Search Results (35)

Background/Objectives: Although vascular mechanisms are increasingly implicated in the etiology of sudden sensorineural hearing loss (SSNHL), the inability to directly visualize the labyrinthine artery remains a diagnostic obstacle. Sharing embryological and physiological parallels with the inner ear, the eye represents an accessible surrogate organ capable of reflecting systemic microvascular status. This study aimed to evaluate the diagnostic value of ocular hemodynamic and structural parameters in patients with acute unilateral idiopathic SSNHL. Methods: This prospective, comparative, cross-sectional study enrolled 30 patients with acute unilateral idiopathic SSNHL and 25 age and sex matched healthy controls. Three groups were defined: the affected eye, the contralateral eye, and the control eye. Retrobulbar hemodynamics (PSV, EDV, RI, PI) were assessed by color Doppler imaging; peripapillary choroidal thickness, RNFL, GCC+, and macular thickness by swept-source OCT; and macular microvascular perfusion by OCT angiography. Results: End diastolic velocity in the posterior ciliary arteries was significantly reduced in both patient eye groups relative to controls (p < 0.001), while RI and PI were significantly elevated (p = 0.001 and p = 0.004, respectively). Comparable hemodynamic impairment was observed in the ophthalmic artery. Peripapillary choroidal thickness was bilaterally reduced in the inferior and temporal quadrants in both patient groups (p = 0.003 and p = 0.010). No significant difference was detected between affected and contralateral eyes in any parameter. RNFL, GC+, and macular thickness remained comparable across all groups. Conclusions: The bilateral symmetry of hemodynamic impairment and choroidal thinning suggests that SSNHL arises against a background of systemic microvascular disease. The combined use of OCT and color Doppler ultrasonography holds clinical potential as a non-invasive biomarker panel for defining the vascular phenotype of the condition. Full article

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

Background/Objectives: The study aimed to evaluate the efficacy of hearing gain using hyperbaric oxygen (HBO) therapy on patients with idiopathic sudden sensorineural hearing loss (ISSHL), and to provide recommendations with reference to treatment session, intervention time and the impairment severity. Methods: In this retrospective chart-review study, we analyzed data of ISSHL patients referred to us from the department of Ear, Nose and Throat (ENT) between January 2016 and December 2024. Hearing sensitivity improvements were assessed using pure-tone audiometry (PTA). Results: We found that 50.7% of patients (n = 148) had improved after 5 HBO sessions and 47.7% (n = 107) had improved after 10 HBO sessions. While no difference was found between different treatment cycles, we found treatment effects varied significantly depending on disease severity. Specifically, 64.3% of patients with profound ISSHL had improved (p = 0.010) after 5 sessions, and 69.2% (p = 0.002) after 10 sessions. Such improvements in patients with profound ISSHL were 3.681-fold larger than in those with mild to moderate ISSHL. In addition, patients who had received HBO therapy within 12 days of diagnosis showed the best response, with an odds ratio (OR) of 7.768 (95% CI: 2.785–21.664) (p = 0.000 *); those receiving HBO between 13 and 27 days had an OR of 3.974 (95% CI: 1.243–12.702), (p = 0.020 *); both groups were compared with those receiving HBO after 27 days. Conclusions: Patients with more severe ISSHL showed greater improvement with HBO therapy. Also, patients who started HBO therapy earlier showed better response; those who started later, like after 27 days, showed poorer or even no response at all. Full article

Background/Objectives: Patients with Idiopathic sudden sensorineural hearing loss (ISSNHL) are often devastated by the unknown etiology coupled with the unknown pathway to recovery. The aim of this study was to evaluate whether abnormalities on the video Head Impulse Test (vHIT) performed early in the course of ISSNHL are associated with poorer hearing recovery. Methods: Forty-four patients with ISSNHL were prospectively enrolled between 2019 and 2022 following exclusion of differential diagnoses on clinical and MRI evaluation. vHIT was performed within 1–14 days of symptom onset and within 48 h of hospitalization. Recovery at six months was assessed both as a dichotomous outcome and by change in pure tone average (PTA). Group differences were analyzed using Fisher’s exact and Mann–Whitney U tests. A two-predictor logistic regression model examined the association between vHIT results, dizziness, and recovery. Results: Twelve patients exhibited abnormal vHIT findings. Abnormal vHIT was strongly associated with the presence of dizziness and with markedly poorer hearing recovery at six months. Patients with normal vHIT demonstrated substantially greater improvement in PTA thresholds compared with those showing abnormal results. Logistic regression further confirmed that abnormal vHIT was an independent predictor of reduced likelihood of hearing recovery, whereas dizziness alone did not independently influence outcomes. Conclusions: Our findings suggest that abnormal vHIT results in ISSNHL patients are linked to poor hearing recovery, which can enhance patient counseling regarding expectations. Although promising as a prognostic tool, we acknowledge our limited sample size and recommend validation in larger prospective cohorts. Full article

Background: Idiopathic sudden sensorineural hearing loss (ISSNHL) is an abrupt unilateral hearing loss of unknown origin. Combination therapy with hyperbaric oxygen (HBO), systemic steroids (SS), and prostaglandin E1 (PGE1) has been used in Japan; however, its prognostic factors remain unclear. Objective: To evaluate the efficacy of HBO combined with SS and PGE1 and to identify prognostic factors for hearing recovery in patients with ISSNHL. Methods: This retrospective study included 116 patients treated within 14 days of ISSNHL onset. Sixty patients received HBO, SS, and PGE1 (HBO group), and 56 received SS and PGE1 alone (No-HBO group). Hearing outcomes were assessed using PTA (arithmetic mean hearing at 250–4000 Hz) and graded by Siegel’s criteria. Prognostic factors were analyzed by multivariate logistic regression. Results: The HBO group showed significantly better hearing grade outcomes (p = 0.007) and greater PTA improvement (p = 0.003) than the No-HBO group. Vertigo and higher initial PTA were identified as independent predictors of poor hearing outcomes. Patients without vertigo showed significantly greater improvement at 2000 Hz (p = 0.009). Receiver operating characteristic analysis revealed an optimal initial PTA cutoff of ≥90.5 dB for predicting poor hearing outcome. Conclusions: HBO combined with SS and PGE1 significantly improves hearing outcomes in ISSNHL. However, the presence of vertigo and severe initial hearing loss remain poor prognostic indicators. These findings suggest that while the addition of HBO may enhance hearing outcomes, prognosis remains limited in severe cases. Further prospective studies are needed to confirm these results. Full article

Objective: Although the etiologies and pathogenesis of common hearing disorders—noise-induced hearing loss (NIHL), age-related hearing loss (ARHL), and idiopathic sudden sensorineural hearing loss (ISSNHL)—are diverse, accumulating evidence indicates that reactive oxygen species (ROS) contribute to hearing loss and that antioxidants may help prevent or treat it. We conducted a literature review to examine the relationship between hearing loss and ROS/free radicals in both humans and animal models. Methods: We performed a comprehensive literature search of PubMed/MEDLINE, Embase, the Cochrane Library, Scopus, and Google Scholar to evaluate the induction and role of ROS in the development and treatment of hearing loss. Results: We synthesized evidence across NIHL, ARHL, and ISSNHL. Factors and reactive species implicated in hearing loss included cytomegalovirus infection, genetic polymorphisms, NADPH oxidase 4 (NOX4), NOX transgenic models (NOX-Tg), lipid hydroperoxides (LOOH), and malondialdehyde (MDA). Antioxidant strategies examined for prevention or treatment included vitamins A, C, and E with magnesium; rebamipide; α-lipoic acid; LLY-283; edaravone; melatonin; glutathione peroxidase; superoxide dismutase; glucose; hydrogen-saturated saline; activation of nuclear factor erythroid 2-related factor 2 (Nrf2); inhaled hydrogen gas; and caffeic acid. Conclusions: Elevated ROS and free radicals appear to contribute to the pathogenesis of hearing loss. Although definitive conclusions cannot yet be drawn, current evidence suggests that antioxidant approaches may aid in prevention and treatment. Further studies are needed to elucidate underlying mechanisms, refine therapeutic targets and dosing, and validate efficacy in rigorously designed clinical trials. Full article

Objective: We aimed to explore the efficacy of repetitive transcranial magnetic stimulation (rTMS) for idiopathic sudden sensorineural hearing loss (ISSNHL) and evaluate the correlation between treatment courses of rTMS and hearing outcomes. Methods: A prospective observational study was conducted at West China Fourth Hospital, Sichuan University, from January 2018 to January 2025. The study enrolled 339 patients (342 affected ears) diagnosed with ISSNHL. Among them, 67 patients (group A) received standard therapy combined with rTMS, while the control group (group B) received conventional therapy only. To verify the correlation between different treatment courses of rTMS and hearing outcomes, patients in Group A were divided into Group A1 (treatment courses ≤ 10) and Group A2 (treatment courses > 10). Hearing thresholds and clinical characteristics were evaluated at admission, discharge day and 6 months post-treatment. The SDRG’s criteria were used for the grading of hearing recovery. Results: Tinnitus (79.89% vs. 75.32%, p = 0.361) and sleep disorders (33.70% vs. 41.14%, p = 0.178) were highly prevalent among patients in group A and group B. 1 Hz rTMS significantly improved these symptoms (PSQI: 52.32% vs. 44.44%, p = 0.032; THI: 16.67 ± 19.41 vs. 8.22 ± 12.77, p = 0.002). Compared to high-tone hearing loss patients, those with low-tone loss in groupA2 showed a more rapid improvement (250 Hz: 17.66 ± 16.59 vs. 14.09 ± 15.58, p = 0.041; 500 Hz: 21.20 ± 18.03 vs. 17.31 ± 16.24, p = 0.036) than grouA1, with benefits sustained at 6-month follow-up (250 Hz: 27.79 ± 18.74 vs. 22.71 ± 18.31, p = 0.012; 500 Hz: 31.89 ± 19.73 vs. 26.49 ± 20.08, p = 0.013). Conclusions: rTMS at 1 Hz, administered in courses > 10 sessions, demonstrated both short-term and long-term beneficial effects in the ISSNHL. Those with low-tone hearing loss exhibit better recovery but a higher chance of relapse than high-tone loss patients. As a non-invasive approach with minimal side effects, rTMS is suitable for routine ISSNHL treatment. 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: The vestibulo-respiratory reflex regulates the tension of the respiratory muscles, which prevents apneas and awakenings during sleep. This study aimed to determine whether functional deficits in the inner ear disturb sleep quality. Methods: We compared sleep parameters in patients with their first episode of acute inner ear deficit (Group A: sudden idiopathic vertigo attack, sudden sensorineural hearing loss), chronic functional inner ear impairment (Group B: chronic peripheral vertigo, permanent hearing loss), and in healthy individuals (Group C). Polygraphy recordings were performed twice, in Group A at the onset of acute otoneurological symptoms and the second time after their withdrawal with an interval of 1 to 13 days, in Group B after 1 to 6 days, and in Group C after 1 to 8 days. Results: In Group A during the symptomatic night, overall and central apnea-hypopnea indices were significantly higher and snoring time was longer. Group A also had higher central apnea-hypopnea index on the first night compared to healthy individuals. In chronic disorders, sleep recordings showed lower autonomic arousal index than in controls or symptomatic nights in Group A. Conclusions: These findings highlight the severity of sleep apnea indicators in Group A. Our results suggest that acute dysfunction of the inner ear substantially impacts central neuronal signaling responsible for regulating normal sleep-related breathing and leads to a deterioration in sleep quality in contrast to individuals with chronic inner ear impairments. It can also be assumed that people with chronic vertigo or hearing loss experience less interrupted sleep than healthy individuals. Full article

Sudden sensorineural hearing loss (SSHNL) is an abruptly appearing hearing loss. The etiology remains unclear, although vascular pathologies, viral infections, or autoimmune disease contribute to the understanding of this pathology. Systematic steroids are often used as the first-line treatment because of their anti-inflammatory effect. However, there remains controversy about the use of steroids and other alternative treatments, as hyperbaric oxygen therapy (HBOT), exploratory tympanotomy, prostaglandin, N-acetylcysteine, or defibrinogenation therapy. In this study, we aim to review the various treatment options currently available for sudden hearing loss, with the objective of advancing our understanding of this condition and clarifying information to guide future clinical practice guidelines. 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 and Objectives: Although different hypotheses have been proposed over time, there is a dearth of information on factors able to predict the response to treatment for idiopathic sudden sensorineural hearing loss (ISSNHL) and hearing recovery. The aim of this study was to apply univariate and multivariate statistical models in a retrospective clinical setting of patients given therapy for ISSNHL at our tertiary academic audiological centers to investigate the prognostic value of clinical signs, symptoms, and comorbidities in relation to hearing recovery. Materials and Methods: The inclusion criteria were: history of ISSNHL diagnosed and treated at the Padova or Modena tertiary academic audiological centers; age ≥ 18 years; availability of clinical and audiological outcome data. The exclusion criteria were: hearing loss in acoustic schwannoma, endolymphatic hydrops, meningitis, trauma (head trauma, temporal bone fracture, acoustic trauma), barotrauma, perilymphatic fistula; exposure to noise levels ≥ 80 dB in the work environment; any unilateral or bilateral hearing loss (except for presbycusis) prior to ISSNHL diagnosis; any disorders affecting the external or middle ear; any previous ear surgery; refusal to make medical data available for research purposes. Eighty-six consecutive patients (38 females, 48 males; median age: 58 years; interquartile range: 47.00–69.00 years) were included. A systemic steroid therapy was administered to all patients, either orally with prednisone or intravenously with methylprednisolone. Second-line therapy included intratympanic steroid injections and/or hyperbaric oxygen therapy. Results: A multivariate logistic regression model was used, including the non-multicollinear clinical and audiological variables, which showed a p-value < 0.10 at the univariate analyses (namely age at diagnosis, time to diagnosis, oral steroid dose, and PTA on the affected side). Only PTA on the affected side retained its statistical significance (OR: 1.0615, 95% CI: 1.0185–1.1063, p = 0.005). Conclusions: The analysis of our data showed an association between the hearing threshold before treatment and the recovery from ISSNHL. Further studies on larger cohorts (especially in a prospective setting) are needed to shed more light on the prognostic role of clinical parameters in patients with ISSNHL. In a correct counseling setting, with regard to the patient’s concern about not being able to recover hearing, it is important to offer perspectives of appropriate hearing rehabilitation approaches. Full article

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. Full article