Arnold Chiari malformation is a disease which is characterized by herniation of a portion of the cerebellum through the foramen magnum. Symptoms vary depending on the extent of the affected area, including posterior neck pain, upper limb pain, paralysis, paresthesia, weakness, dizziness, and ataxia. Among the patients presenting with dizziness, nystagmus is frequently observed, which is primarily characterized by down-beating nystagmus. We experienced a 42- years-old female patient presented with vertigo and gait disturbance, who were diagnosed with type 1 Arnold-Chiari malformation and treated by surgical decompression.
Seesaw nystagmus (SSN) is characterized by conjugate torsional nystagmus with opposite vertical components in the two eyes. The waveform may be pendular or jerk (hemi-seesaw nystagmus, HSSN), in which the slow phase corresponds to one half-cycle and the quick phase to the other. Pendular SSN and HSSN have distinct clinical presentations and underlying causes. The pathophysiology of pendular SSN may be instability of visuovestibular interactions, while the underlying mechanism for HSSN may be related to the ocular tilt reaction or an imbalance in vestibular pathways. We report a patient with HSSN due to unilateral mesodiencephalic infarction that becomes apparent during visual fixation only.
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Midbrain lesion-induced disconjugate gaze: a unifying circuit mechanism of ocular alignment? Maximilian U. Friedrich, Laurin Schappe, Sashank Prasad, Helen Friedrich, Michael D. Fox, Andreas Zwergal, David S. Zee, Klaus Faßbender, Klaus-Ulrich Dillmann Journal of Neurology.2024;[Epub] CrossRef
Ocular flutter is a rare, horizontal eye movement disorder characterized by intermittent bursts of conjugate horizontal saccades without intersaccadic intervals. It can occur in various clinical conditions such as metabolic dysfunction, infection and paraneoplastic syndrome. Herein, a 50-year-old male showed ocular flutter in parainfectious meningoencephalitis and immunoglobulin therapy led to an improvement of symptoms. This case can improve the understanding of the pathological mechanisms of ocular flutter.
Sarcoidosis is a rare, multisystem granulomatous disease. Neurological complications occur in about 5% of patients and vestibulocochlear involvement is even rarer. A 27-year-old woman presented with acute spontaneous vertigo for 5 days. She was diagnosed with pulmonary sarcoidosis 4 months ago, but specific treatments have not yet started. She had preceding otologic symptoms including bilateral tinnitus and ear fullness in the right for 3 months without hearing loss. Initial bedside examinations revealed spontaneous right-beating nystagmus and abnormal catch-up saccades in the left during head impulse tests (HIT). After 2 weeks, video-oculography documented the direction of spontaneous nystagmus was changed into left-beating. Caloric test showed canal paresis in the left, and video HIT showed subtle covert saccades. After starting oral prednisolone, her symptoms improved rapidly. In our case, acute vestibular syndrome and otologic symptoms might be associated with sarcoidosis when considering clinical course and treatment response. Sarcoidosis may be considered as a cause in cases with audiovestibulopathy of unknown etiology.
The upward deviation could be explained by loss of inhibitory inputs from the cerebellum onto the brainstem anterior semicircular canal projections for upward vestibulo-ocular reflex, which would lead to an upward bias in static eye position. Therefore, upward gaze deviation has been reported in comatose patients after resuscitation and diffuse cerebrocerebellar damage sparing the brainstem. Herein, we report a patient with ingestion of glufosinate ammonium presented with cerebellar ataxia and ocular motor findings suggestive of cerebellum involvement such as upward gaze tendency, spontaneous downbeat, gaze-evoked nystagmus, perverted head impulse test, and impaired smooth pursuit.
Objectives This study was performed to assess the efficacy and feasibility of mobile application-based Frenzel tests in dizziness clinics.
Methods We performed an investigator-initiated, blinded-outcome assessor, parallel, randomized controlled crossover trial at Chungnam National University Hospital between August 2019 and October 2019. Certified medical staffs were randomly assigned to the intervention group (i.e., a mobile application-based Frenzel glass system, n=15) or the observation group (i.e., a conventional desktop-based Frenzel glass system, n=15); the groups applied the respective systems for the preparation of eye movement recording and switched systems. The primary outcome was the elapsed time in seconds it took the participants to prepare the system for eye recording simulation. The secondary outcomes were perceived stress and satisfaction scores after completion of the operation, as measured by a questionnaire using 10-point Likert scales.
Results The mean time of machine preparation for eye recording simulation was reduced by 50% in the mobile application group compared to the desktop group in both study periods (38.0±7.1 sec vs. 76.0±8.7 sec). We detected no carryover effect. Participants also reported lower stress while using application than while using the desktop system (2.3±1.3 vs. 4.6±2.4; p<0.001). The application obtained a mean overall satisfaction score of 9.2 out of 10.
Conclusions The implementation of an eye movement recording application in a dizziness examination was well adopted by users and decreased the time and stress related to machine operation.
Relapsing polychondritis is a rare multisystemic autoimmune disorder of unknown etiology and characterized by recurrent episodes of inflammation affecting the cartilaginous tissues. Otologic manifestation such as auricular chondritis is one of the most frequent presenting symptoms in relapsing polychondritis, and inner ear symptoms, such as hearing loss, tinnitus, and vertigo, may develop in 7% to 42% of the patients. In this study, we present a 42-year-old male patient with relapsing polychondritis, who experienced two separate episodes of acute vestibular syndrome at the interval of 6 years. At the first vertigo attack, the patient showed left-beating spontaneous nystagmus with sudden hearing loss on the right side, and a bithermal caloric test revealed canal paresis on the right side. At the second vertigo attack, he showed right-beating spontaneous nystagmus, and a bithermal caloric test, compared to that during the first vertigo attack, revealed additional decrease in caloric response on the left side.
Relapsing polychondritis is a rare multisystemic autoimmune disorder of unknown etiology and characterized by recurrent episodes of inflammation affecting the cartilaginous tissues. Otologic manifestation such as auricular chondritis is one of the most frequent presenting symptoms in relapsing polychondritis, and inner ear symptoms, such as hearing loss, tinnitus, and vertigo, may develop in 7% to 42% of the patients. In this study, we present a 42-year-old male patient with relapsing polychondritis, who experienced two separate episodes of acute vestibular syndrome at the interval of 6 years. At the first vertigo attack, the patient showed left-beating spontaneous nystagmus with sudden hearing loss on the right side, and a bithermal caloric test revealed canal paresis on the right side. At the second vertigo attack, he showed right-beating spontaneous nystagmus, and a bithermal caloric test, compared to that during the first vertigo attack, revealed additional decrease in caloric response on the left side.
Ataxia with ocular motor apraxia type 2 (AOA2) is an autosomal recessive disorder that is characterized by adolescent-onset gait ataxia, peripheral neuropathy, ocular apraxia, and cerebellar atrophy. A 19-year-old male with AOA2 from a novel SETX mutation showed distinct oculomotor abnormalities that included spontaneous and gaze-induced downbeat nystagmus, impaired smooth pursuit, and reversed catch-up saccades during horizontal head impulse tests, as well as peripheral neuropathy involving the lower extremities and mild slowing of frontal processing. He also showed positional hemiseesaw nystagmus in the supine and straight head-hanging positions. Positional hemiseesaw nystagmus is a new manifestation of hereditary cerebellar ataxia and may be explained by a gravity-dependent position-induced error in estimating the tilt in the roll plane due to dysfunction of the tilt-estimator circuit.
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A Case of AOA2 With Compound Heterozygous SETX Mutations Hee Jin Chang, Ryul Kim, Minchae Kim, Jangsup Moon, Man Jin Kim, Han-Joon Kim Journal of Movement Disorders.2022; 15(2): 178. CrossRef
Down-beat nystagmus in peripheral vestibulopathy may rarely occur in selective involvement of inferior division. Diagnosis of isolated inferior vestibular neuritis is based on torsional down-beating spontaneous nystagmus, abnormal head-impulse test for the posterior semicircular canal, and abnormal cervical vestibular evoked myogenic potentials (cVEMPs) with normal bithermal caloric tests and ocular vestibular evoked myogenic potentials (oVEMPs). Herein we report a 50-year-old male patient with spontaneous down-beat nystagmus who demonstrated vestibular dysfunction involving only the posterior semicircular canal. The patient showed normal results in the bithermal caloric test, the oVEMPs and cVEMPs test. Video head impulse test showed decreased gain and corrective saccades only in the posterior semicircular canal. This case is significant in showing that peripheral dizziness with spontaneous down-beating nystagmus can occur as a result of an abnormality involving only a single semicircular canal.
This case report describes a patient who developed positional vertigo after surgery for chronic otitis media on the right side. Canal wall up mastoidectomy was performed, and the stapes was moderately mobilized during removal of the inflammatory granulation tissues that were attached to it. Immediately after the surgery, positional vertigo developed. The patient showed weakly left-beating spontaneous nystagmus in a seated position. Examination of positional nystagmus revealed geotropic direction-changing positional nystagmus with a prolonged duration and weak intensity in a supine head-roll test, which may be caused by a change in inner ear fluids due to a disruption of inner ear membrane around the oval window or penetration of toxic materials into the labyrinth during surgery.
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Light cupula phenomenon: a systematic review Nilüfer Bal, Melike Altun, Elif Kuru, Meliha Basoz Behmen, Ozge Gedik Toker The Egyptian Journal of Otolaryngology.2022;[Epub] CrossRef
Direction-Changing Positional Nystagmus in Acute Otitis Media Complicated by Serous Labyrinthitis: New Insights into Positional Nystagmus Jin Woo Choi, Kyujin Han, Hyunjoo Nahm, Jung Eun Shin, Chang-Hee Kim Otology & Neurotology.2019; 40(4): e393. CrossRef
Dialysis disequilibrium syndrome revisited: Feeling “Disequilibrated” due to inner ear dyshomeostasis? Chang-Hee Kim, Jung Eun Shin, Jung Hwan Park Medical Hypotheses.2019; 129: 109262. CrossRef
This case report describes a patient who developed positional vertigo after surgery for chronic otitis media on the right side. Canal wall up mastoidectomy was performed, and the stapes was moderately mobilized during removal of the inflammatory granulation tissues that were attached to it. Immediately after the surgery, positional vertigo developed. The patient showed weakly left-beating spontaneous nystagmus in a seated position. Examination of positional nystagmus revealed geotropic direction-changing positional nystagmus with a prolonged duration and weak intensity in a supine head-roll test, which may be caused by a change in inner ear fluids due to a disruption of inner ear membrane around the oval window or penetration of toxic materials into the labyrinth during surgery.
Citations
Citations to this article as recorded by
Light cupula phenomenon: a systematic review Nilüfer Bal, Melike Altun, Elif Kuru, Meliha Basoz Behmen, Ozge Gedik Toker The Egyptian Journal of Otolaryngology.2022;[Epub] CrossRef
Direction-Changing Positional Nystagmus in Acute Otitis Media Complicated by Serous Labyrinthitis: New Insights into Positional Nystagmus Jin Woo Choi, Kyujin Han, Hyunjoo Nahm, Jung Eun Shin, Chang-Hee Kim Otology & Neurotology.2019; 40(4): e393. CrossRef
Dialysis disequilibrium syndrome revisited: Feeling “Disequilibrated” due to inner ear dyshomeostasis? Chang-Hee Kim, Jung Eun Shin, Jung Hwan Park Medical Hypotheses.2019; 129: 109262. CrossRef