Glucocorticoids（GC） are widely used in the clinical treatment of autoimmune inner ear diseases, sudden sensorineural hearing loss, Meniere\'s disease, sinusitis and other otolaryngology diseases. However, GC resistance remains a major factor contributing to the poor efficacy of clinical treatments. The mechanism of GC resistance is still unclear. This paper reviews the related mechanisms of GC resistance from the perspectives of GC receptor factors and non-GC receptor factors. Additionally, it summarizes the latest research progress on GC resistance in otolaryngological diseases, with the aim of identifying effective clinical alternative treatment options for reversing GC resistance in the future.
摘要: 糖皮质激素（glucocorticoids，GC）临床上广泛应用于自身免疫性内耳病、突发性聋、梅尼埃病及鼻窦炎等耳鼻咽喉疾病的治疗，部分患者存在GC抵抗现象，但导致GC抵抗因素机制尚不清楚，本文从GC受体因素和非GC受体因素两方面阐述了GC抵抗的相关机制，并综述其在耳鼻咽喉科疾病的最新研究进展，旨在未来寻找GC抵抗生物标志物，提高GC疗效提出新策略。.

Objective: To investigate the disease composition, clinical features, diagnosis, and treatment characteristics of vertigo in children. Methods: A total of 120 children with vertigo diagnosed and treated in the Department of Otorhinolaryngology, Children\'s Hospital, Capital Institute of Pediatrics in Beijing from February 2018 to February 2022 were retrospectively analyzed to explore the clinical characteristics of common peripheral vertigo in children and to summarize the experience of diagnosis and treatment. Results: The etiological composition of 120 cases of vertigo in children are as follows: 63 (52.5%) cases of vestibular migraine of childhood (VMC), 19 (15.8%) of recurrent vertigo of childhood (RVC), 11 (9.2%) of probable vestibular migraine of childhood (PVMC), 10 (8.3%) of secretory otitis media (SOM), 6 (5.0%) of persistent postural-perceptual dizziness (PPPD), 4 (3.3%) of benign paroxysmal positional vertigo (BPPV), 2 (1.7%) of vestibular neuritis (VN), 2 (1.7%) of Meniere\'s disease (MD), 2 (1.7%) of inner ear malformation (IEM), and 1 (0.8%) of vestibular paroxysmal syndrome (VP).The major cause of vertigo in children of different ages was different. SOM was the most important cause in preschool children, followed by RVC and VMC; VMC was the most important cause in school-age children, followed by RVC; and MD and BPPV were exclusive found in adolescents. The incidence rate of PPPD was higher in adolescents than in preschool and school-age children. Children with vertigo had good prognosis in general. Conclusions: VMC, RVC and SOM are the most common causes in vertigo in children, and their proportion was different in different aged children. Transforming abstract feelings into specific information is the skill required for collecting medical history of children with vertigo. Considering the age and cooperation of children, appropriate hearing and vestibular examination techniques are recommended. We should pay more attention to the mental health of children with vertigo and their parents.
目的： 了解儿童眩晕的病因构成、临床表现及诊疗特点。 方法： 病例系列研究。回顾性分析2018年2月至2022年2月首都儿科研究所附属儿童医院耳鼻喉科诊治的120例眩晕患儿的临床资料，探讨儿童常见眩晕的临床特点、总结诊疗经验。 结果： （1）120例儿童眩晕的病因构成：儿童前庭性偏头痛（VMC）63例（52.5%）、儿童复发性眩晕（RVC）19例（15.8%），可能性前庭性偏头痛（PVMC）11例（9.2%），分泌性中耳炎（SOM）10例（8.3%），持续性姿势-感知性头晕（PPPD）6例（5.0%）、良性阵发性位置性眩晕（BPPV）4例（3.3%），前庭神经炎（VN）2例（1.7%），梅尼埃病（MD）2例（1.7%），内耳先天性发育畸形2例（1.7%）、前庭阵发症（VP）1例（0.8%）。（2）不同的年龄阶段，儿童眩晕的病因构成不同，学龄前儿童最主要的是SOM，之后是RVC和VMC；学龄儿童则主要是VMC，之后是RVC；相较于其他年龄段，青少年期MD、BPPV，尤其是PPPD发病率更高。（3）眩晕患儿的整体预后较好。 结论： 眩晕患儿病因以VMC、RVC、SOM为主，在不同的年龄阶段病因不同。采集眩晕患儿病史的技巧是将抽象的感受转化为具体的事项。根据儿童的年龄和配合度，选择适合的听力和前庭检查，并关注眩晕患儿及家长的心理健康，将有助于临床诊治。.

BACKGROUND: Ménière\'s disease (MD) is a disorder of the inner ear that causes episodic bouts of severe dizziness, roaring tinnitus, and fluctuating hearing loss. To date, no targeted therapy exists. As such, we have undertaken a large whole genome sequencing study on carefully phenotyped unilateral MD patients with the goal of gene/pathway discovery and a move towards targeted intervention. This study was a retrospective review of patients with a history of Ménière\'s disease. Genomic DNA, acquired from saliva samples, was purified and subjected to whole genome sequencing.
RESULTS: Stringent variant calling, performed on 511 samples passing quality checks, followed by gene-based filtering by recurrence and proximity in molecular interaction networks, led to 481 high priority MD genes. These high priority genes, including MPHOSPH8, MYO18A, TRIOBP, OTOGL, TNC, and MYO6, were previously implicated in hearing loss, balance, and cochlear function, and were significantly enriched in common variant studies of hearing loss. Validation in an independent MD cohort confirmed 82 recurrent genes. Pathway analysis pointed to cell-cell adhesion, extracellular matrix, and cellular energy maintenance as key mediators of MD. Furthermore, the MD-prioritized genes were highly expressed in human inner ear hair cells and dark/vestibular cells, and were differentially expressed in a mouse model of hearing loss.
CONCLUSIONS: By enabling the development of model systems that may lead to targeted therapies and MD screening panels, the genes and variants identified in this study will inform diagnosis and treatment of MD.

BACKGROUND: Diagnostic dilemma between clinical Meniere\'s disease and radiological endolymphatic hydrops (EH) has emerged since the introduction of hydrops magnetic resonance imaging (MRI). The aim of this study is to explore the potential application of hydrops MRI on diagnosing the EH.
METHODS: This review was developed from peer-reviewed articles published in those journals listed on journal of citation reports. The MEDLINE database of the US National Library of Medicine, Scopus, and Google Scholar were used to collect articles based on the guidelines (PRISMA 2020 statement) for reporting reviews.
RESULTS: Initially, 470 articles were retrieved from 1983 to 2023, and 80 relevant articles were ultimately selected. The sensitivity (69%-92%) and specificity (78%-96%) values varied from each laboratory for detecting EH via hydrops MRI, probably due to candidate selection and the grading system employed.
CONCLUSIONS: The application of hydrops MRI allows (1) differentiation between EH and sudden sensorineural hearing loss; (2) determination of the affected side of EH; and (3) confirmation of the diagnosis of EH concomitant with other disorders. Notably, not all differentials for EH can be visualized on MR images. One of the existing gaps to be filled is that updated hydrops MRI fails to identify distortion, that is, rupture, collapse, fistula, or fibrosis of the inner ear compartments, akin to what histopathological evidence can demonstrate. Hence, enhanced ultrahigh resolution of hydrops MRI is required for demonstrating fine structures of the inner ear compartments in the future.

OBJECTIVE: To uncover the context that allowed for the vestibular neurectomy to grow in favor and practice at the Johns Hopkins Hospital in the early 20th century, and the reasons for its broad abandonment since.
METHODS: The Walter E. Dandy (1905-1946) and Samuel J. Crowe collections (1905-1920) at the Alan Mason Chesney Medical Archives were reviewed, as well as the Samuel J. Crowe and Stacy Guild Temporal Bone Collection.
RESULTS: Speculation on the etiology of Menière\'s disease (MD) has been countless, as have the medical and surgical interventions aimed at treating it. At the Johns Hopkins Hospital, Walter Dandy popularized the neurectomy for MD and performed 692 procedures from 1924 to 1946, believing it to be a curative therapy for vertigo. When he later modified the procedure from a total cranial nerve section to a partial vestibular neurectomy preserving auditory function, surgical candidacy expanded to include nearly any patient with vestibular symptoms. After his passing, trainees\' attention shifted to traumatic injuries, likely influenced by WWII. This left the procedure scarcely used until third parties rekindled interest decades later.
CONCLUSIONS: Neurectomy as the preferential treatment for MD at the Johns Hopkins Hospital was not driven by pure scientific reasoning but was rather contingent on historical context and sponsorship by a prominent figure like Walter Dandy. Appreciation of MD\'s natural history has since curtailed the favorability of destructive procedures in preference for conservative management.

UNASSIGNED: Previous studies have highlighted associations between certain inflammatory cytokines and Ménière\'s Disease (MD), such as interleukin (IL) -13 and IL-1β. This Mendelian randomization aims to comprehensively evaluate the causal relationships between 91 inflammatory cytokines and MD.
UNASSIGNED: A comprehensive two-sample Mendelian randomization (MR) analysis was conducted to determine the causal association between inflammatory cytokines and MD. Utilizing publicly accessible genetic datasets, we explored causal links between 91 inflammatory cytokines and MD risk. Comprehensive sensitivity analyses were employed to assess the robustness, heterogeneity, and presence of horizontal pleiotropy in our findings.
UNASSIGNED: Our findings indicate that MD causally influences the levels of two cytokine types: IL-10 (P=0.048, OR=0.945, 95%CI =0.894~1.000) and Neurotrophin-3 (P=0.045, OR=0954, 95%CI =0.910~0.999). Furthermore, three cytokines exhibited significant causal effects on MD: CD40L receptor (P=0.008, OR=0.865, 95%CI =0.777-0.963), Delta and Notch-like epidermal growth factor-related receptor (DNER) (P=0.010, OR=1.216, 95%CI =1.048-1.412), and STAM binding protein (P=0.044, OR=0.776, 95%CI =0.606-0.993).
UNASSIGNED: This study suggests that the CD40L receptor, DNER, and STAM binding protein could potentially serve as upstream determinants of MD. Furthermore, our results imply that when MD is regarded as the exposure variable in MR analysis, it may causally correlate with elevated levels of IL-10 and Neurotrophin-3. Using these cytokines for MD diagnosis or as potential therapeutic targets holds great clinical significance.

OBJECTIVE: To investigate the safety and feasibility of precise delivery of a long-acting gel formulation containing 6% dexamethasone (SPT-2101) to the round window membrane for the treatment of Menière\'s disease.
METHODS: Prospective, unblinded, cohort study.
METHODS: Tertiary care neurotology clinic.
METHODS: Adults 18 to 85 years with a diagnosis of unilateral definite Menière\'s disease per Barany society criteria.
METHODS: A single injection of a long-acting gel formulation under direct visualization into the round window niche.
METHODS: Procedure success rate, adverse events, and vertigo control. Vertigo control was measured with definitive vertigo days (DVDs), defined as any day with a vertigo attack lasting 20 minutes or longer.
RESULTS: Ten subjects with unilateral Menière\'s disease were enrolled. Precise placement of SPT-2101 at the round window was achieved in all subjects with in-office microendoscopy. Adverse events included one tympanic membrane perforation, which healed spontaneously after the study, and two instances of otitis media, which resolved with antibiotics. The average number of DVDs was 7.6 during the baseline month, decreasing to 3.3 by month 1, 3.7 by month 2, and 1.9 by month 3. Seventy percent of subjects had zero DVDs during the third month after treatment.
CONCLUSIONS: SPT-2101 delivery to the round window is safe and feasible, and controlled trials are warranted to formally assess efficacy.

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BACKGROUND: The vestibular aqueduct (VA) serves an essential role in homeostasis of the inner ear and pathogenesis of Ménière\'s disease (MD). The bony VA can be clearly depicted by high-resolution computed tomography (HRCT), whereas the optimal sequences and parameters for magnetic resonance imaging (MRI) are not yet established. We investigated VA characteristics and potential factors influencing MRI-VA visibility in unilateral MD patients.
METHODS: One hundred patients with unilateral MD underwent MRI with three-dimensional sampling perfection with application optimized contrasts using different flip angle evolutions (3D-SPACE) sequence and HRCT evaluation. The imaging variables included MRI-VA and CT-VA visibility, CT-VA morphology and CT-peri-VA pneumatization.
RESULTS: The most frequent type of MRI-VA and CT-VA visualization was invisible VA and continuous VA, respectively. The MRI-VA visibility was significantly lower than CT-VA visibility. MRI-VA visibility had a weak positive correlation with ipsilateral CT-VA visualization. For the affected side, the MRI-VA visualization was negatively correlated with the incidence of obliterated-shaped CT-VA and positively with that of tubular-shaped CT-VA. MRI-VA visualization was not affected by CT-peri-VA pneumatization.
CONCLUSIONS: In patients with MD, the VA visualization on 3D-SPACE MRI is poorer than that observed on CT and may be affected by its osseous configuration. These findings may provide a basis for further characterization of VA demonstrated by MRI and its clinical significance.

暂无摘要。