Content Validity of the Modified Functional Scale for the Assessment and Rating of Ataxia (f-SARA) Instrument in Spinocerebellar Ataxia.

The functional Scale for the Assessment and Rating of Ataxia (f-SARA) assesses Gait, Stance, Sitting, and Speech. It was developed as a potentially clinically meaningful measure of spinocerebellar ataxia (SCA) progression for clinical trial use. Here, we evaluated content validity of the f-SARA. Qualitative interviews were conducted among individuals with SCA1 (n = 1) and SCA3 (n = 6) and healthcare professionals (HCPs) with SCA expertise (USA, n = 5; Europe, n = 3). Interviews evaluated symptoms and signs of SCA and relevance of f-SARA concepts for SCA. HCP cognitive debriefing was conducted. Interviews were recorded, transcribed, coded, and analyzed by ATLAS.TI software. Individuals with SCA1 and 3 reported 85 symptoms, signs, and impacts of SCA. All indicated difficulties with walking, stance, balance, speech, fatigue, emotions, and work. All individuals with SCA1 and 3 considered Gait, Stance, and Speech relevant f-SARA concepts; 3 considered Sitting relevant (42.9%). All HCPs considered Gait and Speech relevant; 5 (62.5%) indicated Stance was relevant. Sitting was considered a late-stage disease indicator. Most HCPs suggested inclusion of appendicular items would enhance clinical relevance. Cognitive debriefing supported clarity and comprehension of f-SARA. Maintaining current abilities on f-SARA items for 1 year was considered meaningful for most individuals with SCA1 and 3. All HCPs considered meaningful changes as stability in f-SARA score over 1-2 years, 1-2-point change in total f-SARA score, and deviation from natural history. These results support content validity of f-SARA for assessing SCA disease progression in clinical trials.

Visually induced dizziness.

PURPOSE OF REVIEW: Visually induced dizziness (VID) is a common phenomenon in vestibular disorders of both peripheral and central causes. This article provides a review of the most updated understandings of definition, pathophysiology, and treatment options. RECENT FINDINGS: The pathophysiology is complex and its severity or persistence may be related both to the underlying cause and heritable factors. Environmental and psychological factors may influence the degree of impact of VID on daily life function. Treatment is mostly empiric at this point but includes pharmacologic, desensitization, cognitive behavioral therapies, visual rehabilitation, and treatment of the underlying cause whenever present. Additional research is needed to clarify the best management of this vestibular symptom as well as some of the other conditions with which it is commonly associated. SUMMARY: VID is a fairly common vestibular syndrome constitutng spatial disorientation without illusory motion. As it is seen in both peripheral and central vestibular disorders, it should be considered a syndrome or constellation of symptoms rather than a discrete disorder. In some cases, it may be the presenting symptom with no other clear disorder linked to it.

Vestibular Rehabilitation for Peripheral Vestibular Hypofunction: An Updated Clinical Practice Guideline From the Academy of Neurologic Physical Therapy of the American Physical Therapy Association.

BACKGROUND: Uncompensated vestibular hypofunction can result in symptoms of dizziness, imbalance, and/or oscillopsia, gaze and gait instability, and impaired navigation and spatial orientation; thus, may negatively impact an individual's quality of life, ability to perform activities of daily living, drive, and work. It is estimated that one-third of adults in the United States have vestibular dysfunction and the incidence increases with age. There is strong evidence supporting vestibular physical therapy for reducing symptoms, improving gaze and postural stability, and improving function in individuals with vestibular hypofunction. The purpose of this revised clinical practice guideline is to improve quality of care and outcomes for individuals with acute, subacute, and chronic unilateral and bilateral vestibular hypofunction by providing evidence-based recommendations regarding appropriate exercises. METHODS: These guidelines are a revision of the 2016 guidelines and involved a systematic review of the literature published since 2015 through June 2020 across 6 databases. Article types included meta-analyses, systematic reviews, randomized controlled trials, cohort studies, case-control series, and case series for human subjects, published in English. Sixty-seven articles were identified as relevant to this clinical practice guideline and critically appraised for level of evidence. RESULTS: Based on strong evidence, clinicians should offer vestibular rehabilitation to adults with unilateral and bilateral vestibular hypofunction who present with impairments, activity limitations, and participation restrictions related to the vestibular deficit. Based on strong evidence and a preponderance of harm over benefit, clinicians should not include voluntary saccadic or smooth-pursuit eye movements in isolation (ie, without head movement) to promote gaze stability. Based on moderate to strong evidence, clinicians may offer specific exercise techniques to target identified activity limitations and participation restrictions, including virtual reality or augmented sensory feedback. Based on strong evidence and in consideration of patient preference, clinicians should offer supervised vestibular rehabilitation. Based on moderate to weak evidence, clinicians may prescribe weekly clinic visits plus a home exercise program of gaze stabilization exercises consisting of a minimum of: (1) 3 times per day for a total of at least 12 minutes daily for individuals with acute/subacute unilateral vestibular hypofunction; (2) 3 to 5 times per day for a total of at least 20 minutes daily for 4 to 6 weeks for individuals with chronic unilateral vestibular hypofunction; (3) 3 to 5 times per day for a total of 20 to 40 minutes daily for approximately 5 to 7 weeks for individuals with bilateral vestibular hypofunction. Based on moderate evidence, clinicians may prescribe static and dynamic balance exercises for a minimum of 20 minutes daily for at least 4 to 6 weeks for individuals with chronic unilateral vestibular hypofunction and, based on expert opinion, for a minimum of 6 to 9 weeks for individuals with bilateral vestibular hypofunction. Based on moderate evidence, clinicians may use achievement of primary goals, resolution of symptoms, normalized balance and vestibular function, or plateau in progress as reasons for stopping therapy. Based on moderate to strong evidence, clinicians may evaluate factors, including time from onset of symptoms, comorbidities, cognitive function, and use of medication that could modify rehabilitation outcomes. DISCUSSION: Recent evidence supports the original recommendations from the 2016 guidelines. There is strong evidence that vestibular physical therapy provides a clear and substantial benefit to individuals with unilateral and bilateral vestibular hypofunction. LIMITATIONS: The focus of the guideline was on peripheral vestibular hypofunction; thus, the recommendations of the guideline may not apply to individuals with central vestibular disorders. One criterion for study inclusion was that vestibular hypofunction was determined based on objective vestibular function tests. This guideline may not apply to individuals who report symptoms of dizziness, imbalance, and/or oscillopsia without a diagnosis of vestibular hypofunction. DISCLAIMER: These recommendations are intended as a guide to optimize rehabilitation outcomes for individuals undergoing vestibular physical therapy. The contents of this guideline were developed with support from the American Physical Therapy Association and the Academy of Neurologic Physical Therapy using a rigorous review process. The authors declared no conflict of interest and maintained editorial independence.Video Abstract available for more insights from the authors (see the Video, Supplemental Digital Content 1, available at: http://links.lww.com/JNPT/A369).

Autoimmune Vestibulocerebellar Syndromes.

Autoimmune disorders affecting the vestibular end organs, vestibular pathways, vestibular nuclei, and vestibulocerebellum are often underrecognized as a cause of chronic dizziness and ataxia. Autoantibodies specific for cell-surface, synaptic, and intracellular neural antigens serve as biomarkers of these disorders. This article describes the epidemiology, clinical presentation, diagnostic considerations, imaging findings, treatment, and prognosis of autoimmune disorders, in which the vestibulocerebellar syndrome is the main or presenting clinical presentation. Antibodies specific for intracellular antigenic targets described in the article are PCA-1 (Purkinje cell cytoplasmic antibody type 1, also known as anti-Yo), ANNA-1 (antinuclear neuronal antibody type 1, also known as anti-Hu), ANNA-2 (antinuclear neuronal antibody type 2, also known as anti-Ri), Ma1/2 (anti-Kelch-like 11/12 antibody), Kelch-like 11, amphiphysin, CV2 (collapsin response 2, also known as collapsin response mediator protein-5 [CRMP5]), VGCC (voltage-gated calcium channel), GAD65 (glutamic acid decarboxylase 65-kDa isoform), AP3B2 (adaptor protein 3B2, also known as anti-Nb), MAP1B (microtubule-associated protein 1B antibody, also known as anti-PCA-2), and neurochondrin antibodies. Antibodies targeting cell-surface or synaptic antigenic targets described in the article include DNER (delta/notchlike epidermal growth factor related receptor; antigen to anti-Tr), CASPR2 (contactin-associated proteinlike 2), septin-5, Homer-3, and mGluR1 (metabotropic glutamate receptor 1). The vestibulocerebellar presentation is largely indistinguishable among these conditions and is characterized by subacute onset of cerebellar symptoms over weeks to months. The diagnosis of autoimmune vestibulocerebellar syndromes is based on a combination of clinical and serological features, with a limited role for neuroimaging. Subtle eye movement abnormalities can be an early feature in many of these disorders, and therefore a meticulous vestibulo-ocular examination is essential for early and correct identification. Cancer occurrence and its type are variable and depend on the autoantibody detected and other cancer risk factors. Treatment comprises immunotherapy and cancer-directed therapy. Acute immunotherapies such as intravenous immunoglobulin, plasma exchange, and steroids are used in the initial phase, and the use of long-term immunosuppression such as rituximab may be necessary in relapsing cases. Outcomes are better if immunotherapy is started early. The neurologic prognosis depends on multiple factors.

Vestibular Rehabilitation for Peripheral Vestibular Hypofunction: An Evidence-Based Clinical Practice Guideline: FROM THE AMERICAN PHYSICAL THERAPY ASSOCIATION NEUROLOGY SECTION.

BACKGROUND: Uncompensated vestibular hypofunction results in postural instability, visual blurring with head movement, and subjective complaints of dizziness and/or imbalance. We sought to answer the question, "Is vestibular exercise effective at enhancing recovery of function in people with peripheral (unilateral or bilateral) vestibular hypofunction?" METHODS: A systematic review of the literature was performed in 5 databases published after 1985 and 5 additional sources for relevant publications were searched. Article types included meta-analyses, systematic reviews, randomized controlled trials, cohort studies, case control series, and case series for human subjects, published in English. One hundred thirty-five articles were identified as relevant to this clinical practice guideline. RESULTS/DISCUSSION: Based on strong evidence and a preponderance of benefit over harm, clinicians should offer vestibular rehabilitation to persons with unilateral and bilateral vestibular hypofunction with impairments and functional limitations related to the vestibular deficit. Based on strong evidence and a preponderance of harm over benefit, clinicians should not include voluntary saccadic or smooth-pursuit eye movements in isolation (ie, without head movement) as specific exercises for gaze stability. Based on moderate evidence, clinicians may offer specific exercise techniques to target identified impairments or functional limitations. Based on moderate evidence and in consideration of patient preference, clinicians may provide supervised vestibular rehabilitation. Based on expert opinion extrapolated from the evidence, clinicians may prescribe a minimum of 3 times per day for the performance of gaze stability exercises as 1 component of a home exercise program. Based on expert opinion extrapolated from the evidence (range of supervised visits: 2-38 weeks, mean = 10 weeks), clinicians may consider providing adequate supervised vestibular rehabilitation sessions for the patient to understand the goals of the program and how to manage and progress themselves independently. As a general guide, persons without significant comorbidities that affect mobility and with acute or subacute unilateral vestibular hypofunction may need once a week supervised sessions for 2 to 3 weeks; persons with chronic unilateral vestibular hypofunction may need once a week sessions for 4 to 6 weeks; and persons with bilateral vestibular hypofunction may need once a week sessions for 8 to 12 weeks. In addition to supervised sessions, patients are provided a daily home exercise program. DISCLAIMER: These recommendations are intended as a guide for physical therapists and clinicians to optimize rehabilitation outcomes for persons with peripheral vestibular hypofunction undergoing vestibular rehabilitation.Video Abstract available for more insights from the author (see Video, Supplemental Digital Content 1, http://links.lww.com/JNPT/A124).

Posttraumatic vertigo and dizziness.

Dizziness and vertigo are common symptoms following minor head trauma. Although these symptoms resolve within a few weeks in many patients, in some the symptoms may last much longer and impede ability to return to work and full functioning. Causes of persisting or recurrent dizziness may include benign paroxysmal positional vertigo, so-called labyrinthine concussion, unilateral vestibular nerve injury or damage to the utricle or saccule, perilymphatic fistula, or less commonly traumatic endolymphatic hydrops. Some dizziness after head trauma is due to nonlabyrinthine causes that may be related to structural or microstructural central nervous system injury or to more complicated interactions between migraine, generalized anxiety, and issues related to patients self-perception, predisposing psychological states, and environmental and stress-related factors. In this article, the authors review both the inner ear causes of dizziness after concussion and also the current understanding of chronic postconcussive dizziness when no peripheral vestibular cause can be identified.

Causes of imbalance and abnormal gait that may be misdiagnosed.

Disorders of gait and balance are common in medicine and often lead to referral for neurologic evaluation. Because the maintenance of balance and normal gait are mediated by complex neurologic pathways as well as musculoskeletal, metabolic, and behavioral considerations, the list of possible contributing causes is very large. Much of the time, the history and neurologic examination reveal the underlying cause or causes. There are instances, however, when there are limited neurologic findings, as well as no structural abnormalities on brain or spine magnetic resonance imaging studies to explain the imbalance or gait difficulty. In this article, selected disorders that may be overlooked in the neurologic examination and imaging studies are reviewed. Possible causes of imbalance include occult drug-induced ataxia, autoimmune ataxia, ataxia associated with tremor, bilateral vestibular hypofunction, and spastic or dystonic gait disorders with normal imaging.

Approach to the History and Evaluation of Vertigo and Dizziness.

PURPOSE OF REVIEW: This article reviews a method of obtaining the medical history of patients presenting with dizziness, vertigo, and imbalance. By combining elements of the history with examination, the goal is to identify patterns and an effective differential diagnosis for this group of patients to help lead to an accurate diagnosis. RECENT FINDINGS: Studies over the past dozen years have changed the historical approach to patients with dizziness from one based primarily on how the patient describes the sensation of dizziness. This older approach can lead to misdiagnosis, so a preferred method puts greater emphasis on whether the dizziness is acute or chronic, episodic or continuous, or evoked by or brought on by an event or circumstance so that a pattern may be derived that better narrows the differential diagnosis and focused examination can further narrow to a cause or causes. SUMMARY: Dizziness is a common symptom of many possible causes. This article will help clinicians navigate gathering the history and examination to formulate a working diagnosis in patients affected by dizziness.

Tinnitus, Hyperacusis, Otalgia, and Hearing Loss.

PURPOSE OF REVIEW: This article reviews the causes of tinnitus, hyperacusis, and otalgia, as well as hearing loss relevant for clinicians in the field of neurology. RECENT FINDINGS: Important causes of unilateral and bilateral tinnitus are discussed, including those that are treatable or caused by serious structural or vascular causes. Concepts of hyperacusis and misophonia are covered, along with various types of neurologic disorders that can lead to pain in the ear. Hearing loss is common but not always purely otologic. SUMMARY: Tinnitus and hearing loss are common symptoms that are sometimes related to a primary neurologic disorder. This review, tailored to neurologists who care for patients who may be referred to or encountered in neurology practice, provides information on hearing disorders, how to recognize when a neurologic process may be involved, and when to refer to otolaryngology or other specialists.

New Anti-CGRP Medications in the Treatment of Vestibular Migraine.

BACKGROUND: Vestibular migraine (VM) is a condition associated with migraine headache, vertigo, dizziness, and balance disturbances. Treatment options are limited. It is unknown if new calcitonin gene-related peptide (CGRP) migraine medications have efficacy in treating VM. METHODS: We retrospectively reviewed all patients with VM who were prescribed one of the new CGRP medications between January 2016 and July 2020. In total, 28 patients met the inclusion criteria. We specifically evaluated the "older" CGRP medications including erenumab, galcanezumab, fremanezumab, and ubrogepant. Medical records for subsequent visits were assessed to monitor improvement described by patients. RESULTS: Of the 28 patients identified, three were lost to follow up. For the remaining 25 patients, we divided the patients based on a scale of "significant improvement," "moderate improvement," "mild improvement," or "no improvement." In total 21 of 25 patients demonstrated some level of improvement in their VM symptoms with 15 having moderate to significant improvement. CONCLUSION: Results demonstrated a trend toward improvement, suggesting that the CGRP medications appear to be a decent treatment option for VM. A prospective study evaluating CGRP medications in patients with VM would provide further information about this treatment option.

Coding and Reimbursement for Vestibular Tests by the U.S. Centers for Medicare and Medicaid Services (CMS).

Coding and insurance reimbursement is a part of the healthcare system in the United States but is subject to periodic modifications. In addition to changes in the evaluation and management (E/M) codes that took effect in 2021, there are some differences in coding for some diagnostic vestibular function test procedures. Two new codes for vestibular myogenic evoked potential testing were added and previous codes for auditory evoked potential codes 92585 and 92586, which some facilities had used to bill for vestibular myogenic evoked potential testing, have been eliminated. This article outlines the current state of coding and reimbursement by CMS for vestibular procedures.

Benign paroxysmal positional vertigo.

Benign paroxysmal positional vertigo (BPPV) is the most common cause of recurrent vertigo and has a lifetime prevalence of 2.4% in the general population. Benign paroxysmal positional vertigo is caused when calcium carbonate material originating from the macula of the utricle falls into one of the semicircular canals. Due to their density relative to the endolymph, they move in response to gravity and trigger excitation of the ampullary nerve of the affected canal. This, in turn, produces a burst of vertigo associated with nystagmus unique to that canal. Recognition of this condition is important not only because it may avert expensive and often unnecessary testing, but also because treatment is rapid, easy, and effective in >90% of cases. Two well-established methods of treating BPPV are discussed and explained in this article along with a brief discussion of the most commonly used method for treatment of horizontal canal BPPV. Recurrence rates approach 50% in those followed for at least 5 years.

Clinical practice guideline: benign paroxysmal positional vertigo.

OBJECTIVES: This guideline provides evidence-based recommendations on managing benign paroxysmal positional vertigo (BPPV), which is the most common vestibular disorder in adults, with a lifetime prevalence of 2.4 percent. The guideline targets patients aged 18 years or older with a potential diagnosis of BPPV, evaluated in any setting in which an adult with BPPV would be identified, monitored, or managed. This guideline is intended for all clinicians who are likely to diagnose and manage adults with BPPV. PURPOSE: The primary purposes of this guideline are to improve quality of care and outcomes for BPPV by improving the accurate and efficient diagnosis of BPPV, reducing the inappropriate use of vestibular suppressant medications, decreasing the inappropriate use of ancillary tests such as radiographic imaging and vestibular testing, and to promote the use of effective repositioning maneuvers for treatment. In creating this guideline, the American Academy of Otolaryngology-Head and Neck Surgery Foundation selected a panel representing the fields of audiology, chiropractic medicine, emergency medicine, family medicine, geriatric medicine, internal medicine, neurology, nursing, otolaryngology-head and neck surgery, physical therapy, and physical medicine and rehabilitation. RESULTS: The panel made strong recommendations that 1) clinicians should diagnose posterior semicircular canal BPPV when vertigo associated with nystagmus is provoked by the Dix-Hallpike maneuver. The panel made recommendations against 1) radiographic imaging, vestibular testing, or both in patients diagnosed with BPPV, unless the diagnosis is uncertain or there are additional symptoms or signs unrelated to BPPV that warrant testing; and 2) routinely treating BPPV with vestibular suppressant medications such as antihistamines or benzodiazepines. The panel made recommendations that 1) if the patient has a history compatible with BPPV and the Dix-Hallpike test is negative, clinicians should perform a supine roll test to assess for lateral semicircular canal BPPV; 2) clinicians should differentiate BPPV from other causes of imbalance, dizziness, and vertigo; 3) clinicians should question patients with BPPV for factors that modify management including impaired mobility or balance, CNS disorders, lack of home support, and increased risk for falling; 4) clinicians should treat patients with posterior canal BPPV with a particle repositioning maneuver (PRM); 5) clinicians should reassess patients within 1 month after an initial period of observation or treatment to confirm symptom resolution; 6) clinicians should evaluate patients with BPPV who are initial treatment failures for persistent BPPV or underlying peripheral vestibular or CNS disorders; and 7) clinicians should counsel patients regarding the impact of BPPV on their safety, the potential for disease recurrence, and the importance of follow-up. The panel offered as options that 1) clinicians may offer vestibular rehabilitation, either self-administered or with a clinician, for the initial treatment of BPPV and 2) clinicians may offer observation as initial management for patients with BPPV and with assurance of follow-up. The panel made no recommendation concerning audiometric testing in patients diagnosed with BPPV. DISCLAIMER: This clinical practice guideline is not intended as a sole source of guidance in managing benign paroxysmal positional vertigo. Rather, it is designed to assist clinicians by providing an evidence-based framework for decision-making strategies. The guideline is not intended to replace clinical judgement or establish a protocol for all individuals with this condition, and may not provide the only appropriate approach to diagnosing and managing this problem.

Vestibular evoked myogenic potential testing: Payment policy review for clinicians and payers.

PURPOSE OF REVIEW: A recent American Academy of Neurology Evidence-Based Practice Guideline on vestibular myogenic evoked potential (VEMP) testing has described superior canal dehiscence syndrome (SCDS) and evaluated the merits of VEMP in its diagnosis. SCDS is an uncommon but now well-recognized cause of dizziness and auditory symptoms. This article familiarizes health care providers with this syndrome and the utility and shortcomings of VEMP as a diagnostic test and also explores payment policies for VEMP. RECENT FINDINGS: In carefully selected patients with documented history compatible with the SCDS, both high-resolution temporal bone CT scan and VEMP are valuable aids for diagnosis. Payers might be unfamiliar with both this syndrome and VEMP testing. SUMMARY: It is important to raise awareness of VEMP and its possible indications and the rationale for coverage of VEMP testing. Payers may not be readily receptive to VEMP coverage if this test is used in an undifferentiated manner for all common vestibular and auditory symptoms.

Bilateral Vestibular Dysfunction Associated With Chronic Exposure to Military Jet Propellant Type-Eight Jet Fuel.

We describe three patients diagnosed with bilateral vestibular dysfunction associated with the jet propellant type-eight (JP-8) fuel exposure. Chronic exposure to aromatic and aliphatic hydrocarbons, which are the main constituents of JP-8 military aircraft jet fuel, occurred over 3-5 years' duration while working on or near the flight line. Exposure to toxic hydrocarbons was substantiated by the presence of JP-8 metabolite n-hexane in the blood of one of the cases. The presenting symptoms were dizziness, headache, fatigue, and imbalance. Rotational chair testing confirmed bilateral vestibular dysfunction in all the three patients. Vestibular function improved over time once the exposure was removed. Bilateral vestibular dysfunction has been associated with hydrocarbon exposure in humans, but only recently has emphasis been placed specifically on the detrimental effects of JP-8 jet fuel and its numerous hydrocarbon constituents. Data are limited on the mechanism of JP-8-induced vestibular dysfunction or ototoxicity. Early recognition of JP-8 toxicity risk, cessation of exposure, and customized vestibular therapy offer the best chance for improved balance. Bilateral vestibular impairment is under-recognized in those chronically exposed to all forms of jet fuel.

Second-generation antiepileptic drugs' impact on balance: a meta-analysis.

OBJECTIVE: To systematically review available evidence regarding whether second-generation antiepileptic drugs (AEDs) contribute to the risk of balance disorders. METHODS: We systematically evaluated data from randomized controlled trials that compared adjunctive therapy with a second-generation AED (gabapentin, lamotrigine, levetiracetam, oxcarbazepine, pregabalin, tiagabine, topIramate, or zonisamide) vs placebo for partial epilepsy and that reported dose-specific rates of ataxia or Imbalance for each group. Random-effects meta-analysis was used to pool ratios (risk ratio [RR]) and associated 95% confidence Intervals to determine whether there was evidence of an overall AED class effect or a dose-response effect and whether there were differences between Individual AEDs. RESULTS: Sixteen studies met inclusion criteria, representing 4279 individuals randomized to a second-generation AED and 1830 patients to placebo. Pooled analyses of all AEDs demonstrated that they Increase imbalance risk at any dose (RR, 2.73; 95% confidence interval, 2.07-3.61) and at lowest dose (RR, 1.76; 95% confidence interval, 1.26-2.46). The highest dose analysis showed heterogeneity; evaluation of individual AEDs revealed that oxcarbamazepine and topiramate increased imbalance risk at all doses, whereas gabapentin and levetiracetam did not increase imbalance risk at any dose. A dose-response effect was observed for most AEDs. CONCLUSION: Second-generation AEDs at standard dosages, except for gabapentin and levetiracetam, increase the imbalance risk, and evidence exists for a dose-response effect. The mechanisms, risk factors, and consequences of this risk for individual AEDs warrant further study.