Recent progress in vestibular prosthesis / 临床耳鼻咽喉头颈外科杂志

Bilateral vestibulopathy（BVP） is one of the common diseases in the vestibular nervous system, with an incidence rate of about 4%-7% in the population, which can lead to a variety of body dysfunctions. At present, there are two main treatment methods for BVP. One is vestibular rehabilitation. However, only part of BVP patients can finally benefit from vestibular rehabilitation, and most patients will remain with permanent vestibular dysfunction. Benefiting from the maturity of cochlear implant technology, European and American countries took the lead in the development of vestibular prosthesis（VP） technology to restore the vestibular function in patients with BVP. This review will focus on the development history, principles, future applications and the related research progress of VP in China.

Video head impulse test in bilateral vestibulopathy / Teste do impulso efálico com vídeo (vHIT) na vestibulopatia bilateral

Abstract Introduction: Bilateral vestibulopathy is a rare chronic condition with multiple etiologies. Bilateral vestibulopathy is characterized mainly by unsteadiness when walking or standing, which worsens in darkness, as well as oscillopsia. The degree of handicap caused by bilateral vestibulopathy is variable and remains controversial. Objectives: To determine the value of the video Head Impulse Test in quantifying vestibular deficit and to establish its impact on the quality of life. Methods: Twenty patients (mean age, 41.9 years; range 14-80 years) fulfilling the recent Barany criteria of bilateral vestibulopathy, responded to the Situational Vertigo Questionnaire and underwent vestibular examination including fixation, positional tests, oculomotor test battery and video head impulse test. Results: The relation between each of the video head impulse test parameters and the scores from the questionnaire were statistically analyzed. We observed that patients with covert saccades on the video head impulse test were more likely to have a better quality of life than those with both covert and overt saccades, regardless of the vestibulo-ocular reflex gain in each semicircular canal. The presence of covert saccades was found to be associated with an improved quality of life regardless of the severity of vestibule ocular reflex-deficit. Our conclusion was that vestibule ocular reflex gain, measured by video head impulse test, does not quantify the severity of affection of quality of life in patients with bilateral vestibulopathy. Conclusion: Covert saccades are strategies aiming at minimizing the blurring of vision during head movement, that is an adaptive mechanism that improves quality of life. Therefore, we recommend that video head impulse test should be a part of the routine diagnostic workup of bilateral vestibulopathy.

Resumo Introdução: A vestibulopatia bilateral é uma condição crônica rara, com múltiplas etiologias. É caracterizada principalmente por instabilidade ao caminhar ou ficar de pé, que piora na escuridão, e oscilopsia. O grau de deficiência causado pela vestibulopatia bilateral é variável e permanece controverso. Objetivos: Determinar o valor do teste do impulso cefálico na quantificação do déficit vestibular e estabelecer seu impacto na qualidade de vida. Método: Vinte pacientes (média de 41,9 anos; variação de 14 a 80) que atendiam aos critérios recentes da Bárány Society de vestibulopatia bilateral responderam ao Situational Vertigo Questionnaire e foram submetidos a exame vestibular, inclusive fixação, testes posicionais, bateria de testes oculomotores e teste do impulso cefálico com vídeo. Resultados: A relação entre cada um dos parâmetros do teste do impulso cefálico com vídeo e os escores do questionário foram analisados estatisticamente. Observamos que pacientes com sacadas corretivas cobertas do tipo covert no teste de impulso cefálico com vídeo tinham maior probabilidade de ter melhor qualidade de vida do que aqueles com ambas sacadas corretivas cobertas e sacadas corretivas abertas do tipo overt, independentemente do ganho no reflexo vestíbulo-ocular em cada canal semicircular. Verificou-se que a presença de sacadas corretivas do tipo covert está associada a uma melhor qualidade de vida, independentemente da gravidade do déficit no reflexo vestibulo-ocular. Concluímos que o ganho no reflexo vestíbulo-ocular, medido pelo teste do impulso cefálico com vídeo, não quantifica a gravidade do comprometimento da qualidade de vida em pacientes com vestibulopatia bilateral. Conclusão: As sacadas corretivas do tipo covert são estratégias que visam minimizar o embaçamento da visão durante o movimento da cabeça, ou seja, um mecanismo adaptativo que melhora a qualidade de vida. Portanto, recomendamos que o teste do impulso cefálico com vídeo faça parte da rotina de diagnóstico da vestibulopatia bilateral.

Síndrome de Miller Fisher: relato de caso / Miller Fisher syndrome: case report

A síndrome de Miller Fisher é uma desmielinização dos nervos cranianos e periféricos, gerando graves consequências para o paciente, como, por exemplo, redução ou ausência dos reflexos, paralisia do III, IV e VI nervos cranianos e ataxia. Este relato descreveu o caso de uma mulher de 51 anos, natural e procedente de Penápolis (SP), admitida em um hospital de Araçatuba (SP) com quadro de arreflexia, ataxia e oftalmoplegia. No contexto clínico, foi suspeitada a hipótese de síndrome de Miller Fisher e, assim, começou o processo de investigação, com base nos critérios diagnósticos. O caso foi diagnosticado como síndrome de Miller Fisher, e o tratamento teve início.

Miller Fisher Syndrome is a demyelinating disease affecting cranial and peripheral nerves, leading to severe problems to the patient, such as reduced or absent reflexes, III, IV and VI cranial nerves palsy, and ataxia. This report describes the case of a 51-year-old woman from the city of Penápolis, in the state of São Paulo, who was admitted to the hospital in the city of Araçatuba, in the same state, with ataxia, areflexia and ophthalmoplegia. In the clinical context, the suspicion of Miller Fisher Syndrome was raised, and then investigation ensued for the disease, based on the diagnostic criteria. After evaluation, Miller Fisher Syndrome was confirmed and treatment was started.

Terapia vestibular en vestibulopatía bilateral por gentamicina: reporte de un caso y revisión de la literatura / Vestibular therapy in a case of gentamicin-induced vestibulopathy: case report and literature revision

La vestibulopatía bilateral es poco frecuente, se caracteriza principalmente por inestabilidad al caminar o al estar de pie, visión borrosa inducida por el movimiento u oscilopsia al caminar o al realizar movimientos rápidos de la cabeza o del cuerpo, empeoramiento de la estabilidad en la oscuridad o terrenos irregulares, reducción de los síntomas al estar en condiciones estáticas, ganancia del reflejo vestíbulo-ocular angular reducida de forma bilateral, entre otros. Existen múltiples causas. Dentro de las causas identificables, se describen principalmente medicamentos ototóxicos, meningitis y enfermedad de Ménière. Se presenta el caso de una paciente de 64 años diagnosticada con vestibulopatía bilateral posterior a tratamiento intramuscular con gentamicina por sobreinfección bacteriana cutánea de las manos. La evaluación vestibular complementada con videonistagmografía y prueba de impulso cefálico asistida por video confirman el diagnóstico y se inicia tratamiento con rehabilitación vestibular enfocada en promover la compensación central a través de estrategias de sustitución principalmente; además de habituación y adaptación vestibular, favoreciendo la estabilización de la mirada, mantención del equilibrio, control postural, marcha y reducción de los síntomas.

Bilateral vestibulopathy is infrequent, and it is characterized mostly by unstable walking or when standing, blurred vision induced by movement, or oscillopsia when walking or performing fast movements; worsening of the stability in darkness or uneven ground, but with lack of symptoms in static conditions. Other symptoms may include bilateral reduction of the oculo-vestibular reflex. Among the identifiable causes, there is the use of ototoxic medication, meningitis, Ménière's disease, although it can be idiopathic or have a neurological cause. We hereby describe the case of a 64-year-old woman, diagnosed with bilateral vestibulopathy secondary to intramuscular treatment with gentamicin due to a bacterial hand infection. Vestibular assessment was complemented with video-nystagmography and video head impulse test which confirmed the diagnosis, and therapy was started with vestibular rehabilitation focused on promoting central compensation mainly, through substitution strategies. Also, habituation exercise and vestibular adaptation strategies were used, thus promoting sight stabilization, balance maintenance, postural control, walking, and reduction of the symptoms.

Asociaciones entre función vestibular y habilidades cognitivas: un enfoque básico-clínico / Associations between vestibular function and cognitive abilities: from basic to clinical approach

RESUMEN El sistema vestibular tiene un rol fundamental en funciones sensorio-motoras, control del equilibrio y estabilidad de la mirada. En las últimas décadas un amplio número de trabajos ha descrito la importancia de las aferencias vestibulares en el funcionamiento de diversas áreas del cerebro relacionadas con funciones cognitivas tales como la atención, memoria, navegación y otras habilidades visuo-espaciales. Estudios en pacientes con vestibulopatía han demostrado que estos individuos presentan disminución de su rendimiento en algunas pruebas neuropsicológicas; y, a su vez, que personas con patología cognitiva como deterioro cognitivo leve y demencia por enfermedad de Alzheimer tienen mayor probabilidad de presentar pruebas vestibulares alteradas. Esta revisión se enfoca en el papel que cumple el sistema vestibular y su asociación con habilidades cognitivas; basándose en estudios básicos y clínicos que describen una red vestibular cerebral y que han llevado a proponer modelos teóricos que relacionan la función vestibular con la cognición.

ABSTRACT The vestibular system is widely known for its role in sensory-motor functions, balance control and gaze stability. In recent decades, several research articles have described the importance of vestibular afferents in the functioning of brain areas related to cognitive skills such as attention, spatial memory, spatial navigation and other visuospatial abilities. Studies involving subjects with vestibulopathy reveal that these individuals show decreased performance on neuropsychological tests; and that patients with neurocognitive pathologies, such as mild cognitive impairment and dementia due to Alzheimer's disease, have a greater probability of producing diminished or absent responses in clinical vestibular electrophysiological tests. This literature review focuses on the role played by the vestibular system and its association with cognitive abilities. The review incorporates a description of basic and clinical research that describe the cortical vestibular network and emerging theoretical models linking vestibular function to cognition.

Isolated hemorrhage in the cerebellar vermis with vertigo and body lateropulsion to the contralesional side / 영남의대학술지

There have been several reports of patients with isolated lesions of the cerebellar vermis presenting with clinical features similar to those of peripheral vestibulopathy. We report a case of small, isolated hematoma in the cerebellar vermis in a patient who presented with vertigo, ipsilesional nystagmus, and body lateropulsion to the contralesional side without the usual signs or symptoms of cerebellar dysfunction. Although they present with symptoms that mimic those of peripheral vestibulopathy, and brain computed tomography shows no abnormality, as there may be a small, isolated hematoma or infarction in the cerebellar vermis. Thus, brain magnetic resonance imaging should be performed in elderly patients with vascular risk factors.

Customized Vestibular Rehabilitation in the Patients with Bilateral Vestibulopathy: A Pilot Study in One Referred Center / 대한평형의학회지

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.

Characteristic of Sudden Sensorineural Hearing Loss with Objective Vestibular Involvement / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: To analyze the difference in audiologic-vestibular and clinical characteristics between acute cochlea-vestibulopathy (ACV) and idiopathic sudden sensorineural hearing loss (ISSNHL). SUBJECTS AND METHOD: We retrospectively analyzed the clinical characteristics of 91 patients diagnosed as sudden hearing loss (ACV; n=20, ISSNHL; n=71). Patients with vestibular hypofunction were categorized as ACV and all others as ISSNHL. Demographics and clinical findings were compared. Audiologic features such as degree of hearing loss, type of audiometric configuration and hearing improvements were analyzed. In addition, vestibular function test results and hearing recovery were further analyzed among ACV group. RESULTS: Demographics and other clinical findings were not much different between groups. There was a significant difference with respect to audiologic features between the ACV group and ISSNHL group: the initial hearing threshold of the ACV group was higher than that of the ISSNHL group, and their treatment onset was also shorter. There was also a significant difference in the hearing outcome showing very low rate of complete recovery in ACV group. The final hearing threshold of the ACV group was higher than that of the ISSNHL group. Dizziness was the only significant variable in the multiple regression analysis. In the ACV group, the cervical vestibular evoked myogenic potential inter-aural amplitude difference (cVEMP IAD) ratio showed a correlation to the hearing recovery in some frequencies; patients with no cVEMP response showed poor outcome compared to those with cVEMP waveform. CONCLUSION: The ACV group shows a poor prognosis just as in the case of sudden hearing loss defined in the traditional sense of vertigo. The IAD value of the vestibular evoked myogenic potentials test will be helpful in assessing hearing improvement, especially when a high IAD value at the middle frequency is associated with a poor prognosis.

Recovery Phase Spontaneous Nystagmus, Its Existence and Clinical Implication

BACKGROUND AND OBJECTIVES: Determination of the lesion side based on the direction of the nystagmus could result in confusions to the clinicians due to mismatch between the vestibular function tests and also between vestibular and audiologic features. To minimize these mistakes, we elucidated the clinical manifestation and vestibular function test results in cases with recovery spontaneous nystagmus (rSN). SUBJECTS AND METHODS: Patients who visited ENT clinic of tertiary referral hospital for acute onset continuous vertigo from January 2008 to December 2011 were enrolled. In these patients, we assessed onset time of vertigo, time point of paralytic spontaneous nystagmus (SN) and time point of rSN. At each time point of SN, vestibular function tests and hearing function tests were performed. RESULTS: We confirmed the rSN among patients with unilateral vestibulopathy and demonstrated that high gain of the rotatory chair test (slow harmonic acceleration) and/or mismatch of the SN direction and contralateral caloric weakness could indicate the recovery state of patients and nystagmus observed in this stage is recovery phase nystagmus. CONCLUSIONS: In acute vestibulopathy patients, recovery phase nystagmus was observed and on this stage of disease vestibular function tests shows several features that could predict recovery state.

Posttraumatic Peripheral Vertigo / 대한평형의학회지

Posttraumatic vertigo can be defined as the vertiginous disorder occurred after head and neck trauma without other pre-existing vestibular disorder. Central, peripheral, and combined deficits might cause this condition. Especially, various peripheral vestibulopathies are possible causes of posttraumatic vertigo; benign paroxysmal positional vertigo, temporal bone fracture, perilymphatic fistula, labyrinthine concussion, posttraumatic hydrops, and cervical vertigo. Since the differential diagnosis of the posttraumatic vertigo is often difficult, it is essential to acquire knowledge of their pathophysiology and clinical features. In this review, peripheral vestibulopathy as the possible causes of posttraumatic vertigo were described according to the current literature.

Clinical Application and Update of Video Head Impulse Test / 대한평형의학회지

Recently with the introduction of video head impulse test (vHIT), it can be easily performed quantitative and objective measurement of vestibulo-ocular reflex (VOR). vHIT has been used as a clinical vestibular function test that can individually evaluate the function of each semicircular canal. Loss of VOR gain and corrective catch-up saccades that occur during the vHIT usually indicate peripheral vestibular hypofunction, whereas in acute vestibular syndrome, normal vHIT should prompt a search for a central lesion. In this study, we will examine the principle of vHIT and its interpretation, and explain its clinical application in peripheral and central vestibulopathy. In addition, we will compare the caloric test and the differences, and review the most recently introduced suppression head impulse paradigm test.

The Clinical Efficacy of Vestibular Function Tests in Patients with Acute Unilateral Vestibulopathy / 대한평형의학회지

OBJECTIVES: Aim of this study is to investigate the clinical efficacy of the vestibular function tests (VFTs) and the predictability of lesion side of vestibular asymmetry parameters in acute unilateral peripheral vestibulopathy. METHODS: Medical records and results of VFTs (caloric, rotatory chair, and head impulse tests) of 57 patients with acute unilateral vestibulopathy were reviewed retrospectively. The VFTs were examined within 7 days after the clinical onset. RESULTS: For the caloric test, 74% showed significant canal paresis and the predictability of lesion side was 88%. For the sinusoidal harmonic acceleration test, 91% had low gain in at least 1 Hz, phase lead showed 70%, 89% showed phase asymmetry and the predictability of lesion side was 90%. For velocity step test, 67% had abnormal Tc asymmetry and the predictability of lesion side was 95%. In bedside head impulse test (HIT), abnormal catch up saccades were observed in 89% and the predictability of lesion side was 100%. For the video HIT, cover or overt catch-up saccades were observed in 95% and the predictability of lesion side was 100%. One hundred percent (100%) had low gain on the video HIT, but the lesion sides were uncertain because of bilateral involvements or artifacts. CONCLUSIONS: The most important things in the diagnosis of acute unilateral vestibulopathy are typical clinical symptoms and spontaneous nystagmus. A combination of rotatory, caloric, and HITs will result in a more complete examination of the vestibular system. Among them, HIT is recommended as the best tool in acute unilateral vestibulopathy.

Clinical Feasibility of Suppression Head Impulse Test in Vestibulopathy Patients / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The suppression head impulse (SHIMP) test has emerged as one of the new vestibular function tests. The SHIMP test is an analysis of anti-compensatory saccadic movements in gazing moving targets as the head moves. In this study, we investigated the accuracy and sensitivity of the test. SUBJECTS AND METHOD: We analyzed the results of SHIMP test (gain, amplitude, and latency) in 24 cases, which included normal, vestibular neuritis, benign paroxysmal positional vertigo (BPPV), Meniere's disease, bilateral vestibular function loss, temporal bone fracture, tuberculosis meningitis and acoustic schwannoma patients. We also carried out a physical exam, audiologic study, video electronystagmography (vENG) and a conventional video head impulse test to evaluate the feasibility of SHIMP test in cases of vesitibulopathy. RESULTS: In patients with vestibulopathy, the analysis of SHIMP test indicated high sensitivity and accuracy, which were supported by accompanying audio-vestibular studies. Patients suspected with BPPV, vestibular neuronitis and Meniere's disease showed low vestibulo ocular reflex (VOR) gain and delayed anticompensatory saccade with smaller and more scattered amplitudes than the control. Patients with bilateral vestibular function loss, temporal bone fracture, tuberculosis meningitis, and acoustic schwannoma showed delayed latency, and small amplitude or few anticompensatory saccades. Finally, changes in the SHIMP test values might be a hint that patients had recovered from vestibular neuronitis and tuberculosis meningitis. CONCLUSION: We found the feasibility of SHIMP test in detecting the pathologic condition of VOR in vestibulopathy patients. We argue that the usefulness of SHIMP test might be extended to evaluating the effectiveness of rehabilitation.

Mastoid Vibration Reduces Ipsilesional Shift of Subjective Visual Horizontal in Patients with Acute Stage of Unilateral Vestibulopathy

BACKGROUND AND OBJECTIVES: To investigate effect of the vibration on subjective visual horizontal (SVH) in patients with acute stage of unilateral vestibulopathy. SUBJECTS AND METHODS: Twenty-five unilateral vestibulopathy patients which analyzed into 42 cases at different time points and suffered from spinning vertigo for more than 24 hours without hearing loss and neurologic abnormality were enrolled. Thirteen subjects with spontaneous nystagmus (>3 degree/sec; averaged symptom onset <1 week) at the time of SVH measurement were classified into the acute unilateral vestibulopathy group (aVU). The other 29 subjects without spontaneous nystagmus were classified into the compensated vestibulopathy group (cVU). SVH was performed with vibration at either mastoid or sterocleidomastoid muscle. RESULTS: In the analysis of overall subjects, vibration did not significantly change the degree of shift of SVH. However, analyzed by group, the shift of SVH with vibration at ipsilesional mastoid was significantly decreased than baseline in aVU (p<0.05). The shift of SVH with vibration at contralesional mastoid was significantly increased than baseline in cVU (p=0.05). CONCLUSIONS: The shift of SVH due to vibration in acute stage of unilateral vestibulopathy showed reduction of the shift, while in compensated stage it showed increase of the shift.

Brain Hypometabolism in Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome / 대한평형의학회지

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (¹⁸F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in ¹⁸F-FDG PET.

Análise quali-quantitativa de idosos submetidos a um programa estruturado de reabilitação vestibular / Quali-quantitative analysis of elderly people submitted to a structured program of vestibular rehabilitation

Introdução: A reabilitação vestibular busca minimizar sintomas vestibulares ajudando a melhorar a qualidade de vida de indivíduos com doenças vestibulares. Objetivo: Analisar qualiquantitativamente a qualidade de vida de idosos submetidos a um programa estruturado de reabilitação vestibular. Métodos: Participaram do estudo cinco indivíduos de ambos os gêneros com idade média de 67,8 ± 4,12 anos. Os participantes foram submetidos a um programa estruturado para reabilitação vestibular durante duas sessões semanais, totalizando 15 sessões. Para avaliação quantitativa, utilizou-se a Escala de Quantificação de Tontura e o Dizziness Handicap Inventory (DHI) pré e pós-reabilitação e para análise qualitativa aplicou-se em forma de entrevista não-diretiva perguntas para coleta de dados sobre melhora do quadro clínico nas atividades cotidianas, sociais e domiciliares pós-reabilitação. Resultados: Quanto aos dados quantitativos, observou-se melhora significativa no domínio físico (p = 0,043) e qualidade de vida geral (p = 0,043), além da melhora de sintomatologia de tontura (p = 0,042). Tais resultados corroboraram a melhora relatada pelos participantes sobre as atividades de vida diária. Conclusão: Concluiu-se que o programa de exercício para reabilitação vestibular contribuiu para melhorar os domínios emocionais, funcionais e físicos e da qualidade de vida geral, bem como a sintomatologia de tontura, o que corroborou os relatos dos participantes. (AU)

Introduction: The vestibular rehabilitation aims to minimize vestibular symptoms helping to improve the quality of life of elderly with vestibular diseases. Objective: To analyze qualiquantitatively the quality of life of elderly submitted to a structured program of vestibular rehabilitation. Methods: Participated in the study five patients of both genders with a mean age of 67.8 ± 4.12 years. The participants were submitted to a structured program for vestibular rehabilitation. For quantitative assessment we used the Dizziness Quantification Scale and Dizziness Handicap Inventory (DHI) before and after rehabilitation and for qualitative assessment was applied an interview non-directive questions to collect data about clinical improvement in daily, social and domiciliary activities after rehabilitation. Results: As regards to quantitative data, we observed significant improvement on the physical domain (p = 0.043) and overall quality of life (p = 0.043), as well as improvement in the symptomatology of dizziness (p = 0.042). These results corroborate the improvement reported by participants about the activities of daily living. Conclusion: We concluded that the exercise program for vestibular rehabilitation contributed to the improvement of the emotional, functional, physical domains and overall quality of life and the symptomatology of dizziness, which corroborated the reports of participants. (AU)

Genetic Approach of Dizziness / 대한평형의학회지

Patients with recurrent vertigo/dizziness or unsteadiness are a heterogeneous group of complex disorders affecting the peripheral and central vestibular system. They represent a diagnostic challenge for the clinicians, and their genetic basis is largely not known. However, there are some cerebellar and vestibular disorders with a strong genetic background, such as episodic ataxia, spinocerebellar ataxia, vestibular migraine, Meniere's disease, and autosomal dominant nonsyndromic deafness. Furthermore, recent advances in next generation sequencing technique are increasing the number of novel genes associated with cerebellar and vestibular disorders. In this article, we have summarized clinical and molecular genetics findings in neuro-otology.

Acute Ischemic Vestibulopathy without Hearing Loss

Acute isolated vestibulopathy could be caused by either viral inflammation or ischemia. However, diagnosing ischemic vestibulopathy is difficult due to the ambiguity of the causal relationship between ischemia and peripheral vestibulopathy. We present here a patient with acute ischemic vestibulopathy without hearing loss caused by internal auditory artery occlusion, indirectly proven by the finding of a simultaneously developed silent cerebellar infarction. The details of this case suggest that patients with acute isolated peripheral vestibulopathy should be carefully evaluated for underlying causes, including vascular risk factors.

Residual Vestibulo-Ocular Reflex Result in Better Functional Outcomes in Bilateral Vestibulopathy / 대한평형의학회지

BACKGROUND AND OBJECTIVES: The patients with bilateral vestibulopathy (BV) suffer from unsteadiness and oscillopsia, and despite of appropriate rehabilitation, permanent disability is inevitable. However, the level of functional outcomes could be influenced by whether there is residual vestibulo-ocular reflex (VOR) or not. Under the hypothesis that residual VOR function could result in better performances, we tried to compare in functional outcomes between complete and incomplete BV. MATERIALS AND METHODS: Fifty patients who have been diagnosed with BV in our institution were retrospectively reviewed retrospectively between 2008 and 2012. We classified them into complete BV group (n=19) and incomplete BV (n=31) group according to the presence of residual VOR. Among them, 31 patients responded to telephone survey (6 in complete group and 25 in incomplete group). The survey includes 5 categories such as the subjective dizziness restriction on daily life, oscillopsia, unsteadiness and depression. Each score ranged from 0 to 4 and patients were asked twice in different time period. RESULTS: There was no difference in etiology between complete and incomplete group. Incomplete group showed significant improvement in dizziness, restriction on daily life, oscillopsia and unsteadiness compared to complete group. Among 5 catergories, dizziness score was significantly improved in incomplete group (each mean improved sore+/-standard deviation; dizziness 1.84+/-0.83, oscillopsia 0.44+/-0.64, unsteadiness 1+/-1.09, depression 0.24+/-0.86 restriction on daily life 1.16+/-0.97). CONCLUSION: The presence of residual VOR function had better functional outcomes in bilateral vestibulopathy.

Bilateral Vestibulopathy with Alcohol Abuse and Vitamin Deficiency / 대한평형의학회지

Bilateral vestibulopathy (BV) is a clinical entity with impaired function of bilateral peripheral vestibular system, which is characterized by movement-induced vertigo, oscillopsia and gait unsteadiness. Among various etiologies of BV, alcohol and vitamin B deficiency has rarely been reported. We experienced a case of BV with vitamin B deficiency in a 24-year-old man who was previously exposed to alcohol. He had osillopsia and gait unsteadiness as a primary symptom, and was treated successfully with vestibular rehabilitation and vitamin supplement. Bithermal caloric test, rotatory chair test and head impulse test showed the result compatible with BV.