Clinical practice guidelines for the diagnosis and management of acute sensorineural hearing loss.

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. CONCLUSION: 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.

The Association Between Late Gadolinium Enhancement by Cardiac Magnetic Resonance and Ventricular Arrhythmia in Patients With Mitral Valve Prolapse: A Systematic Review and Meta-Analysis.

INTRODUCTION: Malignant ventricular arrhythmia (VA) and sudden cardiac death (SCD) have been reported in patients with mitral valve prolapse (MVP); however, effective risk stratification methods are still lacking. Myocardial fibrosis is thought to play an important role in the development of VA; however, observational studies have produced contradictory findings regarding the relationship between VA and late gadolinium enhancement (LGE) in MVP patients. The aim of this meta-analysis and systematic review of observational studies was to investigate the association between left ventricular LGE and VA in patients with MVP. METHODS: We searched the PubMed, Embase, and Web of Science databases from 1993 to 2023 to identify case-control, cross-sectional, and cohort studies that compared the incidence of VA in patients with MVP who had left ventricular LGE and those without left ventricular LGE. RESULTS: A total of 1464 subjects with MVP from 12 observational studies met the eligibility criteria. Among them, VA episodes were reported in 221 individuals (15.1%). Meta-analysis demonstrated that the presence of left ventricular LGE was significantly associated with an increased risk of VA (pooled risk ratio 2.96, 95% CI: 2.26-3.88, p for heterogeneity = 0.07, I2 = 40%). However, a meta-regression analysis of the prevalence of mitral regurgitation (MR) showed that the severity of MR did not significantly affect the association between the occurrence of LGE and VA (p = 0.079). CONCLUSION: The detection of LGE could be helpful for stratifying the risk of VA in patients with MVP.

Effect of combining batroxobin with acupuncture treatment on hearing recovery in patients with sudden sensorineural hearing loss.

BACKGROUND: The effect of batroxobin on hearing recovery in patients with Sudden Sensorineural Hearing Loss (SSNHL) is still controversial, and acupuncture shows auxiliary benefits for SSNHL. OBJECTIVES: To investigate the effectiveness of combining acupuncture with batroxobin therapy for patients with SSNHL. MATERIAL AND METHODS: One hundred and fifty-six patients with SSNHL were retrospectively enrolled in this study, and categorized into the control group (only batroxobin treatment) and observation group (batroxobin and acupuncture treatment). Pure Tone Audiograms (PTA) threshold and clinical outcomes of hearing recovery were compared. Logistic regression analysis was used to evaluate the association between hearing recovery and potential risk factors. RESULTS: Compared to the control group, the observation group had a higher overall effective rate (p = 0.006) and improvement in PTA threshold (p = 0.007). Among SSNHL patients with high-frequency and flat-type hearing loss, observation group demonstrated superior hearing recovery post-treatment compared to the control group (p < 0.05). Additionally, hearing recovery in patient with SSNHL were associated with SSNHL types, disease duration, neutrophil count and acupuncture (p < 0.05). CONCLUSIONS AND SIGNIFICANCE: Combining batroxobin and acupuncture treatments enhences the improvement of hearing recovery in SSNHL patients compared to only batroxobin treatments, especially high-frequency and flat-type hearing loss.

Access to Sudden Hearing Loss Care at Urgent Care Centers.

OBJECTIVES: To compare patient access to urgent care centers (UCCs) with a diagnosis of sudden hearing loss based on insurance. METHODS: One hundred twenty-five random UCCs in states with Medicaid expansion and 125 random UCCs in states without Medicaid expansion were contacted by a research assistant posing as a family member seeking care on behalf of a patient with a one-week history of sudden, unilateral hearing loss. Each clinic was called once as a Medicaid patient and once as a private insurance (PI) patient for 500 total calls. Each phone encounter was evaluated for insurance acceptance and self-pay price. Secondary outcomes included other measures of timely/accessible care. Chi-square/McNemar's tests and independent/paired sample t-tests were performed to determine whether there were statistically significant differences between expansion status and insurance type. Calls ended before answering questions were not included in the analysis. RESULTS: Medicaid acceptance rate was significantly lower than PI (68.1% vs. 98.4%, p < 0.001). UCCs in Medicaid expansion states were significantly more likely to accept Medicaid (76.8% vs. 59.2%, p = 0.003). The mean wage-adjusted self-pay price was significantly greater in states with Medicaid expansion at $169.84 than in states without at $145.34 when called as a Medicaid patient (mean difference: $24.50, 95% Confidence Interval: $0.45-$48.54, p = 0.046). The rates of referral to an emergency department and self-pay price nondisclosure rates were greater for Medicaid calls than for private insurance calls (8.2% vs. 0.4% and 17.4% vs. 5.8%; p < 0.001 for both). CONCLUSION: Medicaid patients with otologic emergencies face reduced access to care at UCCs. LEVEL OF EVIDENCE: NA Laryngoscope, 2024.

Molecular autopsy in Chinese sudden cardiac death in the young.

Inherited cardiovascular conditions are significant causes of sudden cardiac death in the young (SCDY), making their investigation using molecular autopsy and prevention a public health priority. However, the molecular autopsy data in Chinese population is lacking. The 5-year result (2017-2021) of molecular autopsy services provided for victims of SCDY (age 1-40 years) was reviewed. The outcome of family cascade genetic screening and clinical evaluation was reviewed. A literature review of case series reporting results of molecular autopsy on SCDY in 2016-2023 was conducted. Among the 41 decedents, 11 were found to carry 13 sudden cardiac death (SCD)-causative genetic variants. Likely pathogenic (LP) variants were identified in the DSP, TPM1, TTN, and SCN5A genes. Cascade genetic testing identified four family members with LP variants. One family member with familial TPM1 variant was found to have hypertrophic cardiomyopathy upon clinical evaluation. This study provided insight into the genetic profile of molecular autopsy in a Chinese cohort of SCDY. The detection of important SCD-causative variants through molecular autopsy has facilitated family cascade screening by targeted genetic testing and clinical evaluation of at-risk family members. A literature review of the current landscape of molecular autopsy in the investigation of SCDY was conducted.

One mechanism of sudden sensorineural hearing loss after sildenafil and sexual activity.

OBJECTIVES: A case of sudden sensorineural hearing loss following use of sildenafil was examined in detail over a period of three days from first report to recovery. DESIGN: Case study. The subject presented with sudden sensorineural hearing loss and diplacusis a day after onset. Testing involved detailed interview, standard audiometry, detailed inter-octave audiometry, and measurement of detailed psychophysical frequency tuning curves during a two day recovery period. STUDY SAMPLE: One male aged in his thirties with otherwise normal hearing. RESULTS: Although standard audiometry was within normal limits, detailed inter-octave audiometry and psychophysical frequency tuning curves were consistent with a punctate unilateral intra-cochlear lesion that resolved over a period of three days. CONCLUSIONS: This is the first report of such a frequency-specific audiometric shift and diplacusis after sildenafil, and is not consistent with previous reports of direct ototoxic pharmacological effects. We propose that the lesion was most likely caused by a cochlear bleed, and may have been due to physical exertion rather than a direct pharmaceutical effect. The study highlights the important role of additional diagnostic testing that can be easily achieved in a clinical setting with minimal equipment.

Subcutaneous versus transvenous implantable cardioverter defibrillator in hypertrophic cardiomyopathy: a systematic review and meta-analysis.

Background: A subcutaneous implantable cardioverter-defibrillator (S-ICD) is an alternative to a transvenous implantable cardio defibrillator (TV-ICD). An S-ICD reduces the risk of transvenous lead placement. However, further research is required to determine how S-ICDs affect patients with hypertrophic cardiomyopathy (HCM). In this study, we investigated the comparative efficacy and safety of S-ICDs versus TV-ICDs in HCM. Methods: On December 6th, 2023, we performed a comprehensive search of the PubMed, Embase, Scopus, and Cochrane databases to identify randomized clinical trials (RCTs) and observational studies comparing S-ICDs with TV-ICDs in HCM patients published from 2004 until 2023. No language restrictions were applied. The primary outcome was appropriate shocks (AS), with inappropriate shocks (IAS), and device-related complications considered as secondary outcomes. Odds ratios (ORs) and 95% confidence intervals (CIs) were pooled using a random effects model. The ROBINS-I tool was used to assess the risk of bias of the studies. Results: The search yielded 1,114 records. Seven studies comprising 4,347 HCM patients were included, of whom 3,325 (76.0%) had TV-ICDs, and 1,022 (22.6%) had S-ICDs. There were 2,564 males (58.9%). The age range was from 39.1 to 49.4 years. Compared with the TV-ICD group, the S-ICD cohort had a significantly lower incidence of device-related complications (OR 0.52; 95% CI: 0.30-0.89; P=0.02; I2=4%). Contrastingly, there were no statistically significant differences in the occurrences of AS (OR 0.49; 95% CI: 0.22-1.08; P=0.08; I2=75%) and IAS (OR 1.03; 95% CI: 0.57-1.84; P=0.93; I2=65%) between the two device modalities. In the analysis of the overall risk of bias in the studies, we found 42% of them with several, 28% with moderate, and 14% with low risk of bias. Conclusions: In HCM patients, S-ICDs were associated with a lower incidence of device-associated problems than TV-ICDs. AS and IAS incidence rates were similar between groups. These findings may assist clinicians in determining the most suitable device for treating patients with HCM.

Advances and Challenges in the Management of Brugada Syndrome: A Comprehensive Review.

Brugada syndrome (BrS) is an inherited arrhythmogenic disorder marked by distinctive ST-segment elevations on electrocardiograms (ECG) and an increased risk of sudden cardiac death. Characterized by mutations primarily in the SCN5A gene, BrS disrupts cardiac ion channel function, leading to abnormal electrical activity and arrhythmias. Although BrS primarily affects young, healthy males, it poses significant diagnostic challenges due to its often concealed or intermittent ECG manifestations and clinical presentation that can mimic other cardiac disorders. Current management strategies focus on symptom control and prevention of sudden death, with implantable cardioverter-defibrillators (ICD) serving as the primary intervention for high-risk patients. However, the complications associated with ICDs and the lack of effective pharmacological options necessitate a cautious and personalized approach. Recent advancements in catheter ablation have shown promise, particularly for managing ventricular fibrillation (VF) storms and reducing ICD shocks. Additionally, pharmacological treatments such as quinidine have been effective in specific cases, though their use is limited by availability and side effects. This review highlights significant gaps in the BrS literature, particularly in terms of long-term management and novel therapeutic approaches. The importance of genetic screening and tailored treatment strategies to better identify and manage at-risk individuals is emphasized. The review aims to enhance the understanding of BrS and improve patient outcomes, advocating for a multidisciplinary approach to this complex syndrome.

Shifting Paradigms in Hypertrophic Cardiomyopathy: The Role of Exercise in Disease Management.

Hypertrophic cardiomyopathy (HCM) is traditionally associated with exercise restriction due to potential risks, yet recent evidence and guidelines suggest a more permissive stance for low-risk individuals. The aim of this comprehensive review was to examine existing research on the impact of exercise on cardiovascular outcomes, safety, and quality of life in this population and to consider implications for clinical practice. Recent studies suggest that regular exercise and physical activity in low-risk individuals with HCM are associated with positive outcomes in functional capacity, haemodynamic response, and quality of life, with consistent safety. Various studies highlight the safety of moderate-intensity exercise, showing improvements in exercise capacity without adverse cardiac remodelling or significant arrhythmias. Psychological benefits, including reductions in anxiety and depression, were also reported following structured exercise programs. These findings support the integration of individualised exercise regimens in the management of low-risk individuals with HCM, potentially improving overall well-being and cardiovascular health. Adoption of the FITT principle, consideration of individual risk profiles, and shared decision-making are recommended. Future research is warranted to clarify the definition of 'low-risk' for exercise participation and investigate the influence of physical activity on disease progression in HCM. Innovation in therapeutic strategies and lifestyle interventions, alongside improved patient and provider education, will help advance the care and safety of individuals with HCM engaging in exercise.

[Cardiac channelopathies in the context of hereditary arrhythmia syndromes]. / Kardiale Kanalopathien im Kontext hereditärer Arrhythmiesyndrome.

Genetic arrhythmia disorders are rare diseases; however, they are a common cause of sudden cardiac death in children, adolescents, and young adults. In principle, a distinction can be made between channelopathies and cardiomyopathies in the context of genetic diseases. This paper focuses on the channelopathies long and short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). Early diagnosis of these diseases is essential, as drug therapy, behavioral measures, and if necessary, implantation of a cardioverter defibrillator can significantly improve the prognosis and quality of life of patients. This paper highlights the pathophysiological and genetic basis of these channelopathies, describes their clinical manifestations, and comments on the principles of diagnosis, risk stratification and therapy.

Impact of audiovestibular factors on prognosis in patients with sudden sensorineural hearing loss without vertigo.

PURPOSE: This study aimed to investigate the hearing outcomes in patients with sudden sensorineural hearing loss without vertigo (SSNHLwoV). METHODS: Patients with SSNHLwoV managed from December 2016 to March 2020 were prospectively enrolled in an academic tertiary referral center. Fifty-one patients with SSNHLwoV who completed high-dose steroid treatment. The hearing prognosis was analyzed using a multivariate Cox regression model. RESULTS: The rates of complete, partial, and no hearing recovery were 52.9%, 17.6%, and 29.4% in patients with SSNHLwoV, respectively. The video head impulse test (vHIT) of the posterior semicircular canal (PSC), high-tone hearing loss (4-8 kHz) ≥ 30 dB, and average hearing threshold (0.5-1-2-4 kHz) were significantly associated with incomplete recovery of hearing after treatment. In multivariate analysis, the vHIT of the PSC (hazard ratio [HR], 14.502; 95% confidence interval [CI], 1.371-153.355) and high-tone hearing loss ≥ 30 dB (HR, 9.170; 95% CI, 2.283-36.830) remained robust. CONCLUSIONS: Abnormal vestibular function tests were performed in 80.4% of the patients with SSNHLwoV. Abnormal vHIT of the PSC and high-tone hearing loss ≥ 30 dB were independent factors resulting in incomplete recovery of hearing in patients with SSNHLwoV. In the SSNHLwoV cohort, the caloric test was not significantly associated with hearing prognosis, and vHIT was a feasible predictor of treatment outcome.

Distinct Substrates of Idiopathic Ventricular Fibrillation Revealed by Arrhythmia Characteristics on Implantable Cardioverter-Defibrillator.

BACKGROUND: Idiopathic ventricular fibrillation (IVF) can be associated with undetected distinct conditions such as microstructural cardiomyopathic alterations (MiCM) or Purkinje (Purk) activities with structurally normal hearts. OBJECTIVE: This study sought to evaluate the characteristics of recurrent VF recorded on implantable defibrillator electrograms, associated with these substrates. METHODS: This was a multicenter collaboration study. At 32 centers, we selected patients with an initial diagnosis of IVF and recurrent arrhythmia at follow-up without antiarrhythmic drugs, in whom mapping demonstrated Purk or MiCM substrate. We analyzed variables related to previous ectopy, sinus rate preceding VF, trigger, and initial VF cycle lengths. Logistic regression with cross validation was used to evaluate the performance of criteria to discriminate Purk or MiCM substrates. RESULTS: Among 95 patients (35 women, age 35 ± 11 years) meeting the inclusion criteria, IVF was associated with MiCM in 41 and Purk in 54 patients. A total of 117 arrhythmia recurrences including 91% VF were recorded on defibrillator. Three variables were mostly discriminant. Sinus tachycardia (≤570 ms) was more frequent in MiCM (35.9% vs 13.4%, P = 0.014) whereas short-coupled (<350 ms) triggers were most frequent in Purk-related VF (95.5% vs 23.1%, P = 0.001), which also had shorter VFCLs (182 ± 15 ms vs 215 ± 24 ms, P < 0.001).The multivariable combination provided the highest prediction (accuracy = 0.93 ± 0.05, range 0.833-1.000), discriminating 81% of IVF substrates with a high probability (>80%). Ectopy were inconsistently present before VF. CONCLUSIONS: Characteristics of arrhythmia recurrences on implantable cardioverter- defibrillator provide phenotypic markers of the distinct and hidden substrates underlying IVF. These findings have significant clinical and genetic implications.

Heart proteomic profiling discovers MYH6 and COX5B as biomarkers for sudden unexplained death.

Sudden unexplained death (SUD) is not uncommon in forensic pathology. Yet, diagnosis of SUD remains challenging due to lack of specific biomarkers. This study aimed to screen differentially expressed proteins (DEPs) and validate their usefulness as diagnostic biomarkers for SUD cases. We designed a three-phase investigation, where in the discovery phase, formalin-fixed paraffin-embedded (FFPE) heart specimens were screened through label-free proteomic analysis of cases dying from SUD, mechanical injury and carbon monoxide (CO) intoxication. A total of 26 proteins were identified to be DEPs for the SUD cases after rigorous criterion. Bioinformatics and Adaboost-recursive feature elimination (RFE) analysis further revealed that three of the 26 proteins (MYH6, COX5B and TNNT2) were potential discriminative biomarkers. In the training phase, MYH6 and COX5B were verified to be true DEPs in cardiac tissues from 29 independent SUD cases as compared with a serial of control cases (n = 42). Receiver operating characteristic (ROC) analysis illustrated that combination of MYH6 and COX5B achieved optimal diagnostic sensitivity (89.7 %) and specificity (84.4 %), with area under the curve (AUC) being 0.91. A diagnostic software based on the logistic regression formula derived from the training phase was then constructed. In the validation phase, the diagnostic software was applied to eight authentic SUD cases, seven (87.5 %) of which were accurately recognized. Our study provides a valid strategy towards practical diagnosis of SUD by integrating cardiac MYH6 and COX5B as dual diagnostic biomarkers.

Altered ventilatory responses to hypercapnia-hypoxia challenges in a preclinical SUDEP model involve orexin neurons.

Failure to recover from repeated hypercapnia and hypoxemia challenges caused by severe GCS and postictal apneas may contribute to sudden unexpected death in epilepsy (SUDEP). Our previous studies found orexinergic dysfunction contributes to respiratory abnormalities in a preclinical model of SUDEP, Kcna1-/-mice. Here, we developed two gas challenges consisting of repeated HH exposures and used wholebody plethysmography to determine whether Kcna1-/-mice would have detrimental ventilatory responses. Kcna1-/- mice exhibited an elevated ventilatory response to a mild repeated hypercapnia-hypoxia (HH) challenge compared to WT. Moreover, 71% of Kcna1-/- mice failed to survive a severe repeated HH challenge, whereas all WT mice recovered. We next determined whether orexin was involved in these differences. Pretreatment of Kcna1-/- mice with a dual orexin receptor antagonist rescued the ventilatory response during the mild challenge and all subjects survived the severe challenge. In ex vivo extracellular recordings in the lateral hypothalamus of coronal brain slices, we found reducing pH either inhibits or stimulates putative orexin neurons similar to other chemosensitive neurons; however, a significantly greater percentage of putative orexin neurons from Kcna1-/-mice were stimulated and the magnitude of stimulation was increased resulting in augmentation of the calculated chemosensitivity index relative to WT. Collectively, our data suggest that increased chemosensitive activity of orexin neurons may be pathologic in the Kcna1-/- mouse model of SUDEP, and contribute to elevated ventilatory responses. Our data suggest that individuals at high risk for SUDEP may be more sensitive to HH challenges, whether induced by seizures or other means; and the depth and length of the HH exposure could dictate the probability of survival.

Mitral Annulus Disjunction in consecutive patients undergoing Cardiac Magnetic Resonance: where is the boundary between normality and disease?

BACKGROUND: The presence of mitral annulus disjunction (MAD) has been considered a high-risk feature for sudden cardiac death based on selected study populations. We aimed to assess the prevalence of MAD in consecutive patients undergoing clinically-indicated Cardiac Magnetic Resonance (CMR), its association with ventricular arrhythmias, Mitral Valve Prolapse (MVP), and other CMR features. METHODS: This single-center retrospective study included consecutive patients referred to CMR at our Institution between June 2021 and November 2021. The MAD was defined as a ≥1mm displacement between the left atrial wall-mitral valve leaflet junction and the left ventricular wall during end-systole. The MAD extent was defined as the maximum longitudinal displacement. Associates of MAD were evaluated at uni- and multi-variariable regression analysis. A study endpoint including (aborted) sudden cardiac death, unexplained syncope, and sustained ventricular tachycardia was evaluated at 12-month follow-up. RESULTS: Four-hundred-forty-one patients (55±18 years, 61% males) were included, and 29 (7%) had MVP. The prevalence of MAD ≥1mm, 4mm, and 6mm were 214 (49%), 63 (14%), and 15 (3%), respectively. Patients with MVP showed a higher prevalence of MAD greater than 1mm (90% vs. 46%; p<0.001), 4mm (48% vs. 12%; p<0.001), and 6mm (10% vs. 3%; p=0.03), and a greater MAD extent (4.2mm, 3.0-5.7mm vs. 2.8mm, 1.9-4.0mm; p<0.001) than patients without MVP. The MVP was the only morpho-functional abnormality associated with MAD at multivariable analysis (p<0.001). A high burden of ventricular ectopic beats at baseline Holter-ECG was associated with MAD ≥4mm and MAD extent (p<0.05). The presence of MAD ≥1mm (0.9% vs. 1.8%; p=0.46), MAD ≥4mm (1.6% vs. 1.3%; p=0.87), or MVP (3.5% vs. 1.2%; p=0.32) were not associated with the study endpoint, whereas patients with MAD ≥6mm showed a trend towards a higher likelihood of the study endpoint (6.7% vs. 1.2%; p=0.07). CONCLUSIONS: A MAD of limited entity was common in consecutive patients undergoing CMR. Patients with MVP showed higher prevalence and greater extent of MAD. Extended MAD was rarer and showed association with ventricular arrhythmias at baseline. The mid-term prognosis of MAD seems benign, however prospective studies are warranted to search for potential "malignant MAD extents" to improve patients' risk stratification.

The role of coronary CT angiography in athletes.

The sudden death of a young or high-level athlete or adolescent during recreational sports is one of the events with the greatest impact on public opinion in modern society. Sudden cardiac death (SCD) is the principal medical cause of death in athletes and can be the first and last clinical presentation of underlying disease. To prevent such episodes, pre-participation screening has been introduced in many countries to guarantee cardiovascular safety during sports and has become a common target among medical sports/governing organizations. Different cardiac conditions may cause SCD, with incidence depending on definition, evaluation methods, and studied populations, and a prevalence and etiology changing according to the age of athletes, with CAD most frequent in master athletes, while coronary anomalies and non-ischemic causes prevalent in young. To detect silent underlying causes early would be of considerable clinical value. This review summarizes the pre-participation screening in athletes, the specialist agonistic suitability visit performed in Italy, the anatomical characteristics of malignant coronary anomalies, and finally, the role of coronary CT angiography in such arena. In particular, the anatomical conditions suggesting potential disqualification from sport, the post-treatment follow-up to reintegrate young athletes, the diagnostic workflow to rule-out CAD in master athletes, and their clinical management are analyzed.

Serum lipid metabolism characteristics and potential biomarkers in patients with unilateral sudden sensorineural hearing loss.

BACKGROUND: Glycerophospholipids (GPLs) are essential for cell membrane structure and function. Sphingomyelin and its metabolites regulate cell growth, apoptosis, and stress responses. This study aimed to investigate lipid metabolism in patients experiencing sudden sensorineural hearing loss across all frequencies (AF-SSNHL). METHODS: The study included 60 patients diagnosed with unilateral AF-SSNHL, among whom 30 patients had a level of hearing improvement ≥ 15 dB after 6 months of follow-up. A propensity score-matched (2:1) control group was used. Liquid chromatography‒mass spectrometry based untargeted lipidomics analysis combined with multivariate statistics was performed to investigate the lipids change. The "lipidome" R package and weighted gene co-expression network analysis (WGCNA) were utilised to assess the lipids' structural features and the association between lipids and hearing. RESULTS: Lipidomics successfully differentiated the AF-SSNHL group from the control group, identifying 17 risk factors, mainly including phosphatidylcholine (PC), phosphatidylethanolamine (PE), and related metabolites. The ratios of lysophosphatidylcholine/PC, lysophosphatidylethanolamine/PE, and lysodimethylphosphatidylethanolamine/PE were upregulated, while some glycerophospholipid (GPL)-plasmalogens were downregulated in the AF-SSNHL group, indicating abnormal metabolism of GPLs. Trihexosylceramide (d34:1), PE (18:1e_22:5), and sphingomyelin (d40:3) were significantly different between responders and nonresponders, and positively correlated with hearing improvement. Additionally, the results of the WGCNA also suggested that partial GPL-plasmalogens were positively associated with hearing improvement. CONCLUSION: AF-SSNHL patients exhibited abnormally high blood lipids and pronounced GPLs metabolic abnormalities. Sphingolipids and GPL-plasmalogens had an association with the level of hearing improvement. By understanding the lipid changes, clinicians may be able to predict the prognosis of hearing recovery and personalize treatment approaches.

Clinical impact of hyperbaric oxygen therapy combined with steroid treatment for sudden sensorineural hearing loss: A case-control study.

Objectives: The aim of present study was to evaluate the clinical efficacy of hyperbaric oxygen therapy (HBOT) as a primary therapy combined with standard systemic corticosteroid treatment for sudden sensorineural hearing loss (SSNHL) compared to treatment without the use of HBOT (non-HBOT) through clinical data and advanced analytical approaches. Study Design: Case-control study. Methods: Conducted across three Japanese medical centers involving 298 SSNHL patients diagnosed between 2020 and 2023. Inclusion criteria encompassed first onset and treatment, WHO grade 3 or 4 initial hearing impairment, receipt of systemic corticosteroid therapy within 14 days of symptom onset, and initiation of HBOT within the same timeframe for the case group. The primary outcome measure was the difference in hearing improvement (mean hearing level in decibels, dB) between the two groups, assessed by pure-tone audiometry at baseline and 3 months post-treatment, using the inverse probability of treatment weighting (IPTW) method adjusted for covariate differences. Results: The study included 67 patients in the HBOT group and 68 in the non-HBOT group. The HBOT group exhibited significantly greater hearing improvement (IPTW-adjusted difference: 7.6 dB, 95% CI 0.4-14.7; p = 0.038). Patients without vertigo in the HBOT group demonstrated substantial hearing improvement (11.5 dB, 95% CI 2.3-20.6; p = 0.014), whereas those with vertigo showed no significant improvement (-1.8 dB, 95% CI -11.8-8.3; p = 0.729). The HBOT group also had a significantly higher association with complete recovery (IPTW-adjusted odds ratio: 2.57, 95% CI 1.13-5.85; p = 0.025). Conclusion: In SSHNL, HBOT combination therapy yielded slightly but significantly improved hearing outcomes compared to non-HBOT treatment. Level of Evidence: 4.

Assessment of Peripheral and Central Auditory Processing after Treatment for Idiopathic Sudden Sensorineural Hearing Loss.

Introduction When cases of idiopathic sudden sensorineural hearing loss (SSNHL) are treated successfully, most clinicians assume the normality and symmetry of the auditory processing. This assumption is based on the recovery of the detection ability on the part of the patients, but the auditory processing involves much more than detection alone. Since certain studies have suggested a possible involvement of the central auditory system during the acute phase of sudden hearing loss, the present study hypothesized that auditory processing would be asymmetric in people who have experienced sudden hearing loss. Objective To assess the physiologic and electrophysiological conditions of the cochlea and central auditory system, as well as behavioral discrimination, of three primary aspects of sound (intensity, frequency, and time) in subjects with normal ears and ears treated successfully for SSNHL. Methods The study included 19 SSNHL patients whose normal and treated ears were assessed for otoacoustic emissions, speech auditory brainstem response, intensity and pitch discrimination, and temporal resolution in a within-subject design. Results The otoacoustic emissions were poorer in the treated ears compared to the normal ears. Ear- and sex-dependent differences were observed regarding otoacoustic emissions and pitch discrimination. Conclusion The asymmetrical processing observed in the present study was not consistent with the hearing threshold values, which might suggest that the central auditory system would be affected regardless of the status of the peripheral hearing. Further experiments with larger samples, different recovery scenarios after treatment, and other assessments are required.

Clinical characteristics of patients diagnosed with bilateral sudden sensorineural hearing loss.

This study investigated the etiology, clinical features, and prognosis of patients diagnosed with bilateral sudden sensorineural hearing loss (BSSNHL). The clinical data of 100 patients with bilateral sudden hearing loss as a chief complaint treated at Xiangya Second Hospital of Central South University between January 2010 and August 2022, including clinical characteristics, audiometric data, and prognosis, were retrospectively analyzed. These 100 cases accounted for 8.09% (100/1235) of all patients admitted for sudden sensorineural hearing loss (SSNHL) during the same period. Of these, 71 were simultaneous cases and 29 were sequential cases of BSSNHL. Among the 200 ears analyzed in this study, 13, 36, 57, and 94 had mild, moderate, severe, and profound sensorineural hearing loss, respectively. The overall effective rate after comprehensive treatment was 32%, with significant differences in efficacy and prognosis among different degrees of hearing loss (p < 0.05). Comorbidities of hypertension (24 cases), diabetes (14 cases), and coronary heart disease (9 cases) significantly impacted therapeutic efficacy and prognosis in patients with BSSNHL (p < 0.05). Compared to unilateral SSNHL, BSSNHL exhibits distinctive characteristics.