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Ménière's disease (MD) is characterized by loss of balance and hearing disorders. Although there is known to be endolymphatic hydrops involved in the pathological process, the pathogenesis of the disease is still largely unclear. Approximately half of patients with MD suffer from depressive symptoms and high levels of several stress hormones were observed in MD and depression, simultaneously. Recently, gut microbes have been shown to influence the function of the central nervous system in humans through their metabolites. We studied the intestinal microbiota of 10 patients with MD and 11 healthy donors (HD). Significant negative correlations were found between disease duration and alpha diversity indexes of gut microbes in patients with MD. The relative abundance of the species Butyricicoccus ambiguous taxa was increased in patients with MD compared with that of HD. In contrast, Oscillospiraceae UCG-002/UCG-005 ambiguous taxa and Anaerovoracaceae (Eubacterium) brachy group uncultured bacterium were increased in the relative abundance of HD than that of patients with MD. The relative abundance of the Butyricicoccus species was positively correlated with disease duration. Thus, these compositional alterations of gut microbes in patients with MD are associated with inner ear pathologies, such as endolymphatic hydrops, by changing the metabolite profiles in the intestine., Competing Interests: Declarations. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

The persistent stapedial artery (PSA) is an exceedingly vascular anomaly that can lead to hearing loss or pulsatile tinnitus, yet its preoperative diagnosis is often challenging. We report the case of a 24-year-old woman with bilateral PSA and stapes ankylosis who presented with progressive bilateral mixed hearing loss. The patient was initially diagnosed with bilateral congenital stapes ankylosis and stapes surgery was performed on the left side using transcanal endoscopic ear surgery (TEES). Intraoperative observation of the tympanic cavity revealed the presence of a persistent stapedial artery. The stapes surgery was completed successfully while preserving the stapedial artery, resulting in postoperative hearing improvement. Subsequently, surgery was planned for the right ear. Suspecting the possibility of PSA, we performed a contrast CT scan; additionally, we prepared subtraction images and CT angiography. These images strongly suggested the presence of the PSA on the right side as well. This case highlights the superior surgical visualization provided by TEES and underscores the effectiveness of advanced imaging techniques in diagnosing PSA., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Sasano et al.)

Objective: In the present study, we examined the effects of high-dose betahistine on dizziness handicap inventory (DHI) scores in patients with unilateral vestibulopathy., Methods: An uncontrolled, open-label, multicenter clinical trial was conducted. Fifteen patients with unilateral vestibulopathy, such as vestibular neuritis, who complained of intractable dizziness for more than three months were enrolled. Initially, all patients were orally administered betahistine at a dose of 36 mg/day for four weeks, which is the standard dose and dosing period for the treatment of dizziness in Japan. The patients were then administered betahistine at a double dose of 72 mg/day for four weeks. Six patients who became aware of the benefits of high-dose betahistine were further administered betahistine at 72 mg/day for an additional 12 weeks (a total of 16 weeks). Perceived disability due to dizziness was assessed by DHI scores., Results: In all 15 patients, short-term administration with high-dose (72 mg/day) betahistine for four weeks, but not low-dose betahistine (36 mg/day) for four weeks significantly decreased DHI scores. In particular, in six responding patients with self-reported benefits after short-term administration with high-dose betahistine, long-term administration with high-dose betahistine for 16 weeks further significantly decreased DHI scores. However, DHI scores of the remaining nine non-responding patients were not changed after short-term administration with high-dose betahistine for four weeks., Conclusion: Short-term administration with the standard dose and dosing period of betahistine did not improve DHI scores in the enrolled patients, indicating that they were not compensated for unilateral vestibulopathy with intractable dizziness. The present findings suggest that long-term administration with high-dose betahistine facilitates vestibular compensation to improve intractable dizziness in some, but not all patients with uncompensated unilateral vestibulopathy., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest in this study., (Copyright © 2023. Published by Elsevier B.V.)

Objective: To provide diagnostic and therapeutic strategies for vestibular neuritis in accordance with the Japanese Clinical Practice Guidelines for Vestibular Neuritis 2021., Methods: The Committee for Clinical Practice Guidelines for Vestibular Neuritis was entrusted with a review of the relevant scientific literature on the above topic. Clinical Questions (CQs) concerning the treatment of vestibular neuritis were produced, and a search of the literature was conducted to identify studies related to the CQs. The recommendations were based on the literature review and the expert opinion of a subcommittee., Results: We proposed the diagnostic criteria for vestibular neuritis, as well as answers to CQs, recommendations, and evidence levels for the treatment of vestibular neuritis., Conclusion: The diagnostic criteria for vestibular neuritis were based on clinical history and examination findings after completing the differential diagnosis process. The treatment of vestibular neuritis was divided into acute, subacute, and chronic stages. The Japanese Clinical Practice Guidelines for Vestibular Neuritis 2021 should be used as a reference in the diagnosis and treatment of vestibular neuritis., Competing Interests: Declaration of Competing Interest None, (Copyright © 2022. Published by Elsevier B.V.)

A perilymphatic fistula (PLF) presents with abnormal traffic in the otic capsule, causing cochlear and vestibular symptoms. However, the mechanisms underlying symptom recurrence remain controversial. Herein, we report the case of a 27-year-old female who complained of hearing disturbance in her right ear and recurrent vertigo after sudden onset of hearing loss with vertigo. The caloric test revealed unilateral weakness in the right ear, and the video head impulse test (vHIT) showed decreased vestibulo-ocular reflex (VOR) gain. Contrast-enhanced magnetic resonance imaging (MRI) using hybrid of reversed image of positive endolymph signal and negative image of perilymph signal (HYDROPS) indicated a collapsed endolymphatic space. As the vestibular symptoms did not improve, an exploratory tympanotomy was performed on the right ear. Although perilymph leakage was not noted in the oval or round windows, both windows were sealed with connective tissue. The patient's vestibular symptoms rapidly improved after surgery, and postoperative contrast-enhanced MRI showed improvement in the collapsed endolymphatic space. Although the caloric test revealed unilateral weakness, the VOR gain on the vHIT improved to normal on the right side. Thus, these findings indicated that recurrent symptoms caused by PLF are associated with a collapsed endolymphatic space. We speculate that the collapsed endolymphatic space was due to a ruptured Reissner's membrane. We hypothesized that sealing the fistula would promote normalization of perilymph pressure. The ruptured Reissner's membrane may have been gradually repaired as vestibular symptoms improved. This case adds to the existing literature on the occurrence of the "double-membrane break syndrome". Collapse of the endolymph due to a ruptured Reissner's membrane may be the cause of PLF symptoms., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Ito, Seo, Sasano, Mochizuki and Koizuka.)

Background: Delayed endolymphatic hydrops (DEH) is an inner ear disease that causes recurrent vertigo in the ipsilateral ear or fluctuating hearing in the contralateral ear due to endolymphatic hydrops secondary to preceding deafness. There are few reports of large, multicentre studies investigating the clinical-epidemiological characteristics of DEH., Objective: This study aimed to clarify the characteristics of DEH in Japan., Methods: Clinical data on 662 patients with DEH were analysed by nationwide, multicentre surveys conducted by the Peripheral Vestibular Disorders Research Group of Japan., Results: The proportion of ipsilateral DEH (IDEH) was slightly higher than that of contralateral DEH (CDEH) at 55.4%. The time delay between onset of precedent deafness and onset of DEH was significantly longer for CDEH than for IDEH. The most common cause of precedent deafness was a disease of unknown cause with onset in early childhood (33.1%). Epidemiological characteristics were not significantly different between CDEH with and without vertigo., Conclusion: DEH appearing to be caused by viral labyrinthitis has a high rate of onset within 40 years of precedent deafness. Clinical and epidemiological characteristics of IDEH, CDEH with vertigo, and CDEH without vertigo were very similar., Significance: The clinical-epidemiological characteristics of DEH in Japan were clarified.

Background: The aetiology of vestibular migraine (VM) has not yet been defined; endolymphatic hydrops (EH) has been suggested as a candidate., Objectives: This study aimed to clarify the relationship between VM and EH using neuro-otological tests, including the EH presumption test., Materials and Methods: Fourteen patients with VM underwent caloric testing, video head impulse test (vHIT), cervical and ocular vestibular evoked myogenic potential (cVEMP and oVEMP), and EH presumption tests such as the Futaki's test and furosemide loading VEMP., Results: Caloric testing was abnormal in two of the 14 cases (14.3%), and vHIT was abnormal in one of 12 cases (8.3%). Abnormal asymmetry ratios (ARs) of cVEMP and oVEMP were observed in two of 14 cases (14.3%) and six of 13 cases (46.2%), respectively. Futaki's test results were positive in five of 14 cases (35.7%). Furosemide loading VEMP was positive in seven of 14 cases (50.0%). Nine patients (64.3%) were positive for at least one EH presumption test., Conclusions and Significance: EH is not a rare finding in VM; however, the ratio is less than that in Meniere's disease.