Functional to structural plasticity in unilateral sudden sensorineural hearing loss: neuroimaging evidence.

A cortical plasticity after long-duration single side deafness (SSD) is advocated with neuroimaging evidence while little is known about the short-duration SSDs. In this case-cohort study, we recruited unilateral sudden sensorineural hearing loss (SSNHL) patients and age-, gender-matched health controls (HC), followed by comprehensive neuroimaging analyses. The primary outcome measures were temporal alterations of varied dynamic functional network connectivity (dFNC) states, neurovascular coupling (NVC) and brain region volume at different stages of SSNHL. The secondary outcome measures were pure-tone audiograms of SSNHL patients before and after treatment. A total of 38 SSNHL patients (21 [55%] male; mean [standard deviation] age, 45.05 [15.83] years) and 44 HC (28 [64%] male; mean [standard deviation] age, 43.55 [12.80] years) were enrolled. SSNHL patients were categorized into subgroups based on the time from disease onset to the initial magnetic resonance imaging scan: early- (n = 16; 1-6 days), intermediate- (n = 9; 7-13 days), and late- stage (n = 13; 14-30 days) groups. We first identified slow state transitions between varied dFNC states at early-stage SSNHL, then revealed the decreased NVC restricted to the auditory cortex at the intermediate- and late-stage SSNHL. Finally, a significantly decreased volume of the left medial superior frontal gyrus (SFGmed) was observed only in the late-stage SSNHL cohort. Furthermore, the volume of the left SFGmed is robustly correlated with both disease duration and patient prognosis. Our study offered neuroimaging evidence for the evolvement from functional to structural brain alterations of SSNHL patients with disease duration less than 1 month, which may explain, from a neuroimaging perspective, why early-stage SSNHL patients have better therapeutic responses and hearing recovery.

Digging into the Role of Inflammatory Biomarkers in Sudden Sensorineural Hearing Loss Diagnosis and Prognosis: A Systematic Review and Meta-Analysis.

Background and Objectives: Sudden Sensorineural Hearing Loss (SSNHL) is a quite common clinical finding in otolaryngology. Most cases are classified as idiopathic and there is a dearth of information on factors able to predict the response to treatment and hearing recovery. The main aim of this systematic review and meta-analysis was to assess and critically discuss the role of circulating inflammatory biomarkers in SSNHL. Materials and Methods: A search was conducted of the English literature published between 1 January 2009 and 7 July 2022 on Pubmed, Scopus, Web of Science, ScienceDirect, and Cochrane following PRISMA guidelines. Results: A total of 256 titles were retrieved from the search. After full-text screening and application of inclusion/exclusion criteria, 13 articles were included. Twelve out of thirteen studies reported significant differences in biomarkers values in SSNHL patients, of which Tumor Necrosis Factor alpha (TNF-α) and C-reactive Protein (CRP) were the most analyzed. Our meta-analysis for CRP's mean values in SSNHL groups vs. controls showed significantly higher CRP levels with a pooled overall difference of 1.07; confidence interval (CI) at 95%: 0.03; 2.11. For TNF-α, discordant results were found: three studies showed significantly higher levels in SSNHL patients vs. controls, whereas other three investigations showed lower levels in the SSNHL groups (overall pooled difference 1.97; 95% CI: -0.90; 4.84). A high between-study heterogeneity was found. Conclusions: This systematic review pointed out that, although there exists a growing literature in the field of circulatory biomarkers identification in SSNHL, there is a high heterogeneity of results and low quality of evidence. CRP resulted to be higher in SSNHL patients than in controls, while TNF-α showed more heterogeneous behavior. The data reported herein needs to be confirmed in well-designed prospective multicenter randomized studies, with the objective of improving SSNHL treatment and outcome and thereby reducing the social burden of hearing loss.

Long-term exposure to air pollution and the risk of developing sudden sensorineural hearing loss.

BACKGROUND: The association between exposure to air pollution and sudden sensorineural hearing loss (SSNHL) has not been extensively discussed in the literature. Therefore, we conducted this nationwide study to evaluate the risk of SSNHL in Taiwanese residents with exposure to air pollution. METHODS: We enrolled subjects aged older than 20 years with no history of SSNHL from 1998 to 2010, and followed up until developing SSNHL, withdrawn from the National Health Insurance program, and the end of the database (2011/12/31). The air quality data are managed by Taiwan Environmental Protection Administration. The annual concentrations of PM2.5, SO2, CO, NO, and NO2 from 1998 to 2010 were classified into the three levels according to tertiles. We calculated the annual average of pollutants from baseline until the end of the study, and classified into tertiles. The adjusted hazard ratio (aHR) was estimated by using the multivariate Cox proportional hazard model. RESULTS: When considered continuous air pollutants concentration, subjects who exposed with higher concentration of CO (aHR = 2.16, 95% CI 1.50-3.11), NO (aHR = 1.02, 95% CI 1.01-1.03), and NO2 (aHR = 1.02, 95% CI 1.01-1.04) developing significant higher risk of SSNHL. When classified air pollutants concentration into low, moderate and high level by tertiles, and selected low level as reference, patients exposed with moderate (aHR = 1.56, 95% CI 1.20-2.04) or high level (aHR = 1.33, 95% CI 1.01-1.75) of PM2.5 showed significant higher risk of developing SSNHL. CONCLUSION: This study indicated an increased risk of SSNHL in residents with long-term exposure to air pollution. Nevertheless, further experimental, and clinical studies are needed to validate the study findings.

Can isolated sudden sensorineural hearing loss (SSNHL) and idiopathic acute facial paralysis (Bell's palsy) be symptoms of COVID-19?

OBJECTIVES: The symptoms of COVID-19 at the time of presentation mainly include fever, cough, respiratory distress and myalgia. On the other hand, as neurological symptoms, disruption of taste and smell and cerebrovascular pathologies are well-known, whereas other neurological symptoms and signs are being newly recognized. Sudden-onset sensorineural hearing loss (SSNHL) and idiopathic acute facial paralysis (Bell's palsy) are otologic emergencies that are frequently encountered by otorhinolaryngology specialists. Although there are many articles describing SSNHL and Bell's palsy in the literature, the literature describing their relationship to COVID-19 is limited. In our study, we aimed to present the neuro-otologic relationship of SSNHL and Bell's palsy with COVID-19. MATERIAL AND METHODS: The pretreatment real-time oronasopharyngeal PCR tests, COVID-19 symptomatology and COVID-19 infection statuses of patients who presented to our clinic with isolated SSNHL and Bell's palsy between April 2020 and April 2021 were questioned, and the data of the patients were collected. Throughout their treatment, the patients were followed-up in terms of COVID-19 infection. This is a prospective study. Moreover, to observe the change in the incidence, the data of patients visiting between January 2019 and January 2020 were also collected. The data of the patients were statistically analyzed using SPSS. RESULTS: The study included a total of 177 patients. The SSNHL group consisted of 91 patients, and the Bell's palsy group consisted of 86 patients. Neither group showed a statistically significant difference in comparison to the year without the pandemic in terms of the patient numbers (incidence), sex, age, morbidity, response to treatment or social habits. There was a statistically significant difference in age only in the Bell's palsy group, but this difference was not medically significant. CONCLUSION: As a result of our study, we did not observe a relationship between COVID-19 and cases of SSNHL and Bell's palsy. It is recommended to apply standard otologic treatment to isolated SSNHL and Bell's palsy patients whose association with COVID-19 is not determined.

Normal Weight Central Obesity is a Poor Prognostic Factor for Sudden Sensorineural Hearing Loss.

OBJECTIVES: To investigate the role of normal weight central obesity (NWCO) in the prognosis of sudden sensorineural hearing loss (SSNHL). METHODS: We retrospectively investigated 807 cases of SSNHL from January of 2008 to August of 2019 from the Department of Otorhinolaryngology at Kaohsiung Medical University Hospital in southern Taiwan. We analyzed the association between overweight and obesity, NWCO, and the prognosis of SSNHL. The demographic and clinical characteristics, audiometry results, and outcomes were also reviewed. RESULTS: The nonobese (body mass index [BMI] < 24 kg/m2) and overweight and obese groups (BMI ≥ 24 kg/m2) comprised 343 (42.50%) and 464 (57.50%) patients, respectively. The favorable prognosis rates in the nonobese and the overweight and obese groups were 45.48% and 45.91%, respectively, without a significant difference (P = .9048). Multivariate logistic regression revealed that BMI (adjusted odds ratio [aOR] = 1.00, 95% CI = 0.948-1.062, P = .9165) was not significantly associated with SSNHL recovery. The normal weight noncentral obesity (NWNCO) and NWCO groups comprised 266 (77.55%) and 77 (22.45%) patients, respectively, and had favorable prognosis rates of 48.50% and 35.06%, respectively. The difference between the groups was significant (P = .0371). Multivariate logistic regression analysis revealed that NWCO (aOR = 2.51, 95% CI = 1.292-5.019, P = .0075) was significantly associated with SSNHL recovery. CONCLUSIONS: NWCO may significantly affect the prognosis of SSNHL.

Exploring the Impact of Auditory Hallucinations on Sudden Sensorineural Hearing Loss in Adulthood: A Case Report.

Auditory hallucinations are sounds that patients perceive as coming from outside of their body. Though the mechanism causing auditory hallucinations is not entirely understood, there is a significant amount of evidence suggesting that auditory hallucinations leave lasting impacts on the brain in the same regions that are involved in auditory processing. Sudden sensorineural hearing loss (SSNHL) is a poorly understood condition in which patients lose their hearing typically in the fifth decade of life. Here we present a case of a 42-year-old female with a history of schizophrenia with auditory hallucinations who experienced SSNHL at age 40. As the patient had no known risk factors for SSNHL, we propose that this patient's SSNHL is linked to her history of auditory hallucinations. Through the presentation of this case, we hope to explore the pathogenesis of auditory hallucinations and investigate a potentially bidirectional association between auditory hallucinations and SSNHL. This study calls for further investigation into the impacts of auditory hallucinations on the brain, possible etiologies of SSNHL, and the possibility that auditory hallucinations serve as a risk factor for SSNHL.

Unveiling a Rare Phenomenon: Sudden Sensorineural Hearing Loss Following Skin Prick Testing.

Introduction: The skin prick test (SPT) is a standard method for diagnosing allergic diseases, while sudden sensorineural hearing loss (SSNHL) remains a perplexing condition often of unknown etiology. We present a unique case of SSNHL emerging shortly after an SPT, suggesting a potential link between allergic reactions and inner ear disorders. Case Report: A 27-year-old male presented with unilateral hearing loss following an SPT for allergic rhinitis. Audiometric findings revealed SSNHL in the affected ear. Treatment with corticosteroids led to a full recovery of hearing function. Conclusion: This case highlights the rare occurrence of SSNHL possibly triggered by a type 1 allergic reaction to an SPT. Prompt corticosteroid therapy proved effective in restoring hearing. Further research is needed to understand the relationship between allergies and SSNHL and explore alternative treatment options.

Beyond the Usual Suspects: Ethylene Glycol Poisoning Complicated by Rare Neurological Sequelae.

Ethylene glycol poisoning is a known clinical entity with established diagnostic and management protocols. However, instances presenting with rare neurological complications pose diagnostic challenges and necessitate prompt recognition and intervention. This report details the case of ethylene glycol poisoning in a 38-year-old male patient who initially presented with a history of brake oil consumption at his residence, followed by a delayed presentation with vomiting, abdominal pain, and reduced urine output, and subsequently developed unusual neurological sequelae, including unsteadiness, hearing difficulties, and an inability to close his eyes. Diagnostic assessment revealed cerebellar ataxia with bilateral sensory-neural hearing loss and facial nerve palsy. The patient was subsequently managed primarily for ethylene glycol poisoning, with conservative management for the neurological sequelae, and improved with no residual deficits. This case underscores the importance of promptly managing ethylene poisoning to prevent complications and sequelae as well as reduce morbidity for patients.

Omega-3 Adjunctive Therapy in Idiopathic SSNHL: A Randomised, Triple-Blind, Placebo-Controlled Trial.

Introduction: Idiopathic Sudden Sensorineural Hearing Loss as a subset of sensorineural hearing loss will be confirmed by a progressive hearing loss of at least 30 dB at three contiguous frequencies over 72 hours or less. A sudden or abrupt hearing loss correlates with the time course, and a vascular event is presumptive aetiology. There is an inverse association between Omega-3 consumption and hearing loss. This study aimed to evaluate the efficacy of Omega-3 adjunctive therapy in Idiopathic Sudden Sensorineural Hearing Loss by audiometric assessments. Materials and Methods: In this randomised, triple-blind, placebo-controlled trial, all participants aged 18-70 with a history of sudden deafness (within 12 hours and ≤ 30 days) were eligible for enrollment. They were included if audiology diagnostic tests confirmed the SSNHL. Ultimately, they were randomised to the Omega-3 group and the placebo group. Results: Thirty-three patients were randomly allocated to the Omega-3 group and thirty-two to the placebo group. Vertigo (32.3% of all patients) and underlying conditions had significant relationships with complete response (C.R.)-final hearing level ≤of 25 dB in pure-tone average (P < 0.05). There was no significant difference between both groups before and after treatment. Although it was not statistically significant, patients in the Omega-3 group had faster recovery than placebo. Conclusions: Omega-3 adjunctive therapy did not have a therapeutic effect on SSNHL patients. Moreover, C.R. happened in half the patients. Vertigo and underlying conditions considerably worsen the recovery from SSNHL.

Nomogram for predicting the prognosis of sudden sensorineural hearing loss patients based on clinical characteristics: a retrospective cohort study.

Background: Based on the clinical characteristics of patients, a nomogram predicting the prognosis of patients suffering from sudden sensorineural hearing loss (SSNHL) was constructed, which could aid in personalized treatment. Methods: Data on the clinical characteristics of patients with SSNHL were collected and statistically analyzed. A nomogram for predicting the hearing prognosis of SSNHL patients were then constructed. Results: A total of 356 patients were included in this study, including 227 and 129 in the recovery group (63.76%) and non-recovery group (36.24%), respectively. Univariable logistic regression demonstrated that age, gender, body mass index (BMI), marital, Audiogram curve, vertigo, hearing loss degree, and time to initial treatment were associated with hearing outcomes. Multivariate logistic models showed that age [odds ratio (OR): 0.479, 95% confidence interval (CI): 0.301-0.748, P<0.001], descending (OR: 0.116, 95% CI: 0.047-0.275, P<0.001) and flat audiogram curves (OR: 0.397, 95% CI: 0.159-0.979, P=0.045), profound hearing loss (OR: 0.047, 95% CI: 0.013-0.152, P<0.001), and treatment initiation after 1 week (8-14 days: OR: 0.047, 95% CI: 0.013-0.152, P<0.001; >14 days: OR: 0.131, 95% CI: 0.039-0.413) were risk factors for the hearing recovery. Logistic regression analyses were conducted to construct the prognostic nomogram. As estimated by the area under the receiver operating characteristic curve (ROC), the model had an accuracy of 0.867 (95% CI: 0.709-0.747). The validation analysis confirmed the high accuracy of the nomogram, and the decision curve showed that the model has potential clinical application value. Conclusions: This study demonstrated that age, descending and flat audiogram curves, profound hearing loss, and initiating treatment after 1 week of SSNHL onset were independent risk factors associated with a worse hearing recovery prognosis. Using these factors, a nomogram with a high prediction accuracy was developed to predict the hearing recovery rate of SSNHL patients.

The Impact of Intratympanic Steroid Dosage on Hearing Recovery in Sudden Sensorineural Hearing Loss.

OBJECTIVE: To investigate how dexamethasone dosage impacts intratympanic steroid therapy (IST) for treatment of sudden sensorineural hearing loss (SSNHL). STUDY DESIGN: Retrospective review. METHODS: Inclusion criteria identified subjects who received IST between January 1, 2010 and June 1, 2020 for iSSNHL. Subjects with Meniere's disease, autoimmune inner ear disease, subjects who received injections of non-dexamethasone steroid formulations, and subjects with missing audiogram data were excluded. Subjects were stratified by dexamethasone dosage: low-dose (10 mg/ml) versus high-dose (24 mg/ml), time-to-treatment and oral corticosteroid use. Outcome measures included post-treatment improvement in 4-frequency pure tone average (4F-PTA [500, 1000, 2000,4000 Hz]), low- and high-frequency PTA (250-1000 Hz and 2000-8000 Hz, respectively). RESULTS: Of the 55 included subjects (50.9% male, mean age 48.9 ± 16.4 years), 31 received high-dose while 24 received low-dose injections. 90.9% of subjects were treated with oral steroids prior to or during IST. No significant differences in hearing outcomes were observed between low- and high-dose cohorts or when stratifying by oral steroid use. Time-to-treatment analysis comparing ≤1 month (67.3%) versus >1 month (32.7%) groups demonstrated a significant improvement in post-treatment 4F-PTA (P = .01) in the ≤1 month group. CONCLUSIONS: Hearing recovery was not significantly different between the 10 mg/ml versus 24 mg/ml doses of intratympanic dexamethasone, suggesting that steroid dose may not impact the efficacy of IST. A shorter time-to-treatment was observed to be favorable for hearing improvement.

A review of bilateral sudden sensorineural hearing loss in pediatric patients.

OBJECTIVE: Bilateral, sudden sensorineural hearing loss (SSNHL) in the pediatric population is a rare phenomenon potentially detrimental to language acquisition and social development. This study comprehensively reviews and analyzes existing literature to determine any correlation or commonality in etiologies, presentations, and management of this condition. METHODS: PubMed, Cochrane, Scopus, and Web of Science databases were systematically searched for articles related to pediatric SSNHL from 1970 to 2021. Case series, case reports, and cohort studies were included. Data on patient demographics, etiology, diagnostic testing, management, and hearing recovery were collected. RESULTS: Excluding duplicates, 553 unique titles were identified by established search criteria, of which 342 titles were relevant to pediatric sudden hearing loss. Forty-six papers reported cases of bilateral SSNHL, totaling 145 individual cases. Not included in the analysis were 45 cases documented as non-organic hearing loss. The average age of the total 145 included patients was 8.5 years and 51 were male. Reported etiologies included cytomegalovirus (n = 3), meningitis (n = 13), mumps (n = 5), ototoxin exposure (n = 13), and enlarged vestibular aqueduct (n = 9). Tinnitus (n = 30) was the most reported concurrent symptom, followed by vertigo (n = 21). Systemic steroid therapy was the most common treatment and, when follow up was reported, most patients (51.2%) had complete or partial recovery of hearing. CONCLUSIONS: This is a comprehensive review of pediatric bilateral SSNHL. Though often idiopathic, etiologies also include infectious, structural, and autoimmune. Treatment largely consists of systemic steroid therapy, with variables rates of recovery. Further studies on intratympanic administration of steroids may guide future treatment.

Clinical features and prognosis of pediatric idiopathic sudden sensorineural hearing loss: A bi-center retrospective study.

Objective: Limited research has focused on the clinical features of sudden sensorineural hearing loss (SSNHL) in pediatric patients. This study is aimed to investigate the relationship between clinical features and the baseline hearing severity and outcomes of SSNHL in the pediatric population. Method: We conducted a bi-center retrospective observational study in 145 SSNHL patients aged no more than 18 years who were recruited between November 2013 and October 2022. Data extracted from medical records, audiograms, complete blood count (CBC) and coagulation tests have been assessed for the relationship with the severity (the thresholds of the initial hearing) and outcomes (recovery rate, hearing gain and the thresholds of the final hearing). Results: A lower lymphocyte count (P = 0.004) and a higher platelet-to-lymphocyte ratio (PLR) (P = 0.041) were found in the patient group with profound initial hearing than in the less severe group. Vertigo (ß = 13.932, 95%CI: 4.082-23.782, P = 0.007) and lymphocyte count (ß = -6.686, 95%CI: -10.919 to -2.454, P = 0.003) showed significant associations with the threshold of the initial hearing. In the multivariate logistic model, the probability of recovery was higher for patients with ascending and flat audiograms compared to those with descending audiograms (ascending: OR 8.168, 95% CI 1.450-70.143, P = 0.029; flat: OR 3.966, 95% CI 1.341-12.651, P = 0.015). Patients with tinnitus had a 3.2-fold increase in the probability of recovery (OR 3.222, 95% CI 1.241-8.907, P = 0.019), while the baseline hearing threshold (OR 0.968, 95% CI 0.936-0.998, P = 0.047) and duration to the onset of therapy (OR 0.942, 95% CI 0.890-0.977, P = 0.010) were negatively associated with the odds of recovery. Conclusions: The present study showed that accompanying tinnitus, the severity of initial hearing loss, the time elapse and the audiogram configuration might be related to the prognosis of pediatric SSNHL. Meanwhile, the presence of vertigo, lower lymphocytes and higher PLR were associated with worse severity.

Sudden Sensorineural Hearing Loss Associated with Oral Consumption of Hand Sanitizer: A Case Report.

Introduction: Pediatric sudden sensorineural hearing loss (SSNHL) is a rare otological emergency. With the emergence of the Coronavirus 19 pandemic, alcohol-based hand sanitizers are among the essential household items. Many hand sanitizers are frequently coupled with scents that young children may find pleasant. Case Report: A 5-year-old girl presented to our clinic with hearing loss after the consumption of alcohol-based hand sanitizer. A pure tone audiogram showed bilateral SSNHL. The child was prescribed systemic corticosteroids resulted in a slight improvement in hearing thresholds. The child was followed up at 6 and 18 months showing no further improvement in hearing thresholds. Conclusion: Although various infective, vascular, and immune responses have been proposed, alcohol-based hand sanitizer consumption has not been reported to present with SSNHL to the best of our knowledge. In the current scenario of the Coronavirus pandemic, otorhinolaryngologists must keep in mind that SSNHL may occur as a result of hazardous alcohol-based hand disinfectant consumption.

Related factors to the coping style of patients with sudden sensorineural hearing loss.

BACKGROUND: Coping style can affect the patient's physical and mental health management. Therefore this study aimed to identify factors related to the coping style of young and middle-aged sudden sensorineural hearing loss (SSNHL) patients to provide reference for clinical nursing practice. METHODS: A cross-sectional study was conducted on young and middle-aged SSNHL patients hospitalized in the otolaryngology departments of four hospitals in Suzhou City, China. A paper-based self-administered questionnaire investigated the patient's coping style and related factors. Multiple linear stepwise regression analysed the effective related factors in patients' coping styles. RESULTS: Among 872 patients, 866 completed the survey, with an average age of 37.27 y. Factors related to the coping style of these patients included gender, chronic diseases, history of trauma, social support and type D personality (p<0.05). Female patients adopt more negative coping styles than male patients. Patients with chronic diseases or a history of trauma had more positive coping styles. Higher social support scores were related to improvements in coping style. Patients with type D personality were more likely to adopt negative coping styles. CONCLUSIONS: This study suggests that psychological assessment of patients, chronic diseases, history of trauma, social support and type D personality may benefit the understanding of these patients' coping styles and, as a consequence, may improve their stress management.

Diagnosis, differential diagnosis, and treatment for sudden sensorineural hearing loss: Current otolaryngology practices in China.

Introduction: Although sudden sensorineural hearing loss (SSNHL) has been attempted to be understood for 70 years, diagnosis and treatment strategies still have strong heterogeneity worldwide, which are reflected in the guidelines issued by countries and the clinical practice of otolaryngologists. Methods: Questionnaires were sent to registered otolaryngologists nationwide via an online questionnaire system. We investigated the current views and clinical practices of otolaryngologists in mainland China about the diagnosis, examination, and treatment strategies of SSNHL. Results: Most otolaryngologists supported diagnostic classification via audiograms. Regional economic situation and hospital grade affected application strategies for differential diagnosis. Regarding corticosteroid therapy, 54.9% of respondents opted to discontinue the drug 5 days after systemic administration. Both intratympanic therapy and post-auricular injections were selected by more than half of the respondents as initial and salvage treatments. Discussion: Chinese otolaryngologists exhibit heterogeneity in clinical practices for SSNHL, including distinct approaches to combination therapy and local application of steroids. This study pointed out Chinese doctors' similarities, differences, and unique strategies in diagnosing and treating SSNHL and analyzed the possible reasons to help the world understand the current otolaryngology practices in China.

Left-Sided Sensorineural Hearing Loss and Facial Weakness in a 35-Year-Old Patient: A Diagnostic Challenge and Case Report.

We present the case of a 35-year-old patient who presented with a three-month history of left-sided sensorineural hearing loss and left-sided facial weakness. Initial imaging suggested a schwannoma, and the patient underwent ten treatments of intra-tympanic steroid injections and antibiotics, and was scheduled for surgery. However, the planned schwannoma removal surgery with gamma-knife was aborted due to the absence of the previously identified mass on the pre-procedure MRI. Subsequent imaging revealed continued enhancement of the left internal auditory canal (IAC), leading to considerations of lymphoma, sarcoidosis, IgG4 disease, or other inflammatory condition. The patient's symptoms have significantly improved since and are currently being conservatively managed and monitored. However, the patient continues to show persistent findings on MRI. This case highlights the diagnostic challenges faced in identifying the underlying etiology of this patient and emphasizes the need for further investigations and multidisciplinary management in patients with similar presentations.

The role of the stria vascularis in neglected otologic disease.

The stria vascularis (SV) has been shown to play a critical role in the pathogenesis of many diseases associated with sensorineural hearing loss (SNHL), including age-related hearing loss (ARHL), noise-induced hearing loss (NIHL), hereditary hearing loss (HHL), and drug-induced hearing loss (DIHL), among others. There are a number of other disorders of hearing loss that may be relatively neglected due to being underrecognized, poorly understood, lacking robust diagnostic criteria or effective treatments. A few examples of these diseases include autoimmune inner ear disease (AIED) and/or autoinflammatory inner ear disease (AID), Meniere's disease (MD), sudden sensorineural hearing loss (SSNHL), and cytomegalovirus (CMV)-related hearing loss (CRHL). Although these diseases may often differ in etiology, there have been recent studies that support the involvement of the SV in the pathogenesis of many of these disorders. We strive to highlight a few prominent examples of these frequently neglected otologic diseases and illustrate the relevance of understanding SV composition, structure and function with regards to these disease processes. In this study, we review the physiology of the SV, lay out the importance of these neglected otologic diseases, highlight the current literature regarding the role of the SV in these disorders, and discuss the current strategies, both approved and investigational, for management of these disorders.

Vulnerable frequency as an independent prognostic factor for sudden sensorineural hearing loss.

Objectives: Sudden sensorineural hearing loss (SSNHL) is a common otology emergency in the practice. Its severe hearing impairment and prognosis impair the quality of life. Given that cochlear hair cell vulnerability is not consistent across frequencies, this study aims to investigate the impact of frequency-specific hearing loss on prognosis in SSNHL. Methods: The study included 255 patients with full-frequency SSNHL. The baseline, clinical, and hearing characteristics, as well as possible cardiovascular predictors in blood, were collected for analysis. Results: The 4,000 and 8,000 Hz hearing levels in the responder group were significantly lower than those in the non-responder group (p = 0.008, p < 0.001), while the average hearing was not (p = 0.081). Logistic regression showed that only vertigo (OR, 95% CI, 0.265, 0.102-0.684, p = 0.006) and 8,000 Hz hearing level (OR, 95% CI, 0.943, 0.916-0.971, p < 0.001) were strongly associated with treatment outcome. Conclusions: Compared with other frequencies, 8,000 Hz hearing level was closely related to prognosis in SSNHL. In an adjusted model, our study did not find an effect of mean hearing on prognosis in SSNHL. However, further multicenter prospective studies are needed for validation.

Clinical features and prognostic factors of children with profound sudden sensorineural hearing loss.

Objective: To investigate the clinical features and factors affecting the prognosis of children with profound sudden sensorineural hearing loss (SSNHL). Methods: We retrospectively analyzed the clinical data of 147 children with profound SSNHL who received inpatient treatment at our department from January 2016 to January 2021. All children were administered with systemic steroid therapy and/or intratympanic steroid (ITS) treatment for 2 weeks. Statistical analyses were performed for the clinical features, treatment effectiveness, and factors affecting the prognosis using SPSS 23.0. Results: The median age of the study population was 8 (6-10) years. The median treatment onset time was 8 (4-20) days. The most common concomitant symptom was tinnitus (45.58%). Laboratory findings showed that the percentages of children with abnormal leukocytes was 25.85%, abnormal platelet counts was 17.01%, abnormal cytomegalovirus IgG antibodies was 36.73% and abnormal Epstein-Barr (EB) virus IgG antibodies was 41.50%. The overall recovery rate of the treatment was 20.04%. The univariate analysis showed that age, treatment onset time, tinnitus, and ITS treatment were associated with the prognosis (p < 0.05). Regarding laboratory findings, the neutrophil count, lymphocyte count, and neutrophil-to-lymphocyte ratio differed significantly between the effective and invalid treatment effect groups (p < 0.05). The multivariable logistic regression analysis showed that treatment onset time [odds ratio (OR) = 0.936, 95% confidence interval (CI): 0.881-0.994] and ITS treatment (OR = 0.174, 95% CI: 0.044-0.0687) correlated with hearing recovery (p < 0.05). Conclusion: In this study, the earlier the treatment start time of children with profound SSNHL, the better was the prognosis. Further, ITS could be an effective treatment option.