Objectives This study was performed to investigate the efficacy of the fixation index (FI) of the bithermal caloric test for differentiating brain lesions in vestibular disorder.
Methods We reviewed the medical records of 286 consecutive dizzy patients who underwent brain magnetic resonance imaging (MRI) and the bithermal caloric test at department of otorhinolarnygology. Central vestibulopathy (CVP) was defined as when corresponding lesion was identified on brain MRI, otherwise peripheral vestibulopathy (PVP) was defined. The FI was defined as the mean slow phase velocity (SPV) with fixation divided by the mean SPV without fixation, and failure was indicated when the FI exceeded 70%.
Results The CVP confirmed by brain MRI and PVP were 16.8% and 83.2%, respectively. The most common CVPs were cerebellopontine angle tumor (n=19, 39.6%) and chronic cerebellar infarction (n=18, 37.5%). There were 23 cases of CVP (47.9%) and 47 cases of PVP (19.7%) with abnormal number of FI in at least two of the four caloric irrigations. The FI score of right cool (RC), left cool (LC), and right warm (RW) were also increased significantly in patients with CVP (p=0.031 at RCFI, p=0.014 at LCFI, p=0.047 at RWFI, and p=0.057 at LWFI; Mann-Whitney U-test).
Conclusions If two or more abnormal FIs are detected during bithermal caloric testing, there is a high likelihood of CVP. Consequently, additional brain MRI may be necessary for further evaluation.
Superficial siderosis (SS) is a rare neurodegenerative condition characterized by hemosiderin deposition in the central nervous system, which sometimes leads to sensorineural hearing loss and vestibular dysfunction. This case report details the diagnosis and treatment of a 63-year-old female patient presenting with a yearlong history of postural instability and recent symptoms of sudden right-sided hearing loss and imbalance. Physical examination and imaging revealed bilateral deafness and infratentorial SS. Treatment, including medication, vestibular exercises, high-dose steroids, and hyperbaric oxygen therapy, led to a significant alleviation of dizziness but no improvement in hearing. This case emphasizes the need for clinicians to consider SS in patients presenting with sudden-onset hearing loss and dizziness, even without prior any medical history or trauma, to accurately identify the underlying cause.
Thiamine deficiency may cause Wernicke encephalopathy (WE) characterized by ataxia, ophthalmoplegia, and confusion. Aside from the triad of cardinal symptoms, selective or predominant impairments of bilateral horizontal canals may be observed in WE. Here, we report a patient with gastric mucosa-associated lymphoid tissue (MALT)-lymphoma complicated by an early stage of WE due to thiamine deficiency manifesting bilateral vestibular dysfunctions. A 78-year-old man recently diagnosed gastric MALT-lymphoma presented with dizziness and disequilibrium for several weeks. He showed mild imbalance while tandem walking and abnormal catch-up saccades bilaterally during bedside head impulse test (HIT). Video HIT revealed decreased vestibulo-ocular reflex gains with catch-up saccades for the bilateral horizontal and several vertical canals. Diagnostic work-up demonstrated decreased serum thiamine and lesions in the mammillary body and periaqueductal gray matter on brain magnetic resonance images. Furthermore, with thiamine replacement, his clinical symptoms were markedly improved. Also, vestibular symptoms and signs may be an early manifestation of WE, and video HIT could be a useful diagnostic tool to aid early detections.
Bilateral vestibulopathy is a condition with vestibular hypofunction of both inner ears. Patients with this diagnosis will often complain of dizziness and/or imbalance in darkness and when walking in uneven terrain and will often also experience oscillopsia. Predominant etiology is idiopathic. A 73-year old man with complaints of dizziness for 2 days. Objective findings included spontaneous nystagmus, a positive Romberg test with eyes closed, and a pathological video head impulse test. Initial audiometry only revealed bilateral presbycusis. Following gradual non-complete remission of vertiginous symptoms, the patient was discharged and scheduled for follow-up. The patient was later readmitted due to gradual progressive bilateral hearing deterioration alongside persisting vertiginous symptoms. Various additional tests all came out negative, and the condition was classified as idiopathic acute bilateral vestibulopathy with concomitant progressive deterioration of binaural hearing. The patient was later referred to bilateral cochlear implantation. Acute monosymptomatic bilateral vestibulopathy is difficult to diagnose, as it requires very specific tests that are not routinely done by neurologists. Acute bilateral vestibulopathy with concomitant progressive deterioration of binaural hearing leading to bilateral anacusis is indeed so rare that it has not been possible to find any literature describing a similar case.
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.
Ramsay Hunt syndrome is an acquired paralysis of the face specifically caused by a varicella-zoster virus infection in the facial nerve. Other cranial nerves including vestibulo-cochlear disturbance can be affected. Herein we reported a case of Ramsay Hunt syndrome with atypical vestibular syndrome. Although central vestibular signs including direction changing post head-shaking nystagmus or normal head impulse test are generally meaningful, clinicians need to be careful to interpret them because some findings can be observed not only in cases of central disorders but also in peripheral disorders. Clinical findings such as distinct ear pain and close observation of vesicles are important to diagnose Ramsay Hunt syndrome.
A 25-year-old woman presented with atypical dizziness with oscillopsia. The neuro-otological evaluations showed bilateral vestibulopathy with mild sensorineural hearing loss. Computed tomography and magnetic resonance imaging demonstrated bilateral isolated lateral canal dysplasia (LSCD) with normal cochlea. LSCD is relatively common inner ear malformation, but it is rarely found in bilateral vestibulopathy. In case of patients with bilateral vestibulopathy who cannot find the cause, should be considered conducting radiological examinations in mind of the inner ear anomalies.
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.
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.
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.
Citations
Citations to this article as recorded by
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Re-fixation Saccade at Video-Head Impulse Test in Patients with Sudden Sensorineural Hearing Loss Dong Hyuk Jang, Sun Seong Kang, Hyun Joon Shim, Yong-Hwi An Research in Vestibular Science.2023; 22(2): 46. CrossRef
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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.
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.
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.
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 (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.