Search Results (45)

The etiology of idiopathic sudden sensorineural hearing loss (iSSNHL) remains unclear, and genome-wide genetic evidence is limited. We conducted a multicenter Japanese case–control genome-wide association study including 192 clinically defined iSSNHL cases and 15,302 controls aged ≥80 years without a history of hearing loss. After cross-platform SNP harmonization and imputation (Eagle/Minimac4), association testing was performed using dosage-based logistic regression in PLINK 2.0, adjusting for sex and principal components (PC1–PC10). Gene- and pathway-level analyses were conducted using MAGMA and the PANTHER overrepresentation test. Genomic inflation was modest (λ_GC = 1.04). Eight loci reached genome-wide significance (p < 5 × 10⁻⁸), led by FHIT, with additional loci near LHX2, TRMT1L, MEGF10, SPATS1, SAMD5, MYT1L, and ID4; 21 loci met the suggestive threshold (p < 1 × 10⁻⁶). MAGMA identified eight genes at FDR < 0.05 (FHIT, TRMT1L, MEGF10, RNF2, SWT1, VAMP1, TAPBPL, and C9orf3). These findings suggest that immune-inflammatory and cellular stress–homeostasis mechanisms may contribute to iSSNHL susceptibility and provide candidate loci for future replication and functional studies. Full article

Objectives: Patients with severe to profound sudden sensorineural hearing loss (SSNHL) generally experience poorer hearing recovery; however, the associated risk factors have not been identified. This study synthesizes current evidence to explore prognostic risk factors in this patient group. Methods: Databases were systematically searched through PubMed, Embase, Web of Science, and the Cochrane Library, from their inception to 18 October 2025. Three researchers independently extracted and recorded patient information and relevant data from all selected studies. Any inconsistencies were clarified through discussion or by consulting a fourth researcher. Results: The study included 2632 patients from 15 articles published between 2002 and 2025 and evaluated 8 prognostic risk factors. The results showed that profound hearing loss (OR = 4.68; 95% CI: 3.57–6.13; p < 0.001) and vertigo (OR = 1.95; 95% CI: 1.28–2.98; p = 0.002) were correlated with poorer hearing recovery. Subgroup analyses based on different prognostic criteria confirmed the consistent impact of hearing loss severity on poor outcomes. The remaining 6 risk factors did not show statistically meaningful associations. Conclusions: Profound hearing loss and vertigo are significantly associated with poorer prognosis in patients with severe to profound SSNHL. These findings may help identify high-risk patients early and inform the design of personalized therapeutic approaches in clinical settings. Full article

Background/Objectives: Sudden sensorineural hearing loss (SSNHL) represents a challenging clinical entity with variable prognosis. Audiometric curve configuration has been proposed as a predictor of recovery. This study aimed to evaluate the association between audiogram morphology at onset and hearing outcome in patients with idiopathic unilateral SSNHL treated with standardized therapy. Methods: We retrospectively analyzed 156 patients with idiopathic SSNHL. Hearing thresholds at key frequencies were measured at baseline and 4 weeks post-treatment. Patients were categorized into upsloping, flat, downsloping, or U-shaped audiogram subgroups. Recovery was classified into four levels. Comparisons were made across subgroups for audiometric and laboratory data using ANOVA and chi-square tests. Results: Baseline PTA values were comparable across audiogram subgroups (p = 0.12). Hearing recovery differed significantly according to audiogram configuration (chi-square, p < 0.001), with upsloping and U-shaped patterns showing the best outcomes. Flat and downsloping curves were associated with poorer recovery, lower HDL, and elevated NLR values. Conclusions: Audiogram configuration is a relevant prognostic marker in SSNHL. Patterns linked to adverse metabolic and inflammatory profiles may benefit from tailored treatment strategies in a personalized medicine framework. Full article

Acute vertigo or dizziness that is accompanied by a sudden sensorineural hearing loss (SSNHL) often poses a diagnostic challenge. While a combined audiovestibular deficit makes an inner ear pathology most likely, this does not necessarily exclude a vascular pathology that may be a harbinger of future sinister events. This is especially true for strokes within the territory of the anterior inferior cerebellar artery (AICA), because the labyrinth receives its vascular supply most often by branches of the AICA. Thus, acute labyrinthine ischemia may present in combination with focal neurologic deficits, but also in isolation or as a warning sign before focal stroke signs arise. How can labyrinthine ischemia be differentiated from an idiopathic SSNHL? In this critical review, we discuss both the pathophysiology and the differential diagnosis of acute audiovestibular deficits. We will also address the value of state-of-the-art MR imaging in visualizing labyrinthine ischemia. Finally, we will discuss treatment options and review the prognosis of acute audiovestibular deficits. Full article

Background/Objectives: Sudden sensorineural hearing loss (SSNHL) is an acute condition with unclear etiology, commonly hypothesized to be associated with viral infections. Acute respiratory tract infections (RTIs), particularly those of viral origin, have been implicated in SSNHL through proposed mechanisms such as cochlear invasion and immune-mediated damage. However, robust large-scale epidemiological evidence examining this association remains limited. This study aimed to investigate the potential association between acute RTIs and subsequent risk of developing SSNHL across diverse populations. Methods: We conducted a multinational retrospective cohort study using data from the TriNetX Global Collaborative Network. Adults diagnosed with acute RTIs between 1 January 2012 and 30 June 2023 were compared to matched controls without RTI exposure. Patients with predisposing conditions for SSNHL were excluded. Propensity score matching (1:1) was performed by age and sex. SSNHL diagnoses within 60 days post index were analyzed using Cox proportional hazards models. Subgroup and sensitivity analyses were conducted by race, sex, and age strata. Results: Among 37 million patients analyzed, individuals with acute RTIs had a lower incidence of SSNHL compared to matched controls. Hazard ratios (HRs) for SSNHL were significantly reduced across all racial groups: Whites (HR: 0.572), Blacks (HR: 0.563), and Asians (HR: 0.409). Subgroup analyses revealed stronger inverse associations in males and younger age groups, particularly those aged 18–25 years. Conclusions: Contrary to prior assumptions, acute RTIs were associated with a lower incidence of SSNHL in a large, diverse cohort. While the findings raise the possibility of immunological or physiological factors influencing this association, the results should be interpreted with caution due to unmeasured confounding and the observational nature of the study. Full article

Objectives: This study assessed the potential benefits of supplemental flunarizine administration in patients with sudden sensorineural hearing loss (SSNHL) experiencing incomplete recovery with conventional steroid treatment. Methods: Thirty-nine SSNHL patients, unresponsive to conventional steroid therapy, received either oral ginkgo biloba (control group, n = 24) or a combination of oral ginkgo biloba and flunarizine (treatment group, n = 15). Pure-tone average (PTA) evaluation at 0.5, 1, 2, and 4 kHz was conducted upon each patient’s visits. A change of ≥10 dB was considered ‘significant hearing gain’. Results: The treatment group showed a higher rate of hearing gain ≥10 dB (46.7%) compared to the control group (12.5%) (p = 0.03). Additional treatment with flunarizine was associated with greater improvement in hearing thresholds compared with ginkgo biloba alone (p = 0.004), suggesting a potential therapeutic benefit. Conclusion: This retrospective study suggests that flunarizine may provide additional benefit in patients with SSNHL experiencing incomplete recovery following conventional steroid treatment. These findings are preliminary and require validation in larger, controlled studies. Full article

Background: Gout, characterized by serum uric acid accumulation, prompts inflammation, leading to tissue damage and comorbidities. Prior studies reported a higher risk of hearing loss in gout patients; however, the specific risk of sudden sensorineural hearing loss (SSNHL) remains unclear. Method: This population-based study assessed SSNHL incidence and risk in patients aged ≥ 40 years with or without gout, excluding those with prior SSNHL, within the Korean National Health Insurance Service Health Screening Cohort (2002–2019). A total of 24,508 gout patients were matched 1:4 with 98,032 controls by age, sex, income, and region. SSNHL incidence was compared, and Kaplan–Meier curves with log-rank tests evaluated cumulative incidence over 200 months. Hazard ratios (HRs) were calculated using stratified Cox models adjusted for patient characteristics. Results: SSNHL incidence was slightly higher in the gout cohort vs. controls (1.70% vs. 1.96%, SD = 0.02). Kaplan–Meier analysis revealed significantly higher cumulative SSNHL incidence in gout patients (p = 0.009). Patients with gout had a significantly higher risk of developing SSNHL in both the unadjusted (HR [95% CI]: 1.14 [1.03–1.27]; p = 0.010) and adjusted Cox models (1.13 [1.02–1.26]; p = 0.021). Subgroup analyses indicated higher risk in gout patients aged <60 years, males, non-smokers, non-drinkers, moderately-high income, normal BMI, Charlson Comorbidity Index score of 0, or fasting blood glucose < 100 mg/dL (all p < 0.05). Conclusions: Korean adults with gout, particularly younger, healthier patients, face increased SSNHL risk. Early, effective gout management may help mitigate this risk. Full article

Sudden sensorineural hearing loss (SSNHL) is a rapidly developing acquired idiopathic disorder. Differential expressions of microRNAs (miRNAs) have been identified in the acute serum of SSNHL patients. miRNAs are transmitted in both serum and plasma, but it is unknown which better reflects changes associated with inner ear disease. Therefore, we sought to compare the serum and plasma miRNA expression levels in adult SSNHL patients. We extracted and reverse transcribed total RNA from serum and plasma, and analyzed the product with quantitative real-time PCR. hsa-miR-191-5p was used for normalization, and miRNA expression levels were calculated using the delta Ct method. Serum and plasma samples from 17 SSNHL patients (mean age 51.9 years, standard deviation 13.9 years) showed no significant differences in miR-128-3p, miR-132-3p, miR-375-3p, miR-590-5p, miR-30a-3p, miR-140-3p, miR-186-5p, and miR-195-5p expression levels on Wilcoxon signed-rank test analyses. We conclude that plasma and serum are equally suitable for investigating potential miRNA SSNHL disease markers. Full article

Introduction: Based on clinical practice guidelines, the application of corticosteroids as a first-line therapy is common. Although sudden sensorineural hearing loss (SSHL) etiology is primarily idiopathic, hearing loss can result from a perilymphatic fistula (PLF). Recent findings show evidence of a specific rate of PLF based on a cochlin-tomoprotein (CTP) detection test. Based on this rate of PLF treatment, the concepts of SSNHL need to be re-evaluated. The present study aimed to evaluate CTP in SSNHL patients and compare pre-surgical and post-surgical pure tone hearing thresholds after round and oval window sealing as first-line treatment. Material and Methods: A total of 30 patients with unilateral SSNHL with a pure tone average (PTA) (4 Freq. of 60 dB or more were enrolled in a prospective study. All patients underwent tympanoscopy for middle ear exploration as a first-line treatment. After intraoperative observation of a possible PLF, all patients obtained middle ear lavage to gain CTP samples for following ELISA-based CTP detection tests. All patients received round window and oval window sealing with fascia. PTA hearing thresholds were analyzed post-surgically 3 weeks after treatment based on 4-frequency bone conduction (BC). Results: The average preoperative pure tone BC threshold was 97.7 dB compared with the 69 dB postoperative BC threshold. Mean BC improved by 20.3 dB after middle ear exploration and window sealing. A total of 56% (17 of 30) of patients recovered at least 10 dB. The middle ear cochlin-tomoprotein detection rate was 70% positive. Conclusions: The combination of early tympanoscopy and inner ear-specific cochlin-tomoprotein as a detection tool for suspected PLF showed evidence of PLF as a key causative in SSNHL. Full article

Background: While numerous studies have been conducted on sudden sensorineural hearing loss (SSNHL), research on how treatment outcomes and prognosis vary by age remains insufficient. This study aims to investigate the differences in recovery rates among SSNHL patients divided by age groups. Methods: A retrospective study was conducted on 177 patients diagnosed with SSNHL from 2020 to 2023. The patients were categorized into four age groups: under 20, 30–40, 50–60, and over 70. Recovery rates and associated symptoms were compared across these groups. Results: Significant recovery rates were observed in all age groups except for those over 70 (p = 0.006, p = 0.003, p = 0.009). No significant differences were found in recovery rates based on gender (p > 0.75) or symptoms such as tinnitus, ear fullness, and dizziness (p > 0.05). Conclusions: The study revealed that younger and middle-aged adults showed statistically significant improvements in recovery rates, while the elderly exhibited relatively lower recovery rates. Full article

Objectives: The purpose of this study was to investigate the hearing characteristics and causes of sudden sensorineural hearing loss (SSNHL) in patients aged from 15 to 40 years, focusing on audiological outcomes one year after the diagnosis. Methods: The medical records of individuals with SSNHL who were referred to our tertiary-level audiologic center were reviewed. All patients had undergone comprehensive diagnostic evaluations, including high-resolution 3D-FLAIR delayed magnetic resonance imaging (MRI), cone beam computed tomography (CBCT), and screening for coagulation, infectious, and autoimmune diseases. Results: Overall, 56 patients (mean age 28.1 ± 7.6 years) were included in the study. The hearing threshold in the affected ear improved significantly from 56.0 ± 18.0 dB at the diagnosis to 46.9 ± 22.3 dB after one year (p = 0.02). The degree of hearing loss, audiometric configurations, hearing improvements, and adherence to hearing treatments showed considerable variability among patients. Aural fullness, tinnitus, and hyperacusis were the predominant symptoms associated with SSNHL, and their prevalence decreased significantly over time. The diagnostic protocol led to the identification of the specific cause of SSNHL in 75% (42/56) of patients. The known etiology was found to be otological (39.3%), infectious (21.4%), autoimmune (7.1%), vascular (5.4%), or neoplastic (1.8%). In particular, Menière’s disease (n = 12), isolated cochlear endolymphatic hydrops (n = 6), HSV-1 (n = 5), and EBV (n = 4) infections were the most frequent causes of SSNHL. Conclusions: The identification of the specific etiology of SSNHL may facilitate a more personalized approach to management and treatment. Full article

Objective: To compare white matter hyperintensities (WMHs) on T2-weighted magnetic resonance imaging (MRI) of patients with sudden sensorineural hearing loss (SSNHL) and analyze subpopulations with age-matched controls. Methods: T2-weighted MRI scans of 150 patients with SSNHL were assessed for WMHs and compared with the data of 148 healthy age-matched adults. Assessments of WMHs included independent grading of deep white matter hyperintensities (DWMHs) and periventricular hyperintensities (PVHs). WMH severity was visually rated using the Fazekas and Mirsen scales by two independent observers. Results: Fazekas grades for PVHs (p < 0.001) and DWMHs (p < 0.001) of SSNHL patients were found to be significantly greater than those of healthy participants. The average Mirsen grades for DWMHs of healthy and SSNHL patients were evaluated to be 0.373 ± 0.550 and 2.140 ± 0.859, respectively. Mirsen grades for DWMHs of SSNHL patients were found to be significantly greater (p < 0.001) than those of healthy participants. The Mirsen scale was found to have higher sensitivity (p < 0.001) than the Fazekas scale in grading PVHs and DWMHs. No significant difference (p = 0.24) was found in specificities between the two scales. Conclusions: Patients with sudden hearing loss have a much higher likelihood of having periventricular and deep white matter hyperintensities compared to age-matched controls. These findings indicate that sudden hearing loss patients are more likely to have microvascular changes in the brain, which may indicate a vascular and/or migraine origin to sudden sensorineural hearing loss. Full article

Introduction: Hyperbaric oxygen therapy (HBOT) is one of the treatment methods in patients with sudden sensorineural hearing loss (SSNHL). It is recommended as an elective treatment in patients undergoing steroid therapy. According to current scientific reports, HBOT should be implemented within two weeks after the first symptoms. However, as far as the profile of HBOT is concerned, there are no straightforward recommendations. Methods: The data obtained from the medical records of 218 patients undergoing HBOT for SSNHL at the Military Institute of Medicine—National Research Institute were analyzed statistically for the impact of the duration and the delay in implementing HBOT on the end results of pure-tone audiometry (PTA). Results: A statistically significant hearing improvement in patients undergoing more than 15 cycles of HBOT was detected at all frequencies except for 1500 Hz; in the group reporting for treatment with a delay of more than 10 days, hearing improvement was statistically unsignificant at frequencies of 1500, 3000, and 4000 Hz. Conclusions: The statistical analysis showed that the urgent onset of HBOT could be a significant factor in the therapy of SSNHL. Full article

Sudden sensorineural hearing loss (SSNHL), a rare audiological condition that accounts for 1% of all cases of sensorineural hearing loss, can cause permanent hearing damage. Soon after the launch of global COVID-19 vaccination campaigns, the World Health Organization released a signal detection about SSNHL cases following administration of various COVID-19 vaccines. Post-marketing studies have been conducted in different countries using either pharmacovigilance or medico-administrative databases to investigate SSNHL as a potential adverse effect of COVID-19 vaccines. Here, we examine the advantages and limitations of each type of post-marketing study available. While pharmacoepidemiological studies highlight the potential association between drug exposure and the event, pharmacovigilance approaches enable causality assessment. The latter objective can only be achieved if an expert evaluation is provided using internationally validated diagnostic criteria. For a rare adverse event such as SSNHL, case information and quantification of hearing loss are mandatory for assessing seriousness, severity, delay onset, differential diagnoses, corrective treatment, recovery, as well as functional sequelae. Appropriate methodology should be adopted depending on whether the target objective is to assess a global or individual risk. Full article

In multiple sclerosis (MS), the occurrence of sudden sensorineural hearing loss (SSNHL) is considered rare, with reported cases predominantly being unilateral. Bilateral cases are even rarer. Here, we report a case of bilateral SSNHL in a 20-year-old male diagnosed with MS. The patient, undergoing corticosteroid therapy for the management of MS, additionally received an intratympanic dexamethasone injection; however, it could not achieve significant improvement. Subsequently, the systemic dosage was increased for one week, resulting in substantial hearing improvement in both ears after three months. A review of MS-related SSNHL cases from 1987 to 2022 revealed 39 ears in the literature, with only five ears showing no hearing recovery. A remarkable 87.2% exhibited restored hearing, presenting a more favorable prognosis compared with idiopathic SSNHL. Although there were slight variations in administration methods and duration, all documented treatment approaches involve systemic corticosteroids. In some instances, SSNHL manifested as the initial symptom of MS. When SSNHL occurs in MS, auditory brainstem response (ABR) tests may reveal prolonged abnormalities, making ABR testing effective in cases where MS is suspected following SSNHL. In conclusion, the treatment of MS-related SSNHL appears appropriate with systemic corticosteroids, showing a significantly superior prognosis compared with idiopathic SSNHL. Full article