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.

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.

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.

Analysis of Clinical Features in Patients Showing Bilateral Vestibulopathy with Vestibular Function Test / 대한평형의학회지

BACKGROUND AND OBJECTIVES: Caloric test and rotatory chair test have been adopted in diagnosing bilateral vestibulopathy. However, most of patients who were confirmed by the diagnostic testing not complained typical symptoms of bilateral vestibulopathy such as ossilopsia and ataxia. Patients who do not have typical symptoms of bilateral vestibulopathy, were often diagnosed with bilateral vestibulopathy by caloric test and slow harmonic acceleration test (SHA). The aim of this study is to assess the clinical features between groups classified according to the caloric test and SHA test, and possibly to investigate the representative test in the diagnosis of bilateral vestibulopathy. MATERIALS AND METHODS: Seventy-five patients were divided into three groups: (A) patients diagnosed with the caloric test only, (B) patients diagnosed with SHA test only, (C) patients satisfying the diagnostic criteria of both tests. Clinical characteristics, the results of physical examination, hearing test and vestibular function test (VFT) were compared among three groups. Results: There was no difference in clinical characteristics and results of physical examination among three groups. Regarding VFT results, only in step velocity test, The proportion of patients who showed low gain value on both sides were higher in group C than that of group A and B. No difference was observed in the other VFT results among three groups. RESULTS: There was no difference in clinical characteristics and results of physical examination among three groups. Regarding VFT results, only in step velocity test, The proportion of patients who showed low gain value on both sides were higher in group C than that of group A and B. No difference was observed in the other VFT results among three groups. CONCLUSION: We could not predict the clinical features of bilateral vestibulopathy by the results of VFT, and could not find preferable test in diagnosing 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.

Bilateral Vestibulopathy / 대한이비인후과학회지

Bilateral vestibulopathy (BV) is a clinical entity with impaired function of bilateral peripheral vestibular system, which is characterized by movement-induced postural vertigo, oscillopsia and unsteadiness of gait. The possible causes are reported including aminoglycosides, Meniere's disease and meningitis, however, cause of BV remains unclear in up to half. The diagnosis is confirmed by a combination of the vestibulo-ocular reflex tests such as a bilateral head-impulse test, the rotatory chair test and the caloric test. The treatment consists of prophylaxis of progressive vestibular loss, recovery of impaired vestibular function and promotion of central compensation or substitution with rehabilitation therapy.

Bilateral Vestibulopathy due to Bilateral Cerebellopontine Angle Tumors in Neurofibromatosis Type 2

Bilateral vestibulopathy (BV) is characterized by oscillopsia and imbalance during locomotion. Half of the cases of BV are idiopathic, and BV due to brain tumor has been reported only rarely. We report a case of BV due to bilateral cerebellopontine-angle tumors in a patient with neurofibromatosis type 2. Careful history on oscillopsia and imbalance during locomotion, and regular follow-ups using the head-impulse test and dynamic visual acuity are warranted in patients with neurofibromatosis type 2.

Bilateral Vestibular Hypofunction Induced by Unilateral Herpes Zoster Oticus

Herpes zoster oticus usually accompanies vestibulopathy on the ipsilateral ear. However we have encountered two herpes zoster oticus patients with bilateral vestibulopathies. Bilateral vestibulopathy was detected on the 2nd month and 19th day of herpes zoster oticus, respectively. While the contralateral vestibulopathy was detected 2 month after the ipsilateral vestibulopathy in the first patient, the vestibular function deteriorated simultaneously on the 19th day in the second patient. It seems that the bilateral vestibulopathy was caused by an autoimmune process in both cases, but the initiating event is different. The ipsilateral vestibular damage may have sensitized the immune system in the first patient resulting in sympathetic vestibulopathy. But in the second patient, the ipsilateral cochlear damage may have sensitized the immune system resulting in simultaneous bilateral vestibulopathy.

Vestibular Neuritis and Bilateral Vestibulopathy

Vestibular neuritis is the second most common cause of peripheral vestibular vertigo. The key signs and symptoms are the acute onset of sustained rotatory vertigo without hearing loss, postural imbalance with Romberg's sign, and peripheral type nystagmus. Head thrust and caloric tests show ipsilateral hyporesponsiveness, but hearing test shows normal. Either an inflammation of the vestibular nerve or labyrinthine ischemia was proposed as a cause of vestibular neuritis. Recovery after vestibular neuritis is usually incomplete. Despite the assumed viral cause, the effects of corticosteroids, antiviral agents, or the two in combination are uncertain. Bilateral vestibulopathy is a rare disorder of the peripheral labyrinth or the eighth nerve. The most frequent etiologies include ototoxicity, autoimmune disorders, meningitis, neuropathies, sequential vestibular neuritis, cerebellar degeneration, tumors, and miscellaneous otological diseases. The two key symptoms are unsteadiness of gait and oscillopsia associated with head movements or when walking. The diagnosis is made with the simple bedside tests for defective vestibulo-ocular reflex (head thrust and dynamic visual acuity tests). Bilateral vestibulopathy is confirmed by the absence of nystagmus reaction to both caloric and rotatory chair tests. The spontaneous recovery is relatively rare and incomplete. Vestibular rehabilitation is supportive of the improvement, but the efficacy of physical therapy is limited.

A Case of Bilateral Vestibulopathy Caused by Varicella-Zoster Meningitis

Among various etiologies of bilateral vestibulopathy, meningitis has rarely been reported and no case by varicella- zoster meningitis. We experienced a case of bilateral vestibulopathy and aseptic meningitis developed in a 22-year old woman who was previously affected by chicken pox. She had oscillopsia and unsteadiness of gait when the typical skin lesion of chicken pox was improving. Neurootologic examination, bithermal caloric irrigation, and rotary chair test showed the result compatible with complete bilateral vestibulopathy. With mild headache and nausea, her CSF finding revealed aseptic meningitis and the serum antibody for varicella-zoster virus (VZV) was elevated. On the contrary of previous report that associated with bilateral vestibulopathy tend to poorly improved, this patient showed a favorable outcome with recovery of symptoms and caloric test.

Clinical Analysis of Bilateral Vestibulopathy

From retrospective review of medical charts of patients diagnosed as bilateral vestibulopathy during the past seven years, this study investigated incidence, etiologies, symptoms, vestibular function test findings; electronystagmography (ENG) studies, rotatory chair testing, and posturography, and post-vestibular rehabilitation (VRT) follow-up results. we have an incidence of 1.2% of all the 3423 patients who have undergone vestibular function test. Ototoxicity was the first known etiology and we had a lot of patients of idiopathic bilateral vestibulopathy. Most patients in our study had experiences of sudden onset vertigo rather than slowly progressive symptoms. The most frequently complained symptoms were dysequilibrium and oscillopsia. Post-VRT courses were better in patients whose initial gain of vestibulo-ocular reflex(VOR) was high or ascending type.