Pearls & Oy-sters: Ocular Myasthenia Gravis: Central Ocular Motor Signs and Unilateral Visual Loss Caused by the Great Neuro-Ophthalmologic Impersonator.

Myasthenia gravis (MG) has been described as a great mimicker of other neurologic and ocular motility disorders, including centrally mediated ophthalmoplegia. For example, ocular myasthenia gravis (ocular MG) may cause impaired binocular visual acuity for near vision due to reduced accommodation or for distance vision due to accommodative excess. Notably, accommodative excess due to ocular MG is rare, but may occur with exotropia, with or without diplopia. We report 2 cases of ocular MG: First, a 32-year-old man with exotropia, bilateral hypometric and slowed adducting saccades with dissociated abducting nystagmus, miosis, and decreased distance vision in his right eye; second, a 45-year-old man with similar ocular motor deficits, miosis, and myopia. Both patients showed ocular motor deficits which appeared to localize to the pons but were instead due to ocular MG. Ocular MG should be considered in patients who present with reduced visual acuities due to any disruption in accommodation. Any ocular motor deficit, even if appearing to be centrally mediated or occurring without ptosis, may be caused by ocular MG.

Ophthalmologic and neuro-ophthalmologic findings in children with Down syndrome.

Down syndrome, also known as Trisomy 21, is a genetic disorder associated with mild-to-moderate intellectual disability, delays in growth, and characteristic facial features. A wide range of ocular complications are seen in children with Down syndrome, including strabismus, nystagmus, refractive errors, congenital cataracts, the presence of keratoconus, and decreased visual acuity. Early ophthalmic examination is needed for early diagnosis and treatment in patients. This narrative review examines ocular manifestations in children with Down syndrome and the importance of prompt ophthalmic interventions for treatment.

Case series: Downbeat nystagmus in SCA27B.

BACKGROUND: Spinocerebellar ataxia (SCA) 27B, first reported in late 2022, is caused by the abnormal expansion of GAA repeats in the first intron of the FGF14 gene, which encodes the fibroblast growth factor 14. CASE PRESENTATION: We present two late-onset cases, each manifesting mild cerebellar ataxia accompanied by omnidirectional downbeat nystagmus, which was enhanced in a suspended head position. None of the patients exhibited impaired head impulse or caloric tests. Repeat-primed PCR and targeted long-read nanopore sequence analysis of the FGF14 GAA repeat site identified more than 250 repeats, leading to the diagnosis of SCA27B. DISCUSSION: Downbeat nystagmus is reported to be associated with disturbances in the suppression of the vestibulo-ocular reflex (VOR). Our patients with SCA27B demonstrated downbeat nystagmus, likely due to a disruption of the VOR at the level of the cerebellar cortex, a potentially characteristic clinical feature of SCA27B. We have included video footages of eye movements recorded using Frenzel goggles for these cases. CONCLUSIONS: Omnidirectional downbeat nystagmus may be a distinctive clinical feature of SCA27B.

Early treatment for benign paroxysmal positional vertigo secondary to sudden sensorineural hearing loss.

Sudden sensorineural hearing loss (SSNHL) accompanied by benign paroxysmal positional vertigo (BPPV) is relatively common in the clinic. There are unified standards for the treatment of primary BPPV with good reduction effect, while there are few studies on the treatment of BPPV secondary to SSNHL within 1 week of onset. The study was to investigate the treatment of BPPV secondary to SSNHL and compare its manual reduction with that of primary BPPV. We selected 90 patients with BPPV accompanied by SSNHL within a week of onset and 210 primary BPPV patients at Hebei Provincial Eye Hospital from June 2020 to December 2022. The former group was divided into the medicine group and manual reduction plus medicine group. The medicines used were extract of Ginkgo biloba leaves injection, betahistine hydrochloride injection and oral prednisone. We contrasted the efficacy respectively for posterior semicircular canal BPPV (psc-BPPV), horizontal semicircular canal BPPV (hsc-BPPV) and multiple semicircular canal BPPV (msc-BPPV). In addition, we compared the manual reduction effect for primary BPPV and manual reduction group, and the evaluation of efficacy are the intensity of nystagmus and the clinical symptoms. In the secondary BPPV group, there was no difference in efficacy between the medicine group and manual reduction group at the 7th-day after reduction for psc-BPPV, hsc-BPPV, and msc-BPPV (P > .05). The immediate effect of reduction was significantly different between the primary BPPV group and the group with SSNHL and BPPV for both psc-BPPV and hsc-BPPV (P < .05), and the effect of the primary BPPV group was better, but it was no difference for msc-BPPV (P > .05). For the treatment of BPPV accompanied by SSNHL within 1 week of onset, the additional reduction therapy showed no benefit, so we need to apply medication for SSNHL.

The patient with acute vertigo - the role of clinical examination and imaging.

Vertigo/dizziness or balance disorders are among the most common patients complaints in emergency clinics. Up to 25% of them are potentially life-threatening, especially cardiovascular or cerebrovascular events. The combination of a careful history taking (triggers, duration of difficulties, associated symptoms) and the performance of a basic vestibular examination (nystagmus, oculomotor, head impulse test, positional maneuvers, standing and walking examination) leads to a reliable differentiation of central and peripheral vestibular etiology. Standardized diagnostic algorithms (HINTS, HINTS+, STANDING) are used to identify high-risk patients requiring urgent care. Imaging methods must be interpreted with caution to their low sensitivity in acute phase (sensitivity of non-contrast brain CT for ischemia in the posterior cranial fossa is only 16%, MRI of the brain is false negative in up to 20% of cases in stroke patients in the first 48 hours).

[Ictal nystagmus: acquired nystagmus of infrequent cause in pediatrics]. / Nistagmo ictal: causa infrecuente de nistagmo adquirido en pediatría.

Ictal nystagmus is a rare phenomenon secondary to an epileptic seizure, usually in the temporo- occipital region. For its characterization, we must rely on clinical history, examination, and ideally observation of the episodes. OBJECTIVES: To describe a case of this unusual entity and highlight the characteristics that should increase diagnostic suspicion in order to avoid treatment delay. CLINICAL CASE: An 8-year-old schoolboy, with no relevant history, consulted due to 5-6 episodes a day in the last year of conjugate horizontal eye movements with rapid jerks and associated slight miosis, lasting 5-10 seconds, with doubtful disconnection from the environment or consciousness impairment in some of the episodes, with no other accompanying signs or symptoms. Neurological examination between episodes was normal. He was evaluated by ophthalmology and otolaryngology, which ruled out pathology in these areas. Video-electroencephalogram showed electro-clinical correlations, with epileptiform activity in the left temporal and occipital region, which subsequently generalized during episodes. Brain MRI showed no pathological findings. After initiation of carbamazepine treatment, the patient had a good evolution, without recurrence of the episodes at 2 years of follow-up. CONCLUSIONS: When faced with a case of acquired nystagmus, epileptic etiology should be included in the differential diagnosis, especially if the frequency of episodes is high, of short duration, and associated with consciousness impairment. The diagnosis is based on a video-electroencephalogram with electro-clinical correlations and a good response to treatment with antiepileptic drugs is expected.

The "Vestibular Eye Sign"-"VES": a new radiological sign of vestibular neuronitis can help to determine the affected vestibule and support the diagnosis.

INTRODUCTION: Nystagmus is a valuable clinical finding. Although nystagmus is often described by the direction of its quick phases, it is the slow phase that reflects the underlying disorder. The aim of our study was to describe a new radiological diagnostic sign called "Vestibular Eye Sign"-VES. This sign is defined as an eye deviation that correlates with the slow phase of nystagmus (vestibule pathological side), which is seen in acute vestibular neuronitis and can be assessed on a CT head scan. MATERIALS AND METHODS: A total of 1250 patients were diagnosed with vertigo in the Emergency Department at Ziv Medical Center (ED) in Safed, Israel. The data of 315 patients who arrived at the ED between January 2010 and January 2022 were collected, with criteria eligible for the study. Patients were divided into 4 groups: Group A, "pure VN", Group B, "non-VN aetiology", Group C, BPPV patients, and Group D, patients who had a diagnosis of vertigo with unknown aetiology. All groups underwent head CT examination while in the ED. RESULTS: In Group 1, pure vestibular neuritis was diagnosed in 70 (22.2%) patients. Regarding accuracy, VES (Vestibular Eye Sign) was found in 65 patients in group 1 and 8 patients in group 2 and had a sensitivity of 89%, specificity of 75% and a negative predictive value of 99.4% in group 1-pure vestibular neuronitis. CONCLUSION: VN is still a clinical diagnosis, but if the patient undergoes head CT, we suggest using the "Vestibular Eye Sign" as a complementary sign. As per our findings, this is a valuable sign on CT imaging for diagnosing the pathological side of isolated pure VN. It is sensitive to support a diagnosis with a high negative predictive value.

Impact of Clinician Training Background and Stroke Location on Bedside Diagnostic Test Accuracy in the Acute Vestibular Syndrome - A Meta-Analysis.

OBJECTIVE: Acute dizziness/vertigo is usually due to benign inner-ear causes but is occasionally due to dangerous neurologic ones, particularly stroke. Because symptoms and signs overlap, misdiagnosis is frequent and overuse of neuroimaging is common. We assessed the accuracy of bedside findings to differentiate peripheral vestibular from central neurologic causes. METHODS: We performed a systematic search (MEDLINE and Embase) to identify studies reporting on diagnostic accuracy of physical examination in adults with acute, prolonged dizziness/vertigo ("acute vestibular syndrome" [AVS]). Diagnostic test properties were calculated for findings. Results were stratified by examiner type and stroke location. RESULTS: We identified 6,089 citations and included 14 articles representing 10 study cohorts (n = 800). The Head Impulse, Nystagmus, Test of Skew (HINTS) eye movement battery had high sensitivity 95.3% (95% confidence interval [CI] = 92.5-98.1) and specificity 92.6% (95% CI = 88.6-96.5). Sensitivity was similar by examiner type (subspecialists 94.3% [95% CI = 88.2-100.0] vs non-subspecialists 95.0% [95% CI = 91.2-98.9], p = 0.55), but specificity was higher among subspecialists (97.6% [95% CI = 94.9-100.0] vs 89.1% [95% CI = 83.0-95.2], p = 0.007). HINTS sensitivity was lower in anterior cerebellar artery (AICA) than posterior inferior cerebellar artery (PICA) strokes (84.0% [95% CI = 65.3-93.6] vs 97.7% [95% CI = 93.3-99.2], p = 0.014) but was "rescued" by the addition of bedside hearing tests (HINTS+). Severe (grade 3) gait/truncal instability had high specificity 99.2% (95% CI = 97.8-100.0) but low sensitivity 35.8% (95% CI = 5.2-66.5). Early magnetic resonance imaging (MRI)-diffusion-weighted imaging (DWI; within 24-48 hours) was falsely negative in 15% of strokes (sensitivity 85.1% [95% CI = 79.2-91.0]). INTERPRETATION: In AVS, HINTS examination by appropriately trained clinicians can differentiate peripheral from central causes and has higher diagnostic accuracy for stroke than MRI-DWI in the first 24-48 hours. These techniques should be disseminated to all clinicians evaluating dizziness/vertigo. ANN NEUROL 2023;94:295-308.

Diagnosing and localizing the acute vestibular syndrome - Beyond the HINTS exam.

When assessing the acutely dizzy patient, the HINTS 'Plus' (Head Impulse, Nystagmus, Test of Skew, 'Plus' a bedside assessment of auditory function) exam is a crucial component of the bedside exam. However, there are additional ocular motor findings that can help the clinician distinguish peripheral from central etiologies and enable accurate localization, especially when the patient has acute dizziness, vertigo and/or imbalance but without spontaneous nystagmus. We will review the literature on these findings which are 'beyond HINTS' and include saccades/ocular lateropulsion, smooth pursuit, and provocative maneuvers including head-shaking and positional testing (not part of the HINTS exam). Additionally, we will expound on the localizing value of nystagmus, ocular alignment and the ocular tilt reaction (parts of the HINTS exam). The paper has been organized neuroanatomically, based on brainstem and cerebellar structures that have been reported to cause the acute vestibular syndrome.

Infantile nystagmus without overt eye abnormality: Early features and neuro-ophthalmological diagnosis.

AIM: To analyse the neuro-ophthalmological data of children referred for further work-up of infantile nystagmus where ophthalmological evaluation had not achieved a diagnosis. METHOD: We retrospectively reviewed medical records of patients presenting with infantile nystagmus at our institution between 2007 and 2019. Inclusion criteria were onset before 6 months of age, availability of complete ophthalmic examination, visual electrophysiological tests, and neurological examination. Children with a previous definite ophthalmological diagnosis at onset and those with uncertain nystagmus onset age were not recruited. RESULTS: Out of 142 infants (mean age at nystagmus onset 3.6 mo, SD 1.7, range 0-6 mo; 56 females, 86 males), 23% had neurological nystagmus, 7% mixed neurological and sensory nystagmus, 48% sensory defect, and 22% idiopathic infantile nystagmus. The neurological diagnoses were inborn errors of metabolism, white matter genetic disorders, and brain malformations. The prevalent diagnosis in the sensory defect subgroup was retinal dystrophy. INTERPRETATION: Infantile nystagmus without diagnostic ocular findings may be due to neurological, retinal, and optic nerve disorders or be a benign idiopathic condition. In infants with and without neurological abnormalities, the search for a sensory defect should include visual electrophysiology performed early in the diagnostic pathway. WHAT THIS PAPER ADDS: Infantile nystagmus without diagnostic ophthalmological signs has an underlying neurological cause in 30% of cases. Neurological diagnoses include congenital brain malformations, and metabolic and genetic disorders. Sensory defects are part of systemic neurological disorders in 23% of infants. Electrophysiology is useful when ophthalmological examination is uninformative.

Discordant horizontal-torsional nystagmus: a sign of posterior semicircular canal dysfunction.

In central as well as peripheral vestibular lesions, right-beating horizontal nystagmus is almost always associated with clockwise (top poles of the eyes beating to the right ear) torsional nystagmus when observed and vice versa (concordant nystagmus). This study aimed to determine the etiologies and mechanisms of horizontal and torsional nystagmus beating in the opposite directions (discordant nystagmus). We reviewed the medical records of 16 consecutive patients with discordant horizontal-torsional nystagmus who had been evaluated at the dizziness clinics of Seoul National University Bundang Hospital (n = 11, from March 2003 to March 2021) and Korea University Medical Center (n = 5, from March 2019 to March 2021). The underlying etiologies included inferior vestibular neuritis (n = 7), Meniere's disease (n = 4), internuclear ophthalmoplegia (n = 3), medullary hemorrhage (n = 1), and normal pressure hydrocephalus (n = 1). The torsional nystagmus decreased during the gaze in the same direction (for instance, during rightward gaze in clockwise nystagmus) and increased during the gaze in the opposite direction. Head-impulse tests (HITs) were positive for the ipsilesional posterior canal (PC) in all 11 patients with unilateral peripheral vestibulopathy and two of the three patients with unilateral central vestibulopathy. Discordant horizontal-torsional nystagmus may be observed in peripheral as well as central lesions. Given the findings of HITs and modulation of spontaneous nystagmus during lateral gazes, discordant horizontal-torsional nystagmus may be ascribed to selective damage of the excitatory or inhibitory pathway from the PC that innervates the ipsilateral superior oblique and contralateral inferior rectus muscles.

Vestibular Disorders after Kidney Transplantation: Focus on the Pathophysiological Mechanisms Underlying the Vertical Nystagmus Associated with Tacrolimus-Related Hypomagnesamia.

The purpose of this paper is to present the case of a patient undergoing kidney transplantation who developed limb tremor dizziness and vertical nystagmus (ny) during Tacrolimus (TAC) therapy and to investigate the pathophysiological mechanisms underlying the balance disorder. This case study regards a 51-year old kidney transplant male patient with hand tremors and lower limbs asthenia associated with dizziness and nausea. The symptoms started two months after the beginning of intravenous TAC for renal transplantation. The pure-tone audiometry showed a mild symmetrical high-frequencies down-sloping sensorineural hearing loss. Acoustic emittance measures showed a normal tympanogram; stapedial reflexes were normally elicited. The Auditory Brainstem Responses (ABR) and Cervical Vestibular Evoked Myogenic Potentials (c-VEMPs) were bilaterally normally evoked. The bedside vestibular examination showed spontaneous down-beating stationary persistent, omni-positional nystagmus, not inhibited by fixation. The Head-Shaking Test accentuates the spontaneous ny. The horizontal clinical head impulse test was negative, bilaterally. A biochemical blood test revealed a decrease in Magnesium (Mg) levels (0.8 mg/dL; normal range 1.58-2.55). The integration of Mg induced both a plasma levels normalization and an improvement of clinical symptoms. This case suggests that TAC treatment can induce a Mg depletion that caused the transient cerebellar lesion. Therefore, the monitoring of serum electrolytes during immunosuppressive treatment appears to be a useful tool in order to reduce the central system symptomatology.

Clinician's perspectives in using head impulse-nystagmus-test of skew (HINTS) for acute vestibular syndrome: UK experience.

BACKGROUND: Acute vestibular syndrome (AVS) features continuous dizziness and may result from a benign inner ear disorder or stroke. The head impulse-nystagmus-test of skew (HINTS) bedside assessment is more sensitive than brain MRI in identifying stroke as the cause of AVS within the first 24 hours. Clinicians' perspectives of the test in UK secondary care remains unknown. Here, we explore front-line clinicians' perspectives of use of the HINTS for the diagnosis of AVS. METHODS: Clinicians from two large UK hospitals who assess AVS patients completed a short online survey, newly designed with closed and open questions. RESULTS: Almost half of 73 total responders reported limited (n=33), or no experience (n=19), reflected in low rates of use of HINTS (n=31). While recognising the potential utility of HINTS, many reported concerns about subjectivity, need for specialist skills and poor patient compliance. No clinicians reported high levels of confidence in performing HINTS, with 98% identifying training needs. A lack of formalised training was associated with onward specialist referrals and neuroimaging (p=0.044). CONCLUSIONS: Although the low sample size in this study limits the generalisability of findings to wider sites, our preliminary data identified barriers to the application of the HINTS in AVS patients and training needs to improve rapid, cost-effective and accurate clinical diagnosis of stroke presenting with vertigo.

Oncologic causes of oculopalatal tremors: neurophysiology and treatment.

Oculopalatal tremor (OPT) is an acquired pathology characterized by continuous and rhythmical soft palatal movements combined with pendular nystagmus. Aside from vascular lesions, oncological masses affecting the dentatorubro-olivary pathway can impair brainstem and/or cerebellar pathways, manifesting as dyssynchronous movement. In this review, we delve into the neurophysiology of OPT along with oncological causes and treatment options based on the most recent clinical trial data. This literature review includes medication treatment data from clinical trials enrolling individuals with features of OPT, including acquired pendular nystagmus (APN). Trials were deemed eligible for inclusion in this review if one or more participants had symptoms determined by the trial authors to be caused by OPT. Trials investigating the treatment of APN secondary to a separate cause, such as multiple sclerosis, were excluded from this review. Several early treatments failed to demonstrate a benefit for patients with APN due to OPT. Trials of anticholinergic agents were largely ineffective and poorly tolerated. Botulinum toxin A demonstrated improvement in APN symptoms. Most recently, trials including memantine and gabapentin have demonstrated success with attenuation of APN. Surgical modalities such as DBS have yet to show improvement, though with only a single case report as evidence. Oculopalatal tremor is a unique manifestation of posterior fossa tumors disrupting the Guillain-Mollaret triangle. Symptom control through medication management has had limited success attributed to poor response and medication intolerance. Surgical modalities like DBS may have an emerging role in OPT treatment by targeting dyssynchronous activity in the dentatorubro-olivary pathway.

Evident hypopigmentation without other ocular deficits in Dutch patients with oculocutaneous albinism type 4.

To describe the phenotype of Dutch patients with oculocutaneous albinism type 4 (OCA4), we collected data on pigmentation (skin, hair, and eyes), visual acuity (VA), nystagmus, foveal hypoplasia, chiasmal misrouting, and molecular analyses of nine Dutch OCA4 patients from the Bartiméus Diagnostic Center for complex visual disorders. All patients had severely reduced pigmentation of skin, hair, and eyes with iris transillumination over 360 degrees. Three unrelated OCA4 patients had normal VA, no nystagmus, no foveal hypoplasia, and no misrouting of the visual pathways. Six patients had poor visual acuity (0.6 to 1.0 logMAR), nystagmus, severe foveal hypoplasia and misrouting. We found two novel variants in the SLC45A2 gene, c.310C > T; (p.Pro104Ser), and c.1368 + 3_1368 + 9del; (p.?). OCA4 patients of this Dutch cohort all had hypopigmentation of skin, hair, and iris translucency. However, patients were either severely affected with regard to visual acuity, foveal hypoplasia, and misrouting, or visually not affected at all. We describe for the first time OCA4 patients with an evident lack of pigmentation, but normal visual acuity, normal foveal development and absence of misrouting. This implies that absence of melanin does not invariably lead to foveal hypoplasia and abnormal routing of the visual pathways.

Evaluation of the vestibular system in individuals with presbycusis using video head impulse test and videonystagmography.

BACKGROUND: Vestibulo-ocular reflex (VOR) function is expected to be normal in patients with presbycusis during sudden head rotations. AIM: This study aimed to determine whether presbycusis was accompanied by vestibular system pathologies. In addition, it was examined whether there was a difference existed between the patients with and without presbycusis in terms of normative data. MATERIALS AND METHODS: A total of 40 individuals were included in the study: 20 in the presbycusis group and 20 in the control group. The vestibular systems of both groups were evaluated using the video head impulse test and videonystagmography. RESULTS: The right and left lateral VOR gain values were decreased in the group with presbycusis compared to the control group. The difference between the two groups in the mean VOR gains in the right lateral canal and left lateral canal were statistically significant (p = .040 and p = .050, respectively). The air caloric tests of all individuals were found to be normal. CONCLUSIONS: This result suggests that the loss of vestibular hair cells and vestibular nerve degeneration in the lateral semicircular canal may be more severe in presbycusis than in the same age group with normal hearing.

Comparison of the Efficacy of Video Head Impulse and Bi-Thermal Caloric Tests in Vertigo.

OBJECTIVE: To compare video head impulse test (vHIT) and caloric test efficacy in decompensated and compensated vertigo patients and to further investigate whether vHIT alone can be used as a diagnostic tool in vertigo. METHODS: This study included 25 patients diagnosed with vertigo and without any previous history of vertigo or hearing loss before their admission to our clinic. The control group consisted of 16 healthy adult volunteers. Patients were classified into 2 groups, compensated and decompensated. Video head impulse test and caloric tests were performed and the results were compared between the groups. RESULTS: The difference of caloric test values between control-compensated groups and compensated-decompensated groups was statistically significant (P < .001, Pearson χ2). However, there was no statistically significant difference between the compensated and control groups according to vHIT gain asymmetry values (P = .087). In the very early stages of the disease with spontaneous nystagmus, the diagnostic significance of vHIT was similar to that of the caloric test. When both sides were compared, vHIT gain asymmetry values were close to the caloric test asymmetry values. In the compensated stage, caloric test was superior to vHIT in differentiating compensated vestibular pathologies. When vHIT sensitivity was evaluated according to the bi-thermal caloric test results, the sensitivity of the vHIT gain asymmetry value was 85.71% and 23.08% for decompensated and compensated patients respectively. CONCLUSION: In the early decompensated stages of the disease with spontaneous nystagmus, vHIT shows similar diagnostic accuracy to that of the caloric test. Since patients can tolerate vHIT more easily, our results suggest that vHIT can be considered as a primary evaluation method in the early (decompensated) period of the disease and should be preferred over the caloric test during the acute phase. Caloric test is more reliable at the compensated stage. Video head impulse test is inadequate in evaluating the compensated vestibular hypofunctional states after compensation has been restored. LEVEL OF EVIDENCE: Level 2b.

Sparse classification of discriminant nystagmus features using combined video-oculography tests and pupil tracking for common vestibular disorder recognition.

Vertigo is a common sign related to a problem with the brain or vestibular system. Detection of ocular nystagmus can be a support indicator to distinguish different vestibular disorders. In order to get reliable and accurate real time measurements from nystagmus response, video-oculography (VOG) plays an important role in the daily clinical examination. However, vestibular diseases present a large diversity in their characteristics that leads to many complications for usual analysis. In this paper, we propose a novel automated approach to achieve both selection and classification of nystagmus parameters using four tests and a pupil tracking procedure in order to give reliable evaluation and standardized indicators of frequent vestibular dysfunction that will assist clinicians in their diagnoses. Indeed, traditional tests (head impulse, caloric, kinetic and saccadic tests) are applied to obtain clinical parameters that highlight the type of vertigo (peripheral or central vertigo). Then, a pupil tracking method is used to extract temporal and frequency nystagmus features in caloric and kinetic sequences. Finally, all extracted features from the tests are reduced according to their high characterization degree by linear discriminant analysis, and classified into three vestibular disorders and normal cases using sparse representation. The proposed methodology is tested on a database containing 90 vertiginous subjects affected by vestibular Neuritis, Meniere's disease and Migraines. The presented technique highly reduces labor-intensive workloads of clinicians by producing the discriminant features for each vestibular disease which will significantly speed up the vertigo diagnosis and provides possibility for fully computerized vestibular disorder evaluation.

Nystagmus in Ramsay Hunt syndrome with or without dizziness.

BACKGROUND: The presence of dizziness has been reported as a negative prognostic factor for recovery of facial palsy in Ramsay Hunt syndrome (RHS). The aim of this study was to investigate the incidence and patterns of nystagmus in RHS patients without dizziness, and discuss possible mechanisms. We also compared the severity and prognosis of facial palsy between RHS patients with and without dizziness. METHODS: From January 2014 to January 2019, 36 patients diagnosed with RHS (27 with dizziness and 9 without dizziness) were included. Patterns of nystagmus were examined and categorized using video-nystagmography. House-Brackmann(HB) grade of facial palsy was compared between RHS patients with and without dizziness. RESULTS: Not only RHS patients with dizziness exhibited nystagmus in most cases (96%, 26 of 27) but also as many as 67% (6 of 9) of RHS patients without dizziness exhibited nystagmus, though the intensity was remarkably weak. In both groups of RHS with and without dizziness, direction-fixed nystagmus and direction-changing positional nystagmus were observed. Initial HB grade and recovery of facial palsy after treatment were not significantly different between RHS with and without dizziness. CONCLUSION: Various patterns of nystagmus including direction-fixed and positional direction-changing nystagmus were observed in RHS patients, and inflammation of the vestibular nerve and inner ear end organs may be responsible for the production of nystagmus in these patients. The results support that the evaluation of vestibular function may be necessary even in RHS patients who do not complain of dizziness or vertigo.

Epileptic nystagmus due to a large parieto-temporo-occipital multilobar dysplasia.

We report a young female with medically refractory multiple daily seizures since childhood with semiology suggestive of an epileptic nystagmus. She had a large multilobar parieto-temporo-occipital dysplasia and became seizure-free after parieto-temporo-occipital disconnection with preserved visual functions.