Research in Vestibular Science

Case Report

Gaze-holding nystagmus in chronic progressive external ophthalmoplegia: a case report: Eugene Jung, Seo-Young Choi, Jae Hwan Choi, Kwang-Dong Choi; Res Vestib Sci. 2025;24(1):50-53. Published online March 14, 2025; DOI: https://doi.org/10.21790/rvs.2025.005

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Abstract PDF 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.

Review Article

Preventive medical treatment of vestibular migraine: a practical review: Jae-Hwan Choi, Kwang-Dong Choi, Eun Hye Oh, Seo-Young Choi; Res Vestib Sci. 2025;24(1):20-26. Published online March 14, 2025; DOI: https://doi.org/10.21790/rvs.2024.024

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Abstract PDF: 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.

Case Report

Horizontal nystagmus with velocity-increasing waveforms in delayed post-hypoxic leukoencephalopathy: a case report: Eugene Jung, Suk-Min Lee, Seo-Young Choi, Kwang-Dong Choi; Res Vestib Sci. 2024;23(3):111-113. Published online September 15, 2024; DOI: https://doi.org/10.21790/rvs.2024.016

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Abstract PDF 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.

Reviews

The Association between Dizziness and Anxiety: Update to the Treatment: Seo-Young Choi, Kwang-Dong Choi; Res Vestib Sci. 2022;21(2):40-45. Published online June 15, 2022; DOI: https://doi.org/10.21790/rvs.2022.21.2.40

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Abstract PDF Supplementary Material: Anxiety, depression, or other psychiatric symptoms can be the primary cause of dizziness and the secondary complication of dizziness. Regardless of precedence or consequence, dizziness is closely associated with the psychiatric problem. On this ground, this chapter reviews the association between dizziness and anxiety and the treatment for dizziness with psychiatric symptoms.

Medical Treatment of Nonvascular Central Vertigo: Seo-Young Choi, Kwang-Dong Choi; Res Vestib Sci. 2021;20(3):75-80. Published online September 15, 2021; DOI: https://doi.org/10.21790/rvs.2021.20.3.75

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Abstract PDF: Central vertigo is the common symptom resulting from abnormalities in the central nervous system, caused by various diseases, which include neurodegenerative, vascular, inflammation, infection, tumor, paraneoplastic, toxic, or metabolic disorders. Since the treatment of central vertigo depends on the causes, an accurate diagnosis should be preceded the treatment through a detailed neurotologic examination and laboratory evaluation. Also, it is important to identify and document the neurologic findings accompanied by central vertigo, because some medication focuses on the ocular motor abnormalities as nystagmus or saccadic intrusion. Here, we will review the medical treatment for central vertigo.

Medical treatment of central vertigo: Kwang-Dong Choi, Seo-Young Choi; Received August 22, 2021 Accepted September 1, 2021 Published online September 1, 2021; [Accepted]

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Abstract: Central vertigo is the common symptom resulting from abnormalities in the central nervous system, caused by various diseases, which include neurodegenerative, vascular, inflammation, infection, tumor, paraneoplastic, toxic, or metabolic disorders. Since the treatment of central vertigo depends on the causes, an accurate diagnosis must be preceded the treatment through a detailed neurotologic examination and laboratory evaluation. Also, it is important to identify and document the neurologic findings accompanied by central vertigo, because some medication focuses on the ocular motor abnormalities as nystagmus or saccadic intrusion. Here, we will review the medical therapy for central vertigo.

Case Reports

A Case of Spinocerebellar Ataxia Type 28: Jae-Hwan Choi, Eun Hye Oh, Seo Young Choi, Kwang-Dong Choi; Res Vestib Sci. 2021;20(1):33-36. Published online March 11, 2021; DOI: https://doi.org/10.21790/rvs.2021.20.1.33

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Abstract PDF: Spinocerebellar ataxia type 28 (SCA 28) is characterized by young-adult onset, very slowly progressive gait and limb ataxia, dysarthria, nystagmus, ptosis, and ophthalmoplegia. It is caused by a heterozygous pathogenic mutation in the AFG3L2. So far, approximately 80 cases with genetically-confirmed SCA 28 have been reported in the literature. We report a patient with mild gait ataxia and dysarthria who carried a known pathogenic mutation in the AFG3L2. This is the first report of genetically-confirmed SCA 28 in Korea.

Anti-GQ1b Antibody Syndrome Presenting with Severe Headache: Seo-Young Choi, Kyeung-Hae Kim, Jong Kuk Kim, Nam Jun Kim, Young Hee Kim, Kwang-Dong Choi; Res Vestib Sci. 2020;19(4):141-143. Published online December 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.4.141

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Abstract PDF: Anti-GQ1b antibody syndrome, including Miller Fisher syndrome, Guillain-Barré syndrome with ophthalmoplegia, Bickerstaff’s brainstem encephalitis, and acute ophthalmoplegia without ataxia, has overlapped clinical symptoms and mostly associated with anti-GQ1b immunoglobulin (Ig) G antibody. We report two cases of anti-GQ1b antibody syndrome mainly presenting with a severe headache. The 60-year-old man was admitted for severe headache and gait disturbance. Neurological examination revealed limb and truncal ataxia, areflexia, nystagmus, and ophthalmoplegia. Serum IgG anti-GQ1b antibody was positive. He recovered after intravenous (IV) immunoglobulin and steroid. The 23-year-old man suffered from severe headache (visual analogue scale=10) within the periorbital area. Ophthalmoplegia with gaze-evoked nystagmus were revealed. Serum IgG anti-GQ1b and anti-GT1a antibodies were positive. Headache was improved by IV immunoglobulin and steroid. The pathophysiology of headache in anti-GQ1b antibody syndrome is largely unknown. The affected nerve or structures in the brainstem including the trigeminovascular system may induce intractable severe headache.

Original Articles

Satisfaction and Effect Research on Virtual Reality-Based Vestibular Exercise for the Elderly Patients with Chronic Unilateral Vestibulopathy: Kwang-Dong Choi, Seo-Young Choi; Res Vestib Sci. 2020;19(4):127-132. Published online December 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.4.127

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Abstract PDF: Objectives
To survey the satisfaction of customized vestibular exercise using virtual reality system with mobile head-mounted display (HMD) in the elderly patients with chronic unilateral vestibulopathy, we analyzed questionnaires before and after exercise.
Methods
Sixteen patients (male, 6; median age, 69 years [interquartile range, 65–75 years]) with chronic unilateral vestibulopathy were prospectively enrolled from March 1 to December 31 in 2018. They exercised once a week for 20 to 25 minutes for 4 weeks using the HMD inserted the virtual reality exercise program. Dizziness visual analogue scale (DAS), Korean vestibular disorders activities of daily living scale (ADL), and visual vertigo analogue scale (VVAS) were performed before and after the exercise. After all of the program, the patients were surveyed to measure the satisfaction for the tool and effect of exercise.
Results
DAS, ADL, and VVAS were significantly improved after the vestibular exercise. No one answered unsatisfactory, and at least 62.5% of the patients satisfied the used tool and exercise program. The patients of 50% answered that they satisfied or very satisfied to the efficacy of exercise program. The patients who recovered VVAS more after the exercise were more satisfied to our tools and efficacy of exercise program.
Conclusions
Customized vestibular exercise using virtual reality system with HMD can not only improve dizziness and quality of life, but also made more satisfied to the elderly patients with chronic unilateral vestibulopathy.

Customized Vestibular Rehabilitation in the Patients with Bilateral Vestibulopathy: A Pilot Study in One Referred Center: Kwang-Dong Choi, Seo-Young Choi; Res Vestib Sci. 2019;18(3):64-70. Published online September 15, 2019; DOI: https://doi.org/10.21790/rvs.2019.18.3.64

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Abstract PDF: Objectives
Bilateral vestibulopathy is characterized with unsteadiness and oscillopsia when walking or standing, worsening in darkness and/or on uneven ground. To establish the effect of customized vestibular rehabilitation in bilateral vestibulopathy, we analyzed the questionnaires and functional status before and after treatment.
Methods
Among 53 patients with customized vestibular rehabilitation from January 1st to November 30th in 2018, 6 patients (3 males; median age, 71 years; range, 54–75 years) who regularly exercised with good compliance were retrospectively enrolled. They were educated and trained the customized vestibular rehabilitation once a month or two by a supervisor during 40 minutes, and then exercised at home for 30 minutes over 5 days in a week. Dizziness handicap inventory (DHI), Korean vestibular disorders activities of daily living scale (vADL), Beck’s depression index (BDI), test for dynamic visual acuity (DVA), and Timed Up and Go test (TUG) were performed before and after the customized vestibular rehabilitation.
Results
The patients exercised for median 5.5 months (range, 2–10 months) with the customized methods of vestibular rehabilitation, which included gaze and posture stabilization and gait control exercises. DHI score and TUG was improved after rehabilitation (DHI before vs. after rehabilitation=33 vs. 16, p=0.027, TUG before vs. after rehabilitation=12 vs. 10, p=0.026). BDI, DVA, and vADL scores did not differ between before and after treatment.
Conclusions
Customized vestibular rehabilitation can improve dizziness and balance state in bilateral vestibulopathy. The steady exercises adapted individual peculiarities is the most important for vestibular rehabilitation.

Customized vestibular rehabilitation in the patients with bilateral vestibulopathy: A pilot study in one referred center: Kwang-Dong Choi, Seo-Young Choi; Received June 12, 2019 Accepted August 20, 2019 Published online August 20, 2019; [Accepted]

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Abstract: Objective Bilateral vestibulopathy is characterized with unsteadiness and oscillopsia when walking or standing, worsening in darkness and/or on uneven ground. To establish the effect of customized vestibular rehabilitation in bilateral vestibulopathy, we analyzed the questionnaires and functional status before and after treatment.
Methods
Among 53 patients with customized vestibular rehabilitation from January 1st to November 30th in 2018, six patients (men = 3, age = 71 (median, range = 54~75)) who regularly exercised with good compliance were retrospectively enrolled. They were educated and trained the customized vestibular rehabilitation once a month or two by a supervisor during 40 minutes, and then exercised at home for 30 minutes over 5 days in a week. Dizziness Handicap Inventory (DHI), Korean vestibular disorders activities of daily living scale (vADL), Beck’s depression index (BDI), test for dynamic visual acuity (DVA), and timed up to go test (TUG) were performed before and after the customized vestibular rehabilitation.
Results
The patients exercised for 5.5 months (median, range= 2~10 months) with the customized methods of vestibular rehabilitation, which included gaze and posture stabilization and gait control exercises. DHI score and TUG was improved after rehabilitation (DHI before vs. after rehabilitation = 33 vs. 16, p = 0.027, TUG before vs. after rehabilitation = 12 vs. 10, p =0.026). BDI, DVA, and vADL scores did not differ between before and after treatment.
Conclusions
Customized vestibular rehabilitation can improve dizziness and balance state in bilateral vestibulopathy. The steady exercises adapted individual peculiarities is the most important for vestibular rehabilitation.

Case Report

Opsoclonus-Myoclonus Syndrome Associated with Scrub Typhus: You Jin Choi, Seo Young Choi, Jae-Hwan Choi, Kwang-Dong Choi; Res Vestib Sci. 2017;16(1):34-37. Published online March 15, 2017; DOI: https://doi.org/10.21790/rvs.2017.16.1.34

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Abstract PDF

Scrub typhus is an infective acute febrile disorder caused by the intracellular parasite Orientia tsutsugamushi. Neurological manifestations of scrub typhus are meningoencephalitis, cerebellitis, transverse myelitis, papilledema, and cranial nerve palsy. However, opsoclonus-myoclonus syndrome associated with scrub typhus has been rarely reported. A 59-year-old man developed vertigo, nausea, vomiting, and imbalance following scrub typhus infection for eight days. Examination showed eschar at the axilla, decreased mentality, and opsoclonus- myoclonus syndrome. Video-oculography disclosed opsoclonus with an amplitude of 15°–20° and a frequency of 6–8 Hz. The serum antibody titers to Orientia tsutsugamushi were 1:5,120, and cerebrospinal fluid analysis revealed pleocytosis. Brain magnetic resonance imaging was normal. Neurological symptoms and signs completely improved by systemic steroid and antibiotics treatment. Various mechanisms including direct disseminating inflammation or indirect immune modulation may give rise to neurological complications following scrub typhus.

Citations

Citations to this article as recorded by

Scrub typhus meningoencephalitis presenting as opsoclonus myoclonus syndrome: A video-based case
Laxmikant Ramkumarsingh Tomar, Dhrumil Jatinbhai Shah, Utkarsh Agarwal, Atul Gogia, Anshu Rohatgi, CS Agrawal
Tropical Doctor.2022; 52(1): 192. CrossRef
Neurological Manifestations of Scrub Typhus
Sagar Basu, Ambar Chakravarty
Current Neurology and Neuroscience Reports.2022; 22(8): 491. CrossRef
Neurological facets of scrub typhus: A comprehensive narrative review
Divyani Garg, Abi Manesh
Annals of Indian Academy of Neurology.2021; 24(6): 849. CrossRef

Opsoclonus-Myoclonus Syndrome Associated with Scrub Typhus: You Jin Choi, Seo Young Choi, Jae-Hwan Choi, Kwang-Dong Choi; Received November 1, 2016 Accepted January 13, 2017 Published online January 13, 2017; [Accepted]

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Abstract: Scrub typhus is an infective acute febrile disorder caused by the intracellular parasite Orientia tsutsugamushi. Neurological manifestations of scrub typhus are meningoencephalitis, cerebellitis, transverse myelitis, papilledema, and cranial nerve palsy. However, opsoclonus-myoclonus syndrome associated with scrub typhus has been rarely reported. A 59-year-old man developed vertigo, nausea, vomiting, and imbalance following scrub typhus infection for eight days. Examination showed eschar at the axilla, decreased mentality, and opsoclonus-myoclonus syndrome. Video-oculography disclosed opsoclonus with an amplitude of 15-20° and a frequency of 6-8Hz. The serum antibody titers to Orientia tsutsugamushi were 1:5,120, and CSF analysis revealed pleocytosis. Brain MRI was normal. Neurological symptoms and signs completely improved by systemic steroid and antibiotics treatment. Various mechanisms including direct disseminating inflammation or indirect immune modulation may give rise to neurological complications following scrub typhus.

Symposium I

Ataxia and Diplopia: Jae-Deuk Seo, In Soo Moon, Kwang-Dong Choi; Res Vestib Sci. 2013;12:S31-S40. Published online June 1, 2013

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Abstract PDF: Ataxia is a neurological sign consisting of lack of voluntary coordination of muscle movements, which may result from abnormalities in different parts of the nervous system including the cerebellum and its connecting pathway, proprioceptive sensory pathway, and vestibular system. In the diagnosis of disorders characterized by cerebellar ataxia, the mode of onset, rate of development, and degree of permanence of the ataxia are of particular importance. In adults, paraneoplastic and demyelinating cases account for the largest proportion of subacute onset, and hereditary forms are the usual cause of very slowly progressive and chronic ones. The last category of genetic ataxias now constitute a large and heterogeneous group for which the basis has been established in most cases. Misalignment of the visual axes-strabismus-causes the two images of a seen object to fall on noncorresponding areas of the two retinas, which usually causes diplopia, the sensation of seeing an object at two different locations in space. Accurate diagnosis of diplopia and strabismus require detailed history on the symptomatology and ocular motor examination consisting of assessment of the range of eye movements, subjective diplopia testing, and cover and alternate cover tests.

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