OBJECTIVE: Safety and efficacy of SENS-401, a serotonin type 3 (5-HT3) receptor antagonist and calcineurin inhibitor, in patients with acute sudden sensorineural hearing loss (SSNHL).
METHODS: Multicentre randomized, double blind, placebo-controlled trial enrolled adult subjects with sudden sensorineural hearing loss (SSNHL) or unilateral/bilateral acute acoustic trauma leading to SSNHL within 96 h of disease onset. Subjects were randomly assigned to one of the three oral dose groups: 29 mg, 43.5 mg or placebo given twice daily for 28 days. The primary endpoint was the change from baseline in Pure Tone Average (PTA) in the affected ear to the end of treatment visit (day 28). Subjects were further followed up 8 weeks after the end of the treatment period (day 84).
RESULTS: A total of 115 subjects were randomized. SENS-401 was well tolerated. Although the primary efficacy endpoint was not met at day 28, post-hoc analyses revealed clinically significant and meaningful efficacy outcomes with SENS-401 when compared to placebo in a substantial group of participants diagnosed with idiopathic SSNHL and who had received corticosteroid treatment. Notable improvements were observed in the PTA change from baseline, the complete hearing recovery rate, and the Word Recognition Score (WRS), particularly at day 84. The responder rate consistently favored treated subjects over those who received the placebo.
CONCLUSIONS: While the primary endpoint was not achieved at the end of the treatment period, the study revealed consistently positive efficacy results of clinical relevance in patients with idiopathic SSNHL who received SENS-401, particularly in the 8-weeks follow-up phase after the completion of the treatment.

BACKGROUND: ISSNHL, a common clinical condition, can be accompanied by vertigo. Initially, research on sudden deafness primarily focused on the hearing loss itself, with less emphasis on episodic vertigo. However, as vertigo research has advanced, it has been recognized that BPPV is a frequent accompaniment to ISSNHL-associated vertigo. Even after treatment, some patients may experience residual dizziness. This study investigates the characteristics of patients with ISSNHL accompanied by BPPV and the impact of residual dizziness on their lives.
METHODS: This study is being conducted on patients with ISSNHL accompanied by BPPV, analyzing the characteristics of such patients and the impact of residual dizziness on their lives. Overall, 54 adult inpatients with ISSNHL and BPPV were included in this study. All patients received 50 mg of intravenous prednisolone for 5 consecutive days and hemodilution agents for 10 days. At the same time, BPPV was treated with repositioning by the same therapist using the SRM-IV vertigo diagnostic and treatment system, and different repositioning methods were used for different types of otolithiasis. Patients were grouped according to the absence of residual dizziness when the nystagmus disappeared at the time of discharge.
RESULTS: There were 24 cases in the group with residual symptoms, including 10 males and 14 females. The proportion of females was 58.33%, with an average age of 46.75 ± 13.80. The group without residual symptoms consisted of 30 cases, including 13 males and 17 females. The female proportion was 56.67%, with an average age of 45.77 ± 11.86. There is no statistical significance between the two groups in the pre-treatment hearing status and DHI scores. The HAMA (Hamilton Anxiety Rating Scale) scores before treatment were compared, revealing a significant statistical difference.
CONCLUSIONS: ISSNHL-associated BPPV may be caused by vascular embolism or thrombosis in the cochlear or spiral modiolar artery. This disrupts blood flow, leading to ischemia in the otolithic membrane and subsequent detachment of otoconia. Because this detachment often occurs within 24 h of the initial event, patients experience positional vertigo early in the course of the disease.

OBJECTIVE: To provide pooled analyses on the association between COVID-19 vaccine and the incidence of idiopathic sudden sensorineural hearing loss (ISSNHL).
METHODS: \"Medline\" via \"PubMed\", \"EMBASE\", and \"Google scholar\".
METHODS: Data sources were inspected from January 2020 to January 2024 using search terms relevant to vaccines for COVID-19. Included were papers with reported numbers of vaccinated populations and incidence if ISSNHL in those populations. Quality assessment was performed with the Newcastle-Ottawa Quality Assessment Scale Criteria.
RESULTS: Three publications encompassing more than 191.8 million patients and at least 283 million vaccine doses were included in the quantitative data synthesis. The pooled reported incidence (95%confidence interval) of ISSNHL among COVID-19 vaccine recipients was 1.2588 per 100,000 (0.1385-3.4836). This incidence is significantly lower than the incidence of 5-27 and 60 per 100,000/year reported in the United States and in the European Union, respectively (P < 0.0001).
CONCLUSIONS: There is no evidence to indicate that the COVID-19 vaccine is associated with the incidence of ISSNHL.

OBJECTIVE: Acute sensorineural hearing loss represents a spectrum of conditions characterized by sudden onset hearing loss. The \"Clinical Practice Guidelines for the Diagnosis and Management of Acute Sensorineural Hearing Loss\" were issued as the first clinical practice guidelines in Japan outlining the standard diagnosis and treatment. The purpose of this article is to strengthen the guidelines by adding the scientific evidence including a systematic review of the latest publications, and to widely introduce the current treatment options based on the scientific evidence.
METHODS: The clinical practice guidelines were completed by 1) retrospective data analysis (using nationwide survey data), 2) systematic literature review, and 3) selected clinical questions (CQs). Additional systematic review of each disease was performed to strengthen the scientific evidence of the diagnosis and treatment in the guidelines.
RESULTS: Based on the nationwide survey results and the systematic literature review summary, the standard diagnosis flowchart and treatment options, including the CQs and recommendations, were determined.
CONCLUSIONS: The guidelines present a summary of the standard approaches for the diagnosis and treatment of acute sensorineural hearing loss. We hope that these guidelines will be used in medical practice and that they will initiate further research.

Idiopathic Sudden Sensorineural Hearing Loss (ISSHL) is a sudden onset, unexplained sensorineural hearing loss. Depression is a common mental disorder and a leading cause of disability. Here, We used a two-sample Mendelian randomization approach using pooled statistics from genome-wide association studies of ISSHL (1491 cases, 196,592 controls) and depression (23,424 cases, 192,220 controls) in European populations. This study investigated the bidirectional relationship between single nucleotide polymorphisms associated with depression and ISSHL using inverse variance weighting.Additional sensitivity analyses, such as Mendelian randomization-Egger (MR-Egger), weighted median estimates, and leave-one-out analysis, were performed to assess the reliability of the findings. Significant causal association between genetic susceptibility to ISSHL and depression in a random-effects IVW approach (OR = 1.037, 95% CI = 1.004-1.072, P = 0.030). In contrast, genetic depression was not risk factors for ISSHL (OR = 1.134, 95% CI = 0.871-1.475, P = 0.350). After validation by different MR methods and the sensitivity analysis, all of the above results are consistent. The evidence we have gathered suggests a causal relationship between ISSHL and depression. The presence of the former induces or further exacerbates the latter, whereas a similar situation does not exist when the latter is an influencing factor.

BACKGROUND: Accurate prognostic prediction is crucial for managing Idiopathic Sudden Sensorineural Hearing Loss (ISSHL). Previous studies developing ISSHL prognosis models often overlooked individual variability in hearing damage by relying on fixed frequency domains. This study aims to develop models predicting ISSHL prognosis one month after treatment, focusing on patient-specific hearing impairments.
METHODS: Patient-Personalized Seigel\'s Criteria (PPSC) were developed considering patient-specific hearing impairment related to ISSHL criteria. We performed a statistical test to assess the shift in the recovery assessment when applying PPSC. The utilized dataset of 581 patients comprised demographic information, health records, laboratory testing, onset and treatment, and hearing levels. To reduce the model\'s reliance on hearing level features, we used only the averages of hearing levels of the impaired frequencies. Then, model development, evaluation, and interpretation proceeded.
RESULTS: The chi-square test (p-value: 0.106) indicated that the shift in recovery assessment is not statistically significant. The soft-voting ensemble model was most effective, achieving an Area Under the Receiver Operating Characteristic Curve (AUROC) of 0.864 (95% CI: 0.801-0.927), with model interpretation based on the SHapley Additive exPlanations value.
CONCLUSIONS: With PPSC, providing a hearing assessment comparable to traditional Seigel\'s criteria, the developed models successfully predicted ISSHL recovery one month post-treatment by considering patient-specific impairments.

OBJECTIVE: Although corticosteroids and other treatments for idiopathic sudden sensorineural hearing loss (ISSNHL) have been described, understanding its prognosis without intervention provides valuable information for patient management. The objective of this study is to provide a comprehensive, quantitative statistical analysis of the natural history of untreated idiopathic sudden sensorineural hearing loss (ISSNHL).
UNASSIGNED: A systematic review and meta-analyses.
METHODS: Two independent searches of PubMed, Scopus, Web of Science, and Cochrane Library databases up to June 30, 2022, were performed. Adults with idiopathic sudden sensorineural hearing loss who received placebo or were untreated and had audiometric outcome measures in all study types were reviewed. These data indicative of the natural history of ISSNHL were analyzed, as were study characteristics related to risk of bias. Heterogeneity as assessed via I2 and random effects analyses were performed.
RESULTS: Six studies meeting the inclusion criteria yielded 319 untreated patients whose natural history could be assessed. Heterogeneity among studies was moderate, with a variety of reported outcomes. A hearing improvement of at least 30 dB HL was observed in 36% (95% CI 0.28-0.44) of untreated patients, and of at least 10 dB HL was observed in 70% (95% CI 0.57-0.82) of untreated patients at 3 months. The mean hearing gain among untreated patients was 24.0 dB HL (95% CI 2.65-45.37) at 2-3 months.
CONCLUSIONS: The observed natural history of ISSNHL suggests that patients can regain some hearing without active treatment. In the absence of future studies collecting prospective natural history data from untreated or placebo-treated ISSNHL patients, the data presented here provide the best available historical control data for reconsideration of results in past ISSNHL studies, as well as a roadmap for design and interpretation of future ISSNHL treatment clinical trials. Furthermore, knowing there is a statistically significant mean hearing gain of 24.0 dB HL in the untreated/placebo group provides an ethical basis for future placebo study of ISSNHL. The current status on ISSNHL management calls for a multi-institutional, randomized, double-blind placebo-controlled trial with validated outcome measures to provide science-based treatment guidance. Laryngoscope, 134:S1-S15, 2024.

UNASSIGNED: A growing body of evidence suggests that immunological processes have a significant role in developing idiopathic sudden sensorineural hearing loss (SSHL). However, few studies have examined the association between immune cell phenotype and SSHL using Mendelian Randomization (MR).
UNASSIGNED: The online genome-wide association studies (GWAS) database was used to compile data from GWAS covering 731 immunophenotypes and SSHL. Inverse variance weighted (IVW) analysis was primarily used for MR study, and single nucleotide polymorphisms (SNPs) associated with immunophenotypes served as dependent variables. A sensitivity study and the false discovery rate (FDR) correction were used to examine the MR hypothesis. In addition, the possibility of reverse causality between immunophenotype and SSHL was validated by reverse MR. Reverse MR was analyzed in a manner consistent with forward MR.
UNASSIGNED: After FDR correction and sensitivity analysis, we screened 7 immunophenotypes, including IgD+ CD38dim %lymphocyte (95% CI: 1.0019, 1.0742, p = 3.87 × 10-2, FDR = 1.15 × 10-2); Unsw mem AC (95% CI: 1.004, 1.2522, p = 4.23 × 10-2, FDR = 2.25 × 10-2); CD86+ myeloid DC AC (95% CI: 1.0083, 1.1147, p = 2.24 × 10-2, FDR = 4.27 × 10-2); CD33dim HLA DR- AC (95% CI: 1.0046, 1.0583, p = 2.12 × 10-2, FDR = 4.69 × 10-2); SSC-A on CD8br (95% CI: 1.0028, 1.1461, p = 4.12 × 10-2, FDR = 4.71 × 10-2); CD45RA- CD4+ %T cell (95% CI: 1.0036, 1.0503, p = 2.32 × 10-2, FDR = 4.82 × 10-2); DP (CD4+CD8+) AC (95% CI: 1.011, 1.2091, p = 2.78 × 10-2, FDR = 4.97 × 10-2). There was a strong causal relationship with SSHL onset, and the reliability of the results was verified. Furthermore, the immunological cell profile and SSHL did not appear to be closely associated, as shown by reverse MR analysis.
UNASSIGNED: Our study provides more support for the current hypothesis that immunophenotypes and the pathophysiology of SSHL are closely associated. Further validation is needed to assess the role of these immunophenotypes in SSHL.

The cause of sudden sensorineural hearing loss (SSNHL) remains unknown in a significant number of cases, but vascular involvement in its pathophysiology has been proposed. Our study aimed to assess the incidence of stroke following idiopathic SSNHL (iSSNHL) and to evaluate associated cardiovascular risk factors and comorbidities. We extracted electronic medical record data from iSSNHL patients aged ≥ 50 years retrospectively from 84 general practices. Patients were matched for age, sex and general practice in a 1:4 ratio to controls. Primary outcome was the 5-years stroke risk following iSSNHL diagnosis. 480 iSSNHL cases could be matched to 1911 controls. The hazard ratio for iSSNHL compared with controls was 1.25 (95%CI 0.50-3.27; P = 0.646) for CVA (cerebrovascular accident) alone and 0.92 (95% CI 0.50-1.71; P = 0.804) for CVA and TIA (transient ischemic attack) combined. The hazard ratio for the interaction term between iSSNHL and age ≥ 60 years was 4.84 (95% CI 1.02-23.05; P = 0.048) for CVA and TIA combined. Patients with iSSNHL used antihypertensives and beta-blocking agents more frequently than controls (P = 0.006 and P = 0.022, respectively). In conclusion, no overall significant difference in the risk of stroke was observed, but the hazard ratio for stroke increased in iSSNHL patients aged 60 and older, suggesting potential vascular involvement in older subjects presenting with sudden sensorineural hearing loss.

More than 5% of the world\'s population suffers from disabling hearing loss. If the cause of hearing loss is unclear, it is referred to as idiopathic sudden sensorineural hearing loss (ISSNHL). After failure of standard treatment, the use of hearing aids or a cochlear implant is generally recommended. In this case, a 55-year-old patient was treated with cochlear implantation (CI) after ISSNHL and unsuccessful conservative therapy. Approximately 1 year after implantation and 7 years after the sudden hearing loss, subjective measurements revealed restoration of the hearing threshold.
UNASSIGNED: Mehr als 5 % der Weltbevölkerung leiden an einem behindernden Hörverlust. Bei unklarer Ursache des Hörverlusts bezeichnet man dies als „idiopathic sudden sensorineural hearing loss“ (ISSNHL). Nach ausbleibendem Erfolg der Standardtherapie wird in aller Regel die Verwendung von Hörgeräten oder ein Cochleaimplantat (CI) empfohlen. In diesem Fall wurde ein 55-jähriger Patient mit ISSNHL und erfolgloser konservativer Therapie mit einer Cochleaimplantation behandelt. Rund 1 Jahr nach Implantation und 7 Jahre nach dem Hörsturz wurde anhand von subjektiven Messungen eine weitestgehende Wiederherstellung der Hörschwelle festgestellt.