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Yuka Morita, Kuniyuki Takahashi, Shuji Izumi, Yamato Kubota, Shinsuke Ohshima, Arata Horii
OBJECTIVE: Otitis media (OM) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is a novel concept of ear disease that is characterized by progressive mixed or sensorineural hearing loss with occasional systemic involvement. Considering the accumulating knowledge about the characteristics of and treatment for auditory dysfunction in OMAAV, the objective of this study was to investigate the vestibular function and symptoms of patients with OMAAV. STUDY DESIGN: Retrospective chart review...
September 30, 2016: Otology & Neurotology
Laszlo Szpisjak, Nora Zsindely, Jozsef I Engelhardt, Laszlo Vecsei, Gabor G Kovacs, Peter Klivenyi
AARS2 gene (NM_020745.3) mutations result in two different phenotypic diseases: infantile mitochondrial cardiomyopathy and late-onset leukoencephalopathy. The patient's first symptoms appeared at the age of 18 years with behavioral changes and psychiatric problems. Some years later, extrapyramidal symptoms, cognitive impairment, nystagmus, dysarthria and pyramidal symptoms also developed. The brain magnetic resonance imaging (MRI) indicated extensive white matter abnormalities. The diagnosis of AARS2 gene mutations causing leukodystrophy was confirmed by genetic testing...
October 13, 2016: Journal of Human Genetics
Sung-Hee Kim, David S Zee, Sascha du Lac, Hyo Jung Kim, Ji-Soo Kim
OBJECTIVE: To describe the ocular motor abnormalities in 9 patients with a lesion involving the nucleus prepositus hypoglossi (NPH), a key constituent of a vestibular-cerebellar-brainstem neural network that ensures that the eyes are held steady in all positions of gaze. METHODS: We recorded eye movements, including the vestibulo-ocular reflex during head impulses, in patients with vertigo and a lesion involving the NPH. RESULTS: Our patients showed an ipsilesional-beating spontaneous nystagmus, horizontal gaze-evoked nystagmus more intense on looking toward the ipsilesional side, impaired pursuit more to the ipsilesional side, central patterns of head-shaking nystagmus, contralateral eye deviation, and decreased vestibulo-ocular reflex gain during contralesionally directed head impulses...
October 12, 2016: Neurology
Alberto Vogrig, Tiziano Zanoni, Giuseppe Moretto
No abstract text is available yet for this article.
October 11, 2016: JAMA: the Journal of the American Medical Association
Sertac Yetiser
Objective. The aim of this study is to analyze the hearing and vestibular outcome of patients with otosclerosis who have been operated on by fiber optic flexible CO2 laser. Study Design. A preliminary and retrospective study was conducted in 30 patients with otosclerosis. Results. Comparative analysis of average air conduction thresholds (53.41 ± 11.81 dB versus 26.37 ± 11.04 dB) and air-bone gaps (34 ± 9.92 dB versus 12.03 ± 6.02 dB) before and after the surgery were statistically significant (<0...
2016: International Journal of Otolaryngology
D Kaski, A M Bronstein
Functional (psychogenic) eye movement disorders are perhaps less established in the medical literature than other types of functional movement disorders. Patients may present with ocular symptoms (e.g., blurred vision or oscillopsia) or functional eye movements may be identified during the formal examination of the eyes in patients with other functional disorders. Convergence spasm is the most common functional eye movement disorder, but functional gaze limitation, functional eye oscillations (also termed "voluntary nystagmus"), and functional convergence paralysis may be underreported...
2017: Handbook of Clinical Neurology
Vivek P Buch, Peter J Madsen, Kerry A Vaughan, Paul F Koch, David K Kung, Ali K Ozturk
Rotational vertebrobasilar insufficiency, or bow hunter's syndrome, is a rare cause of posterior circulation ischemia, which, following rotation of the head, results in episodic vertigo, dizziness, nystagmus, or syncope. While typically caused by dynamic occlusion of the vertebral artery in its V2 and V3 segments, the authors here describe a patient with dynamic occlusion of the vertebral artery secondary to a persistent first intersegmental artery, a rare variant course of the vertebral artery. In this case the vertebral artery coursed under rather than over the posterior arch of the C-1...
October 7, 2016: Journal of Neurosurgery. Spine
Andoret van Wyk, Carina A Eksteen, Piet J Becker, Barbara M Heinze
INTRODUCTION: Visual impairment, specifically eye movement disorders and vestibular dysfunction may have a negative influence on the functional recovery in post-stroke patients. This type of sensory dysfunction may further be associated with poor functional outcome in patients' post-stroke. METHODS: In phase 1, a cross-sectional survey (n = 100) will be conducted to determine the prevalence of eye movement disorders and vestibular dysfunction in patients who sustained a stroke...
2016: Frontiers in Neurology
Steven C Dakin, Philip R K Turnbull
Although the contrast sensitivity function (CSF) is a particularly useful way of characterising functional vision, its measurement relies on observers making reliable perceptual reports. Such procedures can be challenging when testing children. Here we describe a system for measuring the CSF using an automated analysis of optokinetic nystagmus (OKN); an involuntary oscillatory eye movement made in response to drifting stimuli, here spatial-frequency (SF) band-pass noise. Quantifying the strength of OKN in the stimulus direction allows us to estimate contrast sensitivity across a range of SFs...
October 4, 2016: Scientific Reports
José L Sánchez-Vicente, Miguel Contreras-Díaz, Laura Llerena-Manzorro, Trinidad Rueda, Fernando López-Herrero, Fredy E Molina-Socola, Ana Muñoz-Morales, Enrique Rodríguez de la Rúa-Franch
PURPOSE: To describe the clinical presentations and image findings of a patient with foveal hypoplasia. METHODS: Observational case report of one patient. The clinical presentation of the patient was documented by fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography. RESULTS: A 55-year-old healthy white female with bilateral foveal hypoplasia without nystagmus was presented. There were no signs suggestive of ocular albinism...
September 28, 2016: Retinal Cases & Brief Reports
Thomas Welton, Sarim Ather, Frank A Proudlock, Irene Gottlob, Robert A Dineen
Albinism is a group of congenital disorders of the melanin synthesis pathway. Multiple ocular, white matter and cortical abnormalities occur in albinism, including a greater decussation of nerve fibres at the optic chiasm, foveal hypoplasia and nystagmus. Despite this, visual perception is largely preserved. It was proposed that this may be attributable to reorganisation among cerebral networks, including an increased interhemispheric connectivity of the primary visual areas. A graph-theoretic model was applied to explore brain connectivity networks derived from resting-state functional and diffusion-tensor magnetic resonance imaging data in 23 people with albinism and 20 controls...
September 29, 2016: Human Brain Mapping
Ungsoo Samuel Kim, Eunhae Cho, Hyon J Kim
No abstract text is available yet for this article.
2016: International Journal of Ophthalmology
Keiko Shimojima, Koichi Maruyama, Masahiro Kikuchi, Ayako Imai, Ken Inoue, Toshiyuki Yamamoto
Allan-Herndon-Dudley syndrome (AHDS) is an X-linked disorder caused by impaired thyroid hormone transporter. Patients with AHDS usually exhibit severe motor developmental delay, delayed myelination of the brain white matter, and elevated T3 levels in thyroid tests. Neurological examination of two patients with neurodevelopmental delay revealed generalized hypotonia, and not paresis, as the main neurological finding. Nystagmus and dyskinesia were not observed. Brain magnetic resonance imaging demonstrated delayed myelination in early childhood in both patients...
August 2016: Intractable & Rare Diseases Research
Hyun Ji Kim, Dae-Young Kim, Jun Ha Hwang, Kyu-Sung Kim
Objectives: To analyze the clinical characteristics of vestibular neuritis patients with minimal canal paresis (canal paresis <25%). Methods: Patients clinically diagnosed with vestibular neuritis and treated at our institute (n=201) underwent otoneurological examination and vestibular function tests. Patients were categorized in terms of the. Results.: of caloric testing (canal paresis<25%, n=58; canal paresis≥25%, n=143). Clinical characteristics and laboratory outcomes were compared between two groups...
September 27, 2016: Clinical and Experimental Otorhinolaryngology
Maria Vittoria Cicinelli, Maria Pia Manitto, Maurizio Battaglia Parodi, Francesco Bandello
PURPOSE: To describe a peculiar flecked-retina phenotype in a young female affected by early-onset retinal dystrophy due to a heterozygous mutation in the cone-rod transcription factor CRX gene. CASE REPORT: A 5-year-old girl presented with poor vision and nystagmus from the first month after birth. Opththalmologic examination at baseline revealed an altered foveal reflex, epiretinal membrane, and yellow fleck-like retinal deposits in the mid- and extreme periphery bilaterally that disappeared after 3 years of follow-up...
October 2016: Optometry and Vision Science: Official Publication of the American Academy of Optometry
Akihide Ichimura, Koji Otsuka
A 16-year-old boy with rotatory positional vertigo and nausea, particularly when lying down, visited our clinic. Initially, we observed vertical/torsional (downward/leftward) nystagmus in the supine position, and it did not diminish. In the sitting position, nystagmus was not provoked. Neurological examinations were normal. We speculated that persistent torsional down-beating nystagmus was caused by the light cupula of the posterior semicircular canal. This case provides novel insights into the light cupula pathophysiology...
2016: Case Reports in Otolaryngology
Jeffrey D Sharon, John P Carey, Michael C Schubert
Vertical nystagmus without a torsional component is generally considered a finding indicative of central nervous system pathology. We report two cases of purely upbeat nystagmus elicited with mastoid vibration after bilateral superior canal plugging, to highlight the vestibular pathophysiology involved in this unusual peripheral cause for upbeat nystagmus. Laryngoscope, 2016.
September 26, 2016: Laryngoscope
Kavin Vanikieti, Piyaphon Cheecharoen, Panitha Jindahra, Sirin Lueangaram, Tanyatuth Padungkiatsagul
Vertebrobasilar dolichoectasia (VBD) is a rare dilative arteriopathy defined as elongation or widening of the intracranial vertebral and/or basilar arteries. The prevalence ranges from 0.06% to 5.8%. The majority of VBDs are asymptomatic. Downbeat nystagmus has been reported as a component of an infrequent ocular movement disorder in VBD. Nevertheless, oculopalatal tremor (OPT), delayed sequelae of a brainstem lesion, has never been demonstrated in VBD cases. Synchronized rhythmic involuntary contractions of the soft palate with an ocular pendular nystagmus, predominantly vertical pendular nystagmus, are the hallmark presentation...
2016: International Medical Case Reports Journal
Yuri Nakamura, Kei-Ichiro Takase, Takuya Matsushita, Satoshi Yoshimura, Ryo Yamasaki, Hiroyuki Murai, Kazufumi Kikuchi, Jun-Ichi Kira
A 34-year-old man presented with an acute onset of upbeat nystagmus, slurred speech, and limb and truncal ataxias. The patient had a history of limb ataxia and gait disturbance previously treated as brainstem encephalitis with corticosteroids 3 years previously. Brain magnetic resonance imaging showed pontine developmental venous anomaly (DVA) and hemorrhagic infarction within the drainage territory of the DVA. Three months later, the patient exhibited recurrent limb ataxia, double vision, and numbness of the left side of the body...
September 19, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Yasumitsu Takimoto, Takao Imai, Makoto Kondo, Yukiko Hanada, Atsuhiko Uno, Yusuke Ishida, Takefumi Kamakura, Tadashi Kitahara, Hidenori Inohara, Shoichi Shimada
Cisplatin is a chemotherapeutic agent commonly used for the treatment of solid tumors, and its side-effects include vestibulotoxicity. Previous studies have reported cisplatin-induced vestibulotoxicity in various animal models, but no study has investigated in vivo mouse vestibular dysfunction after cisplatin. The aim of this study was to investigate cisplatin-induced vestibulotoxicity in C57BL/6J mice. Vestibular function was assessed by recording the vestibulo-ocular reflex (VOR). This was done during sinusoidal rotations in the horizontal plane at three frequencies (0...
September 19, 2016: Toxicology Letters
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