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.

[Discussion and analysis the value of supine median³ nystagmus in the diagnosis and treatment of HC-BPPV].

Objective:To explore the clinical value of supine median³ nystagmus in the accurate diagnosis of horizontal semicircular canal benign paroxysmal positional vertigo（HC-BPPV）. Methods:A total of 187 patients with HC-BPPV admitted to the First Affiliated Hospital of Xi'an Jiaotong University from June 2020 to March 2021 were selected. Among them 42 cases of Cupulolithiasis and 145 cases of Canalithiasis. The nystagmus parameters of patients left and right supine position and supine median³ position were recorded in detail by RART. According to the direction of supine median³ nystagmus, patients were divided into three groups: group A（nystagmus to weak side）, group B（nystagmus to strong side）, group C（negative nystagmus）. The canalith repositioning manoeuvres（CRM） was carried out by utility of an automatic vestibular function diagnosis and therapy system（SRM-IV）. The cure rate of CRM in three groups of HC-BPPV patients was compared, Multivariate logistic regression analysis was performed to analyze the influencing factors of CRM for HC-BPPV. Results:The cure rates of group A, group B and group C were 81.58%, 16.13% and 56.25%, respectively. The difference among the three groups was statistically significant. Then a pairwise comparison of group A, B and C, the difference was statistically significant（χ²A-B=40.294,P<0.001,χ²B-C=14.528, P<0.001,χ²A-C=11.606, P=0.001）; the results of multivariate logistic regression analysis showed that the direction of supine median³ nystagmus and BMI were the influencing factors of CRM for HC-BPPV. Conclusion:The direction, intensity and duration of supine median³ nystagmus play an important role in determining the responsibility semicircular canal of HC-BPPV.

[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.

Evaluation of the reliability and validity of the upright head roll test for lateral semicircular canal benign paroxysmal positional vertigo.

BACKGROUND: The upright head roll test (UHRT) is a recently introduced diagnostic maneuver for lateral semicircular canal benign paroxysmal positional vertigo (LSC-BPPV). OBJECTIVE: This study aimed to evaluate the reliability and validity of the UHRT. METHODS: Two separate studies were conducted. Study 1 analyzed 827 results of videonystagmography (VNG) to assess UHRT reliability, and Study 2 analyzed 130 LSC-BPPV cases to evaluate UHRT validity. RESULTS: The inter-test reliability between UHRT and the supine head roll test (SHRT) showed substantial agreement (Cohen's kappa = 0.753) in direction-changing positional nystagmus (DCPN) and almost perfect agreement (Cohen's kappa = 0.836) in distinguishing the direction of DCPN. The validity assessment of UHRT showed high accuracy in diagnosing LSC-BPPV (80.0%) and in differentiating the variant types (74.6%). UHRT was highly accurate in diagnosing the canalolithiasis type in LSC-BPPV patients (Cohen's kappa = 0.835); however, it showed only moderate accuracy in diagnosing the cupulolithiasis type (Cohen's kappa = 0.415). The intensity of nystagmus in UHRT was relatively weaker than that in SHRT (P < 0.05). CONCLUSION: UHRT is a reliable test for diagnosing LSC-BPPV and distinguishing subtypes. However, UHRT has a limitation in discriminating the affected side owing to a weaker intensity of nystagmus than SHRT.

The effect of increased listening effort on the balance performance of patients with compensated vestibular lesion.

OBJECTIVES: This study investigated the effects of listening effort (LE) on balance in patients with compensated vestibular deficits compared to healthy peers. METHODS: The subjects included two main groups: a control group of 15 healthy subjects and a study group of 19 patients with compensated vestibular pathology. The computerized dynamic posturography test (CDP) was conducted without the speech-in-noise task as a baseline, then the participant was subjected to a dual task in which the auditory task (speech-in-noise sentences) was given as the primary task, and the balance function test was the secondary task. RESULTS: WITHIN-GROUP ANALYSIS: The study group showed statistically significantly worse values of all body balance parameters under dual-task than the baseline in all conditions. These differences were much higher under the compliant platform conditions. However, these findings were not statistically significant in the control group. BETWEEN-GROUP ANALYSIS: The study group showed a statistically significant decline in body balance reactions compared to the control group under dual-task with increased listening effort and the compliant platform. Study subgroup analysis revealed statistically significant differences between patients with unilateral vestibular loss (UVL) and those with bilateral vestibular loss (BVL) in the unstable platform condition. CONCLUSION: Our study regarding implementing a dual-tasking paradigm as a measure of LE during the evaluation of chronic vestibular patients with CDP demonstrated how dual-tasking with increased LE affects postural stability. Because of this, patients will probably be more prone to tripping and falling in multitasking situations, as found in real-world settings. This fact should be taken into consideration while testing patients with chronic vertigo and compensated states at VNG. A dual-task paradigm helps uncover the unrevealed pathology.

Persistent postural-perceptual dizziness: A functional neuro-otologic disorder.

Persistent postural-perceptual dizziness (PPPD) is a functional neuro-otologic disorder that is the most frequent cause of chronic vestibular syndrome. The core vestibular symptoms include dizziness, unsteadiness, and non-spinning vertigo, which are exacerbated by an upright posture or walking, active or passive motion, and exposure to moving or complex visual stimuli. PPPD is mostly precipitated by acute or episodic vestibular diseases; however, its symptoms cannot be accounted for by its precipitants. PPPD is not a diagnosis of exclusion, but may coexist with other structural diseases. Thus, when diagnosing PPPD, the patient's symptoms must be explained by PPPD alone or by PPPD in combination with a structural illness. PPPD is most frequently observed at approximately 50 years of age, with a female predominance. Conventional vestibular tests do not reveal any specific signs of PPPD. However, the head roll-tilt subjective visual vertical test and gaze stability test after exposure to moving visual stimuli may detect the characteristic features of PPPD, that is, somatosensory- and visually-dependent spatial orientation, respectively. Therefore, these tests could be used as diagnostic tools for PPPD. Regarding the pathophysiology of PPPD, neuroimaging studies suggest shifts in interactions among visuo-vestibular, sensorimotor, and emotional networks, where visual inputs dominate over vestibular inputs. Postural control also shifts, leading to the stiffening of the lower body. To treat PPPD, selective serotonin reuptake inhibitors/serotonin noradrenaline reuptake inhibitors, vestibular rehabilitation, and cognitive behavioral therapy are used alone or in combination.

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.

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.

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

OBJECTIVE: 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. STUDY DESIGN: Retrospective study. SETTING: Single tertiary medical center. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Relationship between clinical measures of hearing and clinical measures of vestibular function.

PURPOSE: Patients often have basic audiometry (BA) but not objective diagnostic tests of the vestibular system (VNG) when complaining of symptoms of a vestibular disorder. The relationship of BA results to VNG results is unknown. This study sought to determine if BA scores are related to impaired VNG scores. MATERIALS AND METHODS: We reviewed electronic medical records at a tertiary care center, for patients seen between 2015 and 2021 who had had both a BA and a VNG (n = 651). BA subtests were pure tone averages, word recognition, and tympanogram. VNG subtests were cervical vestibular evoked myogenic potentials, Dix-Hallpike maneuvers, and bi-thermal caloric tests. All tests were summarized as normal/abnormal. RESULTS: More subjects had abnormal BA than abnormal VNG scores. Age but not sex was significantly related to abnormal scores. High BP was a significant comorbidity in 15 % of the sample, more in patients with abnormal than normal VNG scores. Although the abnormal BA and abnormal VNG were significantly related, pure tone averages and tympanogram scores were not related to VNG subtests. Abnormal word recognition with both ears combined was significantly related to normal and abnormal bi-thermal caloric tests. CONCLUSIONS: If the clinician needs to know of any VNG impairment, in general, then performing a BA without a VNG might suffice. If the clinician needs information about the details of possible vestibular impairment, then a VNG should be performed.

Evaluation of Mental Rotation Ability in Patients with Unilateral Benign Paroxysmal Positional Vertigo.

BACKGROUND: Our study aims to determine whether there are differences in mental rotation abilities between unilateral benign paroxysmal positional vertigo patients and healthy controls using object-based mental rotation tasks. METHODS: Our study included 17 unilateral posterior canal benign paroxysmal positional vertigo patients and 20 healthy adults. Spontaneous nystagmus test, saccade test, and dynamic positional tests with videonystagmography and object-based mental rotation test with 2-dimensional images of cubes rotated at certain angles in 3-dimensional space were performed on the participants. The mental rotation test response time and the number of correct answers were compared between patients and controls. We also evaluated whether there was a relationship between saccade test parameters and mental rotation test parameters in our study. RESULTS: No significant relationship was found between benign paroxysmal positional vertigo patients and controls on any of the dependent measures (P -gt; .05). When we evaluated the relationship between saccadic latency and accuracy and mental rotation test response time and number of correct answers in benign paroxysmal positional vertigo patients, no significant relationship was found (P -gt; .05). CONCLUSION: Our findings show that unilateral, posterior canal benign paroxysmal positional vertigo does not affect object-based mental rotation performance. In our study, no correlation was found between saccadic function and mental rotation ability in unilateral benign paroxysmal positional vertigo patients.

Variability in the Results of Vestibular Assessment in Patients with Genetically Confirmed Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome.

BACKGROUND: Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) presents an unpredictable and uneven clinical development of cerebellar ataxia, neuropathy, and vestibular areflexia. The aim of this study is to report the variability of vestibular test results in genetically confirmed patients with cerebellar ataxia, neuropathy, and vestibular areflexia syndrome. METHODS: Caloric testing, video head impulse test (vHIT), and rotatory chair testing were performed in 7 patients who presented pathogenic repeat expansions in the replication factor complex unit 1 gene related to cerebellar ataxia, neuropathy, and vestibular areflexia syndrome. RESULTS: Reduced vestibulo-ocular reflex (VOR) gain was observed in 100% of the patients in rotatory chair testing. Three of them had bilateral areflexia in caloric testing while 2 showed unilateral hypofunction and 2 had no alterations in the test. Only 1 patient had bilateral abnormal vHIT with gains under 0.6 in both ears. CONCLUSION: Genetic testing allows an early diagnosis of cerebellar ataxia, neuropathy, and vestibular areflexia syndrome, whereby the vestibular system may be affected to different degrees. Rotatory chair testing has a higher sensitivity for the detection of vestibular hypofunction in these patients. Caloric testing can provide additional information. vHIT might underdiagnose patients with mild-to-moderate vestibulopathy.

Efficacy of the Head Rotation Test With Bowing for the Lateral Canal Benign Paroxysmal Positional vertigo.

OBJECTIVES: To evaluate the efficacy of the head rotation test with bowing (B-HRT) in the sitting position in diagnosing lateral semicircular canal benign paroxysmal positional vertigo (LSC-BPPV). METHODS: The efficacy outcomes of lateralization of 25 patients with LSC-BPPV were prospectively evaluated using B-HRT. Traditional head toration in the supine position (S-HRT) and the bow and lean test were also assessed for comparative effectiveness. RESULTS: Direction-changing nystagmus was detected in all patients with LSC-BPPV (100%) using B-HRT. The nystagmus direction (geotropic or apogeotropic) determined by B-HRT was consistent with that determined by S-HRT with a perfect level of agreement (Cohen κ = 1.0, p < 0.001**). In 76.0% of the cases, the determination of the affected ear was concordant between B-HRT and S-HRT (Cohen κ = 0.409, p = 0.037*). The concordance rate between B-HRT and bow and lean test showed a fair level of agreement (68.0%; Cohen κ = 0.286, p = 0.126) with no statistical significance. On comparing the peak slow-phase velocity (SPV), SPVs of positional nystagmus on the stronger side and weaker side did not differ statistically significantly between S-HRT and B-HRT. In 12 of the 25 cases, in which the peak SPV asymmetry was determined as less than 30% by S-HRT (average, 11.00 ± 6.87%), the asymmetry determined by B-HRT (average, 47.31 ± 34.78%) was significantly higher, facilitating lesion identification by performing B-HRT together ( p = 0.001*). CONCLUSION: B-HRT in the sitting position identified direction-changing nystagmus in LSC-BPPV. B-HRT is helpful in facilitating the diagnosis of LSC-BPPV in the sitting position and determination of the affected ears in cases with nonprominent differences in bilateral nystagmus intensity according to S-HRT.

Capturing nystagmus during vertigo attacks using a smartphone: adherence, characteristics, pearls and pitfalls.

OBJECTIVE: To investigate adherence, characteristics, and first clinical experiences of capturing ictal nystagmus at home, which can be performed to complement the diagnostic process in patients with episodic vestibular symptoms. METHODS: Patients were recruited at a tertiary referral center in case capturing ictal nystagmus could contribute to the diagnostic process (e.g., to detect or rule out BPPV). They were asked to capture ictal nystagmus with their own smartphone at home, using a smartphone-based adapter (Nystagmocatcher, Balansdiagnos, Stockholm, Sweden). All recordings were analyzed by the last author (RvdB), and the adherence, characteristics, and first clinical experiences were evaluated. RESULTS: Seventy patients with vestibular symptoms were asked to participate in this study. Sixty-two (89%) agreed to participate. The median period of participation was 86 days. Fifty-one patients experienced attacks during the study period. Eventually, 51% of them provided eye movement recordings sufficient for analysis. Different types of nystagmus were observed: positional nystagmus related to BPPV, positional nystagmus not related to BPPV, functional eye movements, and the absence of nystagmus or functional eye movements. Capturing ictal nystagmus could contribute to the diagnostic process in several ways, including to detect or rule out BPPV, to detect or rule out vestibular origin of symptoms, to determine the affected side, telemedicine, to monitor attack frequency, and to detect malingering. Furthermore, strict guidance of patients was necessary, which could be time-consuming. CONCLUSION: Capturing ictal nystagmus can contribute to the diagnostic process in several ways, which motivates to rethink current clinical workflow in vestibular medicine. However, strict guidance is necessary and not all patients provide ictal recordings. In an outpatient setting, it would be advised to use ictal nystagmus recordings on indication, to complement the diagnostic process.

Characteristics and Possible Mechanisms of Direction-Reversing Nystagmus During Positional Testing in Patients With Benign Paroxysmal Positional Vertigo.

OBJECTIVES: The occurrence of direction-reversing nystagmus during positional testing in patients with benign paroxysmal positional vertigo (BPPV) is not uncommon. Further in-depth analysis of the characteristics and possible mechanisms of direction-reversing nystagmus will help us to diagnose and treat BPPV more precisely. The study aimed to analyze the incidence and characteristics of direction-reversing nystagmus during positional testing in BPPV patients, evaluate the outcomes of canalith repositioning procedure for these patients, and further explore the possible mechanism of reversal nystagmus in BPPV patients. STUDY DESIGN: Retrospective study. SETTING: Single-center study. PATIENTS: A total of 575 patients with BPPV who visited the Vertigo Clinic of our hospital between April 2017 and June 2021 were enrolled. MAIN OUTCOME MEASURES: Dix-Hallpike and supine roll tests were performed. The nystagmus was recorded using videonystagmography. The characteristics of direction-reversing nystagmus and the possible underlying mechanism were analyzed. RESULTS: Patients with BPPV who showed reversal nystagmus accounted for 9.39% (54 of 575) of all BPPV patients visiting our hospital during the same period, of which 5.57% (32 of 575) had horizontal semicircular canal BPPV (HC-BPPV), and 3.83% (22 of 575) had posterior semicircular canal BPPV (PC-BPPV). The maximum slow-phase velocities (mSPVs) of the first-phase nystagmus were greater in HC-BPPV and PC-BPPV patients with reversal nystagmus than those without ( p = 0.04 and p = 0.01, respectively). In all HC-BPPV and PC-BPPV patients with reversal nystagmus, the mSPV of the first-phase nystagmus was greater than that of the second-phase nystagmus ( p < 0.01). The duration of the second-phase nystagmus was longer than 60 seconds in 93.75% (30 of 32) of the HC-BPPV patients and 77.27% (17 of 22) of the PC-BPPV patients ( p = 0.107, Fisher exact test). HC-BPPV and PC-BPPV patients with reversal nystagmus both required more than one canalith repositioning procedure compared with those without (HC-BPPV: 75 versus 28.13%, p < 0.001; PC-BPPV: 59.09 versus 13.64%, p = 0.002). CONCLUSIONS: The cause of second-phase nystagmus in BPPV patients with direction-reversing nystagmus may be related to the involvement of central adaptation mechanisms secondary to the overpowering mSPV of the first-phase nystagmus.

Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS): diagnostic contribution of vestibular function tests.

Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a recently recognised but underdiagnosed cause of late-onset hereditary ataxia. Symptoms may vary, and differential diagnoses can span several specialties. We report the case of a man in his 60 s who presented with a 10 year history of imbalance and progressive gait disturbance associated with a chronic spasmodic cough that preceded these symptoms by almost 30 years. He had previously undergone extensive testing for acquired and genetic causes of ataxia without a conclusive diagnosis. Brain MRI revealed cerebellar atrophy, and nerve conduction tests suggested a sensory ganglionopathy. Vestibular function testing was crucial for diagnosis, identifying a severe bilateral vestibulopathy. This led to the consideration of CANVAS, which was finally confirmed by genetic testing. This case raises awareness of this novel genetic disease, highlighting the importance of objective vestibular function tests in establishing an early diagnosis.

Paediatric vestibular assessment in French cochlear implant centres: Challenges and improvement areas.

OBJECTIVE: Survey of paediatric vestibular activity in all 30 French paediatric cochlear implant (CI) centres to identify challenges and areas of improvement. METHOD: All 30 French CI centres answered a 29-question questionnaire about their paediatric vestibular activity, equipment, and management in different clinical situations (e.g. vestibular assessment before a cochlear implantation or in cases of vertigo) at different ages. RESULTS: Eighteen CI centres had dedicated paediatric vestibular clinics and 12 did not. Minimum age required for vestibular testing was 3 years in eight centres. Four vestibular tests stood out: caloric tests, video Head Impulse Test (vHIT), rotating chair, vestibular evoked myogenic potentials (VEMP). Depending on the centre's experience, the use of vestibular tests in clinical routine was very heterogeneous. Expert centres mostly used vHIT and cervical VEMP (in bone conduction) for assessments before the first cochlear implantation in 1-year-old children. Dizziness assessment in 4-year children was based on the use of vHIT, cervical VEMP on bone conduction, rotatory test, and caloric test. Ocular VEMP was rarely used. CONCLUSIONS: Paediatric vestibular assessment requires specific expertise compared to adults. Due to a lack of specialised human resources, some centres may be unable to follow French paediatric CI guidelines. International recommendations could help standardise paediatric vestibular management and public health policies should be discussed to improve training and access for children.

Comparison of clinical characteristics and vestibular function test results in patients with vestibular migraine and Menière's disease.

OBJECTIVES: The differentiation between Vestibular Migraine (VM) and Meniere's Disease (MD) is difficult because of overlapping symptoms. The study aimed to compare the clinical characteristics and vestibular function test results between VM and MD patients. METHODS: Seventy-one patients with definite VM and 31 patients with definite unilateral MD were included. All patients received Caloric Test (CT), Video Head Impulse Test (vHIT) and Vestibular Evoked Myogenic Potential (VEMP) test within 7 days after visiting the hospital. Results of these tests were compared between groups. RESULTS: Most VM patients (64.0%) experienced spontaneous internal vertigo, while most MD patients (66.7%) experienced spontaneous external vertigo. MD patients had more severe vestibular symptoms and autonomic responses compared to VM patients during attacks (p =  0.03, p = 0.00, respectively). The nystagmus intensity of CT-induced was greater in VM patients than in MD patients (p = 0.003). More VM patients had CT intolerance and Central Positional Nystagmus (CPN) compared to MD patients (p = 0.002, p = 0.006, respectively). More MD patients had CT(+) and vHIT saccades wave compared to VM patients (p < 0.001, p = 0.002, respectively). The non-elicitation rate of cervical VEMP was higher, and the ocular VEMP amplitudes were lower in MD patients than in VM patients (p = 0.002, p = 0.018). CONCLUSIONS: Vestibular symptoms during attacks combined with the results of vestibular function tests may be used to differentiate between VM and MD. The diverse nature of vestibular symptoms (especially internal vertigo), history of motion sickness and CT intolerance may provide clues to the diagnosis of VM, whereas spontaneous external vertigo, CT(+) with vHIT(-), and the presence of saccades may provide clues to the diagnosis of MD.

[Further exploration of the classification and clinical value of head-shaking nystagmus].

Objective:To investigate the classification of head shaking nystagmus（HSN） and its clinical value in vestibular peripheral diseases. Methods:Clinical data of 198 patients with peripheral vestibular disorders presenting with HSN were retrospectively analyzed. Video Nystagmograph（VNG） was applied to detect spontaneous nystagmus（SN）, HSN, and Caloric Test（CT）. The intensity and direction of SN and HSN as well as the unilateral weakness（UW） and direction preponderance（DP） values in caloric test was analyzed in patients. Results:Among the 198 patients with vestibular peripheral disease, there were 105 males and 93 females, with an average age of（49.1±14.4） years （range: 14-87 years）. One hundred and thirty seven patients were diagnosed as Vestibular Neuritis（VN）, 12 as Meniere's Disease（MD）, 41 as sudden deafness（SD） and 8 as Hunt's syndrome accompanied by vertigo. Among them, there were 116 patients in the acute phase, including 68 cases（58.6%） with decreased HSN, 4 cases（3.4%） with increased HSN, 5 cases（4.3%） with biphasic HSN, 38 cases（32.8%） with unchanged HSN, and 1 case（0.9%） with perverted HSN. There were 82 cases in the non-acute phase, 51 cases（62.2%） with decreased HSN, 3 cases（3.6%） with increased HSN, 9 cases（11.0%） with biphasic HSN, and 19 cases（23.2%） with unchanged HSN. In biphasic HSN, the intensity of phase I nystagmus was usually greater than that of phase II, and the difference was statistically significant（P<0.01）. There was no correlation between HSN type and course of disease or DP value. The intensity of HSN was negatively correlated with the course of disease（r=-0.320, P<0.001） and positively correlated with DP value（r=0.364, P<0.001）, respectively. The intensity of unchanged nystagmus and spontaneous nystagmus were（8.0±5.7） °/s and（8.5±6.4）°/s, respectively. There was no statistically significant difference in the intensity of nystagmus before and after shaking the head. Conclusion:HSN can be classified into five types and could be regarded as a potential SN within a specific frequency range （mid-frequency）. Similarly, SN could also be considered as a common sign of unilateral vestibular impairment at different frequencies. HSN intensity can reflect the dynamic process of vestibular compensation, and is valuable for assessing the frequency of damage in peripheral vestibular diseases and monitoring the progress of vestibular rehabilitation.