEMP and ocular VEMP abnormalities, and winter onset, were significantly associated with the risk of residual dizziness. CONCLUSIONS: Residual dizziness is a frequent sequel of BPPV that may relate to otolithic dysfunction. VEMP changes were revealed in the form of delayed latencies.

Comparison of vestibular function in hereditary hearing loss patients with GJB2, CDH23, and SLC26A4 variants.

To investigate the association between hereditary hearing loss and vestibular function, we compared vestibular function and symptoms among patients with GJB2, SLC26A4, and CDH23 variants. Thirty-nine patients with sensory neural hearing loss (11 males and 28 females) with biallelic pathogenic variants in either GJB2, SLC26A4, or CDH23 were included in this study (13 GJB2, 15 SLC26A4, and 11 CDH23). The patients were examined using caloric testing and cervical and ocular vestibular-evoked myogenic potentials (cVEMP and oVEMP). We also compared vestibular function and symptoms between patients with these gene variants and 78 normal-hearing ears without vestibular symptoms as controls. The frequency of semicircular canal hypofunction in caloric testing was higher in patients with SLC26A4 variants (47%) than in those with GJB2 (0%) and CDH23 variants (27%). According to the cVEMP results, 69% of patients with GJB2 variants had saccular hypofunction, a significantly higher proportion than in those carrying other variants (SLC26A4, 20%; CDH23, 18%). In oVEMP, which reflects utricular function, no difference was observed in the frequency of hypofunction among the three genes (GJB2, 15%; SLC26A4, 40%; and CDH23, 36%). Hence, discernable trends indicate vestibular dysfunction associated with each gene.

[Evaluation of semicircular canal and otolith graviceptive pathway function in patients diagnosed with motion sickness disorder based on the diagnostic criteria of the Bárány society].

Objective: To investigate the altered function of the semicircular canal and otolith graviceptive pathway in patients diagnosed with motion sickness disorder (MSD) based on the diagnostic criteria of the Bárány society, and explore its relevance to the pathogenesis of MSD. Methods: This is a case-control study. Twenty patients with MSD and age-and sex-matched healthy controls without a history of MSD from the Department of Neurology of Aerospace Center Hospital between March and August 2022 were recruited. All subjects completed the motion sickness susceptibility questionnaire-short version (MSSQ-short) and the motion sickness assessment questionnaire (MSAQ). Canal function was evaluated using caloric stimulation test and video head impulse test (vHIT), and subjective visual vertical/horizontal (SVV/SVH) and vestibular evoked myogenic potential (VEMP) were employed to assess otolith graviceptive function. Differences in vestibular function and correlations between the two groups were analyzed. Results: Each group consisted of 20 cases (9 males and 11 females). The mean age of the MSD and control groups was (26.9±3.9) years and (27.0±3.4) years, respectively. The scores of MSSQ-short [27.0 (22.5, 38.8) vs 1.2 (0, 3.2), P<0.001] and MSAQ [70.1 (54.5, 78.1) vs 11.8 (11.1, 13.9), P<0.001] were significantly higher in the MSD group compared with those of the control group. Evaluation of canal function revealed a significantly higher incidence of caloric stimulation intolerance in MSD patients (60.0%, 12/20) compared with that of the control group (20.0%, 4/20) (P=0.010). Evaluation of otolith graviceptive pathway indicated no significant difference in SVV, SVH and cervical VEMP (cVEMP) abnormality rates between the two groups (all P>0.05). The ocular VEMP (oVEMP) abnormality rate was significantly higher in the MSD group (55.0%, 11/20) than that of the control group (10.0%, 2/20) (P=0.002), with a delayed P1-wave latency compared with the control group [(18.4±1.2) ms vs (17.6±0.8) ms, P=0.018]. Further correlation analysis revealed that P1-wave latency in oVEMP was positively correlated with MSSQ-short (r=0.486, P=0.002) and MSAQ (r=0.391, P=0.015) scores, and duration of caloric intolerance symptoms (r=0.377, P=0.004). Conclusion: The presence of hypersensitivity to caloric stimulation and delayed latency of otolith function in patients with MSD suggests a "separation" between semicircular canal and otolithic function, which may be related to sensory conflict.

Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

BACKGROUND AND OBJECTIVES: We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. METHODS: In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. RESULTS: Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. DISCUSSION: The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. TRIAL REGISTRATION INFORMATION: ClinicalTrials.gov: NCT03049956.

Evaluation of Masseteric Vestibular Evoked Myogenic Potentials in Patients With Migraine.

PURPOSE: Masseter vestibular evoked myogenic potentials (mVEMP) involve the connection between the vestibular complex and trigeminal nerve nuclei. Given the theory that migraine is caused by increased activation of the trigeminal nerve, it is believed that mVEMP responses may have influenced in migraine patients. METHOD: The study included 20 individuals with migraine and 20 healthy controls. Latency, amplitude, and interaural amplitude asymmetry ratio of mVEMP responses recorded in migraine patients were compared with control group. RESULTS: Considering the mVEMP normalization study conducted by Basöz et al. (2021) in a similar age group and in the same clinic, latency prolongation and amplitude decrease were observed in subjects with migraines. Migraine is considered a central pathology, as shown in the cervical and ocular VEMP (cVEMP/oVEMP) literature. No difference was observed in the interaural amplitude asymmetry ratio, which is important in peripheral pathologies. Additionally, when the number of pathological ears was examined in order to understand the total exposure, it was observed that the number of pathological ears was significantly higher in the migraine group. CONCLUSION: In future studies, using mVEMP together with cVEMP and oVEMP tests, which allow evaluation of otolith organs and vestibular nuclei, will be valuable in determining the lesion location. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.25607901.

Efficacy of vestibular rehabilitation program in children with balance disorders and sensorineural hearing loss.

OBJECTIVE: Asses the efficacy of a Vestibular-balance rehabilitation program to minimize or reverse balance disability in children with sensorineural hearing loss. METHOD: Forty-five hearing-impaired children with balance deficits (i.e., variable degrees of sensorineural hearing loss or auditory neuropathy). Thirty-five were rehabilitated with cochlear implants, and ten with hearing aids. Their age ranged from 4 to 10 years old. A Pre-rehab evaluation was done using questionnaires, neuromuscular evaluation, vestibular and balance office testing, and vestibular lab testing (using cVEMP and caloric test). Customized balances, as well as vestibular rehabilitation exercises, have been applied for three months. That was followed by post-rehab assessment, including the Arabic DHI questionnaire, PBS, BESS, HTT, and DVA test. RESULTS: There was a statistically significant difference in all measured parameters (including the Arabic DHI questionnaire, PBS, BESS, HTT, and DVA test) after rehabilitation. CONCLUSIONS: Vestibular-balance rehabilitation intervention positively impacts vestibular and balance functions in hearing-impaired children.

[Practical instructions for recording vestibular evoked myogenic potentials]. / Praktische Hinweise zur Messung vestibulär evozierter myogener Potenziale.

Recording of vestibular evoked myogenic potentials (VEMPs) is a well-established method for functional diagnostics of the otolith organs. VEMPs are vestibular reflexes of the sacculus und utriculus to acoustic stimulation by air-conducted sound or bone-conducted vibration and are recorded by surface electrodes from the cervical (cVEMP) and ocular (oVEMP) muscles. The results of VEMP recordings are part of the neuro-otologic test battery and enable diagnosis of various vestibular disorders or differentiation between non-vestibular and peripheral vestibular vertigo. However, the methods for recording VEMPs vary substantially, although recording and stimulation parameters as well as methods of data analysis have a significant influence on the results. This article provides an overview of recommended parameters as well as practical instructions for the recording, analysis, and interpretation of VEMPs.

Impaired vestibular function associated with orthostatic hypotension in patients with multiple system atrophy.

BACKGROUND: Orthostatic hypotension (OH) is one of the most common symptoms in patients with multiple system atrophy (MSA). Vestibular system plays an important role in blood pressure regulation during orthostatic challenges through vestibular-sympathetic reflex. The current study aimed to investigate the relationship between vestibular function and OH in patients with MSA. METHODS: Participants with MSA, including 20 with OH (mean age, 57.55 ± 8.44 years; 7 females) and 15 without OH (mean age, 59.00 ± 8.12 years; 2 females) and 18 healthy controls (mean age, 59.03 ± 6.44 years; 8 females) were enrolled. Cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) tests were conducted to evaluate vestibular function. RESULTS: Patients with MSA presented with significantly higher rate of absent cVEMPs (57.1% vs 11.1%, p = 0.001) and oVEMPs (25.7% vs 0, p = 0.021) than controls. MSA patients with OH showed more absent cVEMPs (75.0% vs 11.1%, Bonferroni corrected p < 0.001) and oVEMPs (40.0% vs 0, Bonferroni corrected p = 0.003) than controls. Patients with OH also showed higher rate of absent cVEMPs than those without OH (33.3%, Bonferroni corrected p = 0.014). CONCLUSIONS: Our results demonstrated that impairment of vestibular function was associated with MSA, particularly in those with OH. Absent VEMPs may be a potential marker for MSA severity. Our findings suggest that impaired vestibular function is involved in OH development and may serve as an intervention target.

Vestibular-evoked myogenic potential abnormalities in Parkinson's disease with freezing of gait.

BACKGROUND: Vestibular dysfunction is closely associated with the pathophysiology of Parkinson's disease (PD) accompanied by freezing of gait (FOG); however, evidence supporting this clinical association is lacking. Vestibular-evoked myogenic potentials (VEMPs) have been widely acknowledged as a crucial electrophysiological parameter in the clinical evaluation of vestibular function. OBJECTIVE: The present study investigated the possible correlation of FOG occurrence with VEMP observations in patients diagnosed with PD. METHODS: Altogether, 95 idiopathic PD patients were recruited into the present cross-sectional study. All patients underwent motor and non-motor assessments using serial scales. In addition, the electrophysiological vestibular evaluation was conducted, which included cervical (cVEMP) and ocular VEMP (oVEMP) assessments. Furthermore, the correlations of bilateral c/oVEMP absence with clinical phenotypes, especially FOG, among the PD patients were analyzed. RESULTS: Among the 95 patients with PD, 44 (46.3%) had bilateral oVEMP absence and 23 (24.2%) had bilateral cVEMP absence, respectively. The proportions of patients with bilateral oVEMP absence (77.8% vs 30.9%, p = 0.004) and bilateral cVEMP absence (44.4% vs 19.5%, p = 0.035) were higher in the patient group exhibiting FOG than in the group without FOG. Following the adjustment of confounding variables, bilateral oVEMP absence (OR = 8.544, p = 0.007), rather than bilateral cVEMP absence, was shown to independently predict FOG occurrence in patients with PD. CONCLUSION: The close correlation between bilateral oVEMP absence and FOG in PD patients sheds new light on the possible role of central vestibular/upper brainstem dysfunction in FOG development in patients with PD.

Effects of Age on the Frequency Amplitude Ratio of Cervical and Ocular Vestibular Evoked Myogenic Potentials.

PURPOSE: An increase in the 1000/500 Hz frequency amplitude ratio (FAR) of the cervical and ocular vestibular evoked myogenic potentials (cVEMP and oVEMP, respectively) may serve as a potential biomarker for diagnosing Meniere's disease (MD). However, the aging process can also result in an increased FAR for VEMPs. In older patients, distinguishing whether changes in VEMP FAR are due to MD or aging processes becomes difficult. We aimed to investigate the effects of age on VEMP FARs and establish a FAR-normative range for different age groups. METHOD: cVEMP and oVEMP were recorded from a total of 106 participants grouped as young, middle-aged, and older adults using air-conducted tone bursts at 500, 750, and 1000 Hz at 125 dB pSPL. The FAR was calculated for the cVEMP and oVEMP for the following frequencies: FAR1 = 1000/500, FAR2 = 1000/750, and FAR3 = 750/500. RESULTS: A significant age-related effect was observed on the cVEMP FAR. Although the oVEMP FAR showed an increasing trend with age, it was not statistically significant. Age-based normative FAR values are provided. CONCLUSIONS: Drawing from the normative FAR from this study, there is evidence that the existing MD diagnostic criteria would misidentify a considerable number of older adults. Therefore, to reduce false positives, we recommend a more stringent cVEMP and oVEMP FAR criterion in older adults.

The role of vestibular function tests in nontuberculous mycobacterial otomastoiditis: A case report.

BACKGROUND: Nontuberculous mycobacteria (NTM), an extremely rare pathogen causing cervicofacial infections, may result in permanent hearing impairment or intracranial complications. Due to the lack of specific manifestations during the initial onset of NTM otomastoiditis, physicians may misdiagnose it as cholesteatoma or other common bacterial infections. PATIENT CONCERNS: A 44-year-old male who complained of left-sided aural fullness, otalgia, and dizziness for 2 months. DIAGNOSIS: The initial diagnosis was hypothesized to be cholesteatoma based on a whitish mass with mucoid discharge filling the entire outer ear canal on otoscopy and left-sided mixed hearing loss. However, NTM was identified by microbial culture at the 2-month follow-up after surgery. INTERVENTIONS: The patient underwent a left-sided exploratory tympanotomy. Because NTM otomastoiditis was diagnosed, 3 weeks of starting therapies were administered with azithromycin (500 mg/day, oral administration), cefoxitin (3 g/day, intravenous drip), and amikacin (750 mg/day, intravenous drip). The maintenance therapies were azithromycin (500 mg/day, oral administration) and doxycycline (200 mg/day, oral administration) for 7 months. OUTCOMES: The patient's clinical condition improved initially after surgery, but the otomastoiditis gradually worsened, combined with subtle meningitis, 2 months after surgery. The external auditory canal became swollen and obstructed, making it difficult to monitor the treatment efficacy through otoscopy. Thus, we used regular vestibular function tests, including static posturography, cervical vestibular evoked myogenic potentials, and video Head Impulse Test, to assess recovery outcomes. After antibiotic treatment, the infectious symptoms subsided significantly, and there was no evidence of infection recurrence 7 months after treatment. Improvements in static posturography and cervical vestibular evoked myogenic potentials were compatible with the clinical manifestations, but video Head Impulse Test showed an unremarkable correlation. LESSONS: The clinical condition of NTM otomastoiditis may be evaluated using vestibular tests if patients have symptoms of dizziness.

[The "difficult" patient-pearls and pitfalls of vestibular diagnostic tests: Part 2 : Difficult aspects of vestibular laboratory testing]. / "Schwierige" Patient:innen ­ Vestibularisdiagnostik unter erschwerten Bedingungen: Teil 2 : Schwierige Aspekte der apparativen Vestibularisprüfung.

Patients with the cardinal symptoms "vertigo" or "dizziness" may be a real challenge for the treating otorhinolaryngologist. While the first part of this educational series was focused on history taking and bedside neurotological examination, the present paper is devoted to difficult aspects of vestibular laboratory testing, including getting the indication right, what to do if my patient is not able to fully cooperate during the tests, how to choose the adequate diagnostic procedure depending on the patient's comorbidities, how to interpret discordant results of various tests. Finally the paper addresses which conclusions can be drawn (and cannot be drawn) from normal findings in vestibular testing and how to communicate this result to the dizzy patient.

Abnormal vestibular-evoked myogenic potentials as a risk factor for unpredicted falls in spinocerebellar ataxia: a preliminary study.

OBJECTIVE: This study aimed to correlate the symptoms and signs with the findings of laboratory vestibular function tests in patients with spinocerebellar ataxia (SCA). METHOD: We retrospectively recruited 26 patients with SCA (9 men, median age: 52, age range: 21-67). Assessments included Dizziness Handicap Inventory, EuroQoL Five-Dimension, symptom questionnaires manifesting during walking in daily life, the Scale for the Assessment and Rating of Ataxia (SARA), and vestibular function tests including 3D video-oculography, video head impulse test, subjective visual vertical, and cervical and ocular vestibular evoked myogenic potentials (VEMP). RESULTS: Cross-analyses revealed that the patients with VEMP abnormalities showed higher SARA (p = 0.014) and prevalence of unpredictable falls (p = 0.046). The patients with SCA1 more frequently had unpredictable falls (75%, p = 0.038) and VEMP abnormalities (88%, p = 0.001) compared to SCA2 (29% falls, 17% VEMP abnormalities) and SCA6 (no falls or VEMP abnormalities). CONCLUSION: Abnormal VEMPs are strongly associated with unpredicted falls in patients with SCA, particularly in those with SCA1. Impaired processing of otolithic information may contribute to falls in SCAs, and VEMP may help identifying the patients with a risk for unpredicted falls and preventing fall-related injuries in SCA. Limited number of patients with lower SARA scores warrant further confirmatory studies.

Diagnostic value of vestibular evoked myogenic potentials in benign paroxysmal positional vertigo.

OBJECTIVES: Vestibular evoked myogenic potentials (VEMPs) are useful for studying the disturbances along nerve pathways implicated in the transmission of neurological information from otolithic organs related to vestibular function. This study aims to determine the differences in VEMPs in patients affected with benign paroxysmal positional vertigo (BPPV). METHODS: We recruited 36 patients, 9 diagnosed with recurrent BPPV (rBPPV), 9 with only one episode of vertigo (iBPPV), and 18 as a control group. We performed cervical and ocular VEMPs (cVEMPs and oVEMPs). RESULTS: We observed differences in asymmetry ratio, which was 41.82% in cVEMPs in iBPPV and 68.27% in oVEMPs in rBPPV, while no asymmetry was found in control cases. Also, there was a lack of both VEMP responses in 22.2% of cases and an absence of cVEMP in 11.1% in iBPPV; in rBPPV, 11.1 % presented no responses in cVEMPs or oVEMPs, 22.2% showed no oVEMP, and 11.1% showed no cVEMP. These values were normal in the control group. CONCLUSION: The value of VEMPs in BPPV demonstrates the implication of vestibular damage, mainly utricle damage. For better sensitivity in detecting otolith abnormalities, we should perform oVEMPs and cVEMPs in recurrent BPPV and early stages of BPPV.

Evaluating Fixed Single-Point Parameters When Applied to Vestibular Evoked Myogenic Potentials: The Effect of Single Point and Signal Window.

OBJECTIVES: Several studies have applied a common objective detection algorithm (fixed single point [ Fsp ]) for detection of the vestibular evoked myogenic potential (VEMP). However, fundamental parameters of Fsp , such as establishing the location and duration of a signal window, have not been examined. In addition, Fsp criterion values used for response detection have not been established for cervical VEMPs (cVEMPs) or ocular VEMPs (oVEMPs). The purpose of this article was to investigate the effect of various single points and signal windows on Fsp , as well as determining Fsp criteria to determine response presence for cVEMP and oVEMP in a group of young healthy participants. DESIGN: Twenty young healthy adults under the age of 30 and with no history of hearing or balance concerns were enrolled in the study protocol. Air-conducted cVEMPs and oVEMPs were evoked using 500 Hz tone bursts at 123 dB pSPL recorded at a fixed electromyography activation of 50 µV for cVEMPs and 35° gaze angle for oVEMPs. Responses were analyzed off-line using visual and objective detection. Fsp was applied to cVEMPs and oVEMPs using a range of single points and signal windows. RESULTS: Noise variance was lowest for cVEMPs at the latency of P1, and for oVEMPs noise variance was not significantly different across the single-point latencies. On average, extending the length of the signal window lowered the Fsp value in cVEMPs and oVEMPs. An Fsp value of 2.0 was chosen as the criterion cutoff associated with the 95th percentile during no-response conditions using group data for cVEMPs and oVEMPs, respectively. Fsp values for cVEMPs and oVEMPs were not significantly different from each other. DISCUSSION: This study established single-point latency and time-window parameters for VEMP-related applications of the Fsp detection algorithm. Fsp criteria values were established for cVEMP and oVEMP. Using these parameters, responses were detected in all participants.

Does Location of Intralabyrinthine Vestibular Schwannoma Determine Objective and Subjective Vestibular Function?

BACKGROUND AND OBJECTIVE: The incidence of intralabyrinthine schwannomas is increasing, and a growing attention is given to the detrimental effects on hearing function. On the contrary, the vestibular profile of intralabyrinthine vestibular schwannomas (VSs) is still not well understood. We aimed to investigate and report the observed relationships between the intralabyrinthine location of the schwannomas and objective and subjective vestibular profile of the patients. METHODS: Retrospective cohort study of 20 consecutive individuals with sporadic intralabyrinthine schwannomas and grouped according to the intralabyrinthine location of the schwannomas. Vestibular testing consisted of the video head impulse test of all three semicircular canals, the caloric test, cervical and ocular vestibular evoked myogenic potentials, and the dizziness handicap inventory. A nonparametric unpaired t test was performed to compare groups, and Fisher's exact test was used for categorical data. RESULTS: The median video head impulse test gains (lateral, anterior, posterior) were 0.40, 0.50, and 0.75 for intravestibular schwannomas and 0.93, 1.52, and 0.91 for intracochlear schwannomas ( p = 0.0001, p = 0.009, p = 0.33), respectively. Caloric unilateral weakness had a median of 100% for intravestibular schwannomas and 14% for intracochlear schwannomas ( p = 0.0001). The mean dizziness handicap inventory was 21 for intravestibular schwannomas and 1 for cochlear schwannomas ( p = 0.02). There were no significant differences in vestibular evoked myogenic potentials according to intralabyrinthine location. CONCLUSION: By both objective and subjective measures, intralabyrinthine schwannomas with an intravestibular component has significantly worse vestibular function than schwannomas with purely cochlear involvement.

Audiovestibular Findings in Patients with Concurrent Superior Canal Dehiscence and Vestibular Schwannoma.

OBJECTIVE: To describe the clinical-instrumental findings in case of concurrent superior canal dehiscence (SCD) and ipsilateral vestibular schwannoma (VS), aiming to highlight the importance of an extensive instrumental assessment to achieve a correct diagnosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Five patients with concurrent SCD and VS. INTERVENTION: Clinical-instrumental assessment and imaging. MAIN OUTCOME MEASURE: Clinical presentation, audiovestibular findings, and imaging. RESULTS: The chief complaints were hearing loss (HL) and unsteadiness (80%). Other main symptoms included tinnitus (60%) and pressure-induced vertigo (40%). Mixed-HL was identified in three patients and pure sensorineural-HL in 1, including a roll-over curve in speech-audiometry in two cases. Vibration-induced nystagmus was elicited in all cases, whereas vestibular-evoked myogenic potentials showed reduced thresholds and enhanced amplitudes on the affected side in three patients. Ipsilesional weakness on caloric testing was detected in three patients and a bilateral hyporeflexia in one. A global canal impairment was detected by the video-head impulse test in one case, whereas the rest of the cohort exhibited a reduced function for the affected superior canal, together with ipsilateral posterior canal impairment in two cases. All patients performed both temporal bones HRCT scan and brain-MRI showing unilateral SCD and ipsilateral VS, respectively. All patients were submitted to a wait-and-scan approach, requiring VS removal only in one case. CONCLUSION: Simultaneous SCD and VS might result in subtle clinical presentation with puzzling lesion patterns. When unclear symptoms and signs occur, a complete audiovestibular assessment plays a key role to address imaging and diagnosis.

Evidence of Vestibular and Balance Dysfunction in Patients With Mild Cognitive Impairment and Alzheimer's Disease.

OBJECTIVES: Given the expected rise in dementia prevalence, early diagnosis is vital. As a growing body of literature has identified a potential association between vestibular function and cognition, vestibular assessment may aid in early screening. The aim of the study was to better comprehend the proposed association between vestibular function and Alzheimer's disease (AD) by comparing vestibular parameters (vestibular function testing and clinical balance measures) between a group with mild cognitive impairment (MCI), AD, and healthy controls with age-normal cognition. DESIGN: Cross-sectional analysis of the GECkO study, an ongoing prospective single-center longitudinal cohort study. This study included 100 older adults (55 to 84 years). A total of 33 participants with MCI, 17 participants with AD, and 50 participants of age, sex, and hearing-matched healthy controls were included. RESULTS: Participants with AD demonstrated a delayed latency of the p13 component measured by cervical vestibular-evoked myogenic potentials (cVEMP) compared with healthy controls and participants with MCI. Other measures including n23 latency, presence of intact responses, rectified amplitude, mean rectified voltage (measured by cVEMP) and lateral vestibulo-ocular reflex gain (measured by video Head Impulse Test [vHIT]) did not differ between groups. The Timed Up and Go (TUG), Performance-Oriented Mobility Assessment-Balance subscale (POMA-B), and Functional Gait Assessment (FGA) differed significantly between the three groups. Here, more cognitively impaired groups were associated with worse clinical balance scores. CONCLUSIONS: Vestibular and balance deficits were more prevalent in groups with increasing cognitive decline. Regarding vestibular function testing, p13 latency as measured by cVEMP was delayed in participants with AD. Other cVEMP or vHIT measures did not differ between groups. All three clinical balance assessments (TUG, POMA-B, and FGA) resulted in worse scores along the AD continuum. Future research integrating vestibular parameters that add value (including otolith function testing, balance, and spatial navigation) is recommended to validate the association between vestibular function and cognition while avoiding redundant testing.