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).

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.

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.

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.

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.

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.

Practice guideline: Cervical and ocular vestibular evoked myogenic potential testing: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

OBJECTIVE: To systematically review the evidence and make recommendations with regard to diagnostic utility of cervical and ocular vestibular evoked myogenic potentials (cVEMP and oVEMP, respectively). Four questions were asked: Does cVEMP accurately identify superior canal dehiscence syndrome (SCDS)? Does oVEMP accurately identify SCDS? For suspected vestibular symptoms, does cVEMP/oVEMP accurately identify vestibular dysfunction related to the saccule/utricle? For vestibular symptoms, does cVEMP/oVEMP accurately and substantively aid diagnosis of any specific vestibular disorder besides SCDS? METHODS: The guideline panel identified and classified relevant published studies (January 1980-December 2016) according to the 2004 American Academy of Neurology process. RESULTS AND RECOMMENDATIONS: Level C positive: Clinicians may use cVEMP stimulus threshold values to distinguish SCDS from controls (2 Class III studies) (sensitivity 86%-91%, specificity 90%-96%). Corrected cVEMP amplitude may be used to distinguish SCDS from controls (2 Class III studies) (sensitivity 100%, specificity 93%). Clinicians may use oVEMP amplitude to distinguish SCDS from normal controls (3 Class III studies) (sensitivity 77%-100%, specificity 98%-100%). oVEMP threshold may be used to aid in distinguishing SCDS from controls (3 Class III studies) (sensitivity 70%-100%, specificity 77%-100%). Level U: Evidence is insufficient to determine whether cVEMP and oVEMP can accurately identify vestibular function specifically related to the saccule/utricle, or whether cVEMP or oVEMP is useful in diagnosing vestibular neuritis or Ménière disease. Level C negative: It has not been demonstrated that cVEMP substantively aids in diagnosing benign paroxysmal positional vertigo, or that cVEMP or oVEMP aids in diagnosing/managing vestibular migraine.

ACR Appropriateness Criteria® Tinnitus.

Tinnitus is the perception of sound in the absence of an external source. It is a common symptom that can be related to hearing loss and other benign causes. However, tinnitus may be disabling and can be the only symptom in a patient with a central nervous system process disorder. History and physical examination are crucial first steps to determine the need for imaging. CT and MRI are useful in the setting of pulsatile tinnitus to evaluate for an underlying vascular anomaly or abnormality. If there is concomitant asymmetric hearing loss, neurologic deficit, or head trauma, imaging should be guided by those respective ACR Appropriateness Criteria® documents, rather than the presence of tinnitus. Imaging is not usually appropriate in the evaluation of subjective, nonpulsatile tinnitus that does not localize to one ear. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Dizziness in the Outpatient Care Setting.

PURPOSE OF REVIEW: This article summarizes an approach to evaluating dizziness for the general neurologist and reviews common and important causes of dizziness and vertigo. RECENT FINDINGS: Improved methods of diagnosing patients with vertigo and dizziness have been evolving, including additional diagnostic criteria and characterization of some common conditions that cause dizziness (eg, vestibular migraine, benign paroxysmal positional vertigo, chronic subjective dizziness). Other uncommon causes of dizziness (eg, superior canal dehiscence syndrome, episodic ataxia type 2) have also been better clarified. Distinguishing between central and peripheral causes of vertigo can be accomplished reliably through history and examination, but imaging techniques have further added to accuracy. What has not changed is the necessity of obtaining a basic history of the patient's symptoms to narrow the list of possible causes. SUMMARY: Dizziness and vertigo are extremely common symptoms that also affect function at home and at work. Improvements in the diagnosis and management of the syndromes that cause dizziness and vertigo will enhance patient care and cost efficiencies in a health care system with limited resources. Clinicians who evaluate patients with dizziness will serve their patient population well by continuing to manage patients with well-focused workup and attentive care.

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).

Clinical perspectives on medical marijuana (cannabis) for neurologic disorders.

The American Academy of Neurology published an evidence-based systematic review of randomized controlled trials using marijuana (Cannabis sativa) or cannabinoids in neurologic disorders. Several cannabinoids showed effectiveness or probable effectiveness for spasticity, central pain, and painful spasms in multiple sclerosis. The review justifies insurance coverage for dronabinol and nabilone for these indications. Many insurance companies already cover these medications for other indications. It is unlikely that the review will alter coverage for herbal marijuana. Currently, no payers cover the costs of herbal medical marijuana because it is illegal under federal law and in most states. Cannabinoid preparations currently available by prescription may have a role in other neurologic conditions, but quality scientific evidence is lacking at this time.

Benign Paroxysmal Positional Vertigo in the Acute Care Setting.

Benign paroxysmal positional vertigo (BPPV) is a common cause of vertigo characterized by brief episodes provoked by head movements. The first attack of BPPV usually occurs in bed or upon getting up. Because it often begins abruptly, it can be alarming and lead to emergency department evaluation. The episodes of spinning often last 10 to 20 seconds, but may occasionally last as long as 1 minute. There are several forms of BPPV. In nearly all cases, highly effective treatment can be offered to patients. This article reviews the current state of our understanding of this condition and its management.