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.

Study on the correlation of C-reactive protein/albumin ratio with sudden sensorineural hearing loss complicated by hypertension: a prospective study.

BACKGROUND: Understanding the pathophysiology of sudden sensorineural hearing loss (SSNHL) and identifying its clinical symptoms and associated risk factors are crucial for doctors in order to create effective prevention and therapeutic methods for this prevalent otolaryngologic emergency. METHODS: This study focuses on investigating the correlation between the C-reactive protein/albumin ratio (CAR) and SSNHL complicated by hypertension. In this study, 120 patients diagnosed with SSNHL were divided into groups with and without hypertension, and propensity score matching was used to compare and analyze the severity, type, prognosis, and CAR levels in SSNHL. RESULTS: The results showed that the SSNHL group with hypertension had significantly higher CAR levels, age, hearing curve abnormalities, and more severe hearing loss compared to the control group with isolated SSNHL. These differences were statistically significant (p < 0.001). Among different subtypes of SSNHL, CAR levels increased progressively with the advancement of the condition, and these differences were also statistically significant (p < 0.001). CONCLUSION: In summary, in patients with SSNHL, those with hypertension had higher CAR levels than those without a history of hypertension, and they experienced more severe hearing loss. Moreover, there was a clear correlation between CAR levels and the extent of SSNHL, indicating that greater CAR levels in patients with SSNHL are connected to more severe hearing loss in various hearing patterns and perhaps indicative of a poorer prognosis.

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.

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.

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.

LINE-1 global DNA methylation, iron homeostasis genes, sex and age in sudden sensorineural hearing loss (SSNHL).

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) is an abrupt loss of hearing, still idiopathic in most of cases. Several mechanisms have been proposed including genetic and epigenetic interrelationships also considering iron homeostasis genes, ferroptosis and cellular stressors such as iron excess and dysfunctional mitochondrial superoxide dismutase activity. RESULTS: We investigated 206 SSNHL patients and 420 healthy controls for the following genetic variants in the iron pathway: SLC40A1 - 8CG (ferroportin; FPN1), HAMP - 582AG (hepcidin; HEPC), HFE C282Y and H63D (homeostatic iron regulator), TF P570S (transferrin) and SOD2 A16V in the mitochondrial superoxide dismutase-2 gene. Among patients, SLC40A1 - 8GG homozygotes were overrepresented (8.25% vs 2.62%; P = 0.0015) as well SOD2 16VV genotype (32.0% vs 24.3%; P = 0.037) accounting for increased SSNHL risk (OR = 3.34; 1.54-7.29 and OR = 1.47; 1.02-2.12, respectively). Moreover, LINE-1 methylation was inversely related (r2 = 0.042; P = 0.001) with hearing loss score assessed as pure tone average (PTA, dB HL), and the trend was maintained after SLC40A1 - 8CG and HAMP - 582AG genotype stratification (ΔSLC40A1 = + 8.99 dB HL and ΔHAMP = - 6.07 dB HL). In multivariate investigations, principal component analysis (PCA) yielded PC1 (PTA, age, LINE-1, HAMP, SLC40A1) and PC2 (sex, HFEC282Y, SOD2, HAMP) among the five generated PCs, and logistic regression analysis ascribed to PC1 an inverse association with moderate/severe/profound HL (OR = 0.60; 0.42-0.86; P = 0.0006) and with severe/profound HL (OR = 0.52; 0.35-0.76; P = 0.001). CONCLUSION: Recognizing genetic and epigenetic biomarkers and their mutual interactions in SSNHL is of great value and can help pharmacy science to design by pharmacogenomic data classical or advanced molecules, such as epidrugs, to target new pathways for a better prognosis and treatment of SSNHL.

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.

Endothelial Dysfunction and Metabolic Disorders in Patients with Sudden Sensorineural Hearing Loss.

Sudden sensorineural hearing loss (SSNHL) is defined as a sensorineural hearing loss of 30 dB or greater on at least three contiguous audiometric frequencies occurring within a 72 h period. Although SSNHL is commonly encountered in clinical audiology and otolaryngology practice, its etiopathogenesis continues to be poorly understood. Scientific investigations have highlighted the vulnerability of cochlear microcirculation to blood flow alterations. Even mild hypoperfusion can lead to immediate dysfunction in the organ of Corti, given the heightened susceptibility of cochlear hair cells to hypoxia and ischemic damage. The purpose of this review paper is to present evidence of endothelial and vascular involvement in SSNHL and the risk factors, such as metabolic syndrome, that may negatively impact the inner ear's vascular supply, influencing the onset pattern, incidence, and prognosis of SSNHL. By addressing these variables, we can deepen our comprehension of the mechanisms underlying SSNHL and potentially uncover strategies for prevention.

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.

Comparative study of SSNHL with and without tinnitus: audiologic and hematologic differences.

BACKGROUND AND OBJECTIVES: Tinnitus is one of the most common symptoms of sudden sensorineural hearing loss (SSNHL), with the incidence of tinnitus in patients with SSNHL ranging from 60% to 90%. Little is known, however, about the specific audiologic and hematologic factors that may be associated with the development of tinnitus. To better understand the relationship between tinnitus and SSNHL, the present study compared audiologic and hematologic factors in SSNHL patients with tinnitus and without tinnitus. SUBJECTS AND METHOD: The present study compared 120 patients with SSNHL with tinnitus and 59 patients with SSNHL without tinnitus at their initial examination. Their audiology and hematologic test results were analyzed, and hearing recovery was determined by comparing the hearing thresholds before and after treatment. RESULTS: 120 patients with tinnitus showed longer III and V latency in auditory brainstem response (ABR) tests, lower signal-to-noise ratios (SNR) at 2 kHz in transient evoked otoacoustic emissions (TEOAE) tests, and lower response rates at 2 kHz in distortion product otoacoustic emissions (DPOAE) tests of the affected ear (p < 0.05 each) than the 59 patients without tinnitus. However, there were no significant between-group differences in the mean hearing threshold and hearing recovery rate of the affected ear. Patients with tinnitus had significantly worse mean hearing thresholds and hearing thresholds at 4 kHz in the nonaffected ear. The percentages of monocytes and large unstained cells (%LUCs) were higher in the group without tinnitus (p < 0.05), although there were no significant between-group differences in inflammatory markers, such as neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR) and platelet-to-lymphocyte ratio (PLR). CONCLUSION: Tinnitus accompanying SSNHL may be associated with baseline hearing level, as well as being an indicator of damage to outer hair cells and auditory nerves. Additional studies are needed to evaluate hematologic data in SSNHL patients with and without tinnitus.

背景和目的：耳鸣是突发性感觉神经性听力损失（SSNHL）最常见的症状之一。SSNHL 患者中, 耳鸣的发生率为 60% 至 90%。然而, 我们对可能与耳鸣的发生相关的特定听力学和血液学因素知之甚少。 为了更好地了解耳鸣和 SSNHL 之间的关系, 本研究比较了 有耳鸣和无耳鸣的SSNHL患者的听力学和血液学因素。对象和方法：本研究在初次检查时比较了 120 名有耳鸣的 SSNHL 患者和 59 名无耳鸣的 SSNHL 患者。分析了他们的听力学和血液学测试结果, 通过比较治疗前和治疗后的听力阈值来确定听力恢复情况。结果：与 59 名无耳鸣患者相比, 120 名耳鸣患者在听性脑干反应（ABR）测试中表现出较长的 III 和 V 潜伏期, 在瞬态诱发耳声发射 (TEOAE) 测试中 表现出较低的2kHz 时信噪比 (SNR), 在失真产物耳声发射 (DPOAE) 测试中受累耳表现出较低的 2kHz 时响应率（ p<0.05）。然而, 在受累耳的平均听力阈值和听力恢复率方面, 并没有出现重大的组间差异。耳鸣患者的未受累耳的平均听阈和 4kHz 听阈明显较差。 无耳鸣组的单核细胞和未染色大细胞 (%LUC) 的百分比较高（p<0.05）, 尽管在炎症标志物方面, 例如中性粒细胞与淋巴细胞比率 (NLR)、单核细胞与淋巴细胞比率(MLR) 和血小板与淋巴细胞比率 (PLR), 组间差异不显著, 。结论：伴随 SSNHL 的耳鸣可能与基线听力水平相关, 而且还是外毛细胞和听觉神经受损的指标。 需要进一步的研究来评估有和没有耳鸣的 SSNHL 患者的血液学数据。.

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.

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.

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.

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 Incidence of Sudden Sensorineural Hearing Loss (SSNHL) in COVID-19 Patients in Tertiary Care Referral Units.

COVID-19 is a new pandemic infectious disease that emerged in Wuhan, China, at the end of 2019. We aimed to evaluate the sudden sensorineural hearing loss (SSNHL) prevalence after COVID-19 infection or even vaccination. This is a two-center retrospective, observational cross-sectional study performed at tertiary care referral Audiovestibular Medicine Units at the period between August 1, 2020, and October 31, 2021. All SSNHL patients diagnosed in a period of a month with COVID-19 or vaccinated with a COVID-19 vaccine were included in this study. Fifty-three cases with confirmed COVID-19 and one patient vaccinated with a COVID-19 vaccine 1 week before, who reported sudden sensory neural hearing loss, were included in this study. Forty-eight patients had unilateral hearing loss and 6 patients had bilateral hearing loss. Forty-nine patients had typical COVID-19 symptoms; one patient discovered them after complaining of anosmia and ageusia and one patient after COVID-19 vaccination; and three patients were complaining only from hearing loss and had a PCR test for nasopharyngeal swabs to prove infection. Different degrees of SSNHL ranged from mild to severe and most of the patients had severe hearing loss. With more patients, COVID-19 may be a potential factor in sudden sensorineural hearing loss. It should be kept in mind that SSNHL may be the only indicator used to identify COVID-19 cases.

Analysis of NFKB1 and NFKB2 gene expression in the blood of patients with sudden sensorineural hearing loss.

OBJECTIVES: Sudden Sensorineural Hearing Loss (SSNHL) is an increasingly common health problem today. Although the direct mortality rate of this disorder is relatively low, its impact on quality of life is enormous; this is why accurate identification of pathogenesis and influencing factors in the disease process can play an essential role in preventing and treating the disease. Acute inflammation, which leads to chronic inflammation due to aberrant expression of inflammation-mediating genes, may play a significant role in the pathogenesis of the disease. The essential Nuclear factor kappa B (NF-kB) pathway genes, NFKB1 and NFKB2, serve as prothrombotic agents when expressed abnormally, compromising the cochlea by disrupting the endolymphatic potential and causing SSNHL. METHODS: This study investigates the expression levels of NFKB1 and NFKB2 in peripheral blood (PB) through a quantitative polymerase chain reaction in 50 Iranian patients with SSNHL, and 50 healthy volunteers were of the same age and sex as controls. RESULTS: As a result, NFKB2 expression levels in patients were higher than in controls, regardless of sex or age (posterior beta = 0.619, adjusted P-value = 0.016), and NFKB1 expression levels did not show significant differences between patients and controls. The expression levels of NFKB1 and NFKB2 had significantly strong positive correlations in both SSNHL patients and healthy individuals (r = 0.620, P = 0.001 and r = 0.657, P 0.001, respectively), suggesting the presence of an interconnected network. CONCLUSION: NFKB2 has been identified as a significant inflammatory factor in the pathophysiology of SSNHL disease. Inflammation can play an essential role in developing SSNHL, and our findings could be used as a guide for future research.

The relationship between serum homocysteine levels and sudden sensorineural hearing loss: a meta-analysis.

OBJECTIVE: There was disagreement over the association between serum/plasma homocysteine (HCY) levels and sudden sensorineural hearing loss (SSNHL). Through the use of a meta-analysis, this study aims to determine whether there is a significant difference in serum homocysteine levels between the SSNHL group and the control group. DESIGN: The Cochrane Library, EMBASE, and PubMed databases were all thoroughly searched. The two independent reviewers thoroughly examined the initially searched articles. The data results were calculated by standard mean difference (SMD) or odds ratios (OR). Review Manager (version 5.3) was applied to statistical data. STUDY SAMPLE: There were 766 participants in the 6 trials with continuous outcomes that were part of the meta-analysis A. In addition, meta-analysis B, which included 961 people, contained a total of 3 studies with dichotomous results. RESULTS: Both meta-analyses revealed the same conclusion that serum/plasma HCY levels in the SSNHL patients are higher than those in the controls (SMD 0.41, 95 % confidence interval (CI) 0.11 to 0.72, P < 0.01; OR 3.27, 95 % CI 2.16 to 4.94, P < 0.01). CONCLUSION: This study demonstrated that the SSNHL patients' serum/plasma HCY levels were greater than those of the control group.

Application of inner ear MRI after intravenous gadolinium injection in SSNHL.

BACKGROUND: With the development of inner ear gadolinium imaging technology, its clinical application is more and more frequent. OBJECTIVES: To explore the application value of inner ear MRI after intravenous gadolinium injection in sudden sensorineural hearing loss. MATERIAL AND METHODS: The clinical data of 28 patients who were preliminarily diagnosed with sudden sensorineural hearing loss and the results of intravenous gadolinium MRI examination were analyzed to find the relationship between them. RESULTS: Of the 28 patients (30 ears) with idiopathic sudden sensorineural hearing loss, 20 (71.4%) showed normal MRI. MRI abnormalities related to hearing loss were found in 8 cases (28.6%), of which 5 cases (17.9%) showed hydrops in the inner ear membrane labyrinth, 2 cases (7.1%) showed mastoiditis on the affected side, and 1 case (3.6%) showed vestibular schwannoma. CONCLUSIONS AND SIGNIFICANCE: Inner ear MRI after intravenous gadolinium injection can be used as an index to detect the cause of sudden deafness, one of the factors to guide clinical treatment, and an important means to further explore the relationship between hydrops of membranous labyrinth and SSNHL.

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.

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.