Search Results (21)

Background: The posterior labyrinth is particularly vulnerable to ischemic injury. Vertigo can occasionally be the only presenting symptom of acute myocardial infarction (AMI). Acute Bilateral Vestibular Neuropathy (ABVN) is an extremely rare condition, with only three cases previously reported in the literature. Its exact pathophysiological mechanisms remain unclear. Case Presentation: We present the case of a 76-year-old male who presented to the emergency department (ED) with vertigo and severe postural unsteadiness. Subsequently, a silent AMI was diagnosed, prompting cardiac stenting. Vestibular function assessments over the following eight months confirmed the diagnosis of ABVN. A cycle of vestibular rehabilitation yielded limited objective benefit, although the patient reported subjective improvement as measured by the Dizziness Handicap Inventory (DHI). Discussion and Conclusions: This case suggests a potential association between peripheral vestibular dysfunction and acute hemodynamic impairment due to myocardial infarction. Notably, it represents the first reported case of ABVN following a silent AMI, presenting solely with vestibular symptoms. Full article

Background/Objectives: Vestibular dysfunction is one of the main complications after cochlear implant (CI) surgery, and there are currently no standardized protocols for vestibular assessment in CI candidates. Our objectives were to investigate the incidence of vestibular impairment after CI surgery, anamnestic (age, known systemic pathologies and cause of deafness) and surgical (intraoperative complications, malposition of the CI) risk factors, and the role of vestibular assessment in the selection of the suitable ear for implantation. Methods: We included 68 adult patients (80 ears) affected by moderate-to-profound SNHL undergoing CI. The dizziness handicap inventory (DHI), the video head impulse test (VHIT), the caloric test, and dynamic posturography (DP) were used to study the vestibular function and balance before and one month after CI. The DHI was also administered 24 h after surgery. Results: Despite significative impairment 24 h after surgery (29.6 ± 30), the mean DHI score returned to preoperative values (17.9 ± 26) after one month. Dizziness persisted in case of age ≥ 65 years old, surgical difficulties, simultaneous bilateral CI, Meniere’s disease and otosclerosis, comorbidities ≥ 3, anxiety/depression, and neurological diseases. The VHIT significantly worsened in 25% of ears, while the caloric test SPV nystagmus significantly decreased in 30% of ears. In cases of preoperative unilateral weakness, the implantation of the better ear was significantly related to higher DHI scores. Only 4/68 patients had a significant persistent reduction in the postural composite score after surgery, with an increased risk of falls. Conclusions: Medical history and vestibular assessment predict the risk of vestibular damage and help to choose the CI’s side and to manage vertigo after surgery. Full article

Introduction: Masseter vestibular evoked myogenic potentials (M-VEMPs) are a recent tool for assessing a vestibulo-trigeminal pathway departing from the saccule, similarly to cervical VEMPs (C-VEMPs), that evaluate saccular function via the sternocleidomastoid muscle. M-VEMPs may offer a complementary diagnostic value in vestibular neuritis (VN). Methods: This retrospective study analysed M-VEMPs and C-VEMPs in 28 monolateral patients and 1 bilateral (30 ears) diagnosed with VN between 2023 and 2024. Diagnostic evaluation included video head impulse tests (VHIT), caloric tests, ocular VEMPs, and, in a few cases, electromyography (EMG) of the sternocleidomastoid muscle. M-VEMPs were elicited using 500 Hz tone bursts at 97 dB nHL. Results were compared based on the topography of vestibular involvement and muscle response concordance. Results: M-VEMPs were always present in patients with superior VN and intact saccular function, showing consistent results with normal C-VEMPs. In some cases, with saccular dysfunction, M-VEMPs were preserved despite the absence of C-VEMPs, suggesting greater robustness. One patient with herpes zoster (HZ) involving both the VIII and trigeminal nerves showed absent M-VEMPs, indicating trigeminal pathway involvement. Edentulous patients showed reduced or absent M-VEMPs due to compromised masseter muscle electromyography activity. Conclusions: M-VEMPs are reliable and often concordant with C-VEMPs in VN but may reveal additional diagnostic information in discordant or complex cases. They are particularly useful in identifying trigeminal involvement but are limited in patients with poor masseter muscle function. Further studies are needed to clarify their full diagnostic potential. Full article

Background/Objectives: Idiopathic immune-mediated uveitis (IIMU) is an intraocular inflammatory condition affecting the uveal tract and adjacent ocular structures, potentially leading to systemic involvement. Audiovestibular symptoms, such as sensorineural hearing loss (SNHL) and balance disturbances, are often underdiagnosed in these patients. The potential correlation between IIMU and audiovestibular dysfunction remains insufficiently studied. This study aimed to estimate the prevalence and describe the clinical characteristics of audiovestibular manifestations in patients with IIMU. Methods: We conducted a cross-sectional observational study of 34 patients with a confirmed diagnosis of IIMU at a tertiary academic center. All participants underwent a standardized neurootological assessment, including pure-tone audiometry, video head impulse testing (vHIT), and cervical vestibular-evoked myogenic potentials (cVEMP). Demographic and clinical data were also collected. Results: Audiovestibular dysfunction was identified in 41.18% of patients, with bilateral SNHL (B-SNHL) being the most common finding. Patients with B-SNHL had a significantly later age of uveitis onset (52.3 ± 14.4 vs. 35.9 ± 13.9 years, p = 0.003) and a higher incidence of ocular complications (83.3% vs. 59.1%, p = 0.252). Furthermore, worsening ophthalmologic activity was observed in 25% of patients with B-SNHL, compared to 0% in those without B-SNHL (p = 0.037). Vestibular dysfunction was also associated with delayed onset of uveitis (51.0 ± 17.4 vs. 36.0 ± 12.2 years, p = 0.006) and a non-significantly higher complication rate (76.9% vs. 61.9%, p = 0.465). Conclusions: Audiovestibular dysfunction is a frequent finding in patients with IIMU and is associated with delayed uveitis onset and greater ocular morbidity. These results support the inclusion of systematic audiovestibular screening in clinical evaluations of IIMU patients and suggest that earlier detection may inform prognosis and guide multidisciplinary management strategies. Full article

Background/Objectives: Children with congenital or early onset sensorineural hearing loss (SNHL) are at a greater risk of vestibular dysfunction (VD), hypothesized to occur from the close embryological relationship between the cochlear and vestibular systems. Even with increasing focus on early detection and rehabilitation through Universal Newborn Hearing Screening (UNHS) programmes in many countries, few studies have focused on the prevalence and feasibility of vestibular assessment in infant populations. The objectives of this review are to 1. identify the prevalence of VD infants with congenital or early onset (<12 months old) SNHL, 2. identify which vestibular assessment tests/protocols are conducted on this population, 3. report sensitivity and specificity values for identified vestibular assessment tests/protocols. Methods: Studies that included infants aged 0–12 months, with congenital or early onset SNHL of any laterality, degree, or configuration, and who underwent any method of vestibular assessment were included. The review adhered to the Joanna Briggs Institute (JBI) guidance and the PRISMA-ScR extension statement. Results: A total of 18 studies were included in the review. All articles reported that infants with congenital or early onset SNHL are at a greater risk of VD, particularly those with bilateral severe–profound SNHL. The cervical vestibular-evoked myogenic potentials (cVEMP) test was the most frequently identified vestibular assessment tool for this age demographic. Conclusions: Results from the included articles coincide with results from literature assessing older paediatric populations. cVEMPs have been reported to be a feasible, sensitive, and specific screening tool in infants with congenital or early onset SNHL. The prevalence of VD in infants with congenital or early onset SNHL justify further investigation on the feasibility of establishing a pathway for vestibular assessment for all infants referred by UNHS programmes. Full article

Cisplatin is an established component of treatment protocols for various solid malignancies but carries a significant potential for serious adverse effects. Ototoxicity from cisplatin treatment is an important dose-limiting toxicity that manifests as bilateral, progressive, irreversible, dose-dependent sensorineural hearing loss, ear pain, tinnitus, and vestibular dysfunction. Despite the recent approval of sodium thiosulphate for the prevention of cisplatin-induced hearing loss (CIHL) in pediatric patients, structured prevention programs are not routinely implemented in most hospitals, and reducing platinum-induced ototoxicity in adults remains an important clinical problem without established treatment options. Cochlear oxidative stress plays a fundamental role in CIHL. Here, we review the molecular mechanisms leading to oxidative stress in CIHL and the clinical and preclinical studies testing antioxidants in CIHL to guide future clinical trials in assessing the efficacy and safety of candidate antioxidant compounds in this clinical setting. Full article

Background: Bilateral vestibulopathy (BVP) is a disabling condition characterized by a deficit in vestibular function on both sides. Current diagnostic criteria consider instrumental data only from horizontal canals, excluding vertical canals and otolithic function, with the possibility of not including some variants of BVP. This study aims to evaluate vestibular functions in people with chronic vestibular syndrome through a comprehensive battery of tests. Methods: This diagnostic accuracy study included patients who met criteria for probable BVP. The index test included a thorough evaluation of the vestibular system, using the video Head Impulse Test (vHIT) to measure the gain of the angular vestibulo-ocular reflex (aVOR) in all six semicircular canals and the cervical and ocular vestibular-evoked myogenic potentials (VEMPs) to assess otolith function. The diagnostic criteria established by the Barany Society were considered the standard reference, including only the horizontal vHIT as an instrumental assessment. Results: 78 patients (41 male, age 61.40 ± 12.99) were enrolled. The Barany criteria showed a low ability to rule out BPV (sensitivity = 46%). The median Dizziness Handicap Inventory (DHI) varied from 66 to 69 among the models studied, and a significant difference in DHI scores between positive and negative tests was observed for the Barany criteria and the six-canals vHIT model. Conclusions: Our findings highlight the potential to transform BPV diagnostic criteria. The identification of new bilateral vestibular dysfunction variants through improved diagnostic tools calls for revising current criteria, with promising implications for patient care and understanding of etiological and prognostic aspects. Full article

NF2-related schwannomatosis (NF2) is a genetic syndrome characterized by the growth of benign tumors in the nervous system, particularly bilateral vestibular schwannomas, meningiomas, and ependymomas. This review consolidates the current knowledge on NF2 syndrome, emphasizing the molecular pathology associated with the mutations in the gene of the same name, the NF2 gene, and the subsequent dysfunction of its product, the Merlin protein. Merlin, a tumor suppressor, integrates multiple signaling pathways that regulate cell contact, proliferation, and motility, thereby influencing tumor growth. The loss of Merlin disrupts these pathways, leading to tumorigenesis. We discuss the roles of another two proteins potentially associated with NF2 deficiency as well as Merlin: Yes-associated protein 1 (YAP), which may promote tumor growth, and Raf kinase inhibitory protein (RKIP), which appears to suppress tumor development. Additionally, this review discusses the efficacy of various treatments, such as molecular therapies that target specific pathways or inhibit neomorphic protein–protein interaction caused by NF2 deficiency. This overview not only expands on the fundamental understanding of NF2 pathophysiology but also explores the potential of novel therapeutic targets that affect the clinical approach to NF2 syndrome. Full article

Background: Progressive auditory dysfunction is common in patients with generalized neurodegenerative conditions, but clinicians currently lack the diagnostic tools to determine the location/degree of the pathology and, hence, to provide appropriate intervention. In this study, we present the white-matter microstructure measurements derived from a novel diffusion-weighted magnetic resonance imaging (dMRI) technique in a patient with axonal auditory neuropathy and consider the findings in relation to the auditory intervention outcomes. Methods: We tracked the hearing changes in an adolescent with Riboflavin Transporter Deficiency (Type 2), evaluating the sound detection/discrimination, auditory evoked potentials, and both structural- and diffusion-weighted MRI findings over a 3-year period. In addition, we explored the effect of bilateral cochlear implantation in this individual. Results: Between the ages of 15 years and 18 years, the patient showed a complete loss of functional hearing ability. The auditory brainstem response testing indicated an auditory neuropathy with evidence of normal cochlear function but disrupted auditory neural activity. While three structural MRI assessments across this period showed a clinically normal cochleovestibular anatomy, the dMRI evaluation revealed a significant loss of fiber density consistent with axonopathy. The subsequent cochlear implant function was affected with the high levels of current required to elicit auditory sensations and concomitant vestibular and facial nerve stimulation issues. Conclusions: The case study demonstrates the ability of dMRI technologies to identify the subtle white-matter microstructure changes in the auditory pathway, which may disrupt the neural function in patients with auditory axonopathy. Full article

NF2-related Schwannomatosis (NF2-SWN) is a disease that needs new solutions. The hallmark of NF2-SWN, a dominantly inherited neoplasia syndrome, is bilateral vestibular schwannomas (VSs), which progressively enlarge, leading to sensorineural hearing loss, tinnitus, facial weakness, and pain that translates to social impairment and clinical depression. Standard treatments for growing VSs include surgery and radiation therapy (RT); however, both carry the risk of further nerve damage that can result in deafness and facial palsy. The resultant suffering and debility, in combination with the paucity of therapeutic options, make the effective treatment of NF2-SWN a major unmet medical need. A better understanding of these mechanisms is essential to developing novel therapeutic targets to control tumor growth and improve patients’ quality of life. Previously, we developed the first orthotopic cerebellopontine angle mouse model of VSs, which faithfully mimics tumor-induced hearing loss. In this model, we observed that mice exhibit symptoms of ataxia and vestibular dysfunction. Therefore, we further developed a panel of five tests suitable for the mouse VS model and investigated how tumor growth and treatment affect gait, coordination, and motor function. Using this panel of ataxia tests, we demonstrated that both ataxia and motor function deteriorated concomitantly with tumor progression. We further demonstrated that (i) treatment with anti-VEGF resulted in tumor size reduction, mitigated ataxia, and improved rotarod performance; (ii) treatment with crizotinib stabilized tumor growth and led to improvements in both ataxia and rotarod performance; and (iii) treatment with losartan did not impact tumor growth nor ameliorate ataxia or motor function. Our studies demonstrated that these methods, paired with hearing tests, enable a comprehensive evaluation of tumor-induced neurological deficits and facilitate the assessment of the effectiveness of novel therapeutics to improve NF2 treatments. Full article

Migraine pathogenic pathways may selectively target the cochlea. A qualitative and quantitative analysis of cochlear symptoms in migraine patients without vestibular migraine and/or Méniere’s disease was conducted. We examined 60 consecutive patients with history of cochlear symptoms, including fullness, tinnitus, and hearing loss. Patients were divided into two groups based on migraine history: M (migraine) and nM (no migraine). The incidence of migraine was compared to a homogeneous control group with dysfunctional and inflammatory dysphonia without cochlear symptoms. The type, time of onset, recurrence, bilaterality of symptoms, and hearing threshold were analyzed. The incidence of migraine was significantly higher (p = 0.04) in patients with cochlear symptoms than in the control group. The onset of symptoms is significantly earlier (p < 0.05) in the presence of migraine. The fullness, recurrence, and bilaterality of symptoms are associated with migraine in a statistically significant way (p < 0.05). Pure tone audiometry shows a statistically significant increase in the hearing threshold (500–1000 Hz) in group M. Based on developing findings, cochlear migraine may be considered as a novel clinical entity, like vestibular migraine. It would be the expression, in the absence of vertiginous symptoms, of a selective suffering of the anterior labyrinth by known operating mechanisms of migraine. Full article

Background: The vestibulocerebellar tract (VCT) is responsible for maintaining balance, spatial orientation, and coordination. Damage to the vestibular system is accompanied by symptoms of balance disorder or ataxia. This study aimed to compare cerebellar dysfunction according to VCT damage in patients with cerebellar stroke. Methods: Six patients with cerebellum injury were recruited. This study measured ataxia and hand function related to visuomotor integration and manual dexterity using the Purdue pegboard test. The primary and bilateral secondary VCTs were reconstructed to investigate the integrity of pathways using diffusion tensor imaging (DTI). Results: The ataxia sign was positive in five patients (83%) at onset. In the result of the pegboard test, all patients had hand dysfunction in the dominant hand (100%). Likewise, all patients also had non-dominant hand dysfunction (100%). On the DTI tractography, the left and right primary VCTs of the patients demonstrated a 25% injury rate. Furthermore, the injury rates of ipsilateral and contralateral secondary VCTs were 50% and 58%. Conclusions: Ataxia is related to secondary VCTs, and hand dysfunction is also related to VCTs. Therefore, we believe that the current study will be helpful in evaluating and providing a clinical intervention strategy for patients with ataxia and hand dysfunction following cerebellar injury. Full article

(1) Background: Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is characterized by late-onset cerebellar ataxia, bilateral vestibulopathy, and sensory neuronopathy mostly due to biallelic RFC1 expansion. (2) Objectives: The aim of this case series is to describe vestibular, gait, and speech alterations in CANVAS via a systematic approach. (3) Methods: All patients (n = 5) underwent a standardized clinical–instrumental examination, including the perceptual and acoustic analysis of speech, instrumental gait, and balance analysis (posturographic data were acquired using a force plate [Kistler, Winterthur, Switzerland] while 3D gait analysis, inclusive of surface electromyography, was acquired using a motion capture system [SMART DX, BTS Bioengineering, Milan, Italy], a wireless electromyograph [FreeEMG, BTS Bioengineering, Milan, Italy]), and vestibular assessment with video-oculography. (4) Results: Five patients were included in the analysis: three females (patients A, B, C) and two males (patients D and E) with a mean age at evaluation of 62 years (SD ± 15.16, range 36–74). The mean age of symptoms’ onset was 55.6 years (SD ± 15.04, range 30–68), and patients were clinically and instrumentally evaluated with a mean disease duration of 6.4 years (SD ± 0.54, range 6–7). Video-Frenzel examination documented spontaneous downbeat nystagmus enhanced on bilateral gaze in all patients, except for one presenting with slight downbeat nystagmus in the supine position. All patients exhibited different degrees of symmetrically reduced VOR gain for allsix semicircular canals on the video-head impulse test and an unexpectedly normal (“false negative”) VOR suppression, consistent with combined cerebellar dysfunction and bilateral vestibular loss. Posturographic indices were outside their age-matched normative ranges in all patients, while 3D gait analysis highlighted a reduction in ankle dorsiflexion (limited forward rotation of the tibia over the stance foot during the stance phase of gait and fatigue of the dorsiflexor muscles) and variable out-of-phase activity of plantar flexors during the swing phase. Finally, perceptual-acoustic evaluation of speech showed ataxic dysarthria in three patients. Dysdiadochokinesis, rhythm instability, and irregularity were observed in the oral diadochokinesis task. (5) Conclusions: CANVAS is a recently discovered syndrome that is gaining more and more relevance within late-onset ataxias. In this paper, we aimed to contribute to a detailed description of its phenotype. Full article

Background: Children and adolescents with sensorineural hearing loss (SNHL) often experience motor skill disturbances, particularly in balance and gait, due to potential vestibular dysfunctions resulting from inner ear damage. Consequently, several studies have proposed the use of virtual reality-based games as a technological resource for therapeutic purposes, aiming to improve the balance and gait of this population. Objective: The objective of this systematic review is to evaluate the quality of evidence derived from randomized or quasi-randomized controlled trials that employed virtual reality-based games to enhance the balance and/or gait of children and adolescents with SNHL. Methods: A comprehensive search was conducted across nine databases, encompassing articles published in any language until 1 July 2023. The following inclusion criteria were applied: randomized or quasi-randomized controlled trials involving volunteers from both groups with a clinical diagnosis of bilateral SNHL, aged 6–19 years, devoid of physical, cognitive, or neurological deficits other than vestibular dysfunction, and utilizing virtual reality-based games as an intervention to improve balance and/or gait outcomes. Results: Initially, a total of 5984 articles were identified through the searches. Following the removal of duplicates and screening of titles and abstracts, eight studies remained for full reading, out of which three trials met the eligibility criteria for this systematic review. The included trials exhibited a very low quality of evidence concerning the balance outcome, and none of the trials evaluated gait. The meta-analysis did not reveal significant differences in balance improvement between the use of traditional balance exercises and virtual reality-based games for adolescents with SNHL (effect size: −0.48; [CI: −1.54 to 0.57]; p = 0.37; I² = 0%). Conclusion: Virtual reality-based games show promise as a potential technology to be included among the therapeutic options for rehabilitating the balance of children and adolescents with SNHL. However, given the methodological limitations of the trials and the overall low quality of evidence currently available on this topic, caution should be exercised when interpreting the results of the trials analyzed in this systematic review. Full article

(1) Background. Patients with bilateral vestibular disease (BVD) experience oscillopsia with a detriment to visual acuity (VA). This VA is driven mainly by the VOR that has two components: rotational and translational. VA can be tested by using dynamic visual acuity (DVA) on a treadmill because both systems are activated. The aim of this study is to compare VA before and after chronic electrical stimulation of the otolith organ. (2) Materials and Method. Five patients suffering from bilateral vestibular dysfunction (BVD), previously implanted with a new vestibular implant prototype, were included in this study with the aim to check VA with and without vestibular implant use (W and W/O) in static, 2 km/h and 4 km/h walking situations. DVAtreadmill was measured on a treadmill with a dynamic illegible E (DIE) test in static and dynamic conditions (while walking on the treadmill at 2 and 4 km/h). The DVA score was registered in a logarithm of the minimum angle of resolution (LogMAR) for each speed. In addition, every patient completed the oscillopsia severity questionnaire (OSQ) and video head impulse test (vHIT) before and after activation of the vestibular implant. (3) Results. The analysis shows a significant difference in OSQ scores and DVA with an improvement in dynamic conditions. Organized corrective saccades during the use of a vestibular implant with no changes in gain were also detected in the video head impulse tests (vHIT). (4) Conclusion. The vestibular implant with otolithic stimulation offers changes in the response of DVA, which makes this paper one of the first to address the possible restoration of it. It is not possible to rule out other contributing factors (presence of covert saccades, somatosensory system, …). More work seems necessary to understand the neurophysiological basis of these findings, but this implant is added as a therapeutic alternative for the improvement of oscillopsia. Full article