Search Results (40)

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

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

Alcohol use disorder is associated with substantial neurologic and systemic morbidity, but its relationship with audiovestibular dysfunction has not been clearly synthesized. This systematic review summarized human primary clinical evidence on auditory and vestibular manifestations, diagnostic findings, treatment, and prognosis in alcohol use disorder. PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect were searched from inception to 4 February 2026; the review followed PRISMA 2020 guidelines and was registered in PROSPERO (CRD420261301021). Because study designs, clinical contexts, and outcome measures were highly heterogeneous, a structured qualitative synthesis was performed. Twelve human primary clinical studies were included. The available evidence supported objective auditory dysfunction, most commonly sensorineural hearing impairment with frequent high-frequency involvement. Vestibular involvement was also reported, but the evidence was smaller and less phenotypically specific, consisting mainly of syndromic reports, broad peripheral/central classifications, and historical nystagmographic findings. Direct treatment evidence was very limited; improvement after thiamine replacement was reported in alcohol-related Wernicke-spectrum presentations, but no established disease-specific therapy was identified. Overall, current human clinical evidence supports heightened clinical awareness but not disease-specific screening algorithms or targeted therapeutic recommendations. Better prospective studies using contemporary phenotype-based audiovestibular assessment are needed. Full article

Background: In the last few decades, a growing body of evidence has confirmed that vestibular rehabilitation (VR) can improve the symptoms of many unilateral and bilateral vestibular disorders, by facilitating vestibular compensation mechanisms, such as adaptation, substitution, and habituation. However, the usefulness of the vestibular rehabilitation approach in Persistent Postural–Perceptual Dizziness (PPPD) is currently highly debated and unclear. The aim of the present study was to evaluate the efficacy of VR using computerized dynamic posturography in PPPD patients as a single treatment and without other associated psychological or pharmacological therapies. Results were compared with patients with unilateral and bilateral vestibular disfunction, in order to define the role of our rehabilitation model within a framework of personalized therapy for different disorders. Methods: We evaluated 44 patients (23 F, 21 M; ranged from 28 to 82 years; mean age 63.72) affected by unilateral vestibular vestibulopathy (UVP) (n = 19), bilateral vestibular vestibulopathy (BVP) (n = 10) and PPPD (n = 15). For each patient, a comprehensive clinical bedside vestibular assessment was carefully performed by expert clinicians, as well as Bithermal caloric tests with videonystagmography (VNG), Video Head Impulse Test (vHIT) and Computed Dynamic Posturography (CDP). The impact of dizziness on quality of life (QoL) was assessed by the Italian Dizziness Handicap Inventory (DHI). All subjects evaluated in this study underwent five rehabilitative therapy sessions in our centre, once a week for 45 min and exercised daily for 30 min at home. All the exercises progressively became more difficult each week. Results: Our study showed that vestibular rehabilitation improved quality of life and reduced the level of self-perceived handicap in patients affected by unilateral and bilateral vestibular dysfunction, with significant improvement in DHI total score and posturographic parameters. In PPPD patients, rehabilitation did not significantly modify posturographic performances and the improvement in total DHI score did not reach statistical significance, although a significant change was observed in the functional sub-score. Conclusions: Vestibular rehabilitation confirmed its effectiveness in unilateral and bilateral peripheral vestibulopathies. In patients with PPPD, rehabilitation performed with computerized dynamic posturography may reduce subjective handicap and improve some aspects of daily functioning, although the small sample size and the absence of a control group do not allow definitive conclusions about its efficacy. Full article

Background: Turner syndrome is a genotypic disorder in females characterized by the total or partial absence of an X chromosome. While cardiovascular issues and sensorineural hearing loss are well-documented, vestibular system involvement remains understudied. This study aims to examine vestibular system involvement in patients with Turner syndrome and assess if they exhibit a higher prevalence of peripheral vestibular pathology compared to the general population. Methods: A retrospective longitudinal study was conducted with 21 Turner syndrome patients and 21 age-matched controls. Evaluations included clinical history, otoscopy, pure tone audiometry, the Video Head Impulse Test (vHIT) to measure vestibulo-ocular reflex gain, and computerized dynamic posturography, specifically the Sensory Organization Test (SOT) and Stability Limits Analysis. Results: Turner syndrome patients showed significantly higher hearing thresholds across all frequencies compared to controls (p < 0.001). In the vHIT, 30% of the Turner group presented pathological results, with significant gain reductions in the right horizontal and left posterior semicircular canals. Posturography revealed a significant reduction in overall stability (p = 0.006) and a significantly lower vestibular index (p = 0.011) in the Turner group. Additionally, patients with Turner syndrome demonstrated significant impairments in directional control, reaction time, and excursion points during Stability Limits Analysis. Conclusions: Patients with Turner syndrome are more likely to experience vestibular disorders, a finding likely associated with estrogen deficiency and the loss of its protective effect on the inner ear. These results highlight the necessity of including vestibular and posturographic assessments in the routine clinical follow-up of these patients to facilitate early detection and rehabilitation, even in the absence of overt symptoms like vertigo. Full article

Background: Vertigo and dizziness in children represent diagnostically challenging conditions with heterogeneous etiologies. At initial presentation, a substantial proportion of pediatric patients remain without a definitive etiological diagnosis. Evidence on the impact of longitudinal follow-up on etiological classification in pediatric vertigo is limited. Methods: This observational cohort study uses prospectively collected clinical data. Children aged 1–17 years who presented to a tertiary ENT clinic with vertigo and/or dizziness between 2015 and 2020 were systematically enrolled and followed. The present study represents a retrospective revision of a previously published cohort of 257 children. In 2025, extended follow-up data were reviewed to reassess etiological classification using the same diagnostic categories as in the original analysis. Descriptive statistics were applied to compare etiological distributions at initial evaluation and after follow-up revision. Results: After data revision, the proportion of children with unclassified etiology decreased from 44% to 10%. Central etiologies accounted for 35% of cases, peripheral vestibular disorders for 18%, hemodynamic causes for 16%, psychogenic etiologies for 10%, and other specific causes for 7%. Follow-up duration ranged from 0 to 132 months (mean 17.6 months; median 4.5 months). Diagnostic investigations were frequently performed; however, the etiological yield of certain tests, particularly cranial computed tomography, was low. Conclusions: Extended follow-up significantly improves etiological classification in children with vertigo and dizziness, demonstrating that diagnostic uncertainty at initial presentation often reflects evolving clinical phenotypes rather than the absence of an underlying disorder. A longitudinal, clinically guided, and multidisciplinary approach is essential to enhance diagnostic accuracy and optimize the use of diagnostic investigations in pediatric vertigo. Full article

Background: Benign paroxysmal positional vertigo (BPPV) is the most common peripheral vestibular disorder and most frequently involves the posterior semicircular canal (PSC). Atypical apogeotropic variants of PSC-BPPV may present with pure down-beating positional nystagmus, mimicking contralateral anterior semicircular canal involvement and resulting in diagnostic and therapeutic uncertainty. Objective: To assess the effectiveness of the Bascule/Pendular maneuver in managing patients with pure down-beating positional nystagmus and suspected apogeotropic PSC-BPPV. Methods: A total of 178 patients presenting with pure down-beating positional nystagmus without a torsional component were evaluated using a standardized diagnostic protocol under video-Frenzel goggle monitoring. All patients underwent the Bascule/Pendular maneuver, a modification of the classical Semont maneuver designed to mobilize otoconial debris along the vertical canal planes (Left Anterior–Right Posterior and Right Anterior–Left Posterior), regardless of precise lateralization. Conversion of nystagmus from the apogeotropic to the geotropic variant was considered the primary outcome. Results: The maneuver was well tolerated, with no procedural interruptions or complications. Immediate conversion to the geotropic variant was achieved in 86 patients (48.3%) after a single maneuver. In the remaining patients, successful conversion was obtained after additional maneuvers, most commonly following a second application on the contralateral plane. Once geotropization was achieved, all patients were successfully treated using a standard posterior canal repositioning maneuver. Conclusions: The Bascule/Pendular maneuver is a practical and effective approach for patients presenting with pure down-beating positional nystagmus and suspected apogeotropic PSC-BPPV. By facilitating conversion to the geotropic form, it allows prompt treatment with conventional repositioning maneuvers and may represent a useful first-line strategy in atypical BPPV presentations. Full article

NF2-related Schwannomatosis (NF2-SWN) remains a disorder with few effective treatment options. Patients develop vestibular schwannomas (VSs) on both auditory nerves, which gradually impair hearing and often result in significant communication difficulties, social withdrawal, and higher rates of depression. Progress in understanding NF2-SWN biology and translating discoveries into therapies has been slowed by the absence of robust animal models that faithfully reproduce both tumor behavior and the associated neurological deficits. In this review, we summarized the development of animal models that not only reproduce tumor growth in the peripheral nerve microenvironment but also reproduce tumor-induced neurological symptoms, such as hearing loss and ataxia. We further highlight the currently available organotypic models for NF2-SWN. Together, these systems provide an essential foundation for advancing mechanistic studies and accelerating the development of effective therapies for this devastating disorder. Full article

Background and Clinical Significance: Vestibular disorders include a wide range of conditions with overlapping symptoms such as dizziness, vertigo and imbalance, often offering diagnostic challenges when distinguishing between peripheral and central etiology. Accurate differentiation is essential for establishing effective treatment plans. In rare or atypical cases with subtle findings, comprehensive diagnostic tools—such as extended vestibular tests and structured questionnaires like the Dizziness Handicap Inventory (DHI)—are critical for diagnosis and monitoring patient recovery. Case Presentation: A 35-year-old female presented with chronic imbalance and motion-induced dizziness persisting for four years. The patient had a surgical history of right-sided functional neck dissection for a parotid tumor. A comprehensive audiovestibular evaluation was performed, including pure tone audiometry (PTA), tympanometry, videonystagmography (VNG), cervical vestibular evoked myogenic potentials (cVEMP), ocular vestibular evoked myogenic potentials (oVEMP), video head impulse testing (vHIT), computerized dynamic posturography (CDP), and magnetic resonance imaging (MRI). The Dizziness Handicap Index (DHI) was administered at baseline and post-treatment to monitor subjective symptom changes. Objective testing revealed marked right–left amplitude asymmetry on cVEMP, which were recorded from the trapezius muscle due to prior neck dissection surgery, indicating isolated right-sided saccular hypofunction. Following targeted vestibular rehabilitation and pharmacologic treatment, the 3-month reassessment demonstrated resolution of symptoms and a reduction in DHI scores from 24 to 6. Conclusions: Comprehensive vestibular testing, performed in a single diagnostic session, enabled the accurate identification of isolated right-sided saccular hypofunction in this complex post-surgical case. Combining cVEMP, CDP, and DHI assessment provided a complete functional profile, guided targeted rehabilitation, and allowed objective monitoring of recovery. Full article

Background/Objective: The Suppression Head Impulse Paradigm (SHIMP) is a specialized variant of the Head Impulse Test (HIT), designed to evaluate the suppression of the angular Vestibulo-Ocular Reflex (aVOR) by central mechanisms. These mechanisms are primarily mediated by brainstem structures, including the vestibular nuclei, their projections to ocular motor nuclei, and modulatory inputs from the cerebellum. Damage to these areas can impair the generation of anti-compensatory saccades (ACs), even when the peripheral vestibular apparatus remains intact. The present study explores this phenomenon in a cohort of patients with neurological disorders known to potentially involve the brainstem, including multiple sclerosis, severe traumatic brain injury, stroke, and Parkinson’s disease. Methods: This cross-sectional study included 119 patients with multiple sclerosis (PwMS), severe traumatic brain injury (PwTBI), stroke (PwS), and Parkinson’s disease (PwPD). The video Head Impulse Test was performed to assess the aVOR gain across all semicircular canals using both the HIMP and SHIMP. The presence, absence, or delay of ACs was systematically recorded. Results: Among the 119 patients evaluated (238 semicircular canals), 24 (20%) demonstrated normal aVOR gain but failed to generate ACs during SHIMP. The absence of ACs was observed in seven PwMS, five with PwTBI, six with PwS, and six with PwPD. Conclusions: The absence of ACs despite normal aVOR gain suggests a potential impairment in the central pathways controlling saccadic responses, independently of peripheral vestibular function. These findings underscore the clinical relevance of integrating the SHIMP into vestibular assessments to improve the identification of central vestibular dysfunction in neurological disorders. Full article

Neurocutaneous syndromes, known as phakomatoses, encompass a diverse group of congenital conditions affecting the nervous system and skin, with neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2) among the most clinically significant. Both disorders are inherited in an autosomal dominant manner. NF1 presents with café-au-lait macules; cutaneous, subcutaneous, and plexiform neurofibromas; skeletal abnormalities; learning disabilities; and optic pathway gliomas, while NF2 is characterised by bilateral vestibular schwannomas, multiple meningiomas, ependymomas, and peripheral nerve schwannomas. Although germline mutations in the NF1 and NF2 tumour suppressor genes are well established, they do not fully explain the broad clinical variability observed, even among individuals carrying identical mutations. As increasingly recognised in other genetic diseases, epigenetic mechanisms, including DNA methylation, histone modifications, chromatin remodelling, and non-coding RNA (ncRNA) regulation, play a critical role in modulating gene expression and influencing disease severity. Despite important findings, the research remains fragmented, and a unified model is lacking. This review organises the current knowledge, emphasising how epigenetic alterations impact disease behaviour and outlining their potential as prognostic biomarkers and therapeutic targets. A deeper understanding of these mechanisms could lead to improved personalised management and the development of targeted epigenetic therapies for individuals with NF1 and NF2. Full article

Background: The vestibular Romberg test, which assesses the deterioration of balance while standing on rubber foam with closed eyes, is a well-established method in the physical neurological assessment of patients with peripheral vestibulopathy. This study aims to determine whether it can differentiate peripheral vestibulopathy from its main differential diagnosis, namely sensory ataxia, as both conditions typically present with a positive classical Romberg test. Methods: Static balance was assessed in three groups: patients with peripheral vestibulopathy, patients with pure sensory neuropathy, and healthy age-matched controls. Participants stood quietly on a force platform under varying visual and proprioceptive feedback conditions. Conventional and advanced postural sway metrics were investigated to establish a quantitative analogy to both the clinical Romberg and vestibular Romberg tests. Results: Posturographic analysis revealed that, in contrast to healthy controls, patients with vestibular disorders exhibited higher vestibular Romberg quotient values. However, the classical vestibular Romberg quotient did not show diagnostic discrimination between vestibulopathy and sensory neuropathy patients. This lack of discrimination was mainly due to the increased body sway observed in all patient groups under the “eyes open” condition. Nevertheless, a refined vestibular Romberg quotient—comparing standing on foam versus standing on firm support with eyes closed—was able to reliably distinguish vestibulopathy from sensory ataxia. This distinction was evident in both conventional linear sway and spectral postural sway metrics. Conclusions: We conclude that a refined Romberg test, performed solely under conditions of visual deprivation, offers valuable classification potential in differentiating peripheral vestibulopathy not only from healthy controls but also from patients with disequilibrium due to sensory loss. Full article

Background/Objectives: To evaluate the clinical characteristics and diagnostic significance of dissociation between bithermal caloric test and video head impulse test (vHIT) in patients presenting with dizziness. Methods: We retrospectively reviewed 644 patients who underwent bithermal caloric testing and vHIT at a university-affiliated general hospital. Patients were classified into concordant and discordant groups based on test results. The discordant group was further subdivided into those with abnormal caloric test and normal vHIT, and those with normal caloric test and abnormal vHIT. Demographic data, vestibular function test outcomes, and clinical diagnoses were analyzed. Results: Discordant results were observed in 36.5% of patients. Among these, 31.8% had abnormal caloric responses with normal vHIT, and 4.7% had normal caloric responses with abnormal vHIT. Most patients in both discordant subgroups were diagnosed with peripheral vestibular disorders, such as Ménière’s disease and unilateral vestibulopathy. The abnormal caloric/normal vHIT pattern was more common and associated with low-frequency dysfunction. The normal caloric/abnormal vHIT pattern, though less frequent, also involved predominantly peripheral etiologies. Conclusions: Dissociation between caloric and vHIT results is not uncommon and provides important diagnostic insights. Employing both tests in a complementary manner enhances the identification of frequency-specific vestibular deficits and supports more accurate diagnosis and management of vestibular disorders. Full article

This review focuses on the contribution of medical imaging in the diagnosis of peripheral vestibular disorders. This is a narrative review based on a focused literature search conducted using PubMed and the Cochrane Library. Imaging is not usually recommended in initial consultations for vestibular disorders because only 5–10% of MRI scans reveal findings directly related to the disease. The study is a review of the literature that highlights the utility and limitations of imaging such as computed tomography (CT) and magnetic resonance imaging (MRI). It follows the diagnostic approach from history and physical examination to laboratory tests and imaging. Some conditions like vestibular neuritis and benign paroxysmal positional vertigo (BPPV) have limited imaging utility due to the fine details required. Conversely, high-resolution CT and MRI are important for diagnosing Meniere’s disease, acoustic neuroma, and superior canal dehiscence. The role of imaging varies a lot among specific conditions. Advances in imaging technology, particularly high-resolution MRI, promise enhanced diagnostic capabilities. Full article

Introduction: This edition of the Audiology Research journal is dedicated to Dr. John Epley. For this reason, we would like to present a very unusual case involving the development of a bilateral abnormality of the otolith organs. The otoliths are the structures from which calcium particles detach to induce benign paroxysmal positional vertigo, the disorder for which Dr. Epley is famous for managing. Case report: This case report outlines an unusual presentation of vestibular pathology involving the otoliths. Discussion: We suggest that the atypical presentation may be related to a bilaterally asymmetrical insult to the otoliths. Conclusions: The central insult suffered by this patient resulted in bilateral peripheral otolithic pathology. Full article