Dataset Information

Autoinflammatory characteristics and short-term effects of delivering high-dose steroids to the surface of the intact endolymphatic sac and incus in refractory Meniere's disease.

ABSTRACT: Objective:To investigate immune-related genetic background in intractable Meniere's disease (MD) and the immediate results of a novel therapy by delivering steroids to the surface of the intact endolymphatic sac (ES) and incus in a sustainable manner. Case report and methods:Candidate genes involved in immune regulation were sequenced using a next-generation sequencing method in a patient with intractable MD. Mutations were confirmed using the Sanger sequencing method. The ES was exposed, and gelatin sponge particles were immersed in high-dose methylprednisolone solution and placed onto the surface of ES. "L"-shaped gelatin sponge strips were immersed in dexamethasone solution and served as a guiding device for the steroids by touching the incus and gelatin sponge particles on the surface of the ES. Gelatin sponge particles immersed in dexamethasone solution were placed around the gelatin sponge strips and sealed using fibrin glue. Results:Autoinflammation in the refractory MD case was indicated by genotype, including novel heterozygous mutations of PRF1, UNC13D, SLC29A3, ITCH, and JAK3, as well as phenotype. The vertigo was fully relieved immediately after operation. Tinnitus and aural fullness were resolved 3 weeks after operation, whereas hearing improved in 2 mon postoperation. No recurrence was noted during the 5-monfollow-up, and the final MRI supported the novel therapeutic hypothesis. Conclusion:Autoinflammation was involved in a refractory MD. This novel therapy, which involves the delivery of steroids to the surface of the intact ES and incus, is effective in relieving vertigo and tinnitus and improves hearing function of refractory MD.

SUBMITTER: Zou J

PROVIDER: S-EPMC6570643 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

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Autoinflammatory characteristics and short-term effects of delivering high-dose steroids to the surface of the intact endolymphatic sac and incus in refractory Ménière's disease.

Zou Jing J

Journal of otology 20190106 2

<h4>Objective</h4>To investigate immune-related genetic background in intractable Meniere's disease (MD) and the immediate results of a novel therapy by delivering steroids to the surface of the intact endolymphatic sac (ES) and incus in a sustainable manner.<h4>Case report and methods</h4>Candidate genes involved in immune regulation were sequenced using a next-generation sequencing method in a patient with intractable MD. Mutations were confirmed using the Sanger sequencing method. The ES was ...[more]

PMID: 31223300

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Project description:IntroductionOutcomes of surgery for Ménière's disease (MD) remain discordant. Recently, a new surgical procedure in which the endolymphatic duct is clipped was proposed. To date, only one prospective trial assessing this technique was published, yielding promising results. This protocol describes a prospective, double-blinded, randomised controlled trial that will be carried out to assess the effectiveness of this surgical intervention.MethodsEighty-four patients with intractable MD will be recruited from 13 hospitals in the Netherlands. Intraoperatively, randomisation will determine whether endolymphatic duct blockage (EDB) or endolymphatic sac decompression (ESD) will be performed. Randomisation will be 1:1 stratified for gender and duration of MD (recent-onset versus mature MD). All participants receive vestibular rehabilitation after surgery. Patients are followed up during 1 year after surgery. Follow-up visits will take place at 1 week, 3 months, 6 months and 12 months after surgery. The main study endpoint is proportion of patients who are free of vertigo spells at 12 months postoperatively. Secondary parameters include cumulative number of vertigo bouts, co-intervention, tinnitus, hearing, quality of life, cost effectiveness and a budget impact analysis. Total duration of the study is 4 years.AnalysisThe primary analysis will follow the intention-to-treat principle. For the primary outcome, a χ2 test will be performed. Secondary outcomes will be analysed using a linear mixed model (EDB versus decompression group) at the different time measurement point.Ethics and disseminationThis study was reviewed and approved by a board of specialists before funding was obtained, as well as by the Medical Research Ethics Committee Leiden-The Hague-Delft and the boards of all participating centres. Results of this study will be published in international peer-reviewed scientific journals and will be presented on (inter)national scientific conferences and meetings.Trial registration numbersNL9095 and ISRCTN12074571; Pre-Results.

Project description:Objective: Intravenous contrast agent enhanced, high-resolution magnetic resonance imaging of the inner ear (iMRI) confirmed that patients with Menière's disease (MD) and vestibular migraine (VM) could present with endolymphatic hydrops (EH). The present study aimed to investigate EH characteristics and their interrelation to neurotologic testing in patients with VM, MD, or VM with concurrent MD (VM-MD). Methods: Sixty-two patients (45 females, aged 23-81 years) with definite or probable VM (n = 25, 19 definite), MD (n = 29, 17 definite), or showing characteristics of both diseases (n = 8) were included in this study. Diagnostic workup included neurotologic assessments including video-oculography (VOG) during caloric stimulation and head-impulse test (HIT), ocular and cervical vestibular evoked myogenic potentials (o/cVEMP), pure tone audiometry (PTA), as well as iMRI. EH's degree was assessed visually and via volumetric quantification using a probabilistic atlas-based segmentation of the bony labyrinth and volumetric local thresholding (VOLT). Results: Although a relevant number of VM patients reported varying auditory symptoms (13 of 25, 52.0%), EH in VM was only observed twice. In contrast, EH in VM-MD was prevalent (2/8, 25%) and in MD frequent [23/29, 79.3%; χ2(2) = 29.1, p < 0.001, φ = 0.7]. Location and laterality of EH and neurophysiological testing classifications were highly associated (Fisher exact test, p < 0.005). In MD, visual semi-quantitative grading and volumetric quantification correlated highly to each other (r S = 0.8, p < 0.005, two-sided) and to side differences in VOG during caloric irrigation (vestibular EH ipsilateral: r S = 0.6, p < 0.05, two-sided). In VM, correlations were less pronounced. VM-MD assumed an intermediate position between VM and MD. Conclusion: Cochlear and vestibular hydrops can occur in MD and VM patients with auditory symptoms; this suggests inner ear damage irrespective of the diagnosis of MD or VM. The EH grades often correlated with auditory symptoms such as hearing impairment and tinnitus. Further research is required to uncover whether migraine is one causative factor of EH or whether EH in VM patients with auditory symptoms suggests an additional pathology due to MD.

Dataset Information

Autoinflammatory characteristics and short-term effects of delivering high-dose steroids to the surface of the intact endolymphatic sac and incus in refractory Meniere's disease.

Publications

Autoinflammatory characteristics and short-term effects of delivering high-dose steroids to the surface of the intact endolymphatic sac and incus in refractory Ménière's disease.

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