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Case Reports
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Gaze-holding nystagmus in chronic progressive external ophthalmoplegia: a case report
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Eugene Jung, Seo-Young Choi, Jae Hwan Choi, Kwang-Dong Choi
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Res Vestib Sci. 2025;24(1):50-53. Published online March 14, 2025
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DOI: https://doi.org/10.21790/rvs.2025.005
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Abstract
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Supplementary Material
- We report a patient with chronic progressive external ophthalmoplegia (CPEO) who developed bilateral horizontal gaze-holding nystagmus, a previously unreported phenomenon. Video-oculography showed marked slowing of horizontal and vertical saccades, and bilateral horizontal gaze-evoked nystagmus with decreasing-velocity slow-phases waveforms. Horizontal rebound nystagmus was also observed. No abnormalities were identified in the brainstem or cerebellum on brain magnetic resonance imaging. A genetic analysis demonstrated a heterozygous missense mutation c.311A>G (p. D104G, rs28999114) in the SLC25A4 gene. Twitch motoneurons of the global layer receive inputs from premotor areas involved in the generation of eye movement, such as saccadic burst neurons, while non-twitch motoneurons of the orbital layer receive inputs from the medullary structures concerned with gaze-holding. In our patient with CPEO, the presence of omnidirectional ophthalmoplegia and bilateral horizontal gaze-holding nystagmus may indicate the involvement of the global layers of all extraocular muscles, as well as the orbital layers of the horizontal extraocular muscles.
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Miller Fisher syndrome initially presented as bilateral internuclear ophthalmoplegia: a case report
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Sanghyun Kim, Jusuck Lee, Daewoong Bae
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Res Vestib Sci. 2025;24(1):46-49. Published online March 14, 2025
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DOI: https://doi.org/10.21790/rvs.2024.028
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Abstract
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- Miller Fisher syndrome can present with various forms of ophthalmoplegia. This case report describes a 57-year-old woman who initially showed bilateral internuclear ophthalmoplegia (INO), with normal imaging and laboratory findings. Her neurologic symptoms progressed to include hyporeflexia and ataxia, leading to a diagnosis of Miller Fisher syndrome. Intravenous immunoglobulin treatment resulted in gradual improvement. This case report highlights the need to consider rare conditions like Miller Fisher syndrome in patients with INO-like features and unclear etiology.
Original Article
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Seasonal variation in vestibular neuritis in Korea: a nationwide population-based cohort study
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Chul-Young Yoon, Young Jun Seo, Ji-Yun Park
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Res Vestib Sci. 2025;24(1):37-45. Published online March 14, 2025
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DOI: https://doi.org/10.21790/rvs.2025.006
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Abstract
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- Objectives
This study was performed to investigate seasonal variation in the incidence of vestibular neuritis (VN) without recent steroid treatments using nationwide health insurance data. The aim of the study is to elucidate whether seasonal trends can inform optimized diagnostic and treatment strategies for VN.
Methods
A retrospective analysis was conducted using data from the Health Insurance Review and Assessment Service (HIRA) in South Korea from 2007 to 2022. Patients diagnosed with VN were identified using specific operational criteria. Seasonal trends were evaluated by analyzing monthly, quarterly, and seasonal variations in VN incidence, stratified by age and sex.
Results
Out of 237,673 VN patients identified, our analysis revealed significant seasonal variations in incidence, with a notable decline during winter months—especially in February—and an increase during the spring. These patterns were consistent across sex and age groups.
Conclusions
This nationwide study demonstrates that VN exhibits distinct seasonal variations that have significant implications for clinical practice. These results indicate a potential influence of seasonal factors on the occurrence of VN and contribute to more efficient allocation of healthcare resources. Future prospective studies are warranted to further elucidate the mechanisms behind these seasonal differences.
Review Articles
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Oculomotor manifestations in inflammatory central nervous system demyelinating diseases: a narrative review
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Eun-Jae Lee
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Res Vestib Sci. 2025;24(1):27-36. Published online March 14, 2025
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DOI: https://doi.org/10.21790/rvs.2024.022
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Abstract
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- Inflammatory central nervous system (CNS) demyelinating diseases are a group of disorders in which inflammation within the CNS damages the myelin sheath, a protective and conductive layer surrounding nerve fiber in the brain and spinal cord, leading to various neurological symptoms. Common conditions include multiple sclerosis and neuromyelitis optica spectrum disorders, both characterized by relapses and progressive deterioration over time. Given their frequent involvement of the brainstem and cerebellum, these diseases often manifest with oculomotor findings, such as ocular misalignment and instability, providing valuable insights into lesion localization within specific anatomical regions. Recent studies suggest that these findings may also indicate broader pathological burdens, including potential cortical damage, positioning them as potential early biomarkers for disease progression. This review explores oculomotor findings in patients with CNS demyelinating diseases, examining their characteristics, underlying mechanisms, and clinical significance. It also highlights the potential role of these findings as disease biomarkers.
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Preventive medical treatment of vestibular migraine: a practical review
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Jae-Hwan Choi, Kwang-Dong Choi, Eun Hye Oh, Seo-Young Choi
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Res Vestib Sci. 2025;24(1):20-26. Published online March 14, 2025
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DOI: https://doi.org/10.21790/rvs.2024.024
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Abstract
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- This review explores the medical treatment options for vestibular migraine (VM), focusing on preventive strategies and their evidence-based efficacy. VM, characterized by recurrent dizziness associated with migraine, requires tailored management due to its distinct clinical features and pathophysiology. Most VM treatments are derived from migraine management protocols, utilizing medications such as BBs, CCBs, and antiepileptic drugs. Meta-analyses revealed that flunarizine, propranolol, and venlafaxine significantly reduced vertigo frequency and Dizziness Handicap Inventory scores, although the evidence was limited by small sample sizes and methodological inconsistencies. Flunarizine showed benefits in reducing vertiginous episodes but was less effective for headache. Venlafaxine demonstrated additional improvements in depressive symptoms. Observational studies also indicated potential efficacy for propranolol and valproate. Emerging therapies, such as calcitonin gene-related peptide monoclonal antibodies, showed promise in improving both vestibular and headache symptoms in small-scale trials, though further evidence is needed to establish their role in VM management. The selection of preventive medications for VM should be individualized, considering both the frequency and severity of vestibular and headache symptoms, as well as patient comorbidities and preferences. While existing migraine protocols offer some guidance, the need for VM-specific treatment strategies remains critical.
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Models for response dynamics of vestibular end organs: a review
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Jeong‑Yoon Choi
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Res Vestib Sci. 2025;24(1):10-19. Published online March 14, 2025
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DOI: https://doi.org/10.21790/rvs.2024.027
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Abstract
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- The vestibular end organs, consisting of the semicircular canals and otoliths, sense physical variables related to head motion and generate neural signals according to their response dynamics. Therefore, understanding the dynamics is a crucial first step in interpreting vestibular responses under both normal and pathological conditions. This review addresses the response dynamics of vestibular organs using transfer functions. While the semicircular canals and otoliths are originally modeled as second-order overdamped systems, they can be approximated as first-order systems, when considering the range of head motion frequencies typically encountered during daily activities. This review also discusses the clinical implications of time constants in the transfer functions. In both the semicircular canals and otoliths, the dominant time constants are determined by their viscous-to-elastic properties. By analyzing changes in these properties under pathological conditions, the resulting alterations in system responses can be predicted. Such efforts may contribute to bridging the gap between mathematical modeling and clinical understanding.
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Recent advances in diagnostic and therapeutic strategies for management of dizzy patients in Japan
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Noriaki Takeda
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Res Vestib Sci. 2025;24(1):1-9. Published online March 14, 2025
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DOI: https://doi.org/10.21790/rvs.2025.001
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Abstract
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- The present review summarized the recent advances in diagnostic and therapeutic strategies for management of dizzy patients in Japan. Standardization of diagnosis, examination, and treatment of vestibular disorders and nationwide epidemiological surveys of vestibular disorders are high-priority projects of Japan Society for Equilibrium Research (JSER). JSER published clinical practice guidelines for Ménière’s disease, delayed endolymphatic hydrops, benign paroxysmal positional vertigo, vestibular neuritis, vestibular test battery, and vestibular rehabilitation. JSER published a video library of vestibular testing and nystagmus and abnormal eye movements. JSER performed nationwide surveys of Ménière’s disease, delayed endolymphatic hydrops, and bilateral vestibulopathy. Visualization of endolymphatic hydrops in gadolinium-enhanced magnetic resonance imaging (MRI) was developed in Japan. MRI of endolymphatic hydrops becomes an indispensable tool to diagnose Ménière’s disease. The new surgical procedure of endolymphatic sac surgery with steroid instillation and middle ear pressure therapy with a Japanese device become new therapeutic strategies for intractable Ménière’s disease in Japan. Neural and pharmacological mechanisms of static vestibular compensation were revealed in Japan. Diazepam accelerates the initial process of static vestibular compensation and short-term administration with diazepam is recommended for the treatment of acute vertigo in patients with unilateral vestibulopathy. Betahistine accelerates the late process of static vestibular compensation and long-term administration with betahistine is recommended for the treatment of subacute vertigo in patients with unilateral vestibulopathy. Vestibular rehabilitation is effective in developing dynamic vestibular compensation and beneficial for improving chronic dizziness in patients with unilateral vestibulopathy. Vestibular rehabilitation with a Japanese vestibular substitution device is a promising strategy for chronic dizziness in patients with unilateral vestibulopathy.
Letter to the editor
Original Articles
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Associations between Dizziness Handicap Inventory scores and vestibular function tests: a cross-sectional survey
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Eun-Ju Jeon, Chae-Hyun Lim, Eun-Jin Son, Chang-Yeong Jeong, Ji Hyung Lim, Hyun Jin Lee
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Res Vestib Sci. 2024;23(4):156-164. Published online December 15, 2024
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DOI: https://doi.org/10.21790/rvs.2024.023
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Abstract
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- Objectives
This study aims to evaluate the relationship between subjective dizziness disability, as assessed by the Dizziness Handicap Inventory (DHI), and objective vestibular function test (VFT) results in patients presenting with dizziness.
Methods
We conducted a retrospective review of 177 patients who completed the DHI, vertigo visual analog scale, and disability scale at their initial visit. Objective VFTs included videonystagmography with caloric testing, cervical vestibular evoked myogenic potential (cVEMP), and the sensory organization test (SOT). Statistical analyses were conducted to assess correlations and differences in DHI scores based on VFT results and clinical characteristics.
Results
The DHI scores indicated a higher perceived dizziness handicap among female patients compared to males (p=0.012). Chronic dizziness was associated with elevated DHI scores in specific items (DHI-2, DHI-12, and DHI-21; p<0.05). Patients with abnormal caloric responses exhibited higher scores in several DHI items and subscales (DHI-4, DHI-12, DHI-14, DHI-17, DHI-19, DHI-23, physical, emotional, and functional; p<0.05). No significant differences were found in cVEMP results. Only one SOT condition (equilibrium score 5) showed a statistically significant but weak association with DHI scores (r=–0.151, p=0.045).
Conclusions
There were limited correlations between objective vestibular test outcomes and subjective dizziness disability. These findings underscore the multidimensional nature of dizziness and the importance of integrating subjective and objective measures for a comprehensive clinical assessment.
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Various symptoms and signs of vestibular paroxysmia in a tertiary neurotologic clinic: a retrospective comparative study
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Tae Uk Cheon, Eun Hye Cha, Yehree Kim, Hong Ju Park
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Res Vestib Sci. 2024;23(4):147-155. Published online December 15, 2024
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DOI: https://doi.org/10.21790/rvs.2024.021
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Abstract
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Supplementary Material
- Objectives
Vestibular paroxysmia (VP) is characterized by brief episodes of vertigo due to neurovascular cross-compression (NVCC) of the eighth cranial nerve. This study aimed to analyze the clinical features of VP patients in a tertiary neurotologic clinic using the 2008 and 2016 diagnostic criteria and to compare these features.
Methods
A retrospective review was conducted on patients diagnosed with definite or probable VP at the Asan Medical Center from May 2012 to May 2013. Patients underwent comprehensive evaluations including history taking, physical examination, audiometry, vestibular function tests, and magnetic resonance imaging (MRI). The 2008 and 2016 diagnostic criteria for VP were applied, and clinical characteristics were compared.
Results
Nineteen patients were included (14 females and five males; mean age, 57.9±14.5 years). According to the 2008 criteria, 17 patients were diagnosed with definite VP and two with probable VP; however, using the 2016 criteria, nine were definite and two were probable VP. NVCC was observed in 88.9% of patients under the 2016 criteria. MRI revealed NVCC predominantly involving the anterior inferior cerebellar artery. Patients with cerebellopontine angle (CPA) tumors presenting with VP-like symptoms responded to medication.
Conclusions
The 2016 diagnostic criteria for VP allow for a syndromic diagnosis based solely on clinical features. Neurovascular contact is commonly observed in VP patients, and CPA tumor can present VP-like symptoms and respond to carbamazepine. Although MRI is not included in the current criteria, it can be beneficial in diagnosis by identifying neurovascular contact and distinguishing CPA tumors.
Review Articles
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Exploring the nexus: unilateral vestibulopathy and visuospatial cognitive impairments
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Sun-Young Oh, Thanh Tin Nguyen, Marianne Dieterich
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Res Vestib Sci. 2024;23(4):132-146. Published online December 15, 2024
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DOI: https://doi.org/10.21790/rvs.2024.014
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Abstract
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- The interplay between vestibular disorders and visuospatial impairments has long captured scholarly attention. While evidence robustly supports visuospatial deficits in bilateral vestibulopathy, findings regarding unilateral vestibulopathy remain equivocal. Recent studies, both animal-based employing vestibular deafferentation models and human-based involving spatial cognitive tasks, hint at potential visuospatial cognitive impairments in individuals with acute and chronic unilateral vestibulopathy. Nevertheless, these results are preliminary and necessitate further rigorous investigation. The posture-first principle is evident in cognitive-motor dual tasks among patients with vestibular disorders. This review synthesizes these emergent insights, aiming to lay a groundwork for future studies that seek to elucidate this complex relationship further.
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Clinical application of virtual reality for vestibular rehabilitation
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Sung Kwang Hong
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Res Vestib Sci. 2024;23(4):124-131. Published online December 15, 2024
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DOI: https://doi.org/10.21790/rvs.2024.019
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Abstract
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- Virtual reality has emerged as a promising tool in vestibular rehabilitation therapy (VRT), offering immersive and interactive environments that enhance patient engagement and adherence to therapy. Its potential lies in delivering controlled, customizable exercise protocols that simulate real-world challenges in a safe, monitored environment. This allows healthcare providers to tailor exercises based on gaze stabilization, vestibulo-ocular reflex training, and balance training, according to a patient’s specific complaints, symptoms, and progress. While cybersickness is a potential concern with virtual reality interventions, patients with vestibular loss are generally less susceptible to the visual-vestibular mismatch that often causes motion sickness. Studies have shown that side effects, such as nausea or discomfort from cybersickness, are minimal for most patients undergoing virtual reality-based VRT. Despite these promising results, further research is needed to fully validate the effectiveness of virtual reality interventions in VRT. This article will explore the current status and future potential of virtual reality in VRT, including considerations for its clinical application and areas for future research.
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Digital therapeutics and telemedicine for benign paroxysmal positional vertigo and other vestibular disorders: a review
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Eun-Hyeok Choi, Hyo-Jung Kim, Ji-Soo Kim
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Res Vestib Sci. 2024;23(4):115-123. Published online December 15, 2024
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DOI: https://doi.org/10.21790/rvs.2024.018
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Abstract
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- In the recent wave of digital transformation, digital therapeutics and telemedicine are changing the concepts in the management of benign paroxysmal positional vertigo and other vestibular disorders. These technologies provide accessible and cost-effective solutions amid the ongoing digital revolution in healthcare. This review article covers the recently advanced digital therapeutics for vestibular disorders that include questionnaire- or artificial intelligence-based diagnostic algorithms, telemedicine and self-application of canalith repositioning therapy, wearable devices for monitoring of eye movements during the attacks of dizziness/vertigo, and metaverse for vestibular rehabilitation. Integration of these digital technologies would improve diagnostic accuracy and treatment efficacy, reduce the economic burden associated with vestibular disorders, and promise a revolutionary shift in patient care towards personalized medicine.
Case Reports
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Horizontal nystagmus with velocity-increasing waveforms in delayed post-hypoxic leukoencephalopathy: a case report
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Eugene Jung, Suk-Min Lee, Seo-Young Choi, Kwang-Dong Choi
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Res Vestib Sci. 2024;23(3):111-113. Published online September 15, 2024
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DOI: https://doi.org/10.21790/rvs.2024.016
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Abstract
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Supplementary Material
- Abnormal eye movements in unconscious patients serve as crucial diagnostic instruments, offering insights into the function of the central nervous system. Understanding these movements can aid in diagnosing the cause of unconsciousness, localizing brain lesions, and predicting outcomes. We report a patient who presented with spontaneous horizontal nystagmus unaffected by light in delayed post-hypoxic encephalopathy. Video-oculography showed exponentially increasing slow phases, with an amplitude ranging from 3° to 9° and a frequency of 0.5 Hz. Based on the results of oculography and neuroimaging, persistent horizontal nystagmus in our patient may be ascribed to an unstable neural integrator, possibly caused by disrupted cerebellar feedback mechanisms for horizontal gaze holding.
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Fluctuating high-frequency hearing loss with vertigo: is it Menière’s disease? A case report
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Minho Jang, Dong-Han Lee, Jiyeon Lee, Chang-Hee Kim
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Res Vestib Sci. 2024;23(3):106-110. Published online September 15, 2024
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DOI: https://doi.org/10.21790/rvs.2024.011
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Abstract
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Supplementary Material
- This study describes an unusual case of fluctuating unilateral high-frequency hearing loss with vertigo resembling Menière’s disease. The current diagnostic criteria for definite Menière’s disease include audiometrically documented low- to medium-frequency sensorineural hearing loss on at least one occasion before, during, or after an episode of vertigo. This case presented a diagnostic dilemma. Brain MRI was nonspecific, and a bithermal caloric test showed unilateral weakness of 44% on the affected side. The results of electrocochleography and cervical vestibular evoked myogenic potential tests were within the normal ranges. Persistent geotropic or ageotropic positional nystagmus was observed during each vertigo attack; the mechanism underlying this characteristic nystagmus needs further investigation.