Vestibular

前庭
  • 文章类型: Journal Article
    在所有脊椎动物中,维持躯干姿势主要取决于来自脑干前庭脊髓核的下降命令。尽管被广泛地概述了物种,这些前庭脊髓网络的详细解剖和操作结构仍然知之甚少。非洲爪蛙以前曾是探索与动物行为要求相关的解剖和功能方面的优秀模型。在这项研究中,我们检查了前庭刺激引起的前庭运动反应。我们的发现表明,与其他平面的刺激相比,水平面的自然前庭刺激产生更大的功效。通过水平半规管的特定电刺激(GVS)以频率依赖性方式复制的现象。除了接收前庭输入并投射到脊髓的非常前端的神经元簇,在幼年青蛙中观察到的脑干镜中前庭脊髓核的整体解剖分离。然而,我们的结果表明与哺乳动物组织的相似性比以前公认的更接近.此外,我们证明前庭脊髓细胞不仅投射到头端段的脊髓运动神经元,还投射到在变态过程中经历消退的更远段。最后,我们说明了前庭诱导的脊髓反射在幼虫发育过程中如何变化,从基于尾游的活动过渡到延髓躯干爆裂反应,可能会预测变质后青蛙的姿势控制。
    In all vertebrates, maintaining trunk posture primarily depends on descending commands originating from brainstem vestibulospinal nuclei. Despite being broadly outlined across species, the detailed anatomical and operational structure of these vestibulospinal networks remains poorly understood. Xenopus frogs have previously served as an excellent model for exploring such anatomical and functional aspects in relation to the animal\'s behavioral requirements. In this study, we examined the reflex motor reactions induced by vestibular stimulation in pre-metamorphic tadpoles. Our findings indicate that natural vestibular stimulation in the horizontal plane yields greater efficacy compared to stimulation in other planes, a phenomenon replicated in a frequency-dependent manner through specific galvanic stimulation (GVS) of the horizontal semicircular canals. With the exception of a very rostral cluster of neurons that receive vestibular inputs and project to the spinal cord, the overall anatomical segregation of vestibulospinal nuclei in the brainstem mirrors that observed in juvenile frogs. However, our results suggest closer similarities to mammalian organization than previously acknowledged. Moreover, we demonstrated that vestibulospinal cells project not only to spinal motoneurons in rostral segments but also to more distal segments that undergo regression during metamorphosis. Lastly, we illustrated how vestibular-induced spinal reflexes change during larval development, transitioning from tail swim-based activity to rostral trunk bursting responses, likely anticipating postural control in post-metamorphic frogs.
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  • 文章类型: Journal Article
    自我运动感知是所有物种的重要技能。它是一种固有的多感官过程,结合了惯性(基于身体)和相对(相对于环境)运动线索。尽管在人类和非人类灵长类动物中进行了广泛的研究,目前没有使用惯性和相对自运动线索来测试啮齿动物的自运动感知的范例。我们开发了一种新颖的啮齿动物运动模拟器,使用两个同步的机械臂产生惯性,相对,或自运动的组合(惯性和相对)线索。八只老鼠被训练来执行识别航向的任务,类似于流行的灵长类动物范式。引人注目的是,大鼠严重依赖气流进行相对的自我运动感知,从(有限的)光学流线索提供的贡献很小-在黑暗中的性能几乎一样好。相对自运动(气流)被认为具有更大的可靠性与惯性。气流中断,使用风扇或挡风玻璃,相对受损,但不是惯性的,自我运动感知。然而,这个功能不需要胡须。最后,大鼠以基于可靠性(贝叶斯)的方式整合了相对和惯性的自运动线索。这些结果表明,气流是大鼠自我运动知觉的重要线索,并为研究清醒行为啮齿动物的自我运动知觉和多感觉处理的神经基础提供了新的领域。
    Self-motion perception is a vital skill for all species. It is an inherently multisensory process that combines inertial (body-based) and relative (with respect to the environment) motion cues. Although extensively studied in human and non-human primates, there is currently no paradigm to test self-motion perception in rodents using both inertial and relative self-motion cues. We developed a novel rodent motion simulator using two synchronized robotic arms to generate inertial, relative, or combined (inertial and relative) cues of self-motion. Eight rats were trained to perform a task of heading discrimination, similar to the popular primate paradigm. Strikingly, the rats relied heavily on airflow for relative self-motion perception, with little contribution from the (limited) optic flow cues provided-performance in the dark was almost as good. Relative self-motion (airflow) was perceived with greater reliability vs. inertial. Disrupting airflow, using a fan or windshield, damaged relative, but not inertial, self-motion perception. However, whiskers were not needed for this function. Lastly, the rats integrated relative and inertial self-motion cues in a reliability-based (Bayesian-like) manner. These results implicate airflow as an important cue for self-motion perception in rats and provide a new domain to investigate the neural bases of self-motion perception and multisensory processing in awake behaving rodents.
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  • 文章类型: Journal Article
    目的:评估儿科和成人患者的诊断趋势,这些患者进行跨寿命的头晕和失衡的多学科亚专科评估。
    方法:回顾性图表回顾。
    方法:单儿科和单成人学术三级护理医院。
    方法:回顾性回顾2017年至2020年到成人或儿科多学科前庭诊所就诊的患者的电子健康记录,包括临床数据,物理治疗评估,和前庭听力测试。
    结果:共评估了1934名1至95岁的患者。大多数患者是女性(n=1188,61%);最大的队列是在生命的第五个十年(n=321,17%)。76%的患者(n=1470)被指定为病理诊断。头晕的主要原因在儿童和年轻人中最常见,占1至30岁所有诊断的38%至54%。周围性前庭疾病的比例随着年龄的增长而增加,在61至70岁的年龄段达到32%的峰值。前庭性偏头痛是6至20岁(n=110,39%)和31和50岁(n=69,17%)的最常见病理诊断,但在女性中更为普遍(21%vs14%;P<0.0001)。良性阵发性位置性眩晕(BPPV)的患病率在整个生命周期中增加,在71至80岁时达到顶峰。梅尼埃病(MD)在生命的第一个十年内没有发生,但此后增加,在51到60岁之间达到顶峰。
    结论:多学科前庭评估导致大多数患者的诊断。前庭诊断随寿命而变化,然而在大多数年龄组中,中枢紊乱,包括偏头痛,多于外周前庭病变。外周前庭疾病如BPPV和MD的患病率随年龄增加而增加。
    方法:四级。
    OBJECTIVE: To evaluate diagnostic trends in pediatric and adult patients presenting for multidisciplinary subspecialty evaluation of dizziness and imbalance across the lifespan.
    METHODS: Retrospective chart review.
    METHODS: Single pediatric and single adult academic tertiary care hospital.
    METHODS: Retrospective review of electronic health record for patients presenting to an adult or pediatric multidisciplinary vestibular clinic from 2017 to 2020, including clinical data, physical therapy evaluation, and audiovestibular testing.
    RESULTS: A total of 1934 patients aged 1 to 95 were evaluated. Most patients were female (n = 1188, 61%); the largest cohort was in the fifth decade of life (n = 321, 17%). Seventy-six percent of patients (n = 1470) were assigned a pathologic diagnosis. Central causes of dizziness were most common in children and young adults, comprising 38% to 54% of all diagnoses in ages 1 to 30. The proportion of peripheral vestibular disorders increased with age, peaking at 32% in ages 61 to 70. Vestibular migraine was the most common pathologic diagnosis in ages 6 to 20 (n = 110, 39%) and 31 and 50 (n = 69, 17%) regardless of gender, but was more prevalent in females (21% vs 14%; P < .0001). The prevalence of benign paroxysmal positional vertigo (BPPV) increased throughout the lifespan, peaking at age 71 to 80. Meniere\'s disease (MD) did not occur within the first decade of life, but increased thereafter, peaking at ages 51 to 60.
    CONCLUSIONS: Multidisciplinary vestibular evaluation resulted in a diagnosis for the majority of patients. Vestibular diagnoses vary across the lifespan however among most age groups, central disorders, including migraine disorders, outnumber peripheral vestibulopathies. The prevalence of peripheral vestibular disorders such as BPPV and MD increased with age.
    METHODS: Level IV.
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  • 文章类型: Journal Article
    这项回顾性研究描述了我们从2019年12月至2023年10月通过直接经前庭FLC(DTV-FLC)方法接受女性化甲状腺喉软骨成形术(FLC)的前20名变性女性的手术结果。检索并审查所有患者的医疗记录。操作人员的数据,术后,和后续课程,并发症,并检索功能和美容结果。由四名独立的面部整形外科医生评估美容结果。13例患者接受了DTV-FLC联合生殖器成形术或下颌角缩小的生殖器成形术,其中7例接受了孤立的DTV-FLC。DTV-FLC在所有计划的情况下都是可行的。并发症(皮瓣穿孔,甲状软骨骨折,精神知觉减退,血肿,前庭切口开裂,前庭瘢痕粘连,和贫血)是轻微的,可以自发解决。术前甲状软骨突出的分级为1.9±0.9,分级为1~3。18例患者的术后美容结果被判断为改善(从-1到3的评分为2.1±0.8)。18例患者对美容效果满意,一个人不满意(翻修手术患者),一个在后续行动中丢失了。总之,DTV-FTLC是FLC的有效手术方法,产生高患者满意度和良好的美容效果。
    This retrospective study describes the surgical outcomes of our first 20 transgender women to undergo feminization thyroid laryngochondroplasty (FLC) by a direct transvestibular FLC (DTV-FLC) approach from December 2019 to October 2023. The medical records of all patients were retrieved and reviewed. Data on the operative, postoperative, and follow-up courses, complications, and functional and cosmetic outcomes were retrieved. The cosmetic results were evaluated by four independent facial plastic surgeons. Thirteen patients underwent DTV-FLC combined with genioplasty or genioplasty with mandibular angle reduction and seven underwent isolated DTV-FLC. DTV-FLC was feasible in all planned cases. Complications (skin flap perforation, thyroid cartilage fracture, mental hypoesthesia, hematoma, dehiscence of the vestibular incision, vestibular scar adhesions, and anemia) were minor and resolved spontaneously. The preoperative grade of thyroid cartilage protrusion was 1.9 ± 0.9 on a scale from 1 to 3. The postoperative cosmetic results of 18 patients were judged as having improved (a score of 2.1 ± 0.8 on a scale from -1 to 3). Eighteen patients were satisfied with the cosmetic result, one was dissatisfied (the revision surgery patient), and one was lost to follow-up. In conclusion, DTV-FTLC is a valid surgical approach for FLC, yielding high patient satisfaction and good cosmetic results.
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  • 文章类型: Journal Article
    已经提出了电流前庭刺激(GVS)作为一种替代显示模式来传递信息,而不会增加对佩戴者的视觉或听觉感觉模式的要求,或者在无法使用这些模式的环境中(例如,秘密夜间行动)。我们通过四个旨在测试的实验进一步研究了这一概念:(1)受试者可以区分不同GVS电流幅度和极性的阈值,(2)不同双极(即,电流在左右方向之间振荡的正弦波形)电流频率在室温下可区分,热,冷,和多风的环境,(3)单极效应(即正弦波形,电流仅出现在左或右方向)平衡性能上的电流,(4)在一致的视觉搜索任务中,频率和极性调制的GVS条件之间的双任务性能。受试者可靠地区分了从±0.6mA的基座以0.03mA的中值变化的电流幅度(范围为0.02-0.32mA),以及在0.55mA的中值幅度(范围为0.32-0.83mA)的单极电流之间。GVS频率阈值对测试的环境条件具有鲁棒性,没有发现统计学差异。单极电流会增加摇摆和平衡误差。GVS阈值不受双重任务范式的影响,但是,当一致地执行极性阈值任务时,视觉搜索得分略有提高。总体调查结果继续支持GVS用作显示模式,但是注意到一些限制,例如在姿势控制很重要的情况下使用单极电流。
    Galvanic Vestibular Stimulation (GVS) has been proposed as an alternative display modality to relay information without increasing demands on the visual or auditory sensory modalities of the wearer or in environments where those modalities cannot be used (e.g., covert night operations). We further investigated this concept with four experiments designed to test: (1) thresholds at which subjects could distinguish between different GVS current amplitudes and polarities, (2) thresholds at which different bipolar (i.e., sinusoidal waveform with current oscillating between left and right directions) current frequencies were distinguishable among room temperature, hot, cold, and windy environments, (3) effects of unipolar (i.e., sinusoidal waveform with current occurring in only the left or right direction) currents on balance performance, and (4) dual-task performance among frequency and polarity modulated GVS conditions during a concordant visual search task. Subjects reliably distinguished between current amplitudes that varied from a pedestal of ± 0.6 mA by a median of 0.03 mA (range of 0.02-0.32 mA) and between unipolar currents at a median amplitude of 0.55 mA (range of 0.32-0.83 mA). GVS frequency thresholds were robust to the environment conditions tested, with no statistical differences found. Sway and balance errors were increased with unipolar currents. GVS thresholds were not impacted by the dual-task paradigm, but the visual search scores were slightly elevated when congruently performing a polarity thresholding task. Overall findings continue to support GVS use as a display modality, but some limitations are noted, such as the use of unipolar currents under scenarios where postural control is important.
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  • 文章类型: Journal Article
    Usher综合征II型(USH2)是一种罕见的遗传性疾病,包括听力损失,视力障碍,前庭功能明显完整。最近的研究表明,耳石前庭受体可能参与USH2。
    评估USH2中的耳石动态功能。
    22名USH2(中位年龄53.9±2.99)和年龄匹配的对照者接受了完整的电池前庭测试,包括空气传导的颈部和眼部前庭诱发肌源性电位(c-VEMPs和o-VEMPs)。前庭功能测试与活动平衡量表(ABC)和头晕障碍量表(DHI)评分相关。
    十四个USH2报告了先前的眩晕(与无对照)。88只耳朵15例USH2病例和4例对照中c-VEMP缺失(p=0.034),而22例USH2病例和12例对照中缺少o-VEMP(p=0.129)。在右耳o-VEMPN1潜伏期中,USH2与对照组之间存在显着差异(中位数11.60/10.40,p<0.010),N1-P1振幅(中位数5.15/10.10,p<0.003)和o-VEMPN1-P1不对称比率(中位数24.78/40.50,p<0.014)。USH2显示o-VEMP振幅与DHI评分之间有很强的相关性(p=0.003,ρ=0.769)。在眩晕和VEMPs亚组之间没有发现关联。
    我们的发现表明USH2中存在耳石功能障碍,这与主观报告的头晕无关。将前庭测试纳入USH2评估和监测可以增强这种多感觉疾病的表征。
    UNASSIGNED: Usher\'s syndrome type II (USH2) is a rare genetic disorder encompassing hearing loss, vision impairment, and apparent intact vestibular function. Recent research suggests a potential involvement of the otolith vestibular receptors in USH2.
    UNASSIGNED: Evaluate otolith dynamic function in USH2.
    UNASSIGNED: Twenty-two USH2 (median age 53.9 ± 2.99) and age-matched controls underwent a complete battery vestibular testing including air conducted cervical and ocular vestibular evoked myogenic potentials (c-VEMPs and o-VEMPs). Vestibular function tests were correlated with Activities Balance Scale (ABC) and Dizziness Handicap Inventory (DHI) scores.
    UNASSIGNED: Fourteen USH2 reported previous vertigo (vs none control). Among 88 ears, c-VEMPs were absent in 15 USH2 cases and 4 controls (p = 0.034), while o-VEMPs were absent in 22 USH2 cases and 12 controls (p = 0.129). There were significant differences between USH2 vs controls in right ear o-VEMP N1 latencies (median 11.60/10.40, p < 0.010), N1-P1 amplitudes (median 5.15/10.10, p < 0.003) and in o-VEMP N1-P1 asymmetry ratio (median 24.78/40.50, p < 0.014). USH2 showed a strong correlation between o-VEMP amplitude and DHI score (p = 0.003, ρ = 0.769). No association was found between vertigo and VEMPs subgroups.
    UNASSIGNED: Our findings suggest the presence of otolith dysfunction in USH2, which is independent from subjectively reported dizziness. Incorporating vestibular testing into USH2 evaluation and monitoring could enhance characterization of this multisensory disease.
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  • 文章类型: Journal Article
    这项单病例研究描述了一名女性患者的听力学和前庭检查结果,62岁,患有糖尿病和类风湿性关节炎。基于这个案例研究,我们假设糖尿病和类风湿性关节炎患者更容易发生前庭损伤.目前的发现表明,中央前庭病变和双侧感音神经性听力损失与类风湿性关节炎和糖尿病有关。因此,需要总结早期发现和随访以详细了解病理生理学。
    This single case study describes the audiological and vestibular findings of a female patient, aged 62, with diabetes mellitus and rheumatoid arthritis. Based on this case study, we hypothesize that individuals with diabetes mellitus and rheumatoid arthritis are more vulnerable to vestibular impairment. The current findings indicate that central vestibular lesions and bilateral sensorineural hearing loss are related to rheumatoid arthritis and diabetes. Therefore concluding early detection and follow-up is required to understand pathophysiology in detail.
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  • 文章类型: Journal Article
    几十年来,系统性红斑狼疮患者的听觉前庭功能障碍一直被低估。系统性红斑狼疮可同时影响听觉和前庭系统。已经提出了系统性红斑狼疮相关的听觉前庭功能障碍背后的几种潜在的病理生理机制。包括抗体介导的免疫反应,细胞介导的细胞毒性,免疫复合物在微血管中的沉积,中枢参与听觉前庭通路,以及系统性红斑狼疮治疗药物的耳毒性。目前可用的评估系统性红斑狼疮患者听觉前庭功能的测试既不特异也不敏感。然而,对于此类患者的前庭听觉功能障碍的治疗效果尚无共识.在这次系统审查中,我们电子搜索了PubMed,Embase,ClinicalKey,WebofScience,和ScienceDirect平台寻找合格的文章。首次检查日期为2023年12月29日,最终更新搜索日期为2024年6月11日。Further,我们用纽卡斯尔-渥太华量表对收录文章的质量进行了评价。根据上述系统审查过程,我们总结了目前可用的关于这些特征的证据,病理生理学,考试,与系统性红斑狼疮相关的听觉前庭功能障碍的治疗。此外,我们提出了一种特定的类固醇治疗方案来管理与系统性红斑狼疮相关的听觉前庭功能障碍.与系统性红斑狼疮相关的听觉前庭功能障碍可能对适当的治疗有反应,如果及时识别和管理疾病,则可能允许可逆性。因此,为临床医生提供临床相关证据,我们组织了这篇文献综述文章,以总结有关特征的可用证据,病理生理学,考试,系统性红斑狼疮患者的听力前庭功能障碍的治疗。最后,根据我们改良的类固醇治疗方案,我们希望为临床医生提供一种新的治疗策略,以治疗系统性红斑狼疮相关的听前庭功能障碍.
    Audiovestibular dysfunction in patients with systemic lupus erythematosus has been underestimated for decades. Systemic lupus erythematosus can affect both the auditory and vestibular systems simultaneously. Several potential pathophysiological mechanisms behind systemic lupus erythematosus-related audiovestibular dysfunction have been proposed, including antibody-mediated immune responses, cell-mediated cytotoxicity, immune complex deposition in microvessels, central involvement in the audiovestibular pathway, and ototoxicity from medications used in systemic lupus erythematosus treatment. Currently available tests to evaluate audiovestibular function in systemic lupus erythematosus patients are neither specific nor sensitive. Nevertheless, there is no consensus regarding the efficacy of treatments for audiovestibular dysfunction in such patients. In this systematic review, we electronically searched the PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect platforms to find eligible articles. The first inspection date was on 29 December 2023 and the final update search date was on 11 June 2024. Further, we rated the quality of the included articles with Newcastle-Ottawa Scale. Based upon the aforementioned systematic review process, we have summarized the currently available evidence on the characteristics, pathophysiology, examination, and treatment of audiovestibular dysfunction related to systemic lupus erythematosus. Furthermore, we have proposed a specific steroid treatment protocol to manage audiovestibular dysfunction related to systemic lupus erythematosus. Audiovestibular dysfunction related to systemic lupus erythematosus may be responsive to adequate treatments, potentially allowing for reversibility if the disease is recognized and managed in a timely manner. Therefore, to provide clinically relevant evidence to clinicians, we have organized this literature review article to summarize the available evidence on the characteristics, pathophysiology, examination, and treatment of audiovestibular dysfunction in patients with systemic lupus erythematosus. Finally, based on our modified steroid treatment protocol, we would like to provide a new treatment strategy to clinicians to manage systemic lupus erythematosus-related audiovestibular dysfunction.
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  • 文章类型: Journal Article
    本研究探讨了扭转凝视稳定对人体本体感觉颈部激活的存在。13名健康受试者(6名女性,平均年龄25)暴露于被动身体旋转,同时保持头部固定,重力直立,position.参与者坐在机械雪橇上,他们的头部放置在嵌入木梁的下巴支架中,同时佩戴使用强力橡皮筋连接到梁上的眼睛跟踪器,以确保头部稳定。在黑暗中和观看投影的视觉场景时,身体在头部下方被动旋转。静态扭转凝视位置在刺激前的基线位置之间进行比较,在达到最后的身体倾斜后立即。结果表明,与视觉背景结合时,被动颈部屈曲会产生眼扭转。眼睛的旋转方向与颈部的伸展方向相反,匹配与雪橇相同方向的假设头部倾斜。这对应于旨在使头部相对于身体变直的预测头部旋转。在黑暗中的审判中没有看到这样的反应。总之,这些发现表明,本体感觉可能在人类中产生预测性的注视稳定反应。
    The present study explored the presence of torsional gaze-stabilization to proprioceptive neck activation in humans. Thirteen healthy subjects (6 female, mean age 25) were exposed to passive body rotations while maintaining a head-fixed, gravitationally upright, position. Participants were seated in a mechanical sled, their heads placed in a chin rest embedded in a wooden beam while wearing an eye tracker attached to the beam using strong rubber bands to ensure head stability. The body was passively rotated underneath the head both in darkness and while viewing a projected visual scene. Static torsional gaze positions were compared between the baseline position prior to the stimulation, and immediately after the final body tilt had been reached. Results showed that passive neck flexion produced ocular torsion when combined with a visual background. The eyes exhibited rotations in the opposite direction of the neck\'s extension, matching a hypothetical head tilt in the same direction as the sled. This corresponded with a predicted head rotation aimed at straightening the head in relation to the body. No such response was seen during trials in darkness. Altogether, these findings suggest that proprioception may produce a predictive gaze-stabilizing response in humans.
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  • 文章类型: Journal Article
    疾病特异性动眼评估在遗传性小脑共济失调的早期诊断中起着至关重要的作用。尽管一些研究报道了Friedreich共济失调(FRDA)中的定量动眼和前庭测量,特定动眼范式的价值尚不清楚.我们旨在通过系统的文献综述来解决这一知识差距,并为FRDA中量身定制的一组眼动记录提供针对疾病的建议。搜索了MEDLINE和Embase,以研究FRDA患者的定量动眼和/或前庭测量。提取有关动眼和前庭参数的数据,并寻求与一系列临床参数的相关性。纳入研究(n=17)报道了185例患者。观察到的异常情况包括存在扫视侵入(143/161),例如方波急动(SWJ,90/109)和眼部颤振(21/43),偏心凝视受损(40/104),异常追踪(81/93)和前庭眼反射(aVOR)缺陷(39/48)。对于视觉引导扫视(VGS),我们经常观察到扫视潜伏期(27/38)和扫视差(71/93)的增加,而扫视速度更经常被保留(37/43)。增强的防扫视(AS)延迟,眼球震颤和频繁的宏观SWJ与疾病持续时间相关。增加了AS延迟和VGS延迟,频繁的宏-SWJ,aVOR增益降低和aVOR峰期延长与疾病严重程度相关。文献中记载了广泛的动眼和前庭缺陷。追求中的障碍,扫视和AVOR反应是最常见的报道,因此,应优先作为疾病标志物。FRDA中的定量动眼测试可能有助于早期诊断,并证明在监测疾病进展和治疗反应方面很有价值。
    Disease-specific oculomotor assessments play a crucial role in the early diagnosis of hereditary cerebellar ataxias. Whereas several studies have reported on quantitative oculomotor and vestibular measurements in Friedreich\'s Ataxia (FRDA), the value of specific oculomotor paradigms remains unclear. We aimed to address this knowledge gap through a systematic literature review and providing disease-specific recommendations for a tailored set of eye-movement recordings in FRDA. MEDLINE and Embase were searched for studies reporting on quantitative oculomotor and/or vestibular measurements in FRDA-patients. Data on oculomotor and vestibular parameters were extracted and correlations with a range of clinical parameters were sought. Included studies (n = 17) reported on 185 patients. Abnormalities observed included the presence of saccadic intrusions (143/161) such as square-wave jerks (SWJ, 90/109) and ocular flutter (21/43), impaired eccentric gaze-holding (40/104), abnormal pursuit (81/93) and angular vestibulo-ocular reflex (aVOR) deficits (39/48). For visually-guided saccades (VGS), we frequently observed increases in saccade latency (27/38) and dysmetric saccades (71/93), whereas saccade velocity was more often preserved (37/43). Augmented anti-saccade (AS) latency, downbeat nystagmus and frequent macro-SWJ correlated with disease duration. Increased AS-latency and VGS-latency, frequent macro-SWJ, reduced aVOR-gain and augmented aVOR peak-latency correlated with disease severity. A broad range of oculomotor and vestibular deficits are documented in the literature. Impairments in pursuit, saccades and aVOR-responses are most commonly reported, and as such, should be prioritized as disease markers. Quantitative oculomotor testing in FRDA may facilitate early diagnosis and prove valuable in monitoring disease progression and treatment response.
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