Vestibular Disorders.

BACKGROUND: Recent research findings have improved the understanding of the diagnosis, pathophysiology, genetics, etiology, and treatment of peripheral, central, and functional vestibular vertigo syndromes. METHOD: A literature search, with special attention to the current classification, treatment trials, Cochrane analyses, and other meta-analyses. RESULTS: There are internationally accepted diagnostic criteria for benign positional paroxysmal vertigo, Menière's disease, bilateral vestibulopathy, vestibular paroxysmia, and functional dizziness. Whether an acute vestibular syndrome is central or peripheral can usually be determined rapidly on the basis of the history and the clinical examination. "Cere - bellar vertigo" is a clinically important entity. For bilateral vestibulopathy, balance training is an effective treatment. For Menière's disease, preventive treatment with betahistine (48 mg and 144 mg per day) is not superior to placebo. For vestibular paroxysmia, oxcarbazepine has been shown to be effective. Treatments that are probably effective for functional dizziness include vestibular rehabilitation, cognitive behavioral therapy, and serotonin reuptake inhibitors. CONCLUSION: The diagnostic assessment of vestibular syndromes is much easier for clinicians now that it has been internationally standardized. There is still a lack of randomized, controlled trials on the treatment of, for example, Menière's disease, vestibular migraine, and "cerebellar vertigo."

Classification of vestibular signs and examination techniques: Nystagmus and nystagmus-like movements.

This paper presents a classification and definitions for types of nystagmus and other oscillatory eye movements relevant to evaluation of patients with vestibular and neurological disorders, formulated by the Classification Committee of the Bárány Society, to facilitate identification and communication for research and clinical care. Terminology surrounding the numerous attributes and influencing factors necessary to characterize nystagmus are outlined and defined. The classification first organizes the complex nomenclature of nystagmus around phenomenology, while also considering knowledge of anatomy, pathophysiology, and etiology. Nystagmus is distinguished from various other nystagmus-like movements including saccadic intrusions and oscillations.View accompanying videos at http://www.jvr-web.org/ICVD.html.

Vestibular Migraine: How to Sort it Out and What to Do About it.

BACKGROUND: Vestibular migraine (VM) is the most common neurologic cause of vertigo in adults and results in significant utilization of health care resources, but remains under-recognized and underdiagnosed. EVIDENCE ACQUISITION: Review of literature in PubMed using the following terms: vestibular migraine, migraine-associated vertigo, vertiginous migraine, benign recurrent vertigo, migraine-associated dizziness, migraine, migraine treatment, Meniere disease (MD), vertebrobasilar ischemia (VBI), posterior circulation stroke, benign paroxysmal positional vertigo, and episodic-ataxia Type 2 (EA2). RESULTS: VM can manifest with a variety of vestibular symptoms, including spontaneous vertigo, triggered vertigo, positional vertigo, and head-motion dizziness. Patients may report more than 1 vestibular symptom. Episodes of vertigo are often, but not always, accompanied by headache. Auditory symptoms are frequently associated with VM attacks and may mimic the manifestations of MD. Other migrainous features that accompany VM attacks include photophobia, phonophobia, osmophobia, and visual aura. Interictally, patients may suffer from persistent dizziness or isolated paroxysmal vestibular symptoms. Mood disorders (particularly anxiety) are often found in VM. Abnormal neuro-otologic findings are not uncommon in patients with VM. Differential diagnoses for VM include MD, VBI, EA2, and migraine with brainstem aura. For rescue treatment, triptans, vestibular suppressants, and/or antiemetic agents may be considered. Pharmacologic migraine preventives (antiepileptics, beta-blockers, and antidepressants) are often useful. CONCLUSIONS: The keys to correctly diagnosing VM is identifying a relationship between vestibular symptoms and migrainous features and being aware of the heterogeneity of manifestations of this enigmatic, but treatable, condition. The principles of treatment of VM include rescue therapy, lifestyle modification, nonpharmacologic migraine preventives, pharmacologic migraine prophylaxis, and treatment of comorbidities.

Approach to the Examination and Classification of Nystagmus.

BACKGROUND AND PURPOSE: Physical therapists caring for patients with neurologic or vestibular disorders must routinely examine and characterize nystagmus and other oscillatory eye movements. Often, the diagnosis hinges on proper interpretation of the nystagmus pattern. This requires understanding the terminology surrounding the numerous attributes and influencing factors of nystagmus, a systematic approach to the examination, and a classification structure that guides practitioners to the specific nystagmus type and subsequent evaluation and management. SUMMARY OF KEY POINTS: Nystagmus is an involuntary, rapid, rhythmic, oscillatory eye movement with at least 1 slow phase. Jerk nystagmus has a slow phase and a fast phase. Pendular nystagmus has only slow phases. Nystagmus is distinguished from other types of oscillatory eye movements, such as saccadic intrusions or oscillations. Characterizing nystagmus requires clearly describing its trajectory. This includes choosing a reference frame to describe the axes or planes and direction of eye movements. Several attributes are used to describe nystagmus: binocularity, conjugacy, velocity, waveform, frequency, amplitude, intensity, temporal profile, and age at first appearance. Several factors may influence nystagmus, including gaze position, visual fixation, vergence, and a variety of provocative maneuvers. Classification of nystagmus may be organized by physiologic or pathologic nystagmus versus other nystagmus-like movements. Pathologic nystagmus may be spontaneous, gaze-evoked, or triggered by provocative maneuvers. The combination of attributes allows differentiation between the many peripheral and central forms. RECOMMENDATIONS FOR CLINICAL PRACTICE: Therapists should carefully examine and characterize the trajectory and other attributes and influencing factors of nystagmus to accurately classify it and arrive at the correct diagnosis.

Hemodynamic orthostatic dizziness/vertigo: Diagnostic criteria.

This paper presents the diagnostic criteria for hemodynamic orthostatic dizziness/vertigo to be included in the International Classification of Vestibular Disorders (ICVD). The aim of defining diagnostic criteria of hemodynamic orthostatic dizziness/vertigo is to help clinicians to understand the terminology related to orthostatic dizziness/vertigo and to distinguish orthostatic dizziness/vertigo due to global brain hypoperfusion from that caused by other etiologies. Diagnosis of hemodynamic orthostatic dizziness/vertigo requires: A) five or more episodes of dizziness, unsteadiness or vertigo triggered by arising or present during upright position, which subsides by sitting or lying down; B) orthostatic hypotension, postural tachycardia syndrome or syncope documented on standing or during head-up tilt test; and C) not better accounted for by another disease or disorder. Probable hemodynamic orthostatic dizziness/vertigo is defined as follows: A) five or more episodes of dizziness, unsteadiness or vertigo triggered by arising or present during upright position, which subsides by sitting or lying down; B) at least one of the following accompanying symptoms: generalized weakness/tiredness, difficulty in thinking/concentrating, blurred vision, and tachycardia/palpitations; and C) not better accounted for by another disease or disorder. These diagnostic criteria have been derived by expert consensus from an extensive review of 90 years of research on hemodynamic orthostatic dizziness/vertigo, postural hypotension or tachycardia, and autonomic dizziness. Measurements of orthostatic blood pressure and heart rate are important for the screening and documentation of orthostatic hypotension or postural tachycardia syndrome to establish the diagnosis of hemodynamic orthostatic dizziness/vertigo.

On the classification of hydropic ear disease (Menière's disease).

More than 150 years after its initial description by Prosper Menière, the disease named after him is still at the center of scientific debates. Two recent developments have specifically created a breeding ground for controversy: (1) Since its first description 10 years ago, magnetic resonance imaging diagnosis of endolymphatic hydrops in living patients has seen an increasing and worldwide application. (2) The Bárány Society Classification Committee published diagnostic criteria for Menière's disease in 2015 and proposed a concept of the disease that has elicited widespread criticism. In order to promote the understanding of the underlying controversies and arguments, this article gives an overview of and discusses relevant classification proposals for Menière's disease, including the new classification system of hydropic ear disease.

Benign paroxysmal positional vertigo: Diagnostic criteria Consensus document of the Committee for the Classification of Vestibular Disorders of the Bárány Society. / Vértigo posicional paroxístico benigno: criterios diagnósticos. Documento de consenso del Comité para la Clasificación de los Trastornos Vestibulares de la Bárány Society.

This article presents operational diagnostic criteria for benign paroxysmal positional vertigo (BPPV), formulated by the Committee for Classification of Vestibular Disorders of the Bárány Society. The classification reflects current knowledge of clinical aspects and pathomechanisms of BPPV and includes both established and emerging syndromes of BPPV. It is anticipated that growing understanding of the disease will lead to further development of this classification.

Diagnostic criteria for persistent postural-perceptual dizziness (PPPD): Consensus document of the committee for the Classification of Vestibular Disorders of the Bárány Society.

This paper presents diagnostic criteria for persistent postural-perceptual dizziness (PPPD) to be included in the International Classification of Vestibular Disorders (ICVD). The term PPPD is new, but the disorder is not. Its diagnostic criteria were derived by expert consensus from an exhaustive review of 30 years of research on phobic postural vertigo, space-motion discomfort, visual vertigo, and chronic subjective dizziness. PPPD manifests with one or more symptoms of dizziness, unsteadiness, or non-spinning vertigo that are present on most days for three months or more and are exacerbated by upright posture, active or passive movement, and exposure to moving or complex visual stimuli. PPPD may be precipitated by conditions that disrupt balance or cause vertigo, unsteadiness, or dizziness, including peripheral or central vestibular disorders, other medical illnesses, or psychological distress. PPPD may be present alone or co-exist with other conditions. Possible subtypes await future identification and validation. The pathophysiologic processes underlying PPPD are not fully known. Emerging research suggests that it may arise from functional changes in postural control mechanisms, multi-sensory information processing, or cortical integration of spatial orientation and threat assessment. Thus, PPPD is classified as a chronic functional vestibular disorder. It is not a structural or psychiatric condition.

[Video head impulse test for evaluation of vestibular function in patients with vestibular neuritis and benign paroxysmal positional vertigo].

Objective: To assess the clinical application of video head impulse test (vHIT) for vestibular function in vestibular neuritis (VN) and benign paroxysmal positional vertigo (BPPV) patients. Methods: Thirty-three patients with VN and 43 patients with BPPV were enrolled from Sir Run Run Shaw Hospital and Ningbo Second Hospital from March 15 to September 10, 2015; and 50 healthy controls were also enrolled in the study. vHIT was used to quantitatively test the vestibulo-ocular reflex (VOR) gains of a pair of horizontal semicircular canals. VOR gains two pairs of vertical semicircular canals, and the corresponding asymmetrical value of three VOR gains. The saccades information was also recorded. Results: Compared with the healthy control group and BPPV patients, the affected horizontal and vertical VOR gains were declined and the corresponding asymmetries were increased in VN patients (all P<0.01). BPPV group also showed higher vertical VOR gain asymmetries compared with the healthy control group (all P<0.01), but no significant difference was observed in VOR gains and horizontal VOR gain asymmetry (all P>0.05). The sensibility of vHIT in diagnosis of VN was 87.9%. Among 33 VN patients, 22 were diagnosed with superior vestibular nerve dysfunction, 7 were found with inferior vestibular nerve dysfunction and 3 were with both dysfunction; and 1 case was not distinguished. Conclusion: Video head impulse test can quantitatively evaluate the vestibular dysfunction of VN and can help early diagnosis of VN, which may be widely used in clinic.

Chronic subjective dizziness: Analysis of underlying personality factors.

BACKGROUND: Chronic subjective dizziness (CSD) is characterized by persistent dizziness, unsteadiness, and hypersensitivity to one's own motion or exposure to complex visual stimuli. CSD may be triggered, in predisposed individuals with specific personality traits, by acute vestibular diseases. CSD is also thought to arise from failure to re-establish normal balance strategies after resolution of acute vestibular events which may be modulated by diathesis to develop anxiety and depression. OBJECTIVE: To confirm the role of personality traits linked to anxiety and depression (i.e., neuroticism, introversion, low openness) as predisposing factors for CSD and to evaluate how individual differences in these personality traits are associated with CSD severity. METHODS: We compared 19 CSD patients with 24 individuals who had suffered from periferal vestibular disorders (PVD) (i.e., Benign Paroxysmal Postural Vertigo or Vestibular Neuritis) but had not developed CSD as well as with 25 healthy controls (HC) in terms of personality traits, assessed via the NEO-PI-R questionnaire. RESULTS: CSD patients, relative to PVD patients and HCs, scored higher on the anxiety facet of neuroticism. Total neuroticism scores were also significantly associated with dizziness severity in CSD patients but not PVD patients. CONCLUSIONS: Pre-existing anxiety-related personality traits may promote and sustain the initial etiophatogenetic mechanisms linked with the development of CSD. Targeting anxiety-related mechanisms in CSD may be therefore a promising way to reduce the disability associated with CSD.

Genetics of vestibular disorders: pathophysiological insights.

The two most common vestibular disorders are motion sickness and vestibular migraine, affecting 30 and 1-2% of the population respectively. Both are related to migraine and show a familial trend. Bilateral vestibular hypofunction is a rare condition, and some of patients also present cerebellar ataxia and neuropathy. We present recent advances in the genetics of vestibular disorders with familial aggregation. The clinical heterogeneity observed in different relatives of the same families suggests a variable penetrance and the interaction of several genes in each family. Some Mendelian sensorineural hearing loss also exhibits vestibular dysfunction, including DFNA9, DFNA11, DFNA15 and DFNA28. However, the most relevant finding during the past years is the familial clustering observed in Meniere's disease. By using whole exome sequencing and combining bioinformatics tools, novel variants in DTNA and FAM136A genes have been identified in familial Meniere's disease, and this genomic strategy will facilitate the discovery of the genetic basis of familial vestibular disorders.

Overview of the International Classification of Vestibular Disorders.

Classifications and definitions are essential to facilitate communication; promote accurate diagnostic criteria; develop, test, and use effective therapies; and specify knowledge gaps. This article describes the development of the International Classification of Vestibular Disorders (ICVD) initiative. It describes its history, scope, and goals. The Bárány Society has played a central role in organizing the ICVD by establishing internal development processes and outreach to other scientific societies. The ICVD is organized in four layers. The current focus is on disorders with a high epidemiologic importance, such as Menière disease, benign paroxysmal positional vertigo, vestibular migraine, and behavioral aspects of vestibular disorders.

Diagnostic criteria for Menière's disease.

This paper presents diagnostic criteria for Menière's disease jointly formulated by the Classification Committee of the Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society. The classification includes two categories: definite Menière's disease and probable Menière's disease. The diagnosis of definite Menière's disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 minutes and 12 hours. Probable Menière's disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms occurring in a period from 20 minutes to 24 hours.

Benign paroxysmal positional vertigo: Diagnostic criteria.

This article presents operational diagnostic criteria for benign paroxysmal positional vertigo (BPPV), formulated by the Committee for Classification of Vestibular Disorders of the Bárány Society. The classification reflects current knowledge of clinical aspects and pathomechanisms of BPPV and includes both established and emerging syndromes of BPPV. It is anticipated that growing understanding of the disease will lead to further development of this classification.

Doença vestibular em cães: 81 casos (2006-2013) / Vestibular disease in dogs: 81 cases (2006-2013)

Eighty-one cases of vestibular disease in dogs were diagnosed by the neurology service in a veterinary teaching hospital in southern Brazil from 2006 to 2013. Approximately 2/3 of these cases were interpreted as central vestibular disease (CVD) with the remaining cases being considered as peripheral vestibular disease (PVD). Pure breed dogs, especially Dachshunds (PVD) and Boxers (CVD) were more affected than mixed breed dogs. The main clinical signs observed in cases of CVD and PVD included head tilt, vestibular ataxia, and ventral or ventrolateral strabismus. Proprioceptive deficits, cranial nerve V-XII dysfunction, and changes in the levels of conscience were observed only in cases of CVD, whereas absence of palpebral reflex occurred only in cases of PVD. Inflammatory or infectious diseases, especially canine distemper and bacterial otitis were the most commonly observed conditions associated with CVD and PVD, respectively. This article establishes the epidemiology (sex, age, and breed) and prevalence of clinical signs related to canine vestibular disease in the Central Rio Grande do Sul State; discusses the use of the clinical findings in the correct diagnosis and differentiation between CVD and PVD; and defines the main specific diseases responsible for the occurrence of CVD and PVD in dogs.

De 2006 a 2013 foram diagnosticados 81 casos de doença vestibular canina no serviço de rotina em neurologia de um hospital veterinário universitário do sul do Brasil. Desses, aproximadamente dois terços foram diagnosticados com doença vestibular central (DVC) e cerca de um terço como doença vestibular periférica (DVP). Cães com raça definida foram mais acometidos que aqueles sem raça definida, principalmente Dachshund (DVP) e Boxer (DVC). Os principais sinais clínicos observados, tanto na DVP quanto na DVC, incluíram: inclinação de cabeça, ataxia vestibular e estrabismo ventral ou ventrolateral. Deficiência proprioceptiva, disfunção dos nervos cranianos V-XII e alteração de nível de consciência foram vistos apenas em casos de DVC, já a ausência de reflexo palpebral ocorreu apenas em casos de DVP. Doenças inflamatórias/infecciosas, principalmente cinomose e otite bacteriana, foram as condições mais comumente associadas à DVC e à DVP, respectivamente. Esse artigo estabelece os aspectos epidemiológicos (sexo, idade e raça) e a prevalência dos sinais clínicos observados em cães com doença vestibular na Região Central do Rio Grande do Sul, discute a utilização dos achados clínicos no diagnóstico correto e na diferenciação entre DVC e DVP, e define quais as principais doenças responsáveis pela ocorrência dessas duas síndromes clínicas.

Long-term prognosis of patients presenting first-ever vestibular symptoms in a community-based study.

BACKGROUND: Vestibular symptoms (VSs) are frequent complaints in patients attending ambulatory care and the emergency room. They may represent a peripheral vestibular disorder or a stroke/transient ischemic attack (TIA), yet many patients have VSs that cannot be clearly classified at presentation. This study aims to characterize and determine the long-term prognosis of these patients. METHODS: In a prospective community-based study involving 104,700 individuals registered at 4 health centers of Northern Portugal, patients with a first-ever-in-lifetime focal neurologic symptom (FNS) were ascertained using comprehensive methods, including referrals from physicians working in the study area and data retrieved from emergency/discharge records. Physicians were encouraged to report/notify any patient who might have experienced an FNS, including those with vertigo or vertigo-like symptoms, imbalance, presyncope, or nonspecific dizziness. After neurologic assessment patients were classified as having a peripheral vestibular symptom (pVS), a stroke/TIA, or an unclassified vestibular symptom (uVS). They were followed up 7 years after the index event at the outpatient clinic; predictors of survival free from stroke or vascular events were determined using Cox proportional hazards models. RESULTS: Of the 1163 patients with an FNS, 360 (31.0%) were included, 16.7% had a stroke/TIA, 57.8% had pVS, and 25.6% had uVS. Most patients presented only isolated VSs (62.8%); 63% were women and mean age was 60.1 years (standard deviation = 16); hypertension (47.8%), hypercholesterolemia (41.9%), and diabetes (19.2%) were the most prevalent vascular risk factors (VRFs). Cranial computed tomography (CT) scan was performed in 63.3%. Adjusting for age, sex, VRFs, and diagnosis (TIA, pVS and uVS), the long-term risk of stroke was higher when CT showed silent infarctions (hazard rate [HR] = 3.96; 95% confidence interval [CI], 1.63-9.60) and the risk of vascular events (stroke, myocardial infarction, or vascular death) was higher in patients with 2 or more VRFs (HR = 2.70; 95% CI, 1.25-5.86). Identical results were obtained when restricting the model to patients with pVS or uVS. CONCLUSIONS: First-ever-in-lifetime VSs are common in patients with FNS and may represent a good opportunity for preventing a serious vascular event, particularly in patients with vascular comorbidity (silent infarctions and VRFs).

Combined peripheral and central vestibulopathy.

Diagnosis of central vestibulopathy remains a challenge when it is associated with peripheral vestibular dysfunction because neurotological findings from peripheral vestibulopathy may overshadow those from central vestibular involvements. To define the characteristics of disorders involving both peripheral and central vestibular structures, we classified the combined vestibulopathies into four types according to their vestibular manifestations, and describe a typical case in each subtype. Infarction involving the territory of anterior inferior cerebellar artery is the most common cause of acute unilateral cases, whereas tumors involving the cerebellopontine angle should be of prime suspicion in patients with chronic unilateral ones. Wernicke encephalopathy was most common in patients with acute bilateral combined vestibulopathy while degenerative disorders should be considered in chronic bilateral ones. Since the head impulse test (HIT) is mostly positive in combined vestibulopathy, signs of central vestibular dysfunction other than negative HIT should be sought carefully even in patients with obvious clinical or laboratory features of peripheral vestibulopathy.

Vestibular migraine: diagnostic criteria.

This paper presents diagnostic criteria for vestibular migraine, jointly formulated by the Committee for Classification of Vestibular Disorders of the Bárány Society and the Migraine Classification Subcommittee of the International Headache Society (IHS). The classification includes vestibular migraine and probable vestibular migraine. Vestibular migraine will appear in an appendix of the third edition of the International Classification of Headache Disorders (ICHD) as a first step for new entities, in accordance with the usual IHS procedures. Probable vestibular migraine may be included in a later version of the ICHD, when further evidence has been accumulated. The diagnosis of vestibular migraine is based on recurrent vestibular symptoms, a history of migraine, a temporal association between vestibular symptoms and migraine symptoms and exclusion of other causes of vestibular symptoms. Symptoms that qualify for a diagnosis of vestibular migraine include various types of vertigo as well as head motion-induced dizziness with nausea. Symptoms must be of moderate or severe intensity. Duration of acute episodes is limited to a window of between 5 minutes and 72 hours.