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.
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.
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.
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.
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. 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.
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.
Key words: Herpes zoster oticus, Bilateral vestibulopathy, Autoimmune, Sympathetic vestibulopathy
Among various etiologies of bilateral vestibulopathy, meningitis has rarely been reported and no case by varicellazoster 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.
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.