The Light Cupula: An Emerging New Concept for Positional Vertigo

Benign paroxysmal positional vertigo (BPPV) is the most common type of positional vertigo. A canalolithiasis-type of BPPV involving the lateral semicircular canal (LSCC) shows a characteristic direction-changing positional nystagmus (DCPN) which beats towards the lower ear (geotropic) on turning the head to either side in a supine position. Because geotropic DCPN in LSCC canalolithiasis is transient with a latency of a few seconds, the diagnosis can be challenging if geotropic DCPN is persistent without latency. The concept of “light cupula” has been introduced to explain persistent geotropic DCPN, although the mechanism behind it requires further elucidation. In this review, we describe the characteristics of the nystagmic pattern in light cupula and discuss the current evidence for possible mechanisms explaining the phenomenon.

Rotatory Vertebral Artery Syndrome in Foramen Magnum Stenosis / 대한평형의학회지

Rotatory vertebral artery syndrome (RVAS) is characterized by recurrent attacks of vertigo, nystagmus, and syncope induced by compression of the vertebral artery during head rotation. A 60-year-old man with atlas vertebrae fracture presented recurrent attacks of positional vertigo. Left-beat, upbeat and count clock-wise torsional nystagmus occurred after lying down and bilateral head roll (HR) showing no latency or fatigue. Magnetic resonance imaging revealed foramen magnum stenosis (FMS) and dominancy of right vertebral artery (VA). The flow of the right VA on transcranial Doppler decreased significantly during left HR. The slower the velocity was, the more the nystagmus was aggravated. RVAS can be evoked by FMS causing compression of the VA. And the nystagmus might be aggravated according to the blood flow insufficiency.

Abnormal Oculomotor Functions in Amyotrophic Lateral Sclerosis

BACKGROUND AND PURPOSE: Although traditionally regarded as spared, a range of oculomotor dysfunction has been recognized in amyotrophic lateral sclerosis (ALS) patients. ALS is nowadays considered as a neurodegenerative disorder of a third compartment comprising widespread areas of extra-motor brain including cerebellum. Our objective was to perform an observational study to examine for ocular motor dysfunction in patients with ALS and for any differences between bulbar-onset and spinal-onset patients. METHODS: Thirty two ALS patients (bulbar onset: 10, spinal onset: 22) underwent the standardized systemic evaluations using video-oculography. RESULTS: Oculomotor dysfunctions such as square wave jerks, saccadic dysmetria, abnormal cogwheeling smooth pursuits and head shaking and positional nystagmus of central origin have been observed in the ALS patients at a relatively early stage. Abnormal smooth pursuits and saccadic dysmetria were increased in the bulbar-onset compared to the spinal-onset (p < 0.05). CONCLUSIONS: These oculomotor abnormalities may be a marker of neuro-degeneration beyond motor neurons in ALS, especially in bulbar-onset disease. Future longitudinal studies of eye movement abnormalities have provided insights into the distribution and nature of the disease process.

Characteristic analysis of positional nystagmus in patients with vestibular migraine / 第二军医大学学报

Objective To analyze the characteristics of positional nystagmus in patients with vestibular migraine (VM) and to discuss its possible mechanism. Methods We retrospectively analyzed the types, intensities, and time of positional nystagmus recorded by video nystagmography during Dix-Hallpike and Roll-test in 14 patients with acute episode of VM and 16 patients with benign paroxysmal positional vertigo (HSC-BPPV). Results A mixture of geotropic, apogeotropic and downbeat components with a variable combination of each component and various types of positional nystagmus were identified in VM. There was no significant difference in the latencies or the duration of positional nystagmus between VM group and HSC-BPPV group (P>0. 05). The peak slow-phase velocities (SPVmax) in horizontal component of positional nystagmus was (11.4 ± 2.3) °/s in VM group, which was significantly lower than that in the HSC-BPPV group ([56.8±9.4] °/s, P = 0.001). Compared with HSC-BPPV group, the time needed for reaching the SPVmax (tm) was significantly longer in VM group ([18.1±2.0] vs [5.9±1.1] s, P<0. 001), and the rate of velocity change was significantly slower in VM group ([0.54 ± 0.14] vs [7. 21 ±1. 79] °/s2, P<0.001). The asymmetry ratio of the stronger to softer SPVmax in HSC-BPPV group was significantly higher than that in the VM group (P = 0. 02). Conclusion Abnormal central integration mechanism in VM may lead to discordant transmission of semicircular canal rotation information, resulting in mixed nystagmus components and flat nystagmus that appeared at multiple positional maneuver.

Isolated Cerebellar Nodular Infarction with Apogeotropic Central Paroxysmal Positional Nystagmus

No abstract available.

Persistent Positional Vertigo in a Patient with Sudden Sensorineural Hearing Loss: A Case Report

Because inner ear organs are interconnected through the endolymph and surrounding endolymphatic membrane, the patients with sudden sensorineural hearing loss (SSNHL) often complain of vertigo. In this study, we report a patient with SSNHL accompanied by persistent positional vertigo, and serial findings of head-roll tests are described. At acute stage, head-roll test showed persistent geotropic direction-changing positional nystagmus (DCPN), which led to a diagnosis of SSNHL and ipsilateral light cupula. Although vertigo symptom gradually improved, positional vertigo lasted for more than 3 weeks. At this chronic stage, persistent apogeotropic DCPN was observed in a head roll test, which led to a diagnosis of the heavy cupula. Although the mechanism for the conversion of nystagmus direction from geotropic to apogeotropic persistent DCPN is unclear, the change of specific gravity of the endolymph might be one of the plausible hypothetical explanations.

Clinical Characteristics of Horizontal Canal Benign Paroxysmal Positional Vertigo with Persistent Geotropic Direction Changing Positional Nystagmus / 대한평형의학회지

OBJECTIVE: The purpose of this study was to identify the clinical characteristics of horizontal canal benign paroxysmal positional vertigo (h-BPPV) with persistent geotropic direction changing positional nystagmus (DCPN). METHODS: One hundred thirty two patients diagnosed as the geotropic subtype of h-BPPV were analyzed retrospectively. Patients were classified into two groups: persistent h-BPPV (ph-BPPV) group which means h-BPPV showing persistent (>1 minute) geotropic DCPN and short duration h-BPPV (sh-BPPV) group that means h-BPPV with short duration (< or =1 minute) geotropic DCPN. We compared the clinical characteristics and treatment outcomes between the two groups. RESULTS: The study included 34 patients with ph-BPPV and 98 patients with sh-BPPV. There were no differences between the two groups in age, distribution of sex and the affected side. The ph-BPPV group had higher secondary BPPV preponderance and dizziness handicap index (DHI) score compared to the sh-BPPV group. The ph-BPPV group required higher number of canalith repositioning procedures (CRPs) until resolution and higher multiple/single CRP ratio than the sh-BPPV group. In addition, the ph-BPPV group showed longer duration until the remission of subjective symptoms (vertigo, dizziness) compared to the sh-BPPV group. CONCLUSION: ph-BPPV was more frequently associated with secondary causes of BPPV and demonstrated higher DHI score, total number of CRP, and longer remission duration of subjective symptoms compared to sh-BPPV. This information may be helpful for clinicians in counseling and managing the patients with persistent geotropic DCPN h-BPPV.

A Case of Cerebellar Hemangioblastoma Mimicking Benign Paroxysmal Positional Vertigo / 대한이비인후과학회지

The most common cause of positional vertigo is benign paroxysmal positional vertigo (BPPV). BPPV is characterized by brief recurrent spells of vertigo often brought about by certain head position changes that occur when looking up, turning over in bed, or straightening up after bending over. Its diagnosis relies on a thorough history and physical examination, including Dix-Hallpike maneuver. BPPV usually resolves spontaneously, but treatment of persistent cases with canalolith repositioning maneuvers and exercise therapy has been relatively successful. Those patients with atypical findings or whose symptoms fail to respond to medical therapy and otolith repositioning maneuver, however, require further evaluation to rule out intracranial pathologic conditions. We report here on an unusual case of cerebellar hemangioblatoma showing paroxysmal positional vertigo with a review of the related literature.

A Case of Linear Scleroderma Involving Cerebellum with Vertigo

Linear scleroderma is a kind of disease that can cause complication of the central nervous system. Sometimes, ipsilateral intracerebral or white matter lesions in the brain magnetic resonance imaging are noted. Nystagmus is important for the differential diagnosis of dizziness. Positional nystagmus was classified into regular direction nystagmus, direction changing positional nystagmus (DCPN) and irregular nystagmus by their character. DCPN is defined as a nystagmus that changes its direction with different head and body positions, and it can be the sign of lesion in the central vestibular system. Recently, we experienced a 17-year-old woman who had a scalp linear scleroderma, treated for about 10 years and showed DCPN induced by positional nystagmus test. We report a case of linear scleroderma accompanied by cerebellar lesion with a brief review of literature.

Apogeotropic Positional Nystagmus in Pontine Infarction / 대한평형의학회지

It is thought that horizontal canal benign paroxysmal positional vertigo (BPPV) is the most common cause of apogeotropic direction-changing positional nystagmus (DCPN). But there are many reports about cerebellar or brainstem lesions as the cause of apogeotropic DCPN. We also report a 72-year-old male patient who showed apogeotropic DCPN, but was proven to have a pontine infarction. The patients complained of disequilibrium which has lasted for 3-4 years and aggravated recently. The symptom was present only when he stood up, and was absent as soon as he sat down. He was not able to successfully perform the Romberg test and tandem gait on physical examination. Vestibular function test revealed apogeotropic DCPN without spontaneous nystagmus. Rotation chair test and caloric test results were all within normal limit. On the brain magnetic resonance imaging, newly detected infarction in the left basal ganglia, pons and right parietal lobe was found. Although horizontal canal BPPV is the most common cause of apogeotropic DCPN, we should be aware that there can be patients with central origin DCPN. In this report, we present the detailed history of this patient and tried to point out the clues to suspect central lesion in patients with apogeotropic DCPN.

Active head rotation in benign positional paroxysmal vertigo / Da rotação cefálica ativa na vertigem posicional paroxística benigna

Benign Positional Paroxysmal Vertigo (BPPV) is one of the most common vestibular diseases and the active head rotation test one of the most modern methods of vestibular function assessment. AIM: this study aims to verify if the active head rotation test may reveal signs of horizontal and/or vertical vestibulo-ocular reflex dysfunction in vertigo patients suspected for BPPV. STUDY DESIGN: retrospective series study. MATERIALS AND METHOD: Neurotological evaluation including computerized electronystagmography and active head rotation on the horizontal and vertical axes were conducted in 100 patients suspected for BPPV patients. Results: Isolated or associated abnormalities of the horizontal and/or vertical vestibulo-ocular reflex gain, phase and symmetry were indicative of vestibular involvement and found in 77.0 percent of the BPPV patients. CONCLUSION: the active head rotation test revealed horizontal and/or vertical vestibulo-ocular reflex dysfunctions in a relevant number of BPPV patients.

A vertigem posicional paroxística benigna (VPPB) corresponde a uma das vestibulopatias mais comuns e a rotação cefálica ativa um dos métodos mais modernos de avaliação da função vestibular. OBJETIVO: O objetivo desta pesquisa foi verificar se a prova de rotação cefálica ativa pode revelar sinais de disfunção do reflexo vestíbulo-ocular horizontal e/ou vertical em pacientes vertiginosos com hipótese diagnóstica de VPPB. DESENHO DO ESTUDO: Estudo de série retrospectivo. MATERIAL E MÉTODO: Uma avaliação otoneurológica incluindo a eletronistagmografia computadorizada e a prova de rotação cefálica ativa, no plano horizontal e vertical foi conduzida em 100 pacientes com hipótese diagnóstica de VPPB. Resultados: Alterações isoladas ou associadas de ganho, fase e assimetria do reflexo vestíbulo-ocular horizontal e/ou vertical, foram os achados indicativos de comprometimento vestibular em 77,0 por cento dos casos de VPPB. CONCLUSÃO: A prova de rotação cefálica ativa permitiu evidenciar distúrbios do reflexo vestíbulo-ocular horizontal e/ou vertical em relevante proporção dos pacientes com VPPB.

Bilateral Benign Paroxysmal Positional Vertigo of Horizontal Canal and Posterior Canal / 대한이비인후과학회지

Bowing nystagmus, lying down nystagmus, null pointand comparing the slow phase velocity during right and left head roll test may be used to distinguish the side of lesion in lateral canal benign paroxysmal positional vertigo (BPPV). Nonetheless, it is sometimes difficult to distinguish the side of lesion. In particular, when multiple canal BPPV such as lateral and posterior canal BPPV is suspected, the problemis even more complicated. From this reason, usually the side of lesion is first identified for the posterior canal, and the lateral canal BPPV is presumed to be present on the identical side. But is this approachalways correct and justifiable? As there are reports on bilateral posterior canal BPPV and bilateral lateral canal BPPV, there should also be bilateral posterior and lateral canal BPPV cases. We report two cases of bilateral posterior and lateralcanal BPPV, and discuss the grounds for diagnosing these cases as bilateral. The first case is a mixed left posterior canalolithiasis plus right lateral canalolithiasis and the second case is a mixed right posterior canalolithiasis plus left lateral cupulolitiasis. In such cases, mixed nystagmus can make it difficult to directly compare the slow phase velocity during the right and left head roll test. New methods are necessary to distinguish the side of the lesion for the lateral canal. We introduce the concept of AHC (attenuated horizontal component) which seems to be important in deciding the side of lesion in multiple canal BPPV. We also introduce head center nystagmus (HCN) to aid the decision on the side of lesion.

A Case of Vestibular Schwannoma Presenting Vertigo Mimicking Benign ParoxysmalPositional Vertigo / 대한이비인후과학회지

Vestibular symptoms of vestibular schwannoma (VS) can be diverse, though positional vertigo is relatively unusual in VS. We experienced a case with VS presenting positional vertigo mimicking BPPV. A failure of repeated particle repositioning maneuver and observation of atypical nystagmus during follow-up prompted authors to examine the brain through magnetic resonance imaging (MRI). Brain MRI revealed 3.5 mm sized right intracanalicular VS. Diagnostic test showed grossly normal except abolished vestibular evoked myogenic potential response of right side. The patient had been followed-up over 3 years with tests repeated annually. In conclusion, VS may present positional vertigo with positional nystagmus. Ephaptic transmission which may be initiated and changed by head position or partial loss of vestibular tone from inferior vestibular nerve can be suggested as possible mechanisms. Radiologic evaluation should be considered for those patients who showed intractable positional vertigo or atypical positional nystagmus.

Manobra de Epley na vertigem posicional paroxística benigna associada à doença de Ménière / Epleys maneuver in benign paroxysmal positional vertigo associated with Menieres disease

Os efeitos da manobra de Epley na vertigem posicional paroxística benigna (VPPB) associada à doença de Ménière são controvertidos. OBJETIVO: Avaliar a vertigem e o nistagmo de posicionamento após uma ou mais manobras de Epley na vertigem posicional paroxística benigna (VPPB) associada à doença de Ménière e na recorrência da VPPB. MATERISL E MÉTODO: Estudo retrospectivo de 62 pacientes com VPPB associada à doença de Ménière submetidos à manobra de Epley e acompanhados durante 12 meses após a extinção do nistagmo de posicionamento. RESULTADOS: Para abolir o nistagmo de posicionamento, foi necessária uma manobra de Epley em 80,7 por cento dos pacientes, duas em 16,1 por cento e três em 3,2 por cento. A vertigem foi eliminada em 71,0 por cento dos pacientes, melhorou em 27,4 por cento e permaneceu inalterada em 1,6 por cento. Quatro semanas após a extinção do nistagmo de posicionamento, todos os pacientes ficaram assintomáticos. Recorrência da VPPB foi observada em 19,4 por cento dos casos, com eliminação da vertigem e nistagmo de posicionamento à manobra específica para o canal afetado. CONCLUSÕES: Na VPPB associada à doença de Ménière, vertigem e nistagmo de posicionamento foram eliminados com uma, duas ou três manobras de Epley. A recorrência da VPPB foi resolvida com uma manobra para o canal envolvido.

The effects of Epleys maneuver in benign paroxysmal positional vertigo (BPPV) associated with Menières disease are controversial. AIMS: To evaluate the progression of positional vertigo and nystagmus after one or more of Epleys maneuvers in BPPV associated with Menières disease, and the recurrence of BPPV. METHOD: a retrospective study of 62 patients with BPPV associated with MenièreÆs disease, that underwent Epleys maneuver, and that were monitored during 12 months after elimination of positional nystagmus. RESULTS: One Epleys maneuver was required to eliminate positional nystagmus in 80.7 percent of the patients, two in 16.1 percent, and three in 3.2 percent; after elimination of nystagmus, positional vertigo was suppressed in 71.0 percent of the patients, improved in 27.4 percent and remained unaltered in 1.6 percent. Four weeks after elimination of positional nystagmus, all patients were asymptomatic. Recurrence of BPPV was seen in 19.4 percent of the cases, with elimination of the positional vertigo and nystagmus by means of the specific maneuver for the involved canal. CONCLUSION: In BPPV associated with Menières disease, vertigo and positioning nystagmus were eliminated with one, two or three Epley maneuvers. BPPV recurrence was resolved by using a specific maneuver for the affected canal.

Clinical Characteristics of Benign Paroxysmal Positional Vertigo in Korea: A Multicenter Study

Benign paroxysmal positional vertigo (BPPV) is characterized by episodic vertigo and nystagmus provoked by head motions. To study the characteristics of BPPV in a large group of patients in Korea, we retrospectively analyzed clinical features of 1,692 patients (women: 1,146, 67.7%; men: 54.6, 32.3%; mean age: 54.8+/-14.0 yr), who had been diagnosed as BPPV by trained neuro-otologists Dizziness Clinics. The diagnosis of BPPV was based on typical nystagmus elicited by positioning maneuvers. Posterior semicircular canal was involved in 60.9% of the patients, horizontal canal in 31.9%, anterior canal in 2.2%, and mixed canals in 5.0%. The horizontal canal type of BPPV (HC-BPPV) comprised 49.5% of geotropic and 50.5% of apogeotropic types. We could observe significant negative correlation between the proportion of HC-BPPV of each clinic and the mean time interval between the symptom onset and the first visit to the clinics (r=-0.841, p<0.05). Most patients were successfully treated with canalith repositioning maneuvers (86.9%). The high incidence of HC-BPPV in this study may be explained by relatively shorter time interval between the symptom onset and visit to the Dizziness Clinics in Korea, compared with previous studies in other countries.

Traumatic Perilymphatic Fistula Presenting with Direction-Changing Positional Nystagmus

Diagnosis of perilymphatic fistula (PLF) is considered in the patient presenting hearing loss associated with ataxia after penetrating injury of the tympanic membrane. PLF accompanies mixed type hearing loss and paralytic nystagmus. If audiovestibular symptoms and signs are not definite for those patients, in whom PLF is highly suspicious, they can be induced by affected ear down position. The direction of nystagmus induced by position change was reported either toward or away from the affected ear. But the direction changing nature has not been noted in the previous literature. We report on a case of traumatic PLF presented with direction changing positional nystagmus and discuss the possible mechanism involved in this case

A Case of Atypical Benign Paroxismal Positional Vertigo

Paroxysmal positional nystagmus is a common finding in patients with vertigo and can occur in typical and atypical forms. Atypical forms of paroxismal positional nystagmus are thought to represent conditions which are in fact not "benign". This patient was diagnosed as right posterior semicircular canal BPPV at first. After modified Epley maneuver, the type of nystagmus was changed to atypical forms. After left cupulolith reposition maneuver (CRmM), the nystagmus and dizziness were disappeared finally.

Central Positional Nystagmus from Focal Brain Lesion

BACKGROUND AND OBJECTIVES : Central positional nystagmus is induced by positional changes from brainstem or cerebellar lesions. Differentiation central from peripheral positional nystagmus is important in clinical practice. To delineate characteristics of the positional nystagmus observed in central lesions, we analyzed the parameters of positional nystagmus from focal brain lesions. MATERIALS AND METHODS : Ten patients with central positional nystagmus were recruited from the dizziness clinic of Seoul National University Bundang Hospital. All the patients had focal brainstem or cerebellar lesions documented by magnetic resonance imaging. The nystagmus was observed with and without fixation by using Frenzel glasses or Video Goggles. The nystagmus was videotaped or recorded with video-oculography. Provoking positional maneuvers, direction, latency, duration, phase reversal, and fatigue phenomenon of the nystagmus were analyzed. RESULTS : Of the 10 patients, seven had infarctions in the lateral medulla or inferior cerebellum while two experienced cerebellar hemorrhage and remaining one showed a compression of the ventrolateral medulla by cavernous malformation of the vertebral artery. The directions of the positional nystagmus were variable depending on the lesions and provoking maneuvers. Most patients exhibited direction-changing nystagmus without latency, direction-reversal and fatigue phenomenon. However, some of the patients also showed patterns of nystagmus characteristic of peripheral positional nystagmus. In two of the four patients with infarction in the territory of medial posterior inferior cerebellar artery, the positional nystagmus was the only abnormal findings. CONCLUSIONS : Central positional nystagmus may share many characteristics with peripheral type of positional nystagmus. In individual cases, the patterns of nystagmus should be interpreted with caution in differentiating central from peripheral positional nystagmus. Considering the isolated positional nystagmus in some patients with caudal cerebellar lesions, systematic positional maneuvers should be applied to all the patients with vertigo

Mal de Debarquement Syndrome with Apogeotropic Positional Nystagmus

Mal de debarquement syndrome refers to the prolonged sensation of rocking and swaying that is experienced on the return to land following a sea voyage or an air travel. We report a man who developed mal de debarquement syndrome after a 4-day-boat-trip. Apogeotropic nystagmus during head turning in supine position was the only finding. Mal de debarquement syndrome should be considered in the differential diagnosis of direction-changing positional nystagmus, especially when patients do not complain of vertigo with nystagmus.

A Case of Congenital Vestibular Anomaly with Direction Changing Positional Nystagmus / 대한이비인후과학회지

Direction changing positional nystagmus (DCPN) is defined as a nystagmus that changes its direction with different head and body positions. In the past, it was usually thought that DCPN was the sign of central vestibular system lesion. But recently, there have been some reports that DCPN definitely does not localize the site of lesion in the central vestibular pathway, and that it more often indicates a peripheral vestibular site. However, congenital vestibular dys- or hypoplasia was not reported as a cause of DCPN. Recently, we experienced a 17-year-old patient who had a vestibular dys- or hypoplasia and showed a transient geotrophic DCPN with a normal cochlea. We report that congenital vestibular dys- or hypoplasia can be one of the causes of DCPN and present its possible mechanism.