Search Results (276)

Vertigo, dizziness, and oculomotor disturbances may occur as manifestations of immune-mediated disorders affecting the inner ear, central vestibular pathways, or multisystem autoimmune disease. Although uncommon, these conditions are clinically important because delayed recognition may lead to irreversible hearing loss, vestibular dysfunction, or neurological disability. This review summarizes the clinical presentation, diagnostic approach, and treatment of immune-mediated vestibular and oculomotor disorders. We suggest a practical classification into isolated immune-mediated inner ear disease, systemic autoimmune disorders with audio-vestibular involvement, and autoimmune disorders of the central or peripheral nervous system affecting balance and eye movements. Red flags for such conditions include bilateral or progressive symptoms, fluctuating audio-vestibular deficits, associated neurological signs, and accompanied autoimmune disease. Corticosteroids remain the main first-line treatment in many of these disorders, mainly due to missing data from controlled trials. Steroid-sparing immunosuppressants, biologics, and tumor-directed therapies are effective in many cases; however, because of the missing data, they are only used in selected entities without any other choice. A structured neuro-otological and immunological workup is essential to improve diagnostic accuracy and enable timely therapy. Full article

Background/Objectives: This study aimed to examine the associations between diet-related quality of life (DRQOL) and psychological distress, autonomic dysfunction, and migraine in patients with dizziness and balance disorders. Methods: In this retrospective cross-sectional study, 122 patients (56 men, 66 women; mean age 40.4 ± 12.8 years, minimum 14, maximum 65) from the vertigo outpatient clinic at JR Tokyo General Hospital completed self-reported questionnaires. These included the DRQOL scale, Dizziness Handicap Inventory (DHI), Hospital Anxiety and Depression Scale (HADS), Self-rating Depression Scale (SDS), Orthostatic Dysregulation (OD) checklist, and migraine assessments (POUNDing [Pulsating, duration of 4–72 h, Unilateral, Nausea, Disabling], MIDAS, migraine screener). Correlational analyses, group comparisons, and receiver operating characteristic (ROC) analyses were conducted. Results: Higher DRQOL scores indicate poorer DRQOL. DRQOL scores showed positive correlations with psychological distress (SDS: ρ = 0.57; HADS-A: ρ = 0.50; HADS-D: ρ = 0.53; all p < 0.001) and OD severity (ρ = 0.50, p < 0.001) but not with age, DHI, or individual migraine indices. Migraine screener-positive patients had significantly higher DRQOL scores (p < 0.01). DRQOL alone showed modest ability to discriminate migraine screener-positive from migraine screener-negative patients (AUC = 0.65); discrimination improved to an AUC of 0.77 in a multivariable model that also included age and sex. Conclusions: DRQOL appears to capture psychological and autonomic symptom burden rather than vestibular or headache severity, suggesting that it may serve as a complementary, patient-centered metric that adds a multidimensional perspective to conventional vestibular and headache assessments. Full article

Nystagmus, the involuntary rhythmic oscillation of the eyes, is a critical diagnostic marker in vestibular medicine, distinguishing life-threatening central disorders such as stroke from benign peripheral conditions including Benign Paroxysmal Positional Vertigo (BPPV). Despite its clinical importance, accurate nystagmus assessment has long been constrained by expensive infrared video-oculography equipment such as videonystagmography, specialist dependency, and the episodic nature of vestibular symptoms that are often resolved before a clinical encounter. This review synthesizes approximately 50 papers published between 1952 and 2026 across four thematic domains: AI-driven nystagmus analysis, clinical medicine, smartphone and portable hardware innovations, and telemedicine and remote monitoring. On the AI front, classical machine learning models achieve up to 98.77% nystagmus recognition accuracy using ensemble methods, while deep learning frameworks spanning CNNs, U-Nets, LSTMs, and optical flow networks demonstrate clinical-grade slow-phase velocity measurement equivalent to gold standard video-oculography on standard smartphone RGB video. Large language and vision models including GPT-4V and Gemini 2.0 show early-stage promise as zero-shot triage tools but currently fall well below specialist-level diagnostic accuracy. Concurrently, portable hardware innovations ranging from 3D-printed goggle systems to ARKit-based smartphone applications are narrowing the accessibility gap, while telemedicine frameworks enable ictal recording and cloud-based specialist review outside the clinic. Across all domains, the common barriers to clinical translation are dataset scarcity for rare BPPV subtypes, sensitivity to ambient conditions, and the absence of explainable AI mechanisms. This review maps the current state of the field and identifies multimodal data fusion, prospective clinical validation, and interpretable AI as the critical next steps toward equitable, specialist independent vestibular diagnostics. Full article

Horizontal nystagmus is still commonly interpreted at the bedside through a pragmatic peripheral-versus-central dichotomy. Although this heuristic is often clinically useful, it may be misleading because distributed brainstem–cerebellar disorders can generate peripheral-appearing phenotypes. This paper presents a narrative clinical–conceptual review proposing that a substantial subset of horizontal nystagmus patterns may be understood more coherently as expressions of dysfunction within a coupled vestibulo-ocular integrative network rather than as direct signatures of a single lesion site. Within this framework, two core dynamical domains are separated conceptually: a vestibular nuclei (VN)-centered velocity-storage process and an NPH-centered gaze-holding integrator. These processes are proposed to operate under cerebellar regulatory influence, with the nodulus–uvula (NU) acting as a plausible regulator of storage gain, temporal persistence, adaptation stability, and oscillatory behavior. Clinically, the velocity-storage domain is expressed through low-frequency vestibulo-ocular reflex behavior and optokinetic after-nystagmus-related dynamics, whereas the gaze-holding domain is expressed through eccentric gaze stability, gaze-evoked nystagmus, and post-saccadic drift. This framework carries a clinically relevant implication: horizontal nystagmus phenotypes may be interpreted more effectively by asking which functional process is predominantly abnormal—gaze holding, storage-related vestibular persistence, or cerebellar regulatory stability—rather than by relying solely on a binary peripheral–central label. On this basis, we outline a clinician-facing workflow linking gaze dependence, periodicity, direction reversals, head-shaking behavior, and Alexander-law mismatch to operational bedside criteria and candidate quantitative readouts. The proposed model is intended as a clinical–conceptual framework rather than a deterministic localization tool. Its main value lies in organizing discordant vestibular findings, strengthening the mechanistic interpretation of bedside and instrumented observations, and identifying testable directions for future validation studies in acute dizziness and ocular motor disorders. Full article

Eustachian tube dysfunction (ETD) and related pressure-mediated otologic disorders often present with fluctuating auditory, vestibular, and pressure-related symptoms that are difficult to explain using static structural or symptom-based diagnostic labels alone. This conceptual review proposes the Neuro–Vascular–Mechanical–Inflammatory–Autonomic (NVMIA) framework as a hypothesis-generating architecture for organizing such variability. Within this framework, middle ear pressure (MEP) is interpreted as a clinically measurable physiologic variable through which interacting neural, vascular, mechanical, inflammatory, and autonomic influences may become mechanically expressed and clinically observable. The framework does not present NVMIA-based patterns as validated diagnostic categories, clinical decision tools, or treatment algorithms. Rather, it proposes provisional regulatory patterns that may help generate testable hypotheses regarding pressure-regulatory instability, cross-axis coupling, symptom fluctuation, and physiologic reversibility. Mechanical impedance may function as an accessible reference plane for future empirical assessment, while neural, vascular, inflammatory, and autonomic domains are conceptualized as modulatory axes that may alter symptom expression and response variability. The review further outlines future validation needs, including dynamic MEP measurement, patient-reported outcome integration, longitudinal response assessment, and cautious computational modeling. By reframing ETD as a model of state-dependent regulatory instability, the NVMIA framework provides a conceptual basis for future studies in precision otology while emphasizing that prospective validation is required before clinical implementation. Full article

Background/Objectives: Auditory neuropathy spectrum disorder (ANSD) is a well-established hearing disorder in the pediatric population and is estimated to account for at least 10% of children with sensorineural hearing loss. Compared to auditory function, vestibular function in children with ANSD has not been well described in the past. The purpose of this study is to examine vestibular testing results in children with ANSD and to better characterize vestibular dysfunction in these children. Methods: A retrospective review of vestibular laboratory testing results was conducted in pediatric patients diagnosed with ANSD. Vestibular evaluation included vestibular evoked myogenic potential (VEMP), rotary chair test, video head impulse test (vHIT), and videonystagmography (VNG). Results: A total of 30 pediatric patients with ANSD were identified, including 18 boys and 12 girls, with a mean age of 4.5 years. Bilateral ANSD was found in 24 cases, while 6 cases were unilateral. Etiologies of ANSD included a history of hyperbilirubinemia in infancy, cochlear nerve dysplasia, genetic-related conditions, etc. Vestibular dysfunction was found in 12 cases, as indicated by at least one abnormal outcome in VEMP, vHIT, or rotary chair testing. Nineteen children were cochlear implant candidates and eventually underwent unilateral or bilateral implantation. Conclusions: Vestibular dysfunction is significant in pediatric patients with ANSD, and vestibular outcomes appear to be related to underlying etiologies. Formal vestibular evaluation is necessary to identify such vestibular losses, and these findings will be helpful to guide clinical management and rehabilitation strategies for these children. Full article

Background/Objectives: Persistent dizziness after the apparent resolution of an acute or episodic vestibular disorder remains a frequent and clinically challenging condition. In many patients, symptoms persist despite negative positional testing, absence of spontaneous nystagmus, and preserved high-frequency vestibular responses on video head impulse testing. This discrepancy suggests that persistent dizziness may not always be explained by incomplete recovery of a single peripheral vestibular lesion but may reflect a dissociation between transient/high-frequency vestibular responses and sustained/low-frequency or integrative vestibular processing. The aim of this study was to propose a hypothesis-generating, case-based clinical framework for interpreting this dissociation and its implications for targeted vestibular rehabilitation. Methods: This was a retrospective, hypothesis-generating, case-based clinical study derived from routine specialist neuro-otological practice. Four illustrative cases were selected because they represented distinct patterns of persistent dizziness in which preserved or near-preserved transient vestibular responses coexisted with abnormalities in sustained, otolithic, visual–vestibular, or velocity-storage-dependent processing. All patients underwent detailed clinical history assessment, bedside neuro-otological examination, and multidomain vestibular assessment according to clinical indication. The purpose of the study was not to estimate prevalence, validate diagnostic accuracy, or demonstrate treatment efficacy but to illustrate a physiology-based interpretive framework. Results: The four cases showed different patterns of time-domain dissociation. These included low-frequency integrative dysfunction without clear peripheral lateralization, incompletely compensated unilateral vestibular asymmetry, selective unilateral otolithic loss despite preserved semicircular canal high-frequency responses, and bilateral sustained vestibular hypofunction unmasked by an apparently resolved BPPV-like event. Across cases, persistent symptoms were better explained by the relationship between transient and sustained vestibular domains than by any single test result considered in isolation. Conclusions: Persistent dizziness may arise from different combinations of preserved transient vestibular responses and impaired sustained or integrative vestibular processing. The proposed framework does not introduce new vestibular tests and does not claim to validate a new diagnostic entity. Rather, it organizes established vestibular investigations within a time-domain model that may help identify clinically meaningful dissociations and guide individualized, domain-specific vestibular rehabilitation. Prospective studies with larger samples and external validation are required to determine the diagnostic and therapeutic value of this approach. Full article

Background: Vestibular tests are critical for an early detection of balance disorders, thereby reducing the risk of falls, particularly in older adults. The present is an ecologic study where the units of observation are the Italian Regions. Regional scores of access to essential levels of care (LEAs) were employed as predictors to investigate the hypothesis that vestibular examinations supplied by the Italian National Health Service (NHS) were insufficient to meet the needs of the Italian population. Methods: The number of first-level (clinical evaluations of vestibular function) and second-level (recorded spontaneous nystagmus, induced nystagmus, or rotary stimulation) vestibular tests per 100 K population was estimated by Italian region and calendar year during the 2021–2023 period. The odds (i.e., number of any second-level vestibular tests divided by difference between number of first- and second-level tests) by region and calendar year were investigated as a proxy for regional propensity to refer patients to a second-level test following a first-level vestibular assessment. A logistic regression investigated the odds by region and calendar year. Lastly, the regional number × 100 K population (prevalence) of vestibular examinations underwent linear regression analysis, using LEAs as predictors. Results: Descriptive analysis showed that first-level assessments were the most common vestibular tests in Italy during the 2021–2023 period. Prevalence of first-level vestibular examinations was not associated with any indicator of access to healthcare in linear regression. By contrast, the prevalence of second-level vestibular tests decreased with social inequality yet increased with the indicator of higher access to hospital care. In logistic regression, referral propensity to second-level vestibular tests progressively decreased from 2021 to 2023 and exhibited considerable regional variability, being lower than in Lombardy (reference) in all other Italian regions but Veneto (aOR = 4.826; 95%CI: 4.445; 5.329) and Trento autonomous province (aOR = 1.488; 1.363; 1.624). Conclusions: The number of vestibular function tests supplied by the National Health Service in Italy during the 2021–2023 period was probably insufficient to meet the care needs of the general population, forcing more than 66.8% of patients to forgo vestibular evaluation or turn to private audiological services with out-of-pocket payments. The shortfall was greater for more costly instrumental (second-level) vestibular tests, whose supply was influenced by social inequalities and barriers to accessing audiology care at the regional level. The National Recovery and Resiliency Plan has allocated EUR 20.23 billion for healthcare services in Italy, with the aim of addressing patient care requirements in every area of the country. Full article

The calcitonin gene-related peptide (CGRP) is a 37-amino acid neuropeptide belonging to the calcitonin family, discovered as a product of alternative splicing of the calcitonin gene. CGRP has emerged as a pleiotropic signaling molecule with widespread distribution in the central and peripheral nervous systems, particularly within primary sensory neurons. This narrative review synthesizes current knowledge on the CGRP system, integrating recent advances in its molecular structure, gene organization, and post-translational processing with high-resolution structural insights into its heterodimeric receptor complex (CLR-RAMP1) obtained through cryo-electron microscopy. We also include long-term safety data on anti-CGRP monoclonal antibodies, emerging cardiovascular risk signals, and novel therapeutic applications in vestibular migraine and pediatric populations. The intracellular signaling cascades activated by CGRP, including the canonical cAMP-PKA pathway, MAP kinase activation, and context-dependent calcium signaling, are discussed in relation to its diverse physiological functions. These encompass vasodilation, nociception modulation, neurogenic inflammation, gastrointestinal motility, bone metabolism, tissue regeneration, and energy homeostasis. The central role of CGRP in migraine pathophysiology is examined to understand the development of targeted therapies. The current pharmacological landscape is reviewed, including the evolution of small-molecule CGRP receptor antagonists (gepants) through three generations and the four approved monoclonal antibodies targeting CGRP or its receptor, with comparative analysis of their efficacy, safety profiles, and clinical positioning. Beyond migraine, emerging and predominantly preclinical roles of the CGRP system are discussed in chronic pain, osteoarthritis, cardiovascular diseases, sepsis, cancer (particularly bone metastases and tumor microenvironment immunomodulation), and neurodegenerative disorders such as Alzheimer’s disease. In these areas, the available evidence remains heterogeneous and, in most cases, is not yet sufficient to support clinical translation. Finally, future directions are discussed, including the development of stable CGRP analogs, allosteric modulators, and the potential expansion of therapeutic applications into oncology, intensive care medicine, and neuroprotection. Full article

This is a narrative review that explores the development of non-implantable vestibular devices designed to address postural instability, particularly in aging populations and patients with vestibular hypofunction. It establishes that balance relies on complex sensory integration and that the functional decline of this system creates a significant medical need. Three principal technological strategies are examined: sensory substitution devices, galvanic vestibular stimulation (GVS), and immersive visual feedback systems. Sensory substitution devices, which convert balance data into auditory, tactile, or electrotactile cues, demonstrate significant promise. Examples like vibrotactile belts provide feedback that reduces postural sway, enhancing stability and patient confidence. Parallel to this, GVS—using electrical currents applied to the mastoids—emerges as a potent non-invasive method to modulate vestibular pathways, improving balance control and even inducing neuroplastic changes, especially with stochastic “noisy” signals. The most recently developed devices include augmented and virtual reality technologies that offer innovative visual feedback, creating enriched rehabilitation environments that accelerate recovery by promoting sensory reweighting and neural adaptation. This review concludes that while implantable prostheses are advancing, non-invasive devices offer versatile, affordable, and complementary solutions for balance restoration. The future success of non-invasive alternatives hinges on developing more sophisticated stimulation protocols that account for the complexity of natural movement and individual patient contexts, expanding therapeutic options for vestibular disorders. Full article

Background/Objectives: Vestibular evoked myogenic potentials (VEMPs) and the video head impulse test (vHIT) enable receptor-specific assessment of otolithic organs and semicircular canals. Their increasing use has revealed selective or apparently isolated vestibular abnormalities, although the clinical significance of these findings remains uncertain. This mini-review examines selective otolithic and semicircular canal dysfunction, with emphasis on diagnostic interpretation, methodological limitations, and future research needs. Methods: A structured narrative review of PubMed/MEDLINE and Scopus was conducted, focusing on studies reporting isolated, selective, or disproportionate vestibular abnormalities assessed by VEMPs and/or vHIT. Relevant original studies, case series, case reports, reviews, and diagnostic or consensus papers were considered. Results: Selective otolithic dysfunction may involve the utricle, saccule, or both and is often associated with imbalance, tilting, swaying, spatial disorientation, nausea, or postural instability. Selective semicircular canal dysfunction may involve one or more canals and may present with vertigo, dizziness, nystagmus, or gait instability. Similar VEMP and vHIT patterns occur across vestibular neuritis, Ménière’s disease, vestibular migraine, benign paroxysmal positional vertigo, bilateral vestibulopathy, superior semicircular canal dehiscence, vestibular schwannoma, central vestibular disorders, systemic diseases, and idiopathic presentations. Conclusions: Selective vestibular abnormalities should be interpreted as context-dependent laboratory findings rather than discrete disease entities. Their value depends on reproducibility, anatomical plausibility, clinical concordance, complementary testing, and longitudinal follow-up. Full article

Background/Objectives: Diving exposure can cause auditory injury involving both the middle and inner ear structures. Inner ear barotrauma (IEB) and inner ear decompression sickness (IEDCS) are the major inner ear disorders and frequently present with auditory and vestibular symptoms. This study examined how diving characteristics relate to patterns of auditory trauma. Methods: A retrospective chart review of 30 patients, with 36 affected ears, was performed. Diving depth, clinical manifestations, and treatment responses were analyzed to identify factors influencing related prognosis. Results: Diving depth was an important factor associated with symptom severity and the type of injury. Dives deeper than 30 m of seawater were linked to a higher incidence of sudden sensorineural hearing loss and vertigo. In contrast, transient symptoms with minimal objective abnormalities were typically observed in shallow dives. Patients with concomitant decompression sickness (DCS) showed poorer auditory and vestibular recovery following hyperbaric oxygen therapy, while those without DCS showed better hearing improvement. Vertigo was observed in 80% of IEB cases and 66.7% of IEDCS cases. Hearing recovery appeared to be more frequently observed in cases presenting with middle ear symptoms, suggesting a relatively favorable prognosis for IEB compared with IEDCS. Conclusions: The findings suggest potential associations between diving depth and DCS, and its involvement may play a role in the severity and prognosis of diving-related inner ear injury. IEB appeared to be associated with more favorable auditory outcomes compared with IEDCS; however, this observation should be interpreted with caution due to potential diagnostic uncertainty. Given the descriptive nature of the study, further studies with larger cohorts are needed to refine prognostic indicators and optimize management strategies. Full article

Ménière’s disease is a complex disorder of the inner ear, characterized by recurrent episodes of vertigo, progressive hearing loss, tinnitus, and aural fullness. The pathophysiology of this condition is dominated by endolymphatic hydrops, reflecting imbalances in fluid regulation and pressure within the membranous labyrinth, which impair cellular function and the transmission of vestibular and auditory signals. Although genetic predisposition provides a susceptible background, recent studies emphasize that non-genetic factors act as critical triggers of clinical events, determining both the onset of symptoms and the modulation of their severity. These factors directly influence pathophysiology by disrupting endolymphatic homeostasis and altering the intracellular and tissue dynamics of the membranous labyrinth, thereby contributing to the phenotypic variability observed among patients. The key to this process lies in the synergistic interaction between genetic predisposition and external or contextual influences, which determines the threshold at which the compensatory mechanisms of the inner ear fail, triggering the characteristic episodes. Understanding this interdependence, as well as the underlying disease mechanisms, provides essential insights for the identification of preventive and therapeutic strategies aimed not only at symptom control but also at modulating the factors that influence susceptibility to endolymphatic imbalance. Full article

Background/Objectives: The evaluation of cognition is central to many neurological conditions, including traumatic brain injury, Alzheimer’s disease, Lewy body disease, frontotemporal degeneration, and vascular disorders. In clinical practice, particularly in aging populations, cognitive complaints often arise in the context of mixed neurological processes, requiring careful integration of cognitive and non-cognitive findings. Despite this, there remains limited clarity regarding the respective roles of neurologists and clinical neuropsychologists and the distinction between cognitive and neuropsychological assessments, terms that are often used interchangeably despite important differences in methodology and scope. This lack of a shared framework has practical consequences. Cognitive test results, when interpreted in isolation for diagnosis, may be misconstrued as comprehensive measures of brain function, particularly when non-cognitive neurological features such as motor, cerebellar, or vestibular abnormalities should have been considered (but were not). Methods: In this narrative review, we synthesize clinical guidelines, consensus statements, regulatory sources, and representative empirical literature to articulate a competence-based framework in which cognitive assessment is a medically integrated process incorporating history, functional evaluation, neurological examination, and the targeted use of standardized neuropsychological instruments. Results: Neurologists are trained to establish medical diagnoses and integrate cognitive findings into the context of neurological disease, while neuropsychologists contribute detailed psychometric characterization, culturally and demographically informed interpretation, cognitive phenotyping, functional characterization, and validity assessment in complex clinical and medicolegal contexts. Although neuropsychologists are qualified to diagnose neurocognitive disorders using standardized diagnostic criteria, attribution to specific neurological etiologies requires a comprehensive medical evaluation that extends beyond cognitive testing alone. Conclusions: We outline a tiered approach to evaluation that aligns assessment methods with clinical questions and supports accurate diagnosis, interdisciplinary collaboration, and patient-centered care. Full article