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Review Article

Address correspondence to Dr David E. Newman-Toker, Johns Hopkins Hospital, Department of Neurology, 600 North Wolfe Street, Meyer 8-154, Baltimore, MD 21287, toker@jhu.edu. Relationship Disclosure: Dr Newman-Toker has received honoraria for speaking from Janssen Pharmaceuticals, Inc., and consulting fees from Pierre Fabre. He has received research support from the Agency for Healthcare Research and Quality and the NIH. Unlabeled Use of Products/Investigational Use Disclosure: Dr Newman-Toker reports no disclosure. * 2012, American Academy of Neurology.

Symptoms and Signs of Neuro-otologic Disorders


David E. Newman-Toker, MD, PhD, FAAN ABSTRACT
Purpose of Review: Symptoms and signs of neuro-otologic disorders are critical components in the diagnostic assessment of patients with vestibular symptoms such as vertigo, dizziness, unsteadiness, and oscillopsia. Most diagnoses can be accomplished at the bedside. An understanding of key diagnostic principles is essential for all practicing neurologists, who are often faced with determining whether such patients warrant urgent diagnostic testing or hospital admission. This article introduces readers to core concepts and recent advances in the understanding of directed history taking and physical examination in patients with vestibular symptoms or suspected neuro-otologic disorders. Recent Findings: International consensus definitions for vestibular symptoms have recently been published. During the past 5 years, a growing body of scientific evidence has demonstrated that the traditional approach to bedside diagnosis of patients with vertigo and dizziness is inadequate. Former teaching that history taking should first rely on categorizing symptoms by type (eg, vertigo, presyncope, disequilibrium, nonspecific dizziness) has been replaced by an emphasis on categorizing timing and triggers for vestibular symptoms, which focuses the clinicians attention on four key syndromic patterns: (1) acute, spontaneous, prolonged vestibular symptoms; (2) episodic, positional vestibular symptoms; (3) episodic, spontaneous vestibular symptoms; and (4) chronic unsteadiness (with or without oscillopsia). Each of these categories delineates a relatively narrow differential diagnosis within which a focused examination distinguishes between benign common causes and dangerous uncommon ones. Summary: A focused approach to bedside assessment of patients with vestibular symptoms is essential for accurate and efficient diagnosis. All neurologists should be aware of major recent advances.
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INTRODUCTION Patients with neuro-otologic disorders typically present with vertigo, dizziness, unsteadiness, or oscillopsia. Although much confusion and controversy exist regarding these terms, international consensus definitions have recently been published (Box 2-1).1,2 This article will focus on key elements of the history and physical examination that have important diagnostic value in the

assessment of patients with such symptoms. While this article is not intended to cover differential diagnosis per se, a brief discussion about general diagnostic approach is warranted, since the approach influences the choice of what is important in the history and physical examination. In general, the role of the neurologist in vestibular diagnosis is often to differentiate central (ie, neurologic) from
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Definitions for Common BOX 2-1 International Consensus Vestibular Symptomsa,b Term
Vertigo

Definition
The sensation of self-motion when no self-motion is occurring or the sensation of distorted self-motion during an otherwise normal head movement. The sensation of disturbed or impaired spatial orientation without a false or distorted sense of motion. The feeling of being unstable while seated, standing, or walking without a particular directional preference. The false sensation that the visual surround is oscillating. The sensation of impending loss of consciousness. Transient loss of consciousness due to transient global cerebral hypoperfusion characterized by rapid onset, short duration, and spontaneous complete recovery.

Dizziness Unsteadiness Oscillopsia Presyncope Syncope

a b

Data from Bisdorf A, et al, J Vestib Res.1 iospress.metapress.com/content/m127vq45v8l47052/. Data from Task Force for the Diagnosis and Management of Syncope, European Society of Cardiology (ESC), European Heart Rhythm Association (EHRA), et al, Eur Heart J.2 eurheartj.oxfordjournals.org/content/30/21/2631.long.

peripheral (ie, otologic) causes for vestibular symptoms. Although some central causes (eg, multiple sclerosis) are less urgent, and some peripheral causes (eg, bacterial labyrinthitis) are quite urgent, usually the reverse occurs; central causes (eg, stroke) are dangerous and urgent, and peripheral causes (eg, benign paroxysmal positional vertigo [BPPV] and Me nie `re disease) are benign or nonurgent. Thus, much of this article will focus on the utility of symptoms and signs for localizing vestibular lesions as central versus peripheral. Despite this focus, the neurologist must also remain vigilant about general medical disorders (especially cardiac), which, in aggregate, are actually more common causes of dizziness than are otologic or neurologic disorders.3 The article will therefore also make reference to circumstances in which symptoms or signs can, or cannot, be used to identify non-neurovestibular causes, particularly those that are dangerous and urgent (eg, cardiac arrhythmia).
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RECENT TRENDS The traditional approach to diagnosis of patients with vertigo or dizziness relies heavily on the premise that the type of vestibular symptom predicts the underlying etiology. In this approach, the symptom type is classified as (1) vertigo (spinning or motion), (2) presyncope (impending faint), (3) disequilibrium (unsteadiness when walking), or (4) nonspecific dizziness (any other balancerelated sensation not fitting the prior three categories).4 These traditional categories direct subsequent diagnostic inquiry, with vertigo prompting a search for vestibular causes, presyncope a search for cardiovascular causes, unsteadiness a search for neurologic causes, and nonspecific dizziness a search for psychiatric or metabolic causes.5 This approach, described in 1972,6 continues to appear in highimpact medical journals, commonly used medical texts, and Internet-based resources. Recent studies confirm that this diagnostic method for assessing
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Symptoms and Signs


KEY POINTS

h Strong evidence now


indicates that the quality-of-symptoms approach based on categorizing dizziness type is neither valid nor reliable and could be contributing to misdiagnosis.

h Neither patients nor


physicians have a clear understanding of what the terms dizziness and vertigo mean. New consensus definitions have been published.

patients with dizziness represents the current standard of clinical practice in frontline care settings, such as the emergency department.7,8 Unfortunately, strong evidence now indicates that the quality-of-symptoms approach is neither valid nor reliable9Y13 and could be contributing to misdiagnosis.4,8 Patients with dizziness frequently change their report of the type of dizziness when they are asked to describe it 5 to 10 minutes later, and types of dizziness are not valid indicators of the cause.9 Best available evidence instead suggests that a shift of emphasis in clinical assessment away from dizziness type and toward dizziness timing (eg, episode duration) and triggers (eg, changes in head position) will probably yield more accurate and reproducible diagnostic results.4 At its core, this timing-and-triggers approach seeks to classify patients into one of four major clinical categories: (1) acute, spontaneous, prolonged vestibular symptoms (often called the acute vestibular syndrome14,15), as seen with vestibular neuritis and posterior fossa stroke; (2) episodic, positional vestibular symptoms, as seen with BPPV and central mimics; (3) episodic, spontaneous vestibular symptoms, as seen with vestibular migraine, Me nie `re disease, and TIA; and (4) chronic unsteadiness (with or without oscillopsia), as seen with cerebellar degeneration, bilateral vestibular failure, and spinal cord compression. Each of these syndromes will be covered in greater detail in later issue. articles in this VESTIBULAR SYMPTOMS AND HISTORY TAKING As with most other areas of neurology, history taking is paramount in patients presenting with vestibular symptoms. As with other patient symptoms, it is generally important to begin with an open-

ended question about the patients symptoms and allow a narrative to unfold. Nevertheless, it is quite typical with vestibular patients that the critical aspects of the history are not spontaneously reported by the patient, and directed historical questioning is required. This section will describe each major symptom dimension, such as type and duration, and other key historical elements, such as review of symptoms, emphasizing their diagnostic value and some technical pearls about typical patient responses. Symptom Type Neither patients9 nor physicians8,9,16 have a clear understanding of what the terms dizziness and vertigo mean. In the United States, it is traditional for dizziness to serve as an umbrella term that incorporates different forms (eg, vertigo, presyncope, unsteadiness),5,8,10,11,17 and in Europe and Asia this has sometimes,18Y20 but not always,1,21Y25 also been the case. This discrepancy has recently been resolved by an international consensus group in favor of a nonhierarchic approach that segregates the two terms entirely.1 This new approach, however, has not yet fully penetrated the scientific literature.12,13,26 Furthermore, despite an attempt to clarify that vertigo refers to any illusory sense of motion, whether spinning or not,1 many practicing clinicians continue to identify vertigo as a yes answer to the question, Is the world spinning? As suggested above, the traditional approach to diagnosis of vertigo or dizziness relies heavily on vestibular symptom type. It remains standard for textbooks and most clinicians to suggest that the first and most important question to ask a patient presenting with dizziness is, What do you mean by dizzy? While this may be a reasonable question to ask as a conversation starter,
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little evidence suggests that the answer provides much information of any real use to the patient or physician. Systematic reviews have found no published studies that support the predictive validity of symptom type.13,27 Small studies have shown no association between dizziness type and final clinical diagnoses (eg, type of dizziness fails to differentiate central from peripheral causes12 or psychiatric from vestibular causes28). Furthermore, individual patients do not consistently or reliably report their subjective dizziness experience as matching a single category, and different patients with the same condition experience different types of dizziness.9 The exception may be in categorizing patients whose primary symptom is unsteadiness or oscillopsia rather than vertigo, dizziness, or presyncope. Although well-designed prospective studies are lacking, unsteadiness and oscillopsia are likely associated with a somewhat different differential diagnosis than vertigo, dizziness, and presyncope. This occurs partly because of their tendency to be reported as primary complaints in patients with chronic rather than acute or episodic symptoms. It may also reflect the fact that unilateral disorders, whether central or peripheral, are somewhat more likely to produce vertigo, whereas those that are bilateral and symmetric do so only infrequently. Unsteadiness without dizziness or vertigo is most commonly seen in patients with sensory loss (eg, peripheral neuropathy), spinal cord diseases (eg, transverse myelitis, cord compression), and slowly progressive, bilateral cerebellar or vestibular failure (eg, spinocerebellar ataxia syndromes; midline cerebellar neoplasm; bilateral vestibular schwannoma in neurofibromatosis, type 2). Gait unsteadiness that is present only with eyes closed and completely absent with eyes open is more likely to be due to bilateral peContinuum Lifelong Learning Neurol 2012;18(5):10161040

ripheral vestibular failure than a central cause. Oscillopsia present at rest usually indicates the presence of nystagmus of any type, but often vertical pendular nystagmus is present in the straightahead position of gaze, as seen in patients with brainstem lesions caused by multiple sclerosis or stroke. Oscillopsia that occurs only during head motion (eg, while walking) usually indicates bilateral vestibular failure (eg, post gentamicin toxicity). Symptom Timing The timing of vestibular symptoms (including episode, relapse, and illness duration; symptom onset; and frequency) has been studied fairly extensively in individual disease populations28Y31 but not generally as a prospective predictor to help differentiate among diagnoses. Despite the lack of firm evidence, some studies suggest that certain aspects of symptom timing likely have strong predictive power in diagnosis of vestibular symptoms.13 At a minimum, timing of symptoms is more reliably reported by patients than type.9 Inquiry should begin with an attempt to categorize the vestibular symptoms as continuous (with or without fluctuations) or discretely episodic (with virtually no symptoms between episodes). Care should be taken to avoid confusing continuous situational symptoms (eg, I am only unsteady when I am walking, but I am always unsteady when walking.) from those that are truly episodic; such contextdependent symptoms should generally be thought of as continuous from a diagnostic standpoint. When symptoms are episodic, it is useful to try to categorize the episode duration (average and range of durations) as seconds, minutes to hours, or days, with certain implications for the differential diagnosis (Table 2-1).32,33

KEY POINTS

h Oscillopsia present at
rest usually indicates the presence of nystagmus; oscillopsia that occurs only during head motion usually indicates bilateral vestibular failure.

h Care should be taken to


avoid confusing continuous situational symptoms from those that are truly episodic. When symptoms are episodic, it is useful to categorize the episode duration as seconds, minutes to hours, or days.

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Symptoms and Signs

a TABLE 2-1 Common Causes of New Vertigo or Dizziness and Dangerous Mimics, By Duration

Duration
Seconds to hours (episodic: transient or intermittent)

Common, Benignb Causes


Benign paroxysmal positional vertigo(s) Benign orthostatic hypotension (eg, medications) (seconds to minutes) Reflex syncope (seconds to minutes) Panic attack (minutes to hours) nie ` re syndrome (minutes to hours) Me Vestibular migraine (seconds to daysc)

Principal Dangerous Mimics


TIA Cardiac arrhythmia Other cardiovascular disorders (eg, myocardial ischemia, aortic dissection, atrial myxoma, pulmonary embolus, occult gastrointestinal bleeding) Neurohumoral neoplasm (eg, insulinomad, pheochromocytoma) Brainstem, cerebellar, or labyrinthine stroke Bacterial labyrinthitis/mastoiditis or herpes zoster oticus Brainstem encephalitis (eg, Listeria, herpes simplex, paraneoplastic), Miller-Fisher syndrome Wernicke syndrome Medication toxicitye (eg, lithium), drug withdrawal (eg, alcohol), or toxic exposure (eg, carbon monoxide)

Days to weeks (nonepisodic: persistent or continuous)

Vestibular neuritis Viral labyrinthitis Medication toxicity (eg, anticonvulsants)

Reprinted with permission from Newman-Toker DE, The neuro-ophthalmology virtual education library.32 novel.utah.edu/NewmanToker/collection.php. b Although they may be quite disabling during the acute illness phase, diseases classified here as common, benign causes rarely produce severe, irreversible morbidity or mortality, unlike their dangerous mimics counterparts. c Vestibular migraine episodes may last longer than a day in about 25% of cases.33 Rigorous data on the duration of symptoms in this subset of patients with vestibular migraine are lacking, but clinical experience suggests that such patients rarely experience true episodes lasting longer than 48 to 72 hours. d Other causes of hypoglycemia (eg, excess exogenous insulin) are more common but usually simpler to diagnose. e The duration of medication toxicity depends on dosing and the half-life of the medication.

Three clinical nuances about episode duration are worth noting: 1. If the clinician is not careful when inquiring about duration, patients may initially report illness rather than episode duration. For example, in BPPV, episodes generally last seconds but occur repetitively during a bout or relapse for 1 to 2 weeks. 2. Patients may respond to an openended question about episode duration with, I dont know or I wasnt looking at the clock; do not allow them to escape answering the question. It is easy to reframe the question

for the patient by asking, Was it seconds, minutes, hours, days, weeks, months, or years? to help them understand the level of precision required. 3. Patients may initially overestimate episode duration, particularly for brief episodes, either because time seems to slow down during an attack of severe vertigo or dizziness or they are reporting the duration of feeling slightly unwell (eg, nauseated) or mildly unsteady, rather than the core duration of the main symptom. It may be necessary to push the patient toward a shorter duration to test
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whether temporal expansion may be at play. For example, if a patient initially reports a 5-minute duration, ask, Is it possible it might have been just a couple of minutes? If the patient says yes, consider asking, Might it have been less than a minute? Follow-up questions such as, Did the bad vertigo you described last the whole time during the spell, or was the worst of it a lot shorter? may also get to the duration of core symptoms. Less is known about symptom onset and frequency as predictors of underlying disease. Some weak evidence suggests that abrupt-onset (instantaneous or nearly so) dizziness or vertigo may be more likely to result from cerebrovascular than inflammatory causes,13 but it is common for many benign forms of dizziness, such as BPPV, to begin fairly abruptly. Also, in cases in which symptoms were absent prior to sleep and present on awakening, the onset characteristics cannot be determined. Frequency of symptoms is an imprecise predictor, except in cases in which symptoms are frequent during a very long illness duration, such as a decade or longer, without permanent sequelae. In such cases, benign, recurrent disorders, such as vestibular migraine and Me nie `re disease, are highly likely. With an illness duration of weeks or months, frequent or escalating symptoms may actually be harbingers of a dangerous underlying etiology, such as TIA.13 Illness durations of 1 to 5 years are intermediate in risk. Symptom Triggers Vestibular symptom triggers may include positional change (head position or body posture), Valsalva maneuver, and exertion, among others.1,10 Again, despite the lack of prospective studies, some triggers clearly have strong predictive power in diagnosis of neuroContinuum Lifelong Learning Neurol 2012;18(5):10161040

otologic as well as cardiovascular disorders causing episodic vestibular symptoms.10 Similar to timing, symptom triggers are more consistently reported by patients than type.9 Clinicians must carefully distinguish triggers (which provoke new symptoms not present at baseline) from exacerbating features (which worsen preexisting baseline symptoms), since almost all forms of vestibular dizziness, whether central or peripheral, are exacerbated by head movement, at least acutely. The most common triggers are changes in head position or body posture, particularly rising from a recumbent or seated position.34 The most common etiologies are orthostatic hypotension and BPPV. Dangerous etiologies include serious causes of orthostatic hypotension, such as internal bleeding, and central mimics of BPPV, usually due to posterior fossa structural lesions.35,36 BPPV may be confused with orthostatic hypotension if patients report dizziness on rising. The two can generally be differentiated by inquiring about symptoms that occur on reclining or rolling over in bed, which should only be present in patients with BPPV. Forms of BPPV and their diagnoses are described in other articles. However, triggers for symptoms are often canal specific (eg, posterior canal BPPV is triggered by tipping the head back but not by turning the head to one side when upright), and these issues will be alluded to in the Signs section that follows Symptoms since positional testing and treatment maneuvers are also canal specific.37 Vestibular migraine can also produce BPPV-like positional symptoms.38 Care should be taken to avoid confusing brief, triggered episodes (as with BPPV or orthostatic hypotension) with head-motion dizziness (dizziness occurring only during head motion, ie, time locked to the head movement)1 typically seen in patients with chronic dizziness after unilateral or bilateral vestibular

KEY POINTS

h With an illness duration


of weeks or months, frequent or escalating symptoms may actually be harbingers of a dangerous underlying etiology such as TIA.

h Clinicians must carefully


distinguish triggers from exacerbating features, since almost all forms of vestibular dizziness, whether central or peripheral, are exacerbated by head movement.

h Care should be taken to


avoid confusing brief, triggered episodes with head-motion dizziness, typically seen in patients with chronic dizziness after unilateral or bilateral vestibular loss.

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Symptoms and Signs


KEY POINTS

h Clear, reproducible
triggers for nonexertional vestibular symptoms usually indicate a benign underlying cause. Most dangerous causes for episodic symptoms, such as TIA and cardiac arrhythmia, usually occur spontaneously.

h Auditory symptoms may


be peripheral in localization but dangerous in cause.

loss but also in vestibular migraine. Vestibular symptoms triggered by visual motion with head still are commonly encountered in vestibular migraine and may be relatively specific for this condition. Some rare triggers, such as sound, sometimes referred to as the Tullio phenomenon, point to a narrow list of rare causes, such as superior canal dehiscence syndrome, reflex epilepsy, and long QT syndrome. A more thorough discussion of triggers is available elsewhere.10 The most important message about triggered vestibular symptoms is that most patients with clear, reproducible triggers for their symptoms have a benign underlying cause.10 Most dangerous causes for episodic symptoms, such as TIA and cardiac arrhythmia, usually occur spontaneously. A notable exception is exertion, in which cardiac causes remain a serious concern.10 On rare occasions, far lateral head rotation can trigger posterior circulation TIA in the rotational vertebral artery syndrome.39 These unusual cases must be distinguished from the more common carotid sinus hypersensitivity syndrome that also presents with dizziness triggered by lateral head rotation or anterior neck compression. Review of Systems Associated symptoms are often important in the diagnostic assessment of patients with a primary vestibular symptom. They may be divided into para-vestibular, otologic, cephalalgic, neurologic, and other medical symptoms. Symptoms spontaneously reported by the patient, as opposed to elicited on symptom review, are perhaps more likely to be relevant, but it is common for diagnostically critical symptoms to seem mild or irrelevant to patients, eg, mild neck pain in vertebral artery dissection presenting with vertigo.40 The presence of vestibulo-vagal (nausea, vomiting) and vestibulo-spinal (gait

disturbance) symptoms is the rule rather than the exception in patients with acute vestibular pathology, whether new or recurrent and episodic. These three para-vestibular symptoms are often prominent in patients with a report of vertigo, particularly if the vertigo is sustained for more than a few seconds. Although these symptoms usually indicate a vestibular etiology, they do not assist in localization (ie, in distinguishing central from peripheral), except perhaps in cases in which the associated paravestibular symptoms are disproportional in severity to the primary vestibular symptoms.13 With peripheral vestibular pathology, vertigo, nausea, and gait instability tend to be roughly proportional to one another in severity; with central lesions, this need not be so (eg, disproportionate vomiting or gait disturbance relative to dizziness or vertigo41,42 ). This disproportion may explain why acute dizziness dominated by unsteadiness or ataxia is more likely to have a central cause.12,43 Also, the report of vertigo combined with nausea or vomiting is not always due to primary vestibular pathology and may instead be a sign of cardiovascular disease, such as myocardial infarction44 or orthostatic hypotension.45 Otologic symptoms that may accompany vestibular symptoms include both auditory (eg, tinnitus, hearing loss, hyperacusis) and aural (eg, ear fullness or pressure, otalgia) symptoms. The presence of such symptoms substantially increases the odds of a peripheral localization but does not confirm a nonurgent cause except in cases where auditory symptoms indicate clear Me nie `re disease, eg, episodic vertigo lasting hours during several years, with episodes routinely heralded by fluctuating, low-frequency tinnitus and reversible low-frequency hearing loss documented by audiometry. Otherwise, auditory symptoms may be peripheral
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in localization but dangerous in cause,14 eg, TIA or stroke due to ischemia in the anterior inferior cerebellar artery territory.11,46 Auditory symptoms may even occur with dangerous cardiac disorders.44 Extra caution should be taken when dizziness or vertigo occurs in the context of ear or retroauricular pain absent signs of ear disease (eg, erythema, discharge, tenderness), since ear pain is not always due to local pathology, ie, referred otalgia, and vertebral artery dissection is a recognized cause of such pain.47 Vestibular migraine may cause either auditory or aural symptoms.21 Cephalalgic symptoms, whether manifest as headache, neck pain, eye pain, facial pain, or ear pain, are infrequent and, when present, usually not prominent in patients with benign peripheral vestibular diseases such as BPPV, Me nie `re disease, and vestibular neuritis. They are typical in vestibular migraine, but also frequent in dangerous central disorders that cause vestibular symptoms, particularly posterior circulation stroke.13,48 When misdiagnosed, posterior circulation strokes are sometimes mistaken for migraine (Case 2-1).49 Pain that is sudden (abrupt onset), sustained (more than 72 hours), or severe, more likely indicates a dangerous cerebrovascular condition,13 although even the pain of vertebral artery dissection may be mild or intermittent.50 Dizziness and headache are the two most common symptoms of carbon monoxide exposure, and such exposure may be occult. Not surprisingly, the mere presence of headaches in patients with dizziness probably does not discriminate those with central pathology from those with other etiologies.12 General neurologic symptoms (ie, other than audio-vestibular dysfunction and craniocervical pain, described earlier) may be of any type, including both long tract (eg, hemiparesis, limb incoordination) and cranial nerve related
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(eg, diplopia, dysarthria). Such focal symptoms are rare in benign peripheral vestibular disorders and usually indicate a central cause, whether less urgent (eg, basilar migraine, cerebellar degeneration, multiple sclerosis, panic disorder) or more urgent (eg, TIA, stroke, brainstem encephalitis).12,13 Peripheral causes associated with neurologic symptoms are usually not benign and include infectious, infiltrative, or compressive cranial polyneuropathies (eg, herpes zoster oticus, Lyme disease, carcinomatous meningitis, base of skull metastases) as well as local infections that invade the cranial vault and disseminate (eg, otitis media complicated by bacterial labyrinthitis and meningitis). The absence of general neurologic symptoms is weak evidence, at best, in favor of a peripheral vestibular disorder, even in patients with stroke as a cause for their vestibular symptoms.13 Almost any general medical symptom might potentially be relevant to diagnosis of dizziness or vertigo (eg, dysuria as an indicator of urosepsis as the cause for dizziness in an older patient). Nevertheless, some medical symptoms are of particular interest in the evaluation of patients with vestibular presentations. These include cardiorespiratory (eg, chest pain, dyspnea, orthopnea, palpitations, syncope) or hyperadrenergic (eg, fear, tremulousness, hyperventilation, pupillary dilatation, tachycardia) symptoms. Cardiorespiratory symptoms, particularly chest pain, dyspnea, or frank syncope, accompanying dizziness or vertigo could suggest an underlying cardiac or other dangerous etiology, such as aortic dissection or pulmonary embolus.10,51,52 Similar symptoms may also occur in nonurgent cardiovascular disorders, such as reflex syncope,53 and possibly a variety of other general medical disorders also diagnosed in patients with dizziness.12 On occasion, syncope may result from basilar migraine54 or

KEY POINTS

h Cephalalgic symptoms,
whether manifest as headache, neck pain, eye pain, facial pain, or ear pain, are frequent in dangerous central disorders that cause vestibular symptoms, particularly posterior circulation stroke.

h The absence of general


neurologic symptoms is only weak evidence in favor of a peripheral vestibular disorder, since the majority of vestibular presentations caused by stroke have no associated neurologic symptoms.

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Symptoms and Signs

Case 2-1
A 25-year-old woman presented with recent episodic dizziness and headache. The episodes of dizziness occurred spontaneously once or twice a week during the prior 6 weeks and lasted 5 to 10 minutes. No associated neurologic symptoms, including diplopia, were present. The pain was located behind the left ear and had been present throughout the period since symptoms began without substantial variation. She had a personal history of migraine headaches. No history of vascular risk factors was reported. Comment. It is natural to assume that a 25-year-old woman with episodic, spontaneous dizziness and head pain, in the context of a personal history of migraine headaches, is likely to have vestibular migraine as a cause for the vestibular symptoms. In this case, however, the clue to a dangerous underlying cause is the presence of continuous head pain. In patients with migraine and auralike symptoms, both the pain and neurologic symptoms should fluctuate. Here, the patients pain does not fluctuate, suggesting a persistent structural pathology, such as vertebral artery dissection, known to mimic migraine in younger patients. The location of the pain (behind the ear) is one of the common locations for referral of the pain of vertebral dissection. This symptom pattern prompted neuroimaging, and the patient was found to have vertebral artery dissection. The vestibular symptoms were attributed to TIAs in the cerebellum. When the diagnosis was made, the patient disclosed that she had been bungee jumping shortly before the onset of her symptoms. She had not disclosed this previously when asked about any recent injuries, because she did not wish to be blamed for taking the risk and causing her own symptoms. She was treated with anticoagulants for 6 months and made a complete recovery.

vertebrobasilar TIA and stroke,55,56 although generally not in association with chest pain or dyspnea, unless perhaps the source of a TIA or stroke is cardiac or a vascular dissection in the chest.57 The absence of cardiorespiratory symptoms should not be used to exclude cardiovascular causes, since isolated dizziness or vertigo may sometimes be the sole manifestation.11,58 The presence of chest pain or pressure accompanying a primary symptom of gait unsteadiness without other vestibular symptoms should specifically prompt consideration of thoracic spinal cord compression.59 Hyperadrenergic symptoms, with or without cardiorespiratory symptoms, accompanying dizziness or vertigo often indicate panic disorder but can be difficult to distinguish from less common causes such as temporal lobe seizures,60 hypoglyce-

mia,61 and neuroendocrine malignancy, such as pheochromocytoma. Rarely, migraine62 or vertebrobasilar TIA and stroke63 may mimic panic disorder. Medical History Medical history is obviously important in assessing known causes of recurrent vestibular symptoms (eg, Me nie `re disease), conditions that may predispose to vestibular conditions (eg, migraine headaches or childhood motion sickness for vestibular migraine), or risk factors for diseases that may cause vestibular symptoms (eg, dyslipidemia or diabetes for stroke12). It is generally wise to identify major, known, concurrent illnesses (eg, multiple sclerosis, HIV/AIDS, metastatic cancer, severe malnutrition, and major depression) or potentially relevant exposures (eg, viral syndrome, ear surgery, bacterial
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otitis media, ototoxic medications [especially aminoglycoside antibiotics or chemotherapy agents], and toxins) in addition to assessing vascular risk factors.13 No element of a patients history can confidently predict whether present symptoms are causally related to the prior condition or exposure, but they can help establish a baseline prevalence (prior probability) for certain diseases. Generally speaking, less should be inferred diagnostically from common, nonspecific histories and exposures (eg, hypertension, multiple medications, viral syndrome) than infrequent, more specific ones (eg, recent ear trauma,64 high-altitude travel, exposure to sources of carbon monoxide, or severe systemic infection requiring hospitalization and intravenous antibiotics). Others in the household or workplace affected by similar symptoms should spark consideration of infectious, toxic,65 or heritable causes.66,67 VESTIBULAR SIGNS AND PHYSICAL EXAMINATION A thorough treatment of neurovestibular signs and examination techniques is beyond the scope of this article. This section emphasizes the most common and most diagnostically useful bedside signs in neuro-otologic diagnosis, focusing on techniques that assist in localization, and discusses examination for nystagmus, assessment of the vestibuloocular reflex (VOR), and additional oculomotor testing (alignment, saccades, smooth pursuits, and VOR cancellation). The general neurologic and medical examinations are briefly discussed. Disease-oriented articles that follow emphasize and reinforce the principles described here. Nystagmus Nystagmus is an involuntary, rapid, rhythmic, oscillatory eye movement
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with at least one slow phase. Jerk nystagmus has a slow phase and a fast phase (also called quick phase). Pendular nystagmus has only slow phases. The finding may be normal or pathologic, depending on the type of nystagmus and the context in which it occurs. Normal forms of nystagmus include physiologic end-gaze, optokinetic, perrotatory, and postrotatory. Pathologic forms of nystagmus are varied in etiology but generally result from diseases affecting the brainstem, cerebellum, or peripheral vestibular apparatus. Asymmetric lesions of the vestibular system, central or peripheral, cause a slowphase drift that is corrected by quick phases (vestibular nystagmus); other forms of jerk nystagmus result instead from failures of gaze-holding mechanisms. On occasion, cerebral disorders can cause nystagmus, eg, epileptic nystagmus. Nystagmus differs from saccadic oscillations (eg, ocular flutter, opsoclonus) or other fast eye movement disorders (eg, superior oblique myokymia) in that these disorders have no slow phase of movement, ie, backto-back saccades without an intersaccadic interval. Certain eye movements seen only in coma or reduced states of consciousness are given special names, eg, bobbing, dipping, and ping-pong gaze. Although classifications vary, some of these may be considered specific forms of nystagmus. Several nystagmus attributes are typically described either qualitatively at the bedside or quantitatively using oculographic equipment (Table 2-2). The pattern of these attributes ultimately determines the localizing value of the nystagmus. While dozens of nystagmus types and subtypes have been identified,35 only a handful are essential for all neurologists to be able to recognize. A provider who can confidently recognize physiologic nystagmus, nystagmus associated with cerebellar degeneration,

KEY POINT

h Less should be inferred


diagnostically from common, nonspecific histories and exposures (eg, hypertension, multiple medications, viral syndrome) than those that are infrequent and more specific (eg, recent ear trauma).

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Symptoms and Signs


KEY POINT

h A provider who can


confidently recognize physiologic nystagmus, nystagmus associated with cerebellar degeneration, vestibular neuritis, the direction-changing horizontal nystagmus of acute stroke, and posterior canal benign paroxysmal positional vertigo is already well on the way to successful diagnosis of the patient with neurovestibular symptoms.

TABLE 2-2 Nystagmus Attributes Typically Described During a Neurovestibular Examination


b Baseline Attributes Occurrence (eg, spontaneous [in primary gaze without provocation], gaze-evoked, positional) Binocularity (monocular versus binocular) Conjugacy (conjugate versus dysconjugate) Waveform (jerk or pendular, the velocity profile of the slow phase [linear, increasing, decreasing]) Intensity (amplitude and frequency) Plane or axis of rotation (in either head or eye coordinates) Direction (by convention, the direction of jerk nystagmus is named for the fast-phase direction; for torsional quick phases, it is named for whether the top pole of the eye, ie, the 12 oclock position, beats toward the right or left ear) Temporal profile of the nystagmus if it is nonsustained (eg, paroxysmal positional nystagmus of a particular duration) or spontaneously varying over time (eg, periodic alternating nystagmus) Time since onset or age of first appearance if it is persistent (eg, infantile, adult-onset) b Attributes Under Several Different Examination Conditions Under different lighting or examiner viewing conditions (eg, eyes open versus closed, light versus dark, behind Frenzel lenses, with occlusive ophthalmoscopy) In different gaze positions (ie, in all eight secondary and tertiary positions of gaze; in different relative states of convergence or divergence) Response of the nystagmus to various triggering or provocative maneuvers (eg, shaking of the head, mastoid vibration, positioning)

vestibular neuritis, the direction-changing horizontal nystagmus of acute stroke, and posterior canal BPPV is already well on the way to successful diagnosis of the patient with neurovestibular symptoms. Nystagmus may be present continuously or only intermittently. If intermittent, it may be truly episodic and triggered by particular maneuvers or only present in certain gaze positions or under particular viewing conditions (for the patient or the examiner).The first step in assessment for nystagmus is to inspect the eyes carefully in different gaze positions: primary position

(ie, looking straight ahead) plus secondary gaze positions (right, left, up, and down). Tertiary gaze positions (up right, up left, down right, down left) can also be tested, although it is unusual to find nystagmus in these positions if none is present in primary or secondary gaze positions. Physiologic end-gaze nystagmus to the right and left is generally symmetric, nonsustained (lasting just a few beats), and absent in all other gaze positions; the classic nystagmus of cerebellar degeneration looks similar but generally has greater intensity and is sustained. With
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far lateral gaze, particularly outside the binocular field of vision where the nose obscures the medially placed eyes view, a small number of healthy patients will also demonstrate sustained physiologic nystagmus; in such cases, the nystagmus will typically disappear when the eyes are brought back into the binocular field, ie, less extreme gaze. Supraphysiologic nystagmus evoked by eccentric gaze positions and absent in the primary position is often called gaze-evoked nystagmus. Typically the nystagmus beats in the direction of attempted gaze (eg, rightward in right gaze), although it may be exclusively horizontal, exclusively vertical, or even asymmetric. If it is exclusively horizontal and symmetric, pathologic gazeevoked nystagmus must be distinguished from physiologic end-gaze nystagmus; if it is present when the eyes are only partially displaced, eg, within 30- of the primary gaze position, it is invariably pathologic. On occasion, pathologic and physiologic forms are difficult to distinguish. Gaze-evoked nystagmus most often results from failure of the central gaze-holding pathways that generate (or calibrate) the extraocular muscle force required to sustain the eyes eccentrically against the normal elastic restoring forces of the orbital tissues, which bring the eyes passively back to near the midposition. If gaze-evoked nystagmus is purely vertical (upbeat or downbeat) or changes directions in different gaze positions (eg, supraphysiologic, direction-changing horizontal nystagmus), the localization is almost invariably central. The cause, if structural, is usually located in the posterior fossa (eg, cerebellar degeneration, neoplasm, or stroke), although the toxic effects of medications such as alcohol or antiepileptic medications are more common. If the nystagmus is horizontal, asymmetric, unidirectional, and elicited only by gaze in one horizontal direction
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(ie, right-beat nystagmus in right gaze or left-beat nystagmus in left gaze, but not both, and without vertical nystagmus on vertical gaze), it may either be central or peripheral and additional assessments are necessary to localize the cause. Unmasking techniques that block visual fixation, such as use of Frenzel goggles,68 occlusive ophthalmoscopy, or the penlight cover test,69 may be used to elicit nystagmus that has been sufficiently suppressed by visual fixation as to be unapparent clinically. Visual fixation reliably damps spontaneous peripheral vestibular nystagmus.70 When such nystagmus is very mild, it may be completely suppressed by normal vision.69 It is generally the case that peripheral nystagmus is more robustly suppressed than central nystagmus, although the absence of fixation suppression, suggesting a central lesion, is more helpful diagnostically than its presence. For example, many patients with stroke causing acute vestibular syndrome have nystagmus that is only apparent when fixation is blocked.71 Magnification, such as with direct ophthalmoscopy, may also be used to identify subtler, low-amplitude nystagmus. However, very subtle nystagmus, particularly that seen only with fixation blocked, is more nonspecific and may be seen under unremarkable circumstances, eg, after smoking a cigarette. Characteristics of several important vestibular nystagmus forms are described in Table 2-3.72Y79 Many maneuvers are used by specialists to trigger or provoke nystagmus in search of particular diseases. Most of these are beyond the scope of this article, but all neurologists should familiarize themselves with the Dix-Hallpike maneuver (also sometimes called the Nylen-B"r"ny maneuver, or other permutations of these eponyms). The Dix-Hallpike maneuver is a bedside test designed to provoke symptoms

KEY POINTS

h Pathologic gaze-evoked
nystagmus must be distinguished from physiologic end-gaze nystagmus. If the nystagmus is vertical, asymmetric, or present when the eyes are only partially displaced to one side, it is almost invariably pathologic.

h If the nystagmus is
horizontal, asymmetric, unidirectional, and elicited only by gaze in one horizontal direction, it may be either central or peripheral.

h Peripheral nystagmus
is more robustly suppressed than central nystagmus, although the absence of fixation suppression, suggesting a central lesion, is more helpful diagnostically than its presence.

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Symptoms and Signs

a TABLE 2-3 Nystagmus Characteristics in Key Peripheral and Central Vestibular Disorders

Vestibular Condition
Posterior canal benign paroxysmal positional vertigo (BPPV) (80%Y90% of BPPV) Horizontal canal BPPV (10% to 15% of BPPV)

Test Maneuver

Nystagmus Duration

Trajectory/ Direction

Variation in Direction

Positional Vestibular Symptoms (Episodic Nystagmus Triggered by Specific Positional Maneuvers) Head hanging with 5Y30 seconds29 Upbeat-torsionalb Direction reversal 45- turn to each side typical on arising from (torsion toward (Dix-Hallpike37) downfacing ear)29 Dix-Hallpike72 Supine roll with 30Y90 head neutral seconds29 37 c (Pagnini-McClure ) Horizontald (toward downfacing ear much more than toward up-facing ear)29 Downbeat or horizontal much more often than upbeat, torsional, or mixed74j77 Direction reversal typical during test (when switching sides or spontaneously)73

Central paroxysmal positional vertigo

Either test position 5Y60+ seconds; sometimes or midline head sustained (ie, hanging remains while head position is maintained)74

More often direction invariant; occasionally shifts trajectory or direction with head position76

Acute Vestibular Syndrome (Spontaneous Nystagmus That May Be Exacerbated Nonspecifically by Various Head Maneuvers) Vestibular neuritis or neurectomy Gaze testing (increases with gaze toward the fast phase70) Gaze testing (may increase with gaze toward either direction78) Persistent, increases when visual fixation is blocked70,e Persistent, may increase when visual fixation is blocked71,f Dominantly horizontal T small vertical or torsional component70 Dominantly horizontal much more often than vertical, torsional, or mixed15 Direction fixed during first 24Y72 hours13; occasionally switches direction during recovery phase Acutely 38% direction changing with horizontal gaze position,13 (mixed vestibular/gaze holding)

Central acute vestibular syndrome (usually stroke)

Reprinted with permission from Newman-Toker DE, The neuro-ophthalmology virtual education library.32 novel.utah.edu/NewmanToker/collection.php. b A mixed upbeat-torsional vector during the Dix-Hallpike test is a reliable predictor of posterior canal BPPV, since this pattern is highly atypical in central paroxysmal positional vertigo.74 c The conventional supine roll test for horizontal canal BPPV may also provoke dizziness in disorders triggered by lateral neck rotation (eg, carotid sinus hypersensitivity, rotational vertebral artery occlusion). Characteristic nystagmus brought on during a modified, enbloc (head and body together) supine roll test should exclude either mimic and confirm horizontal canal BPPV.79 d Horizontal nystagmus elicited by positional testing, even when transient and triggered by the supine roll test, is not a specific indicator of horizontal canal BPPV since it can occur in other peripheral vestibular disorders and those with central lesions.77 In questionable cases, response to treatment can be used as a diagnostic confirmation, since central paroxysmal positional vertigo symptoms should not improve with canalith repositioning, as would be expected for BPPV.36 e Peripheral vestibular nystagmus generally obeys Alexanders law. It increases in intensity when gaze is directed toward the fast phase and decreases in intensity when gaze is directed toward the slow phase. The nystagmus may only be apparent in lateral gaze to one side (first degree); be apparent in lateral gaze toward the fast phase and in the primary position (second degree); or be apparent in all three gaze positions (third degree). f Contrary to popular belief, spontaneous nystagmus due to acute central lesions can be suppressed by visual fixation and unmasked by fixation-blocking maneuvers.71 Unmasking of nystagmus (ie, presence of fixation suppression of the nystagmus) should only be considered a peripheral sign in acute vestibular syndrome when accompanied by a safe HINTS result.

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KEY POINTS

h The Dix-Hallpike test is


the gold standard diagnostic test for the common, posterior canal variant of benign paroxysmal positional vertigo and should be thought of as a specific test for this disorder. A different diagnostic maneuver is needed to accurately diagnose the uncommon horizontal canal variant. Diagnosing anterior canal benign paroxysmal positional vertigo without neuroimaging by MRI is usually ill advised, given how closely the signs mimic central disease.

FIGURE 2-1

DixYHallpike maneuver for diagnosis of right posterior semicircular canal benign paroxysmal positional vertigo. Step 1: Seat the patient on a table positioned so he or she may be taken back to the head-hanging position with the neck in slight extension. Stabilize the head with your hands and move the head 45- toward the side you will test. Move the head, neck, and shoulders en bloc to the head-hanging position. Step 2: Observe the eyes; hold them open if necessary.

Reprinted from Fife TD, et al, Neurology.37 B 2008, with permission from American Academy of Neurology. www.neurology.org/content/70/22/2067.full?sid=32955bc5-714f-40af-8024-8846a127a47e.

h The Dix-Hallpike test


should generally not be performed in patients with severe, acute vestibular syndrome who already have spontaneous nystagmus. An exacerbation of symptoms or signs may be misinterpreted as support for a benign paroxysmal positional vertigo diagnosis.

and signs, ie, nystagmus, in patients with positional dizziness or vertigo (Figure 2-1). The modern form of the test was first described in detail by Dix and Hallpike in 1952. With minor adjustments to technique, the Dix-Hallpike test remains the gold standard diagnostic test for the condition now known as BPPV of the posterior semicircular canal (p-BPPV).36,37 Note that the Dix-Hallpike test is best thought of as a specific diagnostic test for p-BPPV, and a different diagnostic maneuver is needed to accurately diagnose horizontal canal BPPV (Table 2-3).37 A nuanced point is that the Dix-Hallpike test, particularly if not performed with the head fully hanging, can also trigger the horizontal nystagmus of BPPV of the horizontal canal. The test may also be used in the diagnosis of central positional vertigo syndromes, in which case the nystagmus is typically persistent downbeat, and as a nonspecific provocative maneuver to elicit vestibular nystagmus. Under most circumstances, the rare
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anterior canal variant of BPPV, which also presents with positional downbeat nystagmus, should only be seriously entertained after neuroimaging by MRI has excluded a posterior fossa structural lesion. The Dix-Hallpike test should not generally be performed in patients with acute, continuous vestibular symptoms, such as vertigo, nausea, vomiting, or head motion intolerance, ie, acute vestibular syndrome. Such patients almost invariably have spontaneous nystagmus at baseline, and exacerbation of symptoms or nystagmus by the Dix-Hallpike test offers no further probative diagnostic information in most cases, although it may be mistakenly thought to.8 If the test is applied in a patient with mild or recovering vestibular neuritis with minimal or no spontaneous or gaze-evoked nystagmus, a unidirectional and nonparoxysmal, horizontal pattern of nystagmus can be elicited. A preferred method to provoke nystagmus in such patients, however, would be repetitive side-to-side head shaking.

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Symptoms and Signs


KEY POINTS

h The classic nystagmus


of posterior canal benign paroxysmal positional vertigo has a mixed vertical-torsional vector with the fast phase beating upward and toward the shoulder on the side of the affected ear. This pattern of nystagmus is highly localizing to the posterior semicircular canal and essentially excludes a central cause.

h The two principal


bedside tests of vestibuloocular reflex function are dynamic visual acuity and the horizontal head-impulse test.

The test method was recently described in an AAN practice parameter derived from an evidence-based review of canalith repositioning maneuvers37 and is reproduced in Figure 2-1. Because this is a provocative test, the normal response is no response (no symptoms, no nystagmus). A specific positive response is the development of vertigo or dizziness in conjunction with the classic p-BPPV nystagmus (p-BPPN).72 The classic p-BPPN80,81 has a mixed vertical-torsional vector with the eyes in the primary position, often with the more dominant vector being vertical in one eye and torsional in the other. The direction of the fast phase is a combined upbeat (ie, toward the brow) and ipsitorsional (ie, toward the side of the affected ear); thus, the 12 oclock pole of the eyes moves up and toward the right ear in a right p-BPPV. This pattern of triggered nystagmus is highly localizing to the posterior semicircular canal and essentially excludes a central cause. If the eyes are deviated laterally during the test, the nystagmus may appear almost purely torsional (looking toward the affected ear) or vertical (looking away from the affected ear), but the direction of the nystagmus fast phase will not change. If the test is performed in the light without Frenzel goggles68 or another method to block visual fixation, the patients brain may be able to suppress the vertical vector of the p-BPPN using vision, so the nystagmus may appear almost purely torsional. Beyond the nystagmus vector and direction are several other nystagmus features said to help discriminate between p-BPPN and central mimics that are often assessed during or on repeated trials of the Dix-Hallpike test, although central mimics can be almost indistinguishable on each of these variables. The nystagmus should (1) not begin immediately but after a short

latency (1 to 15 seconds); (2) damp after 5 to 30 seconds; (3) reverse direction, but not change vector, on arising from the Dix-Hallpike position; and (4) fatigue on repeated trials, ie, progressively disappear on successive attempts of the Dix-Hallpike test. Vestibuloocular Reflex Although there are many ways to evaluate labyrinthine function, the most direct is to assess the VOR. The VOR serves to maintain stable visual fixation while the head is moving, particularly when the head is moving too quickly for smooth visual pursuit mechanisms to keep up. Although the VOR can be tested in a number of ways, the two principal bedside tests of VOR function are dynamic visual acuity and the horizontal head-impulse test (h-HIT). Each of these tests involves moving the head rapidly and assessing whether eye movements respond appropriately. For dynamic visual acuity, the normal response is stable visual acuity as reported by the patient while the head is moved; for the h-HIT, the normal response is stable fixation on a target as witnessed by the examiner. Dynamic visual acuity testing generally involves asking the patient to read an eye chart while the examiner oscillates the head rapidly, either vertically or horizontally. Loss of three or more lines of visual acuity (head moving versus head stationary) indicates some degree of vestibular failure.82 This approach requires less examiner skill to perform and interpret than the h-HIT but also offers less diagnostic informationVit does not lateralize the lesion and is often normal in patients with unilateral or partial vestibular failure. The test is probably most effective in identifying bilateral vestibular failure83 and can be used to monitor patients for aminoglycoside vestibular toxicity.84
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In 1988, the h-HIT was described by Halmagyi and Curthoys85 as a bedside test for peripheral vestibular disease. This high-acceleration maneuver (which might be thought of as a dolls eye maneuver on steroids) proved to be capable of identifying patients with vestibular failure in a way that classic oculocephalic maneuvers could not. Moreover, the test is able to interrogate each ear separately because of a quirk of normal vestibular physiology in which excitatory responses carry more weight than inhibitory responses. Since its original description, an abnormal h-HIT has consistently been shown to correlate with ipsilateral vestibular hypofunction and, more specifically, deafferentation of the inputs from the ipsilateral horizontal semicircular canal.85Y87 Although not described further here, the h-HIT has since been adapted for use to interrogate function of the two vertical semicircular canals (anterior and posterior).87 Similar tests using a linear head motion (the head-heave and headsurge tests) have also been developed to interrogate function of the linearacceleration-detecting otolith organs (utricle and saccule). The h-HIT, as originally described, is a rapid, passive head rotation from a center to lateral (10- to 20-) position as a subject fixates at a central target, such as the examiners nose. Technique is important in conducting the h-HIT, and it is generally advisable to watch the maneuver being performed by an experienced examiner before attempting it. Instructional videos are available on the Internet (novel.utah.edu/NewmanToker/collection.php ). Since the response is linked to high acceleration rather than high velocity or amplitude of the head rotation, the technique is properly conducted with a quick flick of the examiners wrists over a very short amplitude. The examiner must warn the patient that the head will be
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rotated very rapidly, and it is generally advisable to rotate the head slowly several times before conducting the highacceleration maneuver and preparing the patient by asking him or her to relax the neck. The test must be performed passively by the examiner, rather than actively by the patient, and the direction of the impulse should be disguised, eg, by using an inconsistent pattern of directions such as left-leftleft-right-left-left-right-right-left. Failure to do so may result in a false-negative test result through the production of covert saccades, in which an anticipatory fast eye movement is unconsciously substituted for the abnormal VOR during the head rotation, making it impossible for the examiner to detect the abnormality. The normal VOR response to a rapid, passive head rotation as a subject fixates on a central target is an equal and opposite eye movement that keeps the eyes stationary in space (normal h-HIT). This is sometimes referred to as a VOR gain equal to 1.0 (ratio of head rotation to eye rotation 1:1). An abnormal response occurs when the head is rapidly rotated toward the side of a vestibular lesion. The loss of vestibular afferent input results in the inability to maintain fixation during the head rotation (gain less than 1.0), requiring a corrective gaze shift (refixation saccade) once the head stops moving in order to reacquire visual fixation on the central target (abnormal h-HIT). A largeamplitude refixation saccade indicates a very low VOR gain and substantial vestibular hypofunction. The test is abnormal with lesions affecting the labyrinth (eg, labyrinthitis or aminoglycoside ototoxicity), vestibular nerve (eg, vestibular neuritis or surgical section), and lateral pons (eg, anterior inferior cerebellar artery stroke or multiple sclerosis plaque). Somewhat paradoxically, a normal response points to a central lesion

KEY POINTS

h An abnormal
head-impulse vestibuloocular reflex results from deafferentation of the inputs from the ipsilateral horizontal semicircular canal, which is most often the result of peripheral vestibular pathology, although lateral pontine lesions may produce the same sign.

h The head-impulse test


must be performed passively by the examiner, rather than actively by the patient, and the direction of the impulse should be disguised. Failure to do so may result in a false-negative test.

h A normal head-impulse
vestibuloocular reflex response usually points to a dangerous central lesion (typically stroke) in patients presenting with acute vestibular syndrome. Note that it is counterintuitive that the normal finding is considered a bad sign in this clinical context.

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Symptoms and Signs


KEY POINT

h Using a centripetal head


rotation for the head-impulse test reduces any theoretical risk of vertebral artery injury with neck overrotation by an overzealous, inexperienced examiner.

(usually stroke) in patients presenting with acute vestibular syndrome because most patients with peripheral causes of acute vestibular syndrome have an abnormal response, but most with central causes do not (Case 2-2).13,15,71 A common adaptation of the h-HIT is to displace the head laterally first, then rotate the head back to the center position. Some examiners find the maneuver easier to conduct using this centripetal head motion, and, more importantly, the results are easier to interpret, especially in the presence of spontaneous nystagmus. Asymmetric nystagmus often varies in intensity with horizontal gaze position. With the centripetal rotation, the globes end in the

primary position in the orbit regardless of the h-HIT direction of rotation (rather than a laterally displaced position either right or left of the midline). Because the end position is the same on the right and left, the impact of any nystagmus on interpreting the h-HIT result is equivalent regardless of the side tested. Using a centripetal head rotation also reduces any theoretical risk of vertebral artery injury with neck overrotation by an overzealous, inexperienced examiner. For the test to be effective, the patient must be able to fixate steadily on a visual target and the extraocular muscles should be working normally. If the patient is visually impaired (eg, blind,

Case 2-2
A 45-year-old man presented to the emergency department with 24 hours of new continuous vertigo, nausea, vomiting, and unsteady gait. He preferred to lie motionless. He denied auditory or neurologic symptoms, headache, neck pain, or recent trauma. He had no relevant medical or exposure history, including no cerebrovascular or vestibular disorders, no recent or remote ear surgery, and no smoking or other vascular risk factors. He took no medications. He had no family history of vestibular disease or recurrent dizziness. The patients general neurologic examination was normal, including no limb ataxia or dysmetria, compatible with a peripheral cause. He felt unsteady when standing but was able to sit with arms crossed unaided. Eye examination revealed direction-fixed, left-beat, horizontal nystagmus that was worse in left gaze and no skew deviation on alternate cover testing. The h-HIT was normal bilaterally. MRI with diffusion-weighted imaging revealed a large acute left posterior inferior cerebellar artery infarction. A complete search for cerebrovascular risk factors identified a left vertebral artery dissection as the underlying cause of stroke. The patient was observed in the hospital while antithrombotic agents were initiated and was discharged uneventfully after the critical 3-day window had passed, during which complications related to swelling were most likely. He made a complete neurologic recovery. Comment. This patient presented with the acute vestibular syndrome (continuous vertigo or dizziness lasting more than 1 day, accompanied by nausea or vomiting, head-motion intolerance, nystagmus, and unstable gait). The most likely etiology is vestibular neuritis, but dangerous causes such as cerebellar stroke must be considered. The absence of vascular risk factors and his age younger than 50 reduce the risk of vertebrobasilar atherosclerosis, but vertebral artery dissection remains a concern, mitigated incompletely by the absence of craniocervical pain. No auditory symptoms were present to raise concerns about inner ear ischemia, all compatible with a leading potential diagnosis of vestibular neuritis. The presence of a normal general neurologic examination is compatible with vestibular neuritis but does not exclude the possibility of a cerebellar infarction, since roughly half of strokes causing this clinical presentation produce no neurologic signs. His direction-fixed, predominantly horizontal nystagmus and absence of skew deviation are also compatible with the benign diagnosis, but also do not exclude possible stroke since more than half of such patients have these benign oculomotor signs. The normal head-impulse result in the setting of acute vestibular syndrome, however, strongly favors a stroke, despite the lack of other symptoms, signs, or risk factors.

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severe refractive error without glasses or contact lenses), cognitively impaired (eg, comatose, inattentive), or otherwise unable to consistently follow directions (eg, 2 years old or with a language barrier and no interpreter), the test cannot be performed. Even awake, alert, and attentive patients must often be reminded to maintain visual fixation during the testing; intermittent inattentiveness can result in extraneous refixation saccades that can be misinterpreted, ie, false-positives. If the patient has extraocular muscle weakness, such as sixth nerve palsy, internuclear ophthalmoplegia, or prior oculomotor surgery, h-HIT VOR test results should be interpreted with caution. Additional Oculomotor Testing In addition to a search for nystagmus and VOR testing, several other oculomotor tests are typically performed in the assessment of patients with vestibular symptoms. A search for ocular misalignment by alternate cover testing is often warranted, particularly in pursuit of skew deviation, ie, vertical ocular misalignment of vestibular cause. Although skew deviation can result from peripheral vestibular disease, it is most often a central vestibular sign, particularly in patients with acute vestibular syndrome.15 If saccades (ie, fast eye movements) are abnormal in patients with vestibular symptoms, the cause is usually central rather than peripheral. The classic finding is saccadic dysmetria (ie, hypometric or hypermetric saccades), which usually results from lesions of the cerebellum or its connections in the brainstem. Smooth pursuit eye movements may be choppy rather than smooth, often known as saccadic breakdown of smooth pursuit. This finding is somewhat nonspecific, but, when combined with directionchanging, gaze-evoked nystagmus, verContinuum Lifelong Learning Neurol 2012;18(5):10161040

tical nystagmus, or failure of VOR cancellation, it typically indicates cerebellar or brainstem disease.35 The VOR is essential for maintaining stable vision on a target when the head is moving, but the brain requires a mechanism for suppressing the VOR during combined eye-head tracking, as in visually pursuing a target in the distance moving slowly from right to left, where the head and eyes rotate together, rather than in opposition. VOR cancellation is managed by the vestibulocerebellum and, in particular, the flocculus and paraflocculus. However, the task is accomplished by a distributed network of neurons that likely include contributions from the parietal and frontal eye fields, as well as the dorsal pontine nuclei. Although many bedside tests of cerebellar function are available, only three are typically abnormal in diseases predominantly affecting the flocculus and paraflocculus (eg, Chiari I malformation): (1) eccentric gaze holding; (2) visual smooth pursuit tracking; and (3) VOR cancellation. The VOR cancellation test, also known as the VOR suppression test, involves a combined eye-head tracking task. It is typically accomplished as follows: (1) While seated, the patient is asked to extend the arms directly in front of the body and clasp the hands together with thumbs abducted and pointed toward the ceiling. (2) The patient is then asked to visually fixate on the thumbs and maintain that fixation. (3) The patients entire body is then rotated en bloc from side to side. The rotation should cover an angle sweep of roughly 30- to either side of the starting position with each 60- pass lasting approximately 1.5 to 6 seconds (rotational velocity 10- to 40- per second). Several passes of increasing velocity should be examined to establish the range within which the VOR cancelling system is able to compensate.

KEY POINT

h Although skew
deviation can result from peripheral vestibular disease, it is most often a central vestibular sign, particularly in patients with acute vestibular syndrome.

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Symptoms and Signs


KEY POINT

h Long tract or cranial


nerve signs accompanying vestibular symptoms indicate a central cause. Many patients with central causes, however, including dangerous ones such as stroke, have no such signs.

This whole-body rotation is most easily accomplished using a swivel chair, although it can also be approximated by asking the patient to twist at the waist from a stationary seat. Particularly in the latter case, the patient must be instructed to keep the head, neck, and shoulders moving together as a unit and the thumbs straight in front of the body and pointed toward the ceiling. Some physical instruction and assistance from the examiner are often required. The test can be adapted for use in interrogating cancellation of the vertical VOR by using the same fixation target (outstretched thumbs) and assisting the patient through several cycles of trunk flexion and extension. The normal response to the VOR cancellation test is that the eyes remain stationary and motionless in the primary position of gaze (midposition within the orbits) throughout the middle of each side-to-side pass. It is common for normal subjects to slip off the target and demonstrate a few jerks of what looks like nystagmus at the turns, ie, during peak deceleration and acceleration. Patients with an abnormal response will develop vestibular nystagmus during the middle of each rotational pass (fixed-velocity segment). This will occur at lower velocities and be more pronounced in cases with more severe failure of cancellation. For the test be effective, the patient must be able to fixate steadily on a visual target (as with the tests of VOR function discussed earlier) and should not have baseline primary position nystagmus. Furthermore, the test is not meaningful in patients who have completely lost VOR function (eg, aminoglycoside toxicity), since there is nothing to suppress. Additional Neurologic and General Examinations Several aspects of the neurologic and general examinations are typically rele-

vant in patients with vestibular symptoms. As with general neurologic symptoms, neurologic signs other than audiovestibular dysfunction and gait imbalance are rare in benign peripheral vestibular disorders. Long tract or cranial nerve signs almost always indicate a central cause, whether less urgent, such as multiple sclerosis, or more urgent, such as stroke and brainstem encephalitis.12,13 Many patients with central causes, however, including dangerous ones such as stroke, have no such signs.13 Almost any general examination finding might prove to be diagnostic, eg, a tender, swollen calf suggesting deep vein thrombosis leading to pulmonary embolism as a cause for dizziness, but some findings should be sought routinely in patients with vestibular symptoms. Aspects of the general examination that are of particular relevance in those with vertigo or dizziness include the following: (1) measurement of heart rate and blood pressure, particularly orthostatic vital signs in patients with symptoms on arising or comparison of blood pressures in the two arms in cases of suspected subclavian steal88 or other upper thoracic vascular pathology; (2) cardiac auscultation for significant valvular murmurs; and (3) inspection of the ear (pinna, mastoid) and otic fundus by otoscopy for signs of trauma, infection, or neoplasm. Syndrome-Specific Approaches to Examination In aggregate, a focused examination approach is usually warranted based on the vestibular syndrome in question. Patients with spontaneous, episodic symptoms usually have no obvious signs, but those with episodic positional, acute prolonged, or chronic persistent symptoms may have examination clues to the underlying localization or diagnosis. For positional vestibular symptoms,
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With Acute Dizziness: History, TABLE 2-4 Safe-to-Go Steps for Bedside Evaluation of Patients Review of Systems, and Physical Examinationa
Step 1 History: Plenty of Protective Ps If symptoms are old and recurrent: Periodic and Prolonged: recurrent, stereotyped episodes or bouts over a protracted period (longer than 2 to 4 years); current episode is typical in all respects If symptoms are more recent: Painless: head or neck pain, if present, should sound like migraine or tension-type headaches, and must not be any of the following: Sudden (peak intensity G30 minutes) Severe (unusually painful) Sustained (duration 972 hours) Plus, if there is vomiting: Proportional Puking: vertigo worse than vomiting might be OK; vomiting worse than vertigo is bad Plus, if there is loss of consciousness: Prototypical Passing out: classic vasovagal syncope (with typical provocation and prodrome) is OK; anything else is probably bad Step 2 Review of Systems: Dearth of Deadly Dsb No vascular brainstem symptoms: Diplopia (double vision) Dysarthria (trouble speaking) Dysphagia (trouble swallowing) Dysphonia (hoarseness/hiccups) Dysmetria (clumsiness) Dysesthesia (facial numbness) Drop attacks (sudden falls without loss of consciousness)c Down-is-up distortions (room tilt and room-inverted illusions)d No vascular inner ear symptoms: Deafness (any transient or bilateral hearing loss is bad; abrupt-onset unilateral loss may also be bad, but could be benign) No cardiovascular symptoms: Dyspnea (any cardiorespiratory symptoms, unless clearly related to vasovagal or panic are bad) Step 3 Physical Examination: Choose either Was Dizzy or Still Dizzy Examination Was Dizzy: If symptoms are intermittent or gone, look for benign paroxysmal positional vertigo (BPPV), orthostasis, or normal examination and classic history P-Power to send patient packing: Position-provoked with Positive Pike (upbeatVtorsional nystagmus on Dix-Hallpike) OR Postural with Predictable Pressure Plunge (symptomatic orthostatic blood pressure drop on arising) OR nie ` re disease, or panic) Pristine examination and Paradigmatic Presentation (BPPV,e vasovagal, migraine, Me Still Dizzy: If symptoms persist, confirm acute peripheral vestibulopathy by excluding brainstem, cerebellar, and middle ear signs

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Symptoms and Signs

TABLE 2-4 Continued


IF SAFE AND CLEAR THEN ILL SEND HIM ON HOMEf Intact Fields (no visual field cut) Stands Alone (able to stand unassisted) Face Even (no weakness, droop, or ptosis) CLear Enunciation (no slurred or hoarse speech) Accurate Reaching (no drift; normal reaching, rapid alternating movements) THErmal Normal (equal thermal or sharp sense) Isocoria in Low Light (equal pupils in dim light)g Straight Eyes (normal ocular alignment, especially vertical)h No Deafness (no moderate to severe hearing loss) Head Impulse Misses (abnormal h-HIT)i One-way Nystagmus (unidirectional, horizontal)j Healthy Otic and Mastoid Examination (pearly; no pimples, pus, perforation, or pain on palpation of mastoid)
a

Modified with permission from Newman-Toker DE, The neuro-ophthalmology virtual education library.32 novel.utah.edu/NewmanToker/collection.php. b Deadly Ds should be excluded both by review of systems and corresponding examination, eg, diplopia and ocular misalignment. c nie ` re disease and occasionally seen in patients with other inner ear diseases.89 Drop attacks are common in patients with Me nie ` re disease history, they should spark concern for an underlying cerebrovascular or However, in the absence of a clear Me cardiovascular90 etiology for dizziness/vertigo. Although drop attacks are also seen in epilepsy and narcolepsy-cataplexy, in such cases, they are generally not associated with dizziness. d nie ` re and other inner ear diseases, but, in the absence of a convincing Room tilt illusions have been reported in patients with Me history pointing to otic involvement (eg, fluctuating tinnitus with recurrent attacks, symptoms provoked by noise, progressive lowfrequency hearing loss), a cerebrovascular cause should be surmised. e Since BPPV may resolve on its own, a typical history alone is sometimes considered sufficient for diagnosis. However, care should be taken to ensure that episodes were reliably triggered by adopting the head positions, rather than exacerbated by head movement after a spontaneous, episodic onset of symptoms (more typical of TIA). f The IF SAFE & CLEAR THEN ILL SEND HIM ON HOME acronym can be abridged to SEND HIM ON HOME by mandating that a thorough general neurologic examination be normal. SEND HIM ON HOME is HINTS plus normal hearing and a normal ear examination. g Pupils must be examined in dim light, since the anisocoria of Horner syndrome (suggesting brainstem pathology in this clinical context) is usually only apparent in dim light or darkness. Note that even in low light, the amount of anisocoria is typically less than 2 mm, and any associated ptosis or anhidrosis is also subtle. h Clinically evident skew deviation (vertical misalignment of vestibular cause) has been reported in occasional patients with peripheral vestibular lesions, but, in the context of acute vestibular syndrome, skew is a strong predictor of stroke.13 Unfortunately, the converse is not true, since most strokes presenting with acute vestibular syndrome have normal vertical ocular alignment clinically.13 Thus, normal vertical ocular alignment is necessary but not sufficient for a safe-to-go departure. i An abnormal h-HIT is seen in most cases of vestibular neuritis but also in some stroke patients (particularly those with lateral pontine involvement). Therefore, in patients with acute vestibular syndrome, the abnormal impulse is necessary but not sufficient for a safe-to-go departure. j In acute vestibular syndrome, peripheral nystagmus should be predominantly horizontal and direction-fixed, regardless of gaze position. Direction-changing, gaze-evoked nystagmus is highly suggestive of brainstem or cerebellar stroke.13 Unfortunately, the converse is not true, since most strokes presenting with acute vestibular syndrome have unidirectional nystagmus mimicking vestibular neuritis.15,41,71,91Y93 So unidirectional nystagmus is necessary but not sufficient for a safe-to-go departure.

provocative positional testing to elicit nystagmus (eg, Dix-Hallpike) and orthostatic cardiovascular assessment are warranted. For acute vestibular syndrome, VOR assessment by h-HIT, a search for direction-changing nystagmus, and alternate cover testing for skew deviation are the signs most likely to discriminate central from peripheral causes.13 This three-test oculomotor battery has been given the acronym HINTS (head

impulse, nystagmus, test of skew) with the findings predicting stroke given the acronym INFARCT (impulse normal, fast-phase alternating, refixation on cover test).15 For chronic persistent vestibular symptoms, VOR testing by h-HIT or dynamic visual acuity combined with assessments for cerebellar-type oculomotor abnormalities, such as gazeevoked horizontal nystagmus, vertical nystagmus, impaired smooth pursuit,
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VOR cancellation failure, and saccadic dysmetria, is likely to be the most effective strategy. CONCLUSIONS A careful history and physical examination will generally permit accurate diagnosis of patients presenting with vestibular symptoms. Patients with conditions producing vestibular symptoms lasting seconds to hours are rarely symptomatic at the time of initial assessment. If they are still symptomatic, it is generally with intermittent symptoms triggered by certain actions, such as head movement and standing up quickly. By contrast, patients with conditions producing vestibular symptoms lasting for days to weeks are usually symptomatic at the time of initial assessment. This clinical distinction is crucial, since the expected bedside examination findings differ dramatically between the two groups. In the former group, with transient or intermittent symptoms, the physician should use physical examination maneuvers to provoke symptoms and signs but should not be surprised to find a completely normal examinationVhere, often the history offers the only clue to differentiate between common, benign causes and their dangerous mimics. In the latter group, with persistent and continuous symptoms, the physician should expect the physical examination findings to distinguish between benign causes and dangerous causes and should be surprised if they do not. More importantly, distinguishing the were dizzy from the still dizzy patients allows the examiner to rapidly and confidently determine who is at risk of serious underlying disease. Table 2-4 89Y93 shows a composite summary of the critical history and examination techniques necessary to determine a benign cause under these two crucial scenarios.
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REFERENCES
1. Bisdorff A, Von Brevern M, Lempert T, Newman-Toker DE. Classification of vestibular symptoms: towards an international classification of vestibular disorders. J Vestib Res 2009;19(1Y2):1Y13. 2. Task Force for the Diagnosis and Management of Syncope, European Society of Cardiology (ESC), European Heart Rhythm Association (EHRA), et al. Guidelines for the diagnosis and management of syncope (version 2009). Eur Heart J 2009;30(21):2631Y2671. 3. Newman-Toker DE, Hsieh YH, Camargo CA Jr, et al. Spectrum of dizziness visits to US emergency departments: cross-sectional analysis from a nationally representative sample. Mayo Clin Proc 2008;83(7):765Y775. 4. Newman-Toker DE. ProQuest: Dissertations & Theses. Diagnosing dizziness in the emergency department: why What do you mean by dizzy? should not be the first question you ask, gateway.proquest.com/openurl?url_ ver=Z39.88-2004&res_dat=xri:pqdiss&rft_val_ fmt=info:ofi/fmt:kev:mtx:dissertation&rft_ dat=xri:pqdiss:3267879. Published 2007. Accessed April 18, 2012. 5. Drachman DA. A 69-year-old man with chronic dizziness. JAMA 1998;280(24):2111Y2118. 6. Drachman DA, Hart CW. An approach to the dizzy patient. Neurology 1972;22(4): 323Y334. 7. Newman-Toker DE. Charted records of dizzy patients suggest emergency physicians emphasize symptom quality in diagnostic assessment. Ann Emerg Med 2007;50(2): 204Y205. 8. Stanton VA, Hsieh YH, Camargo CA Jr, et al. Overreliance on symptom quality in diagnosing dizziness: results of a multicenter survey of emergency physicians. Mayo Clin Proc 2007;82(11):1319Y1328. 9. Newman-Toker DE, Cannon LM, Stofferahn ME, et al. Imprecision in patient reports of dizziness symptom quality: a cross-sectional study conducted in an acute care setting. Mayo Clin Proc 2007;82(11):1329Y1340. 10. Newman-Toker DE, Camargo CA Jr. Cardiogenic vertigoVtrue vertigo as the presenting manifestation of primary cardiac disease. Nat Clin Pract Neurol 2006;2(3): 167Y172; quiz 173. 11. Newman-Toker DE, Dy FJ, Stanton VA, et al. How often is dizziness from primary cardiovascular disease true vertigo? A systematic review. J Gen Intern Med 2008; 23(12):2087Y2094. 12. Cheung CS, Mak PS, Manley KV, et al. Predictors of important neurological causes

KEY POINTS

h A safe (benign) HINTS


result in acute vestibular syndrome is a unilaterally abnormal horizontal head-impulse test plus direction-fixed, predominantly horizontal nystagmus plus normal vertical ocular alignment on alternate cover test. The nystagmus must beat away from the side of the abnormal impulse and should not change direction in different gaze positions or during provocative testing (eg, positional maneuvers or head shaking).

h For spontaneous, episodic


vestibular symptoms, emphasize the history over the examination. For positional symptoms, use the Dix-Hallpike maneuver and orthostatic vital signs. For acute vestibular syndrome, test for HINTS to INFARCT. For chronic, persistent vestibular symptoms, focus on cerebellar eye signs.

h With transient or
intermittent symptoms, the physician should use physical examination maneuvers to provoke symptoms and signs but should not be surprised to find a completely normal examination. With persistent and continuous symptoms, the physician should expect that the physical examination findings will usually distinguish between benign causes and dangerous causes and be surprised if they do not.

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Symptoms and Signs

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