Search Results (12)

Long COVID-19 syndrome (or so-called post-COVID-19) is indicated by miscellaneous symptoms, usually starting 3 months from the COVID-19 infection and lasting for at least 2 months, which cannot be explained by an alternative diagnosis. There has been more and more reports addressing the audiovestibular dysfunction related to long COVID-19 syndrome. Emerging evidence suggests that the linkage between audiovestibular dysfunction and long COVID-19 syndrome might rely on (a) direct inner ear system damage related to viral invasion and consequent inflammation, (b) micro thromboembolic events, which might result from the COVID-19-induced autoimmune reaction against endothelial cells, and consequent transient-ischemia and hypoxia of the auditory pathways, (c) the disturbed nerve conduction in vestibulocochlear nerves due to viral invasion, and finally (d) altered auditory cortex function, either imbalanced central gain or neurotransmitter disturbance. However, most of the aforementioned mechanism remained hypothetic and still needed further studies to approve or refute. This systematic review synthesizes current evidence on the characteristics, pathophysiology, diagnostic approaches, and management of audiovestibular dysfunction related to long COVID-19 syndrome. Literature searches across PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect (up to 15 December 2025) were conducted in accordance with PRISMA guidelines. Through this systematic review, we provided a schematic diagram of the physiopathology of long COVID-19 syndrome-related audiovestibular dysfunction. Further, we summarized the currently available diagnostic tools to explore the audiovestibular function in such patients. The currently available treatment, either pharmacotherapy or nonpharmacotherapy, mainly tackles idiopathic audiovestibular dysfunction but not specifically long COVID-19 syndrome-related audiovestibular dysfunction. Timely recognition and intervention may prevent progression to permanent hearing loss or vestibular disability, improving quality of life. Trial registration: PROSPERO CRD420251265741. Full article

Background/Objectives: As precision medicine advances, attention to sex and gender determinants across epidemiological and clinical domains has intensified. However, in the audio-vestibular field, knowledge on sex- and gender-related aspects remains relatively limited. The main aim of this review has been to analyze the available gender medicine-based evidence in vestibular disorders. In particular, our investigation considered the following: (i) pathophysiology and clinical presentation, including differences in predominant signs and symptoms, diagnostic modalities and findings, underlying biological mechanisms associated with vestibular disorders across sex-specific groups; (ii) prognostic variables, including response to treatment, recovery rates, and long-term functional outcomes; (iii) the potential role of sex- and gender-specific diagnostic and therapeutic approaches in the management of vestibular disorders. Methods: Our protocol was registered on PROSPERO (CRD42025641292). A literature search was conducted screening PubMed, Scopus and Web of Science databases. After removal of duplicates and implementation of our inclusion/exclusion criteria, 67 included studies were identified and analyzed. Results: Several studies reported a higher incidence of vestibular dysfunctions among females, with proposed associations involving hormonal fluctuations, calcium metabolism and vitamin D. Estrogen receptors within the inner ear and their regulatory effects on calcium homeostasis have been proposed as potential mechanisms underlying these sex-specific differences. Furthermore, lifestyle factors, comorbidities and differential health-seeking behaviors between males and females may also modulate disease expression and clinical course. Conclusions: Gender-specific variables could not be independently analyzed because none of the included studies systematically reported gender-related data, representing a limitation of the available evidence. Current evidence suggests the presence of sex-related differences in the epidemiology and clinical expression of vestibular disorders, but substantial gaps remain regarding mechanisms, outcomes, and clinical implications. Future research should prioritize prospective, adequately powered studies specifically designed to assess sex and gender influences, integrating biological, psychosocial, and patient-reported outcomes, and adopting standardized sex- and gender-sensitive reporting frameworks. Full article

Hyper-IgE syndrome (HIES) is a rare genetic immunodeficiency characterized by elevated serum IgE levels and associated immune dysregulation, manifesting in recurrent infections, eczema, and skeletal abnormalities. Emerging evidence suggests a link between HIES and audiovestibular dysfunction, potentially mediated by IgE-driven inflammation in the inner ear, which is not immunologically privileged. However, the nature of this association remains poorly understood. This systematic review synthesizes current evidence on the characteristics, pathophysiology, diagnostic approaches, and management of audiovestibular dysfunction in HIES patients. Literature searches across PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect (up to 6 August 2025) were conducted in accordance with PRISMA guidelines. Key findings indicate that HIES-related audiovestibular issues, including sensorineural hearing loss and vestibular impairment, may arise from IgE-mediated endolymphatic sac inflammation, leading to hydrops and hair cell damage. Diagnostic tools such as audiometry, electrocochleography, and allergen challenge tests show promise, with elevated IgE correlating with abnormal otoacoustic emissions and prolonged auditory brainstem response latencies. Treatment focuses on immunomodulation (e.g., corticosteroids, dupilumab) to mitigate IgE effects, though evidence is limited to case reports. A proposed schematic diagram illustrates pathophysiology, emphasizing IgE’s role in inner ear toxicity. Timely recognition and intervention may prevent progression to permanent hearing loss or vestibular disability, improving quality of life. Future research should explore genetic–immunologic mechanisms and prospective trials for targeted therapies. Trial registration: PROSPERO CRD420251120600. 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

Although the inner ear is considered an immune-privileged organ because of the blood–labyrinth barrier, accumulating evidence has revealed an unexpected relation between Hashimoto’s disease and inner ear damage manifesting as audiovestibular dysfunction. Hashimoto’s disease can simultaneously affect both the auditory and vestibular systems, either through direct autoantibody attacks or through metabolic dysfunction associated with hypothyroidism. Currently, there is no consensus regarding tests or treatments for audiovestibular dysfunction related to Hashimoto’s disease. In this review, we summarize the currently available evidence regarding the characteristics, pathophysiology, diagnostic approaches, and treatment of audiovestibular dysfunction in patients with Hashimoto’s disease. Furthermore, we propose a specific steroid-plus-thyroxine treatment protocol to manage audiovestibular dysfunction associated with Hashimoto’s disease. This condition may respond to adequate treatment, potentially allowing reversibility if it is recognized and managed in a timely manner. Conversely, delayed diagnosis or failure to recognize the subtle presentation of audiovestibular dysfunction in patients with Hashimoto’s disease may lead to progressive hearing loss, immobility, and reduced quality of life. Based on the updated evidence in our review and our modified treatment protocol, we aim to provide new insights and therapeutic directions for clinicians managing audiovestibular dysfunction in patients with Hashimoto’s disease. Trial registration: PROSPERO CRD420250652982. Full article

Background: Anti-phospholipid syndrome (APS) has emerged as a significant issue in autoimmune diseases over recent decades. Its hallmark feature is thromboembolic events, potentially affecting any vascularized area including the microcirculation of the inner ear. Since the first case report of APS-related audiovestibular dysfunction described in 1993, numerous reports have explored the association between APS-related antibodies and audiovestibular dysfunction. These studies indicate a higher prevalence of APS-related antibodies in patients with sensorineural hearing loss compared to healthy controls. Unlike other idiopathic hearing loss disorders, audiovestibular dysfunction associated with APS may respond to appropriate treatments, highlighting the importance of timely recognition by clinicians to potentially achieve favorable outcomes. Therefore, this systematic review aims to consolidate current evidence on the characteristics, pathophysiology, assessment, and management of audiovestibular dysfunction linked to APS. Methods: This systematic review utilized electronic searches of the PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect online platforms. The initial search was performed on 27 January 2024, with the final update search completed on 20 June 2024. Results: Based on theoretical pathophysiology, anticoagulation emerges as a pivotal treatment strategy. Additionally, drawing from our preliminary data, we propose a modified protocol combining anticoagulants, steroids, and non-invasive brain stimulation to offer clinicians a novel therapeutic approach for managing these symptoms. Conclusions: Clinicians are encouraged to remain vigilant about the possibility of APS and its complex audiovestibular manifestations, as prompt intervention could stabilize audiovestibular function effectively. Full article

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. Full article

Background and Objectives: Granulomatosis with Polyangiitis (GPA) is a rare, autoimmune, multisystemic disease characterized by vasculitis and necrotizing granuloma that commonly affects the upper and lower respiratory tract and kidneys. Audiovestibular dysfunction in GPA diseases may have different clinical presentations. The aim of the present study was to evaluate hearing function in patients with GPA and to compare the results with a healthy control group. Materials and Methods: A total of 34 individuals participated in the study. The GPA group consisted of 14 participants, and the control group was composed of 20 healthy participants with no signs or symptoms of ear disease. The ages ranged from 18 to 65 years old, with a mean age of 43.8 years. The participants underwent a complete audiological evaluation using otoscopy, impedance audiometry, pure tone audiometry, speech audiometry—evaluation of speech thresholds, and speech recognition in quiet. Both ears were tested. All of the participants of the study were native Lithuanian speakers. Data were statistically analyzed using the Statistical Analysis System software SAS^® Studio 3.8. A p value < 0.05 was regarded as statistically significant. Results: 92.85% of patients from the GPA group reported hearing-related symptoms: hearing loss, tinnitus, and fullness in the ears. The arithmetic means of all hearing thresholds at frequencies from 125 Hz to 8000 Hz were significantly higher in the GPA group. The results revealed statistically significant differences between the two groups in the Speech Detection Threshold, Speech Recognition Threshold, Speech Discomfort level, and Word Recognition Scores. Conclusions: The frequency of hearing loss, the average hearing thresholds, and speech thresholds were higher in GPA patients than in healthy individuals. The most common type of hearing loss was sensorineural. Audiological assessments should be considered during the routine evaluation of patients with GPA disease to prevent hearing-related disabilities. Full article

Audio-vestibular symptoms can arise from vertebrobasilar dolichoectasia (VBD) and basilar dolichoectasia (BD). Given the dearth of available information, herein we reported our experience with different audio-vestibular disorders (AVDs) observed in a case series of VBD patients. Furthermore, a literature review analyzed the possible relationships between epidemiological, clinical, and neuroradiological findings and audiological prognosis. The electronic archive of our audiological tertiary referral center was screened. All identified patients had a diagnosis of VBD/BD according to Smoker’s criteria and a comprehensive audiological evaluation. PubMed and Scopus databases were searched for inherent papers published from 1 January 2000 to 1 March 2023. Three subjects were found; all of them had high blood pressure, and only the patient with high-grade VBD showed progressive sensorineural hearing loss (SNHL). Seven original studies were retrieved from the literature, overall including 90 cases. AVDs were more common in males and present in late adulthood (mean age 65 years, range 37–71), with symptoms including progressive and sudden SNHL, tinnitus, and vertigo. Diagnosis was made using different audiological and vestibular tests and cerebral MRI. Management was hearing aid fitting and long-term follow-up, with only one case of microvascular decompression surgery. The mechanism by which VBD and BD can cause AVD is debated, with the main hypothesis being VIII cranial nerve compression and vascular impairment. Our reported cases suggested the possibility of central auditory dysfunction of retro-cochlear origin due to VBD, followed by rapidly progressing SNHL and/or unnoticed sudden SNHL. More research is needed to better understand this audiological entity and achieve an evidence-based effective treatment. Full article

The aim of this study is to contribute to a better description of the genotypic and phenotypic spectrum of DFNA6/14/38 and aid in counseling future patients identified with this variant. Therefore, we describe the genotype and phenotype in a large Dutch–German family (W21-1472) with autosomal dominant non-syndromic, low-frequency sensorineural hearing loss (LFSNHL). Exome sequencing and targeted analysis of a hearing impairment gene panel were used to genetically screen the proband. Co-segregation of the identified variant with hearing loss was assessed by Sanger sequencing. The phenotypic evaluation consisted of anamnesis, clinical questionnaires, physical examination and examination of audiovestibular function. A novel likely pathogenic WFS1 variant (NM_006005.3:c.2512C>T p.(Pro838Ser)) was identified in the proband and found to co-segregate with LFSNHL, characteristic of DFNA6/14/38, in this family. The self-reported age of onset of hearing loss (HL) ranged from congenital to 50 years of age. In the young subjects, HL was demonstrated in early childhood. At all ages, an LFSNHL (0.25–2 kHz) of about 50–60 decibel hearing level (dB HL) was observed. HL in the higher frequencies showed inter-individual variability. The dizziness handicap inventory (DHI) was completed by eight affected subjects and indicated a moderate handicap in two of them (aged 77 and 70). Vestibular examinations (n = 4) showed abnormalities, particularly in otolith function. In conclusion, we identified a novel WFS1 variant that co-segregates with DFNA6/14/38 in this family. We found indications of mild vestibular dysfunction, although it is uncertain whether this is related to the identified WFS1 variant or is an incidental finding. We would like to emphasize that conventional neonatal hearing screening programs are not sensitive to HL in DFNA6/14/38 patients, because high-frequency hearing thresholds are initially preserved. Therefore, we suggest screening newborns in DFNA6/14/38 families with more frequency-specific methods. Full article

Background: The world’s age-related health concerns continue to rise. Audio-vestibular disorders, such as hearing loss, tinnitus, and vertigo, are common complaints in the elderly and are associated with social and public health burdens. Various preventative measures can ease their impact, including healthy food consumption, nutritional supplementation, and lifestyle modification. We aim to provide a comprehensive summary of current possible strategies for preventing the age-related audio-vestibular dysfunction. Methods: A PubMed, Embase, and Cochrane review databases search was conducted to identify the relationship between diet, lifestyle, and audio-vestibular dysfunction. “Diet”, “nutritional supplement”, “lifestyle”, “exercise”, “physical activity”, “tinnitus”, “vertigo” and “age-related hearing loss” were used as keywords. Results: Audio-vestibular dysfunction develops and progresses as a result of age-related inflammation and oxidative stress. Diets with anti-inflammatory and antioxidant effects have been proposed to alleviate this illness. A high-fat diet may induce oxidative stress and low protein intake is associated with hearing discomfort in the elderly. Increased carbohydrate and sugar intake positively correlate with the incidence of audio-vestibular dysfunction, whereas a Mediterranean-style diet can protect against the disease. Antioxidants in the form of vitamins A, C, and E; physical activity; good sleep quality; smoking cessation; moderate alcohol consumption; and avoiding noise exposure are also beneficial. Conclusions: Adequate diet or nutritional interventions with lifestyle modification may protect against developing audio-vestibular dysfunction in elderly individuals. Full article

Pathogenic missense variants in COCH are associated with DFNA9, an autosomal dominantly inherited type of progressive sensorineural hearing loss with or without vestibular dysfunction. This study is a comprehensive overview of genotype-phenotype correlations using the PRISMA and HuGENet guidelines. Study characteristics, risk of bias, genotyping and data on the self-reported age of onset, symptoms of vestibular dysfunction, normative test results for vestibular function, and results of audiovestibular examinations were extracted for each underlying pathogenic COCH variant. The literature search yielded 48 studies describing the audiovestibular phenotypes of 27 DFNA9-associated variants in COCH. Subsequently, meta-analysis of audiometric data was performed by constructing age-related typical audiograms and by performing non-linear regression analyses on the age of onset and progression of hearing loss. Significant differences were found between the calculated ages of onset and progression of the audiovestibular phenotypes of subjects with pathogenic variants affecting either the LCCL domain of cochlin or the vWFA2 and Ivd1 domains. We conclude that the audiovestibular phenotypes associated with DFNA9 are highly variable. Variants affecting the LCCL domain of cochlin generally lead to more progression of hearing loss when compared to variants affecting the other domains. This review serves as a reference for prospective natural history studies in anticipation of mutation-specific therapeutic interventions. Full article