OBJECTIVE: To evaluate the differences between audio-vestibular function testing and inner ear gadolinium magnetic resonance imaging (MRI) in distinguishing definite Ménière disease (DMD) and probable Ménière disease (PMD), and to provide a reference for early clinical diagnosis and intervention.
METHODS: A total of 116 patients diagnosed with DMD (n = 80) and PMD (n = 36) were enrolled. The differences in the results of pure tone audiometry, caloric test, and tympanic injection of gadolinium for contrast-enhanced MRI between the two groups were compared and analyzed. Parameters that could differentiate between the two conditions were identified, and the sensitivity and specificity and the area under the curve (AUC) of individual and combined indices in the differential diagnosis of DMD and PMD were evaluated.
RESULTS: The hearing threshold and hearing asymmetry rate of the DMD group were significantly higher than those of the PMD group (p < 0.001), 98.8% and 30.6%, respectively. The abnormal rates of canal paresis (CP) and severity of endolymphatic hydrops in the DMD group were higher than those in the PMD group (p < 0.05). When combined with high-frequency hearing thresholds, hearing asymmetry, hearing curve type, endolymphatic hydrops, and abnormal CP, the diagnostic accuracy of DMD was improved compared to using high-frequency alone (p < 0.05).
CONCLUSIONS: This study showed that PMD and DMD may represent two different stages in the development of MD disease. The comprehensive assessment of audio-vestibular function testing and inner ear MRI proves beneficial for early diagnosis, potentially contributing to the preservation of inner ear function.

OBJECTIVE: To explore the microstate characteristics and underlying brain network activity of Ménière\'s disease (MD) patients based on high-density electroencephalography (EEG), elucidate the association between microstate dynamics and clinical manifestation, and explore the potential of EEG microstate features as future neurobiomarkers for MD.
METHODS: Thirty-two patients diagnosed with MD and 29 healthy controls (HC) matched for demographic characteristics were included in the study. Dysfunction and subjective symptom severity were assessed by neuropsychological questionnaires, pure tone audiometry, and vestibular function tests. Resting-state EEG recordings were obtained using a 256-channel EEG system, and the electric field topographies were clustered into four dominant microstate classes (A, B, C, and D). The dynamic parameters of each microstate were analyzed and utilized as input for a support vector machine (SVM) classifier to identify significant microstate signatures associated with MD. The clinical significance was further explored through Spearman correlation analysis.
RESULTS: MD patients exhibited an increased presence of microstate class C and a decreased frequency of transitions between microstate class A and B, as well as between class A and D. The transitions from microstate class A to C were also elevated. Further analysis revealed a positive correlation between equilibrium scores and the transitions from microstate class A to C under somatosensory challenging conditions. Conversely, transitions between class A and B were negatively correlated with vertigo symptoms. No significant correlations were detected between these characteristics and auditory test results or emotional scores. Utilizing the microstate features identified via sequential backward selection, the linear SVM classifier achieved a sensitivity of 86.21% and a specificity of 90.61% in distinguishing MD patients from HC.
CONCLUSIONS: We identified several EEG microstate characteristics in MD patients that facilitate postural control yet exacerbate subjective symptoms, and effectively discriminate MD from HC. The microstate features may offer a new approach for optimizing cognitive compensation strategies and exploring potential neurobiological markers in MD.

BACKGROUND: Dizziness/vertigo is one of the most common symptoms for which people seek healthcare. However, the healthcare expenditure attributable to dizziness/vertigo in South Korea remains poorly understood. We investigated the healthcare costs due to six major disorders causing dizziness/vertigo using claims data.
METHODS: The healthcare costs were evaluated using all the claims data submitted to the Health Insurance Review and Assessment Service from January 1 to December 31, 2022. The six major vestibular disorders included for analysis were benign paroxysmal positional vertigo (BPPV), psychogenic/persistent postural perceptual dizziness (PPPD), vascular vertigo/dizziness (VVD), vestibular migraine (VM), Meniere\'s disease (MD), and vestibular neuritis (VN).
RESULTS: During the 1-year study period, 4.1% of adults aged 20 or older visited hospitals due to dizziness/vertigo in South Korea. Compared to the general population, the patients with dizziness/vertigo were more often elderly, female, and residents of small towns. The total healthcare cost for the six major vestibular disorders was ₩547.8 billion (approximately $406.5 million). BPPV incurred the highest annual healthcare cost (₩183.5 billion, 33.5%), followed by VVD (₩158.8 billion, 29.0%), MD (₩82.2 billion, 15.0%), psychogenic/PPPD (₩60.3 billion, 11.0%), VN (₩32.9 billion, 6.0%), and VM (₩30.1 billion, 5.5%). The mean healthcare cost per hospital visit due to dizziness/vertigo was ₩96,524 (95% confidence interval, ₩96,194-₩96,855), 30% higher than the average (₩73,948) of the overall healthcare cost per hospital visit over the same period.
CONCLUSIONS: Owing to higher healthcare costs for dizziness/vertigo and increased prevalence of dizziness/vertigo in the aged population, healthcare costs due to dizziness/vertigo will increase rapidly in South Korea. Thus, a guideline for cost-effective management of dizziness/vertigo should be established to reduce the healthcare costs due to these common symptoms.

Bevezetés: A Ménière-betegség gyógyíthatatlan, de menedzselhető betegség. Alapja az endolympha terének „kitágulása”, az endolympha hydrops. Lehet egy- vagy kétoldali. A tünetek megjelenése alapján metakrón vagy szinkrón megjelenésről beszélhetünk. Hasonló tünetekkel jelentkezhet az autoimmun belsőfül-betegség. Célkitűzés: Kétoldali Ménière-betegséggel diagnosztizált betegek (7 beteg: 2 férfi és 5 nő) belső fülének feltérképezése, immunológiai statusuk felderítése, továbbá az egyéb kórképektől, főleg az autoimmun belsőfül-betegségtől való differenciálás, tapasztalatok alapján egy kivizsgálási protokoll felállítása. Módszer: Kétoldali Ménière-betegeknél rohammentes állapotban a fül-orr-gégészeti, otoneurológiai és immunológiai status, valamint az anamnézis felvétele, továbbá az angularis vestibuloocularis reflex (aVOR) nagy és kis frekvenciájú működésének felmérése. Az előbbit videofejimpulzus-teszt (vHIT), míg az utóbbit kalorizációs teszt során vizsgáltuk. Tisztahang-küszöbaudiogramot végeztünk. Mágneses rezonanciás képalkotó segítségével koponyafelvétel készült. Az immunológiai laborvizsgálat vérszérumból történt. Eredmények: A betegeknél szisztémás autoimmun betegség nem igazolódott. Minden esetben kétoldali, a mély frekvenciákat is érintő pancochlearis sensorineuralis halláscsökkenés volt látható. vHIT során két esetben a magas frekvenciájú aVOR érintettsége is igazolható volt, melyet a betegség előrehaladottságának véleményeztünk. A többi esetben ép működésű, magas frekvenciájú aVOR-t találtunk. A kalorizációs teszt során minden esetben kétoldali ívjáratparesis volt látható. Megbeszélés: A szakirodalomban számos tanulmány foglalkozik az általunk megfigyelt, a vHIT és a kalorizációs teszt közötti diszkrepanciával. Ez az aVOR receptorának, a crista ampullarisnak az anatómiájából következhet: míg a magas frekvenciájú aVOR-t a centrálisan elhelyezkedő I-es típusú szőrsejtek érzékelik, addig az alacsony frekvenciájú aVOR-t a perifériásan elhelyezkedő II-es típusú szőrsejtek. Az utóbbiak szelektív károsodását figyelték meg Ménière-betegségben és autoimmun belsőfül-betegségben is. Ugyanakkor segített a differenciálásban a betegek rendezett immunológiai statusa, az intravénás kortikoszteroidra adott gyenge válaszuk, egy esetben pedig a saccotomiát követő állapotjavulás. Következtetés: A tapasztalatok alapján egy kivizsgálási protokollt kíséreltünk meg felállítani olyan betegek esetében, akik kétoldali fültünetekkel, rohamokban jelentkező, forgó jellegű szédüléssel jelentkeznek. Javasoljuk többek között a vHIT, a kalorizációs teszt és az immunológiai kivizsgálás elvégzését is. Megfontolandónak tartjuk egy multicentrikus vizsgálat elvégzését is mindkét kórképpel kapcsolatban. Orv Hetil. 2024; 165(30): 1176–1183.

UNASSIGNED: Meniere disease, characterized by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure, is a common cause of vertigo in humans. The pathogenesis of Meniere disease remains unknown. The current study aimed to describe a novel pathological change discovered in the inner ears of patients with Meniere disease who underwent labyrinthectomy.
UNASSIGNED: This retrospective case-control study was conducted with 21 patients with MD who underwent labyrinthectomy. A total of 15 patients diagnosed with acoustic neuroma or glomus jugular tumor were review over the same period of time as control. The clinical information of the patients and the pathological features of the membrane are described.
UNASSIGNED: The new pathological tissue was a morbid membrane structure sealing the round window, characterized by the formation of lymphatic capillaries. Histochemical and immunofluorescent staining was positive for D2-40, LYVE-1, podoplanin, and PROX1, which are the classical markers of the lymphatic vessels. Transmission electron microscopy revealed that the lymph capillaries lacked a typical basement membrane and that their ends were blind, composed of a single layer of endothelial cells with valval connection structures between adjacent capillary epithelial cells.
UNASSIGNED: This is the first report of lymphatic vessels in the human inner ear, and this pathological structure is a completely new discovery. The lymphatic vessels may develop due to inflammation or decompensation of pressure in the inner ear, suggesting that the inner ear can reactively form lymphatic vessels in some inflammation and fluid flow-dependent pathological conditions. The current findings help in improving our understanding of the pathogenesis of Meniere disease.

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 systematically review how audiometric data change over time in patients with Menière\'s disease (MD) undergoing non-ablative medical therapy.
UNASSIGNED: Medline (via PubMed), Scopus, Web of Science, Cumulated Index to Nursing and Allied Health Literature (CINAHL), Google Scholar.
METHODS: A systematic review and meta-analysis of the literature was performed. Adult patients undergoing non-ablative medical therapy and reported duration of disease or follow-up were included and pooled estimates of pure-tone average (PTA) were tabulated. Studies were excluded if they did not use established MD, did not have pure-tone average (PTA) audiometric data, underwent ear surgery or ablative therapies, and were systematic reviews or case reports.
RESULTS: Out of 198 articles meeting full eligibility, 13 studies, involving 950 patients with MD, were included in the review and further analyzed. No effect on progression of PTA from initial diagnosis was seen between the different medical therapies within 2 years of non-ablative medical treatment. There was a significant worsening of PTA after 2 year, regardless of treatment used. High levels of heterogeneity among studies were noted up to 6 months from diagnosis ( I2 = 79%), likely reflecting differences in patient characteristics, treatment regimens, and study design. Overall, the risk of bias was low for the majority of included studies.
CONCLUSIONS: Patients diagnosed with MD who are undergoing non-ablative medical therapy should be counseled on the likelihood of worsening of hearing loss over the course of the disease despite elected treatment.

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.