[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.

Adult-onset congenital cholesteatoma in the hypotympanum initially presenting as Bell's palsy: A case report.

INTRODUCTION: Cholesteatoma is a rare disease characterized by the accumulation of keratinized squamous epithelial cells in the middle ear or mastoid cavity. Vertigo and facial palsy, which are rare complications, may indicate erosion into the semicircular canals or the fallopian canal. PATIENT CONCERNS: A 40-year-old woman presented to our clinic with progressive right-sided hearing loss over 5 years (primary concern). Approximately 10 years ago, the patient had developed acute right-sided facial weakness with no additional symptoms. A neurologist at another hospital had diagnosed her condition as Bell's palsy and treated it accordingly. DIAGNOSIS: Adult-onset congenital cholesteatoma in the hypotympanum. INTERVENTION: Combined endoscopic and microscopic removal of the cholesteatoma. OUTCOMES: Physical examination revealed slight improvement in right-sided peripheral facial palsy. LESSON: Routine eardrum examination is recommended for patients presenting with isolated peripheral facial palsy. If necessary, a patient should be referred to an otologist for further evaluation and treatment.

Frailty of the.

Vestibular frailty and presbyvestibulopathy, including benign paroxysmal positional vertigo (BPPV), can cause dizziness among elderly patients. Vestibular frailty and presbyvestibulopathy may contribute to the onset of the vicious circle of falling-bone fracture-prolonged bedridden status-senile dementia. Treatment interventions for vestibular frailty and presbyvestibulopathy should be based on vestibular rehabilitation rather than vestibular implantation or regeneration. In acute BPPV, the otolith repositioning maneuver can be used to return otolithic debris to the utricle. At the chronic remission stage, there are nutritional guidelines for improving bone density in otolith organs and rehabilitation guidelines for activating otolith organs to prevent exfoliation. Moreover, sleeping in the head-up position can prevent free-floating debris from entering the semicircular canal. Throughout their old age, the psychiatric care/support is also indispensable to keep their initiative against vestibular frailty.

The vertical computerized rotational head impulse test.

The computerized rotational head impulse test (crHIT) uses a computer-controlled rotational chair to deliver whole-body rotational impulses to assess the semicircular canals. The crHIT has only been described for horizontal head plane rotations. The purpose of this study was to describe the crHIT for vertical head plane rotations. In this preliminary study, we assessed four patients with surgically confirmed unilateral peripheral vestibular abnormalities and two control subjects. Results indicated that the crHIT was well-tolerated for both horizontal head plane and vertical head plane stimuli. The crHIT successfully assessed each of the six semicircular canals. This study suggests that the crHIT has the potential to become a new laboratory-based vestibular test for both the horizontal and vertical semicircular canals.

CHD7 and SOX2 act in a common gene regulatory network during mammalian semicircular canal and cochlear development.

Inner ear morphogenesis requires tightly regulated epigenetic and transcriptional control of gene expression. CHD7, an ATP-dependent chromodomain helicase DNA-binding protein, and SOX2, an SRY-related HMG box pioneer transcription factor, are known to contribute to vestibular and auditory system development, but their genetic interactions in the ear have not been explored. Here, we analyzed inner ear development and the transcriptional regulatory landscapes in mice with variable dosages of Chd7 and/or Sox2. We show that combined haploinsufficiency for Chd7 and Sox2 results in reduced otic cell proliferation, severe malformations of semicircular canals, and shortened cochleae with ectopic hair cells. Examination of mice with conditional, inducible Chd7 loss by Sox2CreER reveals a critical period (~E9.5) of susceptibility in the inner ear to combined Chd7 and Sox2 loss. Data from genome-wide RNA-sequencing and CUT&Tag studies in the otocyst show that CHD7 regulates Sox2 expression and acts early in a gene regulatory network to control expression of key otic patterning genes, including Pax2 and Otx2. CHD7 and SOX2 directly bind independently and cooperatively at transcription start sites and enhancers to regulate otic progenitor cell gene expression. Together, our findings reveal essential roles for Chd7 and Sox2 in early inner ear development and may be applicable for syndromic and other forms of hearing or balance disorders.

[Semicircular canal occlusion].

Semicircular canal occlusion（SCO） is a surgical technique widely used for treating vertigo symptoms. It is primarily aimed at treating benign paroxysmal positional vertigo（BPPV）, Ménière's disease（MD）, labyrinthine fistula, and superior semicircular canal dehiscence syndrome, among others. This review aims to comprehensively summarize the development, evolution, relevant basic research, and clinical applications of semicircular canal occlusion, especially the application of endoscopic technology in recent years, and explore its practical value in the field of surgical treatment for vertigo.

[Dynamic visual acuity screening test results analysis of 25 patients with peripheral vertigo].

Objective:To observe the results of dynamic visual acuity screening tests in patients with peripheral vertigo and explore its clinical significance. Methods:The number of 48 healthy volunteers were enrolled as control group and 25 peripheral vertigo patients as experimental group. In the experimental group, there are 12 patients with vestibular neuritis, 1 patient with Hunt syndrome, 5 patients with sudden deafness with vertigo and 7 patients with bilateral vestibular dysfunction. Horizontal and vertical dynamic visual acuity screening tests were performed on them. The number of lost rows of horizontal and vertical dynamic visual acuity was compared between the control group and the experimental group to figure out if there is a statistical difference. The number of lost rows of horizontal and vertical dynamic visual acuity was compared within the experimental group to figure out if there is a statistical difference. The two groups of 18 cases of unilateral vestibular function decline and 7 cases of bilateral vestibular function decline in the experimental group were compared with the control group, and figure out if there is a statistical difference. Results:The median number of lost rows of horizontal dynamic visual acuity in 48 healthy volunteers was 1.5 and median number of lost rows of vertical dynamic visual acuity was 1.0 in the control group. The median number of lost rows of horizontal dynamic visual acuity of 26 healthy volunteers was 6 and median number of lost rows of vertical dynamic visual acuity was 5 in the experimental group. Compared to the experimental group, the number of lost rows both have statistical significance in horizontal and vertical dynamic visual acuity（P<0.01）. The comparison of horizontal and vertical lost rows within the test group also have statistical significance（P<0.01）. Twenty five patients with exceptional vestibular disease in the experimental group were divided into unilateral vestibular function reduction group（n=18） and bilateral vestibular function reduction group（n=7）. Compared with the control group, there was significant differences in the number of horizontal and vertical lost rows（P<0.01） within the three groups. After pairwise comparison, the number of lost rows of horizontal and vertical in the control group was significantly lower than that in the unilateral vestibular function reduction group and the bilateral vestibular function reduction group（P<0.01）. There was a highly significant correlation between the number of horizontally lost rows of DVA and the mean vHIT values of bilateral horizontal semicircular canals in 25 patients（P<0.01）; and a highly significant correlation between the number of vertically lost rows of DVA and the mean vHIT values of vertical semicircular canals in 4 groups bilaterally（P<0.01）. Conclusion:The Dynamic Visual Acuity Screening Test is a useful addition to existing tests of peripheral vestibular function, particularly the vHIT test, and provides a rapid assessment of the extent of 2 Hz VOR impairment in patients with reduced vestibular function.

Inner ear morphology in wild versus laboratory house mice.

The semicircular canals of the inner ear are involved in balance and velocity control. Being crucial to ensure efficient mobility, their morphology exhibits an evolutionary conservatism attributed to stabilizing selection. Release of selection in slow-moving animals has been argued to lead to morphological divergence and increased inter-individual variation. In its natural habitat, the house mouse Mus musculus moves in a tridimensional space where efficient balance is required. In contrast, laboratory mice in standard cages are severely restricted in their ability to move, which possibly reduces selection on the inner ear morphology. This effect was tested by comparing four groups of mice: several populations of wild mice trapped in commensal habitats in France; their second-generation laboratory offspring, to assess plastic effects related to breeding conditions; a standard laboratory strain (Swiss) that evolved for many generations in a regime of mobility reduction; and hybrids between wild offspring and Swiss mice. The morphology of the semicircular canals was quantified using a set of 3D landmarks and semi-landmarks analyzed using geometric morphometric protocols. Levels of inter-population, inter-individual (disparity) and intra-individual (asymmetry) variation were compared. All wild mice shared a similar inner ear morphology, in contrast to the important divergence of the Swiss strain. The release of selection in the laboratory strain obviously allowed for an important and rapid drift in the otherwise conserved structure. Shared traits between the inner ear of the lab strain and domestic pigs suggested a common response to mobility reduction in captivity. The lab-bred offspring of wild mice also differed from their wild relatives, suggesting plastic response related to maternal locomotory behavior, since inner ear morphology matures before birth in mammals. The signature observed in lab-bred wild mice and the lab strain was however not congruent, suggesting that plasticity did not participate to the divergence of the laboratory strain. However, contrary to the expectation, wild mice displayed slightly higher levels of inter-individual variation than laboratory mice, possibly due to the higher levels of genetic variance within and among wild populations compared to the lab strain. Differences in fluctuating asymmetry levels were detected, with the laboratory strain occasionally displaying higher asymmetry scores than its wild relatives. This suggests that there may indeed be a release of selection and/or a decrease in developmental stability in the laboratory strain.

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.

Scuba Diving-Induced Inner-Ear Pathology: Imaging Findings of Superior Semicircular Canal and Tegmen Tympani Dehiscence.

BACKGROUND Superior semicircular canal dehiscence is an inner-ear pathology which presents with vertigo, disequilibrium, and hearing loss. Although the exact etiology of superior semicircular canal dehiscence is unknown, it is thought that an increase in middle-ear pressure disrupts a thin overlying temporal bone. Superior semicircular canal dehiscence is frequently seen in association with dehiscence of the tegmen tympani, which overlies the middle ear. Here, we present a case report of a 52-year-old Puerto Rican man with vertigo, dizziness, vomiting, and mild hearing loss associated with superior semicircular canal and tegmen tympani dehiscence after performing improper scuba diving techniques. CASE REPORT A 52-year-old Puerto Rican man presented to the emergency department with vertigo, dizziness, vomiting, and mild hearing loss in the right ear. The symptoms began shortly after scuba diving with inadequate decompression techniques on ascent. He was treated with recompression therapy with mild but incomplete improvement in symptoms. Bilateral temporal magnetic resonance imaging was suggestive of segmental dehiscence of the right superior semicircular canal and tegmen tympani. High-resolution computed tomography of the temporal bone confirmed right superior semicircular canal and tegmen tympani dehiscence with an intact left inner ear. CONCLUSIONS The increased inner-ear pressure that occurs during scuba diving can lead to dehiscence of the superior semicircular canal and tegmen tympani, causing vertigo and hearing loss. Performance of improper diving techniques can further increase the risk of dehiscence. Therefore, appropriate radiologic evaluation of the inner ear should be performed in such patients.

The evaluation of superior semicircular canal in patients with unilateral cleft lip and palate using CBCT.

OBJECTIVES: The present study aims to evaluate the thickness and radiological patterns of the superior semicircular canal (SSC) in patients with unilateral cleft lip and palate (CL/P). METHODS: Cone beam computed tomography (CBCT) images of the patients were evaluated in axial and Pöschl planes. CBCT images of 84 patients with unilateral CL/P and 168 healthy individual controls were included in the study. Three study groups were established: the CS-CL/P group (cleft side temporal bones of the CL/P patients), NCS-CL/P (non-cleft side temporal bones of the CL/P patients) and the control group. The radiological patterns of SSCs were categorized as dehiscence, papyraceous, normal, pneumatised and thick. The minimum bone thickness of SSC was measured. RESULTS: It was found that the CS-CL/P group had a higher prevalence for SSCD compared to both the NCS-CL/P group and the control group. CS-CL/P group had a higher prevalence of dehiscence type and papyraceous type compared to the control group. The SSC thickness on the CS-CL/P patients was thinner than the NCS-CL/P patients and the control group sides (p = 0.033 and p < 0.001, respectively). CONCLUSIONS: The mean thickness of SSC was found significantly lower in the CS-CL/P group compared to both the NCS-CL/P group and the control group. The elevated prevalence of dehiscence and papyraceous types in the CS-C/LP group compared to the control group implies that the presence of a cleft may be a predisposing factor for these types.

Trans-mastoid plugging of superior semicircular canal dehiscence: long-term follow-up.

OBJECTIVE: To evaluate the long-term outcomes of trans-mastoid plugging of superior semicircular canal dehiscence (SSCD), focusing on complicated cases. METHODS: In this cohort study, we included all patients who underwent trans-mastoid plugging of SSCD between 2009 and 2019. We evaluated the symptoms (autophony, sound-/pressure-induced vertigo, disequilibrium, aural fullness and pulsatile tinnitus) before and 1 year after surgery in the medical records. We systematically assessed the current symptoms 6.2 ± 3 years postoperative (range 2.2-12.3 years) using questionnaires sent by post and validated by telephone interviews. We also documented any complications and the need for further procedures. We compared pure tone and speech audiometry before and 1 year after surgery. Finally, the degree of mastoid pneumatisation and mastoid tegmen anatomy were reviewed on preoperative CT scans. RESULTS: We included 24 ears in 23 patients. No complications were recorded, and none required a second procedure for SSCD. Following surgery, oscillopsia and Tullio phenomena resolved in all patients. Hyperacusis, autophony, and aural fullness were also settled in all patients except one. Balance impairment persisted to some degree in 35% of patients. No deterioration over the years was reported regarding the above symptoms. On average, bone conduction pure tone average pre- and 1 year postoperative were 13.7 ± 17 and 20.5 ± 18 dB, respectively (P = 0.002). Air bone gaps were reduced from 12.7 ± 8 to 5.9 ± 6 (P = 0.001). Two patients had a significant sclerotic mastoid, three had a prominent low-lying mastoid tegmen, and two had both. Anatomy had no effect on outcome. CONCLUSION: Trans-mastoid plugging of SSCD is a reliable and effective technique which achieves long-lasting symptom control, even in cases with sclerotic mastoid or low-lying mastoid tegmen.

Low-Lying Tegmen and Surgical Outcomes Following the Middle Cranial Fossa Repair of Superior Canal Dehiscence.

OBJECTIVE: To compare treatment response from the middle cranial fossa repair of superior canal dehiscence (SCD) between cases with and cases without low-lying tegmen (LLT). STUDY DESIGN: Cohort study. SETTING: Single tertiary care institution. METHODS: Two investigators independently reviewed preoperative high-resolution temporal bone computed tomography images and classified the ipsilateral tegmen as either "low-lying" or "control." Patients completed a symptom questionnaire and underwent audiometric testing pre- and post-operatively. Multivariable regression models assessed for symptomatic resolution and audiometric improvement following surgery with tegmen status as the primary predictor. Models controlled for patient age, sex, bilateral SCD disease, dehiscence location, prior ear surgery status, surgery duration, and follow-up duration. RESULTS: Among a total of 410 cases included, we identified 121 (29.5%) LLT cases. Accounting for all control measures, patients with LLT were significantly less likely to experience overall symptom improvement (adjusted odds ratio: 0.32, 95% confidence interval [CI]: 0.18-0.57, p < .001) and reported a significantly lower proportion of preoperative symptoms that resolved following surgery (adjusted ß: -25.6%, 95% CI: -37.0% to -14.3%, p < .001). However, audiometric outcomes following surgery did not differ significantly between patients with and patients without LLT. CONCLUSION: This is the first investigation on the relationship between LLT and surgical outcomes following the middle fossa repair of SCD. Patients with LLT reported less favorable symptomatic response but exhibited a similar degree of audiometric improvement.

Association of the superior semicircular canal and tegmen tympani dehiscences and its relationship with the pneumatisation of the temporal bone.

PURPOSE: To analyse the degree of pneumatisation of the temporal bone when there is an association between dehiscence of the superior semicircular canal and dehiscence of the tegmen tympani. MATERIALS AND METHODS: We analysed a retrospective CT study of 124 selected cases. A single inclusion criterion was applied: the presence of a dehiscence of the tegmen tympani. On the other hand, the degree of temporal pneumatisation was assessed by axial and coronal planes, and has been divided into the following grades O, I, II and III, according to the status and relationship of the mastoid, the bony labyrinth, the petrous segment of the carotid canal and sigmoid sinus. RESULTS: Of the 124 cases studied, 35 (28.2%) presented both dehiscences. In 26 of the 35 (47.3%), grade II pneumatisation, 4 (14,8%), grade I, and 5 (11,9%) grade III was observed, with a statistically significant relationship (p < 0.001). On the other hand, we did not find a significant relationship when relating both dehiscences in any age or sex group. However, when relating the degree of pneumatisation to sex, among those with grade III pneumatisation, the proportion of men (52.4%) was significantly higher than that of women (47.6%) (p = 0.017). CONCLUSION: We have detected a statistically significant relationship between the coexistence of grade II pneumatisation and the presence of both dehiscences in the temporal bone.

Changes in vestibular-related responses to combined noisy galvanic vestibular stimulation and cerebellar transcranial direct current stimulation.

Vestibular nuclei and cerebellar function comprise vestibular neural networks that control vestibular-related responses. However, the vestibular-related responses to simultaneous stimulation of these regions are unclear. This study aimed to examine whether the combination of noisy galvanic vestibular stimulation (nGVS) and cerebellar transcranial direct current stimulation (ctDCS) using a complex transcranial electrical stimulation device alters vestibular-dominant standing stability and vestibulo-ocular reflex (VOR) function. The center of foot pressure (COP) sway and VOR of participants (28 healthy, young adults) were assessed under four conditions of transcranial electrical stimulation using nGVS and ctDCS. The COP was calculated with the participant standing on a soft-foam surface with eyes closed using a force plate to evaluate body sway. VOR measurements were collected via passive head movements and fixation on a target projected onto the front wall using a video head impulse test (vHIT). VOR gain was calculated in six directions using a semicircular canal structure based on the ratio of eye movement to head movement. The nGVS + ctDCS and nGVS + sham ctDCS conditions decreased COP sway compared to the sham nGVS + ctDCS and sham nGVS + sham ctDCS conditions. No significant differences were observed in the main effect of stimulation or the interaction of stimulation and direction on the vHIT parameters. The results of this study suggest that postural stability may be independently affected by nGVS. Our findings contribute to the basic neurological foundation for the clinical application of neurorehabilitation using transcranial electrical stimulation of the vestibular system.

Semicircular canals are long in patients with benign paroxysmal positional vertigo.

OBJECTIVE: To search for any morphological variation contributing to aetiopathogenesis and the diagnosis of benign paroxysmal positional vertigo, we measured the sizes of the semicircular canals in patients with and without benign paroxysmal positional vertigo using multidetector computed tomography. METHODS: Cranial bone computed tomography images of 30 benign paroxysmal positional vertigo patients and 30 control patients were acquired with a 128-slice computed tomography scanner and a transverse plane with a thickness of 0.67 mm. The inner diameter, height and width of the canals were measured. RESULTS: The width of the anterior semicircular canals, and the width and height of the posterior semicircular canals of the affected ears in benign paroxysmal positional vertigo patients (n = 30) were significantly greater than in the control patients (n = 90; p = 0.001, p = 0.023, p = 0.003, respectively). CONCLUSION: In benign paroxysmal positional vertigo patients, the posterior and anterior semicircular canals are longer than those in people without benign paroxysmal positional vertigo. These morphological changes may contribute to elucidating the aetiopathogenesis and be used as a radiological sign for diagnosis of benign paroxysmal positional vertigo disease.

A comparison of the video head impulse test and the functional head impulse test in chronic unilateral vestibular loss.

OBJECTIVE: To examine the correlation of video head impulse test, functional head impulse test and Dizziness Handicap Inventory results in patients with chronic unilateral vestibular loss, and to compare the results with healthy controls. METHODS: Forty-eight patients diagnosed with chronic unilateral vestibular loss and 35 healthy individuals, aged 18-65 years, were included. The video head impulse test, functional head impulse test and Dizziness Handicap Inventory were administered. RESULTS: A significant positive correlation was found between functional head impulse test and video head impulse test results for the study group in all semicircular canals (p < 0.05). There was no significant correlation between Dizziness Handicap Inventory, functional head impulse test and video head impulse test results (p > 0.05). The functional head impulse test and video head impulse test results of the control group were significantly higher than those of the study group in all semicircular canals planes (p < 0.05). CONCLUSION: In chronic unilateral vestibular loss patients, with high head accelerations, the functional head impulse test indicates deterioration in vestibulo-ocular reflex functionality. It would be beneficial to include the video head impulse test and functional head impulse test in clinical practice as complementary tests in vestibulo-ocular reflex evaluation.