Vestibular migraine

INTRODUCTION — Vestibular migraine is a syndrome characterized by recurrent episodes of vertigo or other vestibular symptoms attributed to migraine.

This topic will review the clinical features, diagnosis, and management of vestibular migraine. Other aspects of migraine are discussed separately.

●(See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults".)

●(See "Acute treatment of migraine in adults".)

●(See "Preventive treatment of episodic migraine in adults".)

●(See "Acute treatment of migraine in children".)

●(See "Preventive treatment of migraine in children".)

Migraine with brainstem aura is a related condition featuring a complex aura that may include vertigo along with other brainstem symptoms and is discussed separately. (See "Migraine with brainstem aura".)

Other causes of vertigo are discussed elsewhere. (See "Causes of vertigo".)

TERMINOLOGY — Vestibular migraine was previously identified by other terms including migraine-associated vertigo, migraine-associated dizziness, migraine-related vestibulopathy, and migrainous vertigo [1,2].

Childhood precursors to migraine and vestibular migraine in adulthood include vestibular migraine of childhood, benign paroxysmal vertigo of childhood, and recurrent vertigo of childhood [2-5]. (See "Types of migraine and related syndromes in children", section on 'Recurrent vestibular symptoms'.)

Vestibular migraine is considered to be distinct from migraine with brainstem aura, previously known as basilar migraine [2,6,7]. (See "Migraine with brainstem aura".)

PATHOPHYSIOLOGY AND DISEASE ASSOCIATIONS — The pathophysiology of vestibular migraine is incompletely understood. The wide spectrum of clinical and laboratory features of vestibular dysfunction that overlap with migraine and other vestibular disorders suggests a heterogeneous pathophysiology [8-10]. Several factors have hindered the study of vestibular migraine including the lack of biologic markers or specific tests, lack of standard nomenclature, and evolving consensus diagnostic criteria. Several potential mechanisms are proposed; these are not mutually exclusive.

Vestibular dysfunction and migraine — An association between episodic vertigo and migraine was noted as early as 1873 [11]. A contemporary association between vertigo and migraine has been noted by observational studies reporting a high prevalence of vestibular symptoms in patients with migraine and other studies identifying a high prevalence of migraine in patients with episodic vestibular symptoms.

Among patients with migraine, a history of episodic vertigo is reported by 20 to 33 percent [8,12-15]. This contrasts with studies reporting the prevalence of vertigo for patients with tension-type headaches and the lifetime prevalence of vertigo in the general population, both estimated at generally less than 8 percent [8,16,17]. One-half to two-thirds of migraineurs also report motion sickness [8,12,18-21]. This prevalence is probably two to five times greater than in the general population. Patients with migraine also appear more susceptible to visually induced motion sickness and have more severe symptoms [22,23]. (See "Motion sickness".)

Nonlocalizing vestibular signs and signs suggestive of central vestibular dysfunction are common but also quite variable among studies of patients with migraine. Mild interictal vestibular and oculomotor signs (spontaneous, positional, or gaze-evoked nystagmus; abnormal vestibulo-ocular reflex; impaired ocular pursuit) have been reported in 20 to 66 percent of patients with migraine [6,12,24-28].

Among patients with episodic vertigo, the prevalence of migraine has been estimated between 38 and 87 percent [29-31]. In one study, family members of patients with vertigo also had a higher-than-expected prevalence of migraine [32].

Pathogenesis — Several hypotheses have been proposed that describe the onset of vestibular migraine symptoms as a component of episodic migraine.

●Precursor to migraine – Vestibular migraine may be a precursor to more typical forms of migraine in adults, similar to the relationship between benign paroxysmal vertigo of childhood and migraine in the pediatric population [33-35]. Long-term follow-up of children with benign paroxysmal vertigo suggests that most will go on to develop migraine later in life [35]. (See "Types of migraine and related syndromes in children", section on 'Benign paroxysmal vertigo of childhood'.)

●Migraine aura – For some patients, vestibular migraine may represent a typical migraine aura. Patients may report transient vestibular symptoms, sometimes accompanied by visual or other neurologic symptoms and followed by onset of a migraine headache. However, this sequence occurs only in a minority of patients. In case series, only 2 to 30 percent of patients with vestibular migraine had vestibular symptoms meeting this description [6,7,29,36,37].

●Migraine trigger – Episodic vertigo may act as a migraine trigger. Evidence for this is limited but includes a report of three individuals who had migraine attacks shortly after caloric testing [38]. In another observational study based in a neurotology clinic, nearly half of migraine patients who underwent vestibular testing experienced a migraine within 24 hours of testing, compared with 12 percent of patients without a migraine history and 5 percent among those who did not receive vestibular testing [39].

Migrainous mechanisms — Pathophysiologic processes that account for migraine include vestibulocerebellar pathways that may be preferentially implicated in patients with vestibular migraine.

●Trigeminovascular system – Trigeminal ganglion excitation and release of vasoactive neuropeptides such as substance P, pituitary adenylate-cyclase-activating polypeptide, and calcitonin gene-related peptide (CGRP) are thought to contribute to the headache pain of migraine. Vestibular symptoms may also be attributed to trigeminovascular activation [37]. The presence of receptors to vasoactive neuropeptides and heightened immunoreactivity have been shown to alter function in vestibular nuclei [40,41]. Activation of central vestibular nuclei can also alter monoaminergic activation that in turn modulates migrainous symptoms and pain pathways [25]. In addition, transcutaneous electrical stimulation of the trigeminal nerve has been shown to produce nystagmus in patients with migraine [42]. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Trigeminovascular system'.)

●Spreading depression – Cortical spreading depression describes a wave of neuronal and glial depolarizations that may account for the migraine aura by activating trigeminal afferent pathways. Vestibular migraine has been hypothesized to be a "brainstem aura" in the vestibular nuclei due either to noncortical spreading depression or to cortical spreading depression via direct projections from the posterior parietal cortex [6,25]. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Cortical spreading depression'.)

●Sensory sensitivity – Neuronal sensitization involves a progressive increased responsiveness to peripheral stimulation leading to migraine by a reduction of response thresholds, increased magnitude of response, and spontaneous neuronal activity. This process may account for the sensitivities many patients with migraine report including motion, lights, sound, tactile stimuli, and smells. In addition, these patients may be more likely to exhibit motion sickness or other subclinical vestibulocerebellar abnormalities [23,25,43-46]. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Sensitization'.)

Some experts believe vestibular migraine is related to both central sensitization and aberrant sensory integration in vestibulo-thalamo-cortical processing because multisensory integration occurs at the level of the thalamus and the cortex [47]. Functional imaging studies have shown altered functional connectivity between the thalamus and brain regions involved in visual and vestibular processing and pain [48]. In one study, cortical structural abnormalities and resting state functional connectivity abnormalities were found in areas of the temporal and parietal lobes, areas that participate in multisensory integration [49].

●Channelopathies – Vestibular symptoms may arise from an inherited channelopathy for some patients with migraine [18]. One channelopathy, episodic ataxia type 2 (EA2), manifests clinically with episodic vertigo. Half of patients with this disorder have migraine [50,51]. Another disorder, familial hemiplegic migraine (FHM), is associated with a genetic abnormality of the same calcium channel [52]. Both of these disorders have been linked to a genetic variant on chromosome 19p. (See "Overview of the hereditary ataxias", section on 'Episodic ataxias' and "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Familial hemiplegic migraine'.)

Kindreds with apparently dominantly inherited syndromes that include both migraine and episodic vertigo have also been described [53-55]. While a similar ion channel genetic defect is suspected, a gene locus in these families is not yet identified; linkage analysis ruled out a linkage to the 19p locus associated with EA2 and FHM.

Other sources of vestibular dysfunction — Nonmigrainous mechanisms have also been proposed to account for symptoms of vestibular migraine.

●Ischemic damage – Migraine-induced ischemia of the inner ear may lead to cochlear and labyrinth injury, altering the excitability of these structures [56,57]. Migraine appears to be a risk factor for ischemic stroke, perhaps related to vasospasm. However, given the duration and recurrence of these symptoms and the absence of evidence of ischemia, vestibulocochlear ischemia is an unlikely explanation for most cases of vestibular migraine.

●Endolymphatic hydrops – Endolymphatic hydrops, a distention of the membranous, endolymph-containing portions of the labyrinthine system, is the pathologic lesion underlying Meniere disease. Some experts suggest that vestibular migraine and Meniere disease are associated, while others suggest that the overlap in presentation may lead to misdiagnosis [58,59]. (See 'Differential diagnosis' below and "Meniere disease: Evaluation, diagnosis, and management".)

The attacks of vertigo that characterize Meniere disease and migraine are frequently similar in duration. One study observed that attacks of vertigo in patients with Meniere disease have migrainous features (headache, photophobia, or visual aura) in 45 percent of patients [56]. This study also reported that patients with Meniere disease had an increased lifetime prevalence of migraine compared with controls (56 versus 25 percent). In another study, approximately one-quarter of patients with either condition met criteria for both diagnoses [58].

A channelopathy causing migraine could also cause an osmotic disequilibrium in the inner ear, resulting in endolymphatic hydrops [56]. Alternatively, migraine-associated ischemia could damage the labyrinth and produce endolymphatic hydrops on that basis [57].

EPIDEMIOLOGY — Vestibular migraine is considered to be a common vestibular disorder, but its prevalence is not known. A population-based study in Germany estimated a lifetime prevalence of vestibular migraine of approximately 1 percent [36], while a survey in the United States found a one-year prevalence of 2.7 percent [60]. Other epidemiology studies have used different diagnostic criteria but reported similar results [24,61]. However, epidemiologic studies may be limited by several factors including case misattribution, evolving diagnostic criteria, and referral biases [59]. Vestibular migraine may account for up to 30 percent of patients seeking treatment for episodic dizziness [62]. While two-thirds of patients identified as having vestibular migraine had consulted a clinician regarding this problem, only 20 percent were given that diagnosis [60].

Vestibular migraine is more common among females (60 to 83 percent) than males [6,12,29,36,37,63]. This sex ratio is similar to that reported in migraine. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Epidemiology'.)

Vestibular migraine may be diagnosed more frequently in children than adults [25]. In adult case series, the mean age of onset of vertigo in migraine is approximately 40 years old [6,12,36]. However, in one series, a first attack occurred in a patient who was 72 years old [6].

CLINICAL FEATURES — Vestibular migraine is characterized by recurrent episodes of transient vertigo. Most patients also have migraine-type headaches.

A history of migraine headaches (without vestibular symptoms) typically precedes the development of vestibular migraine, often by several years [64]. However, in some patients, episodes of vertigo precede onset of migraine headaches, such as those with paroxysmal vertigo of childhood who later develop migraine headaches with features consistent with vestibular migraine. Sometimes headache episodes are replaced by vertigo episodes in later life, such as around menopause.

Evolving diagnostic criteria that require migrainous history or features may also influence understanding of these clinical presentations [29,36,65]. (See 'Diagnostic criteria' below.)

Clinical symptoms

Episodic vertigo — The vestibular symptoms that characterize episodes of vestibular migraine can be perceived as internal vertigo (a false sensation of self-motion) or external vertigo (a false sensation that the visual surround is spinning or flowing). The character of the vertigo is often rotational but can also be rocking, swaying, tilting, falling, or floating [6,66].

Other commonly described vestibular symptoms include unsteadiness, visually induced vertigo (provoked by complex or large moving visual scenes, such as traffic or moving trains), head motion intolerance (a sense of motion and nausea provoked or aggravated by head movement), and nonvertiginous "dizzy" symptoms such as lightheadedness [12,67-69].

●Duration – The duration of an episode of vestibular migraine is variable but typically last minutes to several hours. However, symptoms may last only a few minutes for some patients (<15 percent) and more than 24 hours for others (up to 25 percent) [2,6,12,25,29,36,37,63,67,70].

Following the resolution of an acute episode, most patients have motion intolerance and unsteadiness for a day or more. It is rare for symptoms to persist for more than 72 hours.

●Frequency – The typical frequency of attacks varies from daily to one or two each month [6,12,37,71]. However, episodes may occur several times each day or on a near constant basis for some patients [12].

●Triggers – Vestibular migraine episodes are often spontaneous. However, they may also be triggered by environmental stimuli similar to patients with other forms of migraine [24,72]. These triggers include [68,70,73,74] (see "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Precipitating and exacerbating factors'):

•Sleep deprivation

•Stress

•Certain foods

•Visual stimuli (eg, fluorescent lights, moving trains, crowds, busy visual patterns)

•Change of head position

Symptoms that are triggered by specific head positions typically persist as long as the precipitating head position is maintained or until the episode resolves, in contrast to patients with benign paroxysmal positional vertigo who typically report improvement of symptoms after head movement ceases. (See 'Differential diagnosis' below.)

Migraine headache — While vestibular migraine episodes may occur either with or without headache, migrainous headaches are associated with at least some of the vestibular episodes in 50 to 94 percent of patients [12,29,36,63,66,70,71,73]. However, it is uncommon for patients with vestibular migraine to report a consistent temporal relationship between the vertigo and headache [12,37,75]. The vertigo may occur prior to or during a migraine headache. Few patients experience vertigo consistently as a typical aura immediately prior to headache onset [7,36].

Other ictal features — Additional migrainous symptoms other than headache (photophobia, phonophobia, visual aura) often occur during the episodes of vertigo [12,29,36,70]. Sensitivity to sound, or phonophobia, occurs in two-thirds of patients [18]. However, visual and other auras are uncommon [7,36].

Auditory symptoms such as bilateral or unilateral tinnitus, aural fullness, or subjective hearing impairment are relatively common during vestibular migraine attacks. However, transient auditory symptoms are typically mild, unlike some patients with Meniere disease with more severe or persisting symptoms [8,12,15,18,36,58,71]. (See 'Differential diagnosis' below.)

Nausea and vomiting are frequent, but nonspecific, and may be attributed to vertigo of any cause. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Clinical features'.)

Interictal symptoms — Although vestibular migraine is considered an episodic vestibular disorder, many patients report a high prevalence of mild vestibular symptoms between attacks. There is controversy surrounding whether these interictal vestibular symptoms should be considered chronic vestibular migraine or a comorbid vestibular disorder such as persistent postural-perceptual dizziness (PPPD) [7,66,76,77]. (See 'Differential diagnosis' below.)

Patients with vestibular migraine frequently report visually induced and head motion-induced dizziness even when such stimuli do not trigger a full episode of vestibular migraine [78]. A susceptibility to motion sickness may be more prominent in patients with vestibular migraine rather than other forms of migraine [21,78,79]. (See "Motion sickness", section on 'Patient factors'.)

Patients with vestibular migraine have higher rates of anxiety and depression compared with controls or those with other vestibular disorders [66,80-83]. Anxiety may be a consequence of unpredictable, disabling symptoms and may also exacerbate symptoms during acute episodes [24,73].

Examination findings

●Nystagmus – Nystagmus is typically present during an episode of vestibular migraine. Features may be suggestive of either peripheral or central origin (table 1); they may be spontaneous, gaze-evoked, or solely positional. Nystagmus can be identified during routine neurologic examination with tools to block visual fixation such as occlusive ophthalmoscopy, the penlight-cover test, Frenzel lenses, or video oculography [24,84-87]. (See "Evaluation of the patient with vertigo", section on 'Nystagmus'.)

Spontaneous horizontal nystagmus is common during vestibular migraine episodes, but intense spontaneous horizontal nystagmus is more suggestive of Meniere disease; vertical nystagmus is more typical with vestibular migraine [88]. Interictal nystagmus is uncommon in vestibular migraine. (See 'Differential diagnosis' below.)

During the symptom-free period between episodes, the bedside vestibular and oculomotor examination is generally normal. However, patients may exhibit very low-velocity spontaneous or positional nystagmus when examined with vision denied [85].

●Other examination findings – Impaired smooth pursuit may be seen on extraocular muscle examination and abnormalities of the vestibulo-ocular reflex may be seen during episodes by video head impulse testing (figure 1). Interictal testing usually reveals a normal symmetric vestibulo-ocular reflex, although these and other vestibular laboratory tests such as vestibular-evoked myogenic potentials reveal nonspecific abnormalities in a small minority of patients with vestibular migraine [10,37,85]. (See "Evaluation of the patient with vertigo", section on 'Head impulse test'.)

Most patients have impaired gait and stance on examination during episodes.

DIAGNOSIS — The diagnosis of vestibular migraine should be considered in patients with recurrent episodes of vertigo associated with a history of migraine headaches. The diagnosis of vestibular migraine is made clinically in patients who fulfill diagnostic criteria, after excluding alternative causes of episodic vestibular symptoms.

Diagnostic criteria — Clinical criteria have been jointly developed by the Bárány Society and the International Classification of Headache Disorders (ICHD-3) [2,89]:

●At least five episodes of vestibular symptoms of moderate or severe intensity, lasting between 5 minutes and 72 hours

●One or more migrainous feature with at least 50 percent of vestibular episodes, including:

•Headache with at least two of the following characteristics (unilateral, pulsating, moderate or severe intensity, aggravation by routine physical activity)

•Photophobia and phonophobia

•Visual aura

●A current or past history of migraine (with or without aura)

●Symptoms not better accounted for by another diagnosis

Qualifying vestibular symptoms include spontaneous vertigo, positional vertigo, visually induced vertigo, head-motion-induced vertigo, or head-motion-induced nonvertiginous dizziness with nausea. Vestibular symptoms are considered moderate to severe if they interfere with daily activities.

The Bárány Society also developed criteria for probable vestibular migraine that can be applied when the patient has either migrainous features during the vestibular episodes or a current or past history of migraine, but not both [1]. For these patients, we perform diagnostic testing to evaluate for alternative causes of symptoms and reassess the diagnosis if the patient does not respond to treatment or if the clinical features change. In one study, 75 patients diagnosed with vestibular migraine according to similar criteria were reassessed after a mean of nine years [70]. More than 80 percent of patients continued to be considered as having probable or definite vestibular migraine, while the diagnosis was revised in 12 patients, most often to Meniere disease.

Evaluation to exclude alternative conditions

Indications for diagnostic testing — There are no conclusive diagnostic tests for migraine or vestibular migraine. We perform diagnostic testing to exclude alternative causes of episodic vertigo for patients with new-onset symptoms that do not fulfill diagnostic criteria. In addition, diagnostic testing is warranted for those with atypical clinical features including any of the following:

●Vestibular symptoms that are either very brief (eg, seconds up to a few minutes) or prolonged (eg, >72 hours)

●Sudden, fluctuating, or progressive hearing loss or other auditory symptoms

●Loss of consciousness during episode

●Systemic signs on general examination (eg, fever, rash)

●Abnormal signs on neurologic examination (eg, limb weakness, cranial nerve palsies)

Diagnostic testing may be unnecessary for patients with a longstanding history of episodic vertigo meeting diagnostic criteria for vestibular migraine, no atypical features to suggest an alternative diagnosis, and a normal examination between episodes.

Neuroimaging — We suggest neuroimaging to evaluate for transient ischemic attack (TIA) or stroke when acute vestibular episodes are either new onset, associated with other abnormal neurologic findings on examination, or occur in patients with risk factors for stroke. Noncontrast computed tomography (CT) of the head is often performed as an initial imaging study for patients with acute stroke symptoms including those with isolated vertigo because it is typically easier to perform quickly, but it has poor sensitivity for detecting acute ischemia in the posterior fossa. Magnetic resonance imaging (MRI) of the brain without contrast with diffusion-weighted sequences is more sensitive for detecting acute ischemia. Vascular imaging with CT or MR angiography of the head and neck is performed to identify vascular stenoses or occlusion as a cause of symptoms.

Brain MRI with contrast including high-resolution 3D protocols that include submillimeter slices through the internal auditory canal should be performed for patients with acute persisting or chronic vestibular symptoms to evaluate for structural causes to symptoms such as tumors of the posterior fossa.

Audiometry — We suggest audiometry for patients with prominent or monaural auditory symptoms during episodes and those with symptoms that persist after episodes resolve to assess for features of Meniere disease or superior semicircular canal dehiscence syndrome. A follow-up audiometry study can be helpful when an initial study is negative and the diagnosis remains unclear [70]. (See "Meniere disease: Evaluation, diagnosis, and management" and "Causes of vertigo", section on 'Semicircular canal dehiscence syndrome'.)

Other testing for patients with atypical symptoms

●Patients with very brief attacks of vertigo that are positionally induced should be evaluated clinically for benign paroxysmal positional vertigo (BPPV). (See "Benign paroxysmal positional vertigo".)

●Vestibular laboratory testing is not typically required for the diagnosis of vestibular migraine and frequently reveals nonspecific abnormalities in patients with vestibular migraine [10,37,85]. Testing can be helpful in selected patients for identifying significant peripheral or central vestibular dysfunction from an alternative diagnosis.

For patients with symptoms atypical for vestibular migraine, and for patients who do not respond adequately to treatment for vestibular migraine, it is appropriate to broaden the extent of testing to evaluate alternative or comorbid conditions. The evaluation of a patient with vertigo is discussed in more detail separately. (See "Evaluation of the patient with vertigo".)

DIFFERENTIAL DIAGNOSIS — There are many causes of vertigo, but common causes of episodic vertigo can be distinguished by the duration of attacks and the presence of associated symptoms (table 2). The major considerations in the differential diagnosis of vestibular migraine are those disorders that produce vertigo with a similar tempo and duration of symptoms. These most often include Meniere disease, other migraine variants, and vertebrobasilar transient ischemic attacks (TIAs).

●Meniere disease – Symptoms associated with Meniere disease include recurrent episodes of vertigo, unilateral tinnitus, and unilateral hearing loss. These symptoms can also occur in patients with vestibular migraine, making it difficult to distinguish these conditions [56,58]. In addition, some typically migrainous symptoms, including headache, are common in attacks of Meniere disease [2,56,76]. The clinical presentation of Meniere disease may also be variable. In rare patients with Meniere disease, vestibular symptoms predominate and audiologic symptoms may be minor or absent [36,56,58,90]. (See "Meniere disease: Evaluation, diagnosis, and management".)

However, Meniere disease is approximately one-tenth as common as vestibular migraine in population studies [36]. Patients with Meniere disease frequently report transient ear fullness preceding the attack or at attack onset. Fluctuating and progressive hearing loss and other monaural symptoms are common in Meniere disease; various auditory symptoms have also been described in vestibular migraine, but they are typically transient. Audiometry examinations showing progressive or fluctuating low- to medium-frequency hearing loss are most useful in identifying the condition as Meniere disease [70]. Nystagmus occurs during both Meniere disease and vestibular migraine attacks, but very intense spontaneous horizontal nystagmus (>12 degrees per second) may suggest a diagnosis of Meniere disease over vestibular migraine [88].

Meniere disease and migraine can be comorbid [76].

●Migraine with brainstem aura – Migraine with brainstem aura (MBA) is a form of migraine with an aura that features two or more focal brainstem symptoms. Vertigo is a common aura symptom but never the only aura symptom in MBA. In addition, most patients with MBA develop a migraine headache within 5 to 60 minutes of onset of an aura. Only a small fraction of patients with vestibular migraine meet criteria for MBA [6,7]. (See "Migraine with brainstem aura".)

●Brainstem stroke – Vertigo due to TIA or stroke is frequently accompanied by other symptoms such as dysarthria, diplopia, weakness, or numbness. However, isolated vertigo may be an uncommon presentation of ischemic stroke involving vestibular or cerebellar brainstem pathways [91-96]. Some patients with isolated vestibular symptoms may be found to have other neurologic signs on examination such as limb ataxia, Horner syndrome, or sensory dysfunction. Sudden onset of symptoms and the presence of cerebrovascular risk factors may suggest TIA or stroke as the diagnosis for vestibular symptoms. Hemorrhagic and some ischemic strokes may be identified by head CT; brain MRI is the preferred study to identify acute ischemic infarction. Vascular imaging may help identify stenoses or occlusion as the cause to symptoms for patients with TIA or stroke. (See "Posterior circulation cerebrovascular syndromes" and "Neuroimaging of acute stroke".)

●Benign paroxysmal positional vertigo – Benign paroxysmal positional vertigo (BPPV) is the most common cause of episodic vertigo. BPPV typically presents with recurrent episodes of isolated vertigo that may be similar to vestibular migraine attacks. However, BPPV is often triggered by specific head movements and improves by remaining still. Attacks frequently resolve within one minute, unless head movement triggers another BPPV attack. Migrainous symptoms are not features of BPPV attacks. (See "Benign paroxysmal positional vertigo".)

BPPV and vestibular migraine can be comorbid, and migraine has been linked to an increased risk of BPPV [29,74,97,98].

●Superior semicircular canal dehiscence syndrome – Superior semicircular canal dehiscence (SSCD) syndrome refers to an abnormal thinning or opening in the bone covering the superior semicircular canal that can allow pressure to move fluid in the semicircular canal and cause vertigo by activating hair cells that sense rotation. Loud sounds can induce pressure waves to trigger a transient sense of vertigo. Vertigo may also be triggered by coughing or Valsalva maneuver. Patients may have pulsatile tinnitus and increased sensitivity to bone-conducted sound with autophony (hearing their own voice as loud or distorted on the affected side) and increased loudness of internal sounds (eg, chewing, eye movements, footsteps). SSCD may be identified by CT of the temporal bone. (See "Causes of vertigo", section on 'Semicircular canal dehiscence syndrome'.)

SSCD and vestibular migraine can be comorbid, as illustrated by a retrospective review of 91 patients with SSCD that identified vestibular migraine in 36 patients (40 percent) [99].

●Persistent postural-perceptual dizziness – Persistent postural-perceptual dizziness (PPPD) refers to a syndrome of chronic functional nonvertiginous dizziness and unsteadiness that combines core features of other previously described distinct syndromes such as phobic postural vertigo and chronic subjective dizziness [100-104]. PPPD typically presents with chronic, fluctuating nonvertiginous dizziness rather than spontaneous vertigo episodes associated with vestibular migraine [7,76].

PPPD is often comorbid with vestibular migraine [105]. Whether these symptoms represent a chronic form of vestibular migraine that should be defined separately from comorbid PPPD is uncertain [66,77,105]. However, it is important to distinguish symptoms due to vestibular migraine from the chronic dizziness and unsteadiness due to PPPD, as their management is different.

●Episodic ataxia – Episodic ataxia type 2 (EA2) is a rare genetic disorder involving a pathologic variant in the CACNA1A gene coding for the Ca_v2.1 pore-forming subunit of the neuronal P/Q-type calcium channel. Patients can present with recurrent disabling episodes of vertigo, nausea/vomiting, ataxia, and nystagmus, often provoked by exertion or emotional stress. Symptoms typically begin in childhood, and brain imaging with MRI may show cerebellar vermis atrophy. Acetazolamide may be effective in reducing the frequency of symptoms. (See "Overview of the hereditary ataxias", section on 'Episodic ataxias'.)

●Vestibular paroxysmia – Vestibular paroxysmia is a term sometimes used to describe very brief (one to several seconds), frequent (up to several times a day) episodes of vertigo without migraine symptoms. It is most commonly attributed to a microvascular compression of the eighth cranial nerve. (See "Causes of vertigo", section on 'Vestibular paroxysmia'.)

●Recurrent vestibulopathy – The entity benign recurrent vertigo refers to spontaneous episodes of vertigo without other migrainous, neurologic, or otologic symptoms or sequelae but has strong links to migraine [8,30,32]. (See "Causes of vertigo", section on 'Recurrent vestibulopathy'.)

The differential diagnosis of vertigo also includes several conditions that present with persistent symptoms or vertigo along with other clinical features (table 3). These disorders are discussed separately. (See "Causes of vertigo".)

TREATMENT — The management of vestibular migraine symptoms includes treatment of acute symptoms and prevention of future attacks. Most of the evidence for treatment of vestibular migraine is from case reports and series [6,12,18,53,63,106-111]. The efficacy of treatments for vestibular migraine has not been established in large, well-designed controlled trials [24,112-114]. Current practice recommendations are based on a synthesis of available studies, expert opinion, anecdotal experience, and adaptation from the robust migraine headache literature along with accepted treatments for acute vertigo [114,115].

Indications for treatment — Pharmacotherapy for patients with acute vestibular migraine symptoms are typically used for patients with prolonged or severe symptoms. Medications are typically not helpful for episodes that resolve more quickly.

We suggest preventive treatment for vestibular migraine for patients with episodes that are frequent (three to six per month or more) or if acute treatments are ineffective [63,108]. Indications for prophylactic therapy should mirror those for other forms of migraine headache, which consider the frequency, duration, and disabling nature of the attacks.

Acute treatment

Initial therapies — For most patients with vestibular migraine with acute symptoms, we suggest initial treatment with vestibular suppressants when the episode is prolonged (eg, more than 30 minutes) or when vertigo or nausea is severe. Options for acute symptoms include:

●Antihistamines (eg, meclizine, diphenhydramine)

●Antiemetics (eg, prochlorperazine, promethazine)

●Benzodiazepines (eg, diazepam, lorazepam)

The selection of agent is based largely on individual patient symptoms, comorbidities, and risk of adverse effects as studies on acute treatment for patients with vestibular migraine are limited. However, a meta-analysis of 17 clinical trials including 1586 patients that assessed the efficacy of antihistamines and benzodiazepines for acute vertigo found that single-dose antihistamines provided greater acute symptom relief at two hours than single-dose benzodiazepines [116].

Agents with intravenous or rectal formulations may be preferred if vomiting prevents oral administration (table 4) [12,18]. (See "Treatment of vertigo", section on 'Symptomatic treatment'.)

Alternative options — Triptans may be tried for patients who do not respond to initial acute treatment options and for those who have headache symptoms that accompany vertigo attacks. However, data on the efficacy of triptans for vestibular migraine are limited. In a placebo-controlled trial of 134 patients with vestibular migraine, those who received rizatriptan had a similar rate of symptom improvement at one hour as those who received placebo [117]. However, those who received rizatriptan reported improved unsteadiness/dizziness and motion sensitivity at 24 hours and for physical well-being at 48 hours. In a pilot study of zolmitriptan for vestibular migraine that enrolled 10 patients treating 17 attacks, there was a numerically higher response rate with treatment versus placebo (38 versus 22 percent) [107]. Low enrollment because of infrequent or brief attacks limited the power of this study.

Noninvasive vagal nerve stimulation and external trigeminal nerve stimulation have also been reported in single-center retrospective case series as potentially shortening attack duration or reducing vertigo intensity [118,119].

Preventive treatment

General measures for all patients

●Lifestyle modifications – Lifestyle modification alone can be effective at reducing dizziness and headaches for some patients [120]. Adequate and restful sleep is an important factor to address and reduce the severity and frequency of episodes. Dietary and environmental triggers should be identified and avoided where possible, although there are limited efficacy data for this approach in vestibular migraine [12,121]. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Precipitating and exacerbating factors'.)

●Treat comorbid conditions – Identifying and treating comorbidities that may mimic or trigger vestibular migraine episodes may help guide appropriate preventive therapies [76]. Common comorbidities include anxiety, benign paroxysmal positional vertigo (BPPV), motion sickness, and persistent postural-perceptual dizziness (PPPD). Meniere disease can be comorbid or confused with vestibular migraine. Such patients warrant careful medical management of both conditions, sometimes using headache symptoms as a marker for treatment response for vestibular migraine. (See "Benign paroxysmal positional vertigo", section on 'Treatment' and "Treatment of vertigo" and "Generalized anxiety disorder in adults: Management" and "Meniere disease: Evaluation, diagnosis, and management".)

Patients with vestibular migraine are susceptible to motion sickness. The prevention and treatment of motion sickness is described separately. (See "Motion sickness".)

Prophylactic migraine medications — For most patients who take preventive therapy for vestibular migraine, we suggest medications that are effective for migraine prophylaxis. Since high-quality efficacy data are limited in vestibular migraine, and no one therapy has been shown to be superior to another, the choice of medication is driven primarily by the patient's comorbidities and concern for adverse effects. This approach to choosing a prophylactic medication is described in detail separately. (See "Preventive treatment of episodic migraine in adults", section on 'Choosing pharmacologic therapy'.)

●Initial medication options – We suggest beta-blockers, antiseizure medications, or antidepressants that are used for prophylaxis for other types of migraine and have been shown to have benefit for vestibular migraine, unless specific medical comorbidities or contraindications exist.

•Beta-blockers – Several beta-blockers may be used for vestibular migraine prevention. We typically use propranolol or metoprolol.

-Propranolol extended-release formulation is typically used and started at 60 mg once daily and titrated if needed after two to three weeks to 60 mg twice daily. The dose may be titrated further if needed every two to three weeks by 40 to 80 mg to a typical maximum dose of 120 mg twice daily.

-Metoprolol is started at 50 mg daily and titrated as needed by 50 mg every two to three weeks to a maximum dose of 200 mg daily.

Beta-blockers are used with caution in patients with cardiovascular risk factors and those with asthma or depression due to risk of adverse events. (See "Major side effects of beta blockers".)

Propranolol is generally well tolerated and has been associated with reductions in vertigo frequency and severity in uncontrolled before-and-after comparisons and one randomized trial [122-125]. Metoprolol is also commonly used for migraine prophylaxis. A multicenter prevention trial with metoprolol for vestibular migraine failed to show benefit over placebo; however, the trial was terminated due to slow patient accrual after less than 50 percent enrollment [126].

•Antiseizure medications – Valproate, topiramate, and lamotrigine are frequently used for migraine prevention. The typical starting, titration, and target dose ranges for these medications are as follows:

-Topiramate is started at 25 mg daily; titrate as needed by up to 25 to 50 mg weekly to 200 mg in two divided doses.

-Valproate is started at 500 mg daily; titrate as needed by 250 to 500 mg weekly up to 1500 mg daily in two divided doses.

-Lamotrigine is started at 25 mg daily; titrate as needed by 50 mg after two weeks, then by 50 mg every one to two weeks thereafter up to a target dose of 200 mg daily in two divided doses.

Common adverse effects of topiramate include cognitive impairment, paresthesias, and mood disorders. Valproic acid can commonly cause weight gain or tremors and is avoided in females of childbearing age due to risk of teratogenicity. Lamotrigine may cause a cardiac conduction disorder or rash, especially if the dose is titrated rapidly. The adverse effects of these medications are discussed in greater detail separately. (See "Antiseizure medications: Mechanism of action, pharmacology, and adverse effects".)

The evidence to support the use of antiseizure medications for vestibular migraine is limited to small trials and open-label studies. Topiramate was found to be associated with a reduction in vertigo and headache frequency and severity in a small unblinded trial, with the lower 25 mg twice-daily dose better tolerated and no less effective than 50 mg twice daily [127]. Other small multiple-arm studies without placebo also reported benefit on vertigo and headache severity with topiramate [124,125,128]. Valproic acid was compared with venlafaxine and flunarizine in a small single-blinded randomized trial and found similar reductions in total dizziness handicap inventory (DHI) scores among all three groups and reduced vertigo attack frequency with valproic acid and venlafaxine [129]. Lamotrigine reduced monthly vertigo and headache frequency and was well tolerated in one small retrospective study [130].

•Antidepressants – Multiple classes of antidepressant medications may be used for migraine prophylaxis. We prefer venlafaxine or amitriptyline for patients with vestibular migraine.

-Venlafaxine is started at 37.5 mg daily and increased as needed by 37.5 mg weekly up to 150 mg daily.

-Amitriptyline is started at 10 mg at bedtime and increased as needed by 25 mg weekly up to 100 mg at bedtime.

Venlafaxine was associated with similar improvements as propranolol in vertigo frequency and severity and overall dizziness handicap but greater improvement in depression measures in an unblinded randomized trial [122]. The venlafaxine group had greater improvement in the emotional domain of the DHI compared with valproic acid and flunarizine groups but similar reductions in the vertigo attack frequency and severity in another small single-blinded randomized trial [129]. Amitriptyline and nortriptyline have also been reported to be beneficial in small studies [124,125,128].

Amitriptyline may cause dry mouth, constipation, orthostatic hypotension, or confusion. Venlafaxine can cause elevation in blood pressure and may worsen headaches in some patients. The adverse effects of these medications are discussed in greater detail separately. (See "Tricyclic and tetracyclic drugs: Pharmacology, administration, and side effects", section on 'Amitriptyline' and "Serotonin-norepinephrine reuptake inhibitors: Pharmacology, administration, and side effects", section on 'Venlafaxine'.)

●Alternative options

•Calcium channel blockers – Several calcium channel blockers are used for migraine prevention. However, there is little evidence to support the use for vestibular migraine.

Flunarizine, cinnarizine, and lomerizine have been evaluated for vestibular migraine in several single-treatment and multiple-treatment arm studies. Flunarizine has slightly stronger evidence at reducing vertigo frequency, vertigo severity, DHI, and headache severity [125,129,131,132]. Cinnarizine and lomerizine have less data supporting their reported reductions in headache and vertigo frequency and severity [133-135]. None of these agents are available in the United States.

Verapamil is frequently used for other forms of migraine, but the evidence to support the use for vestibular migraine is modest and some studies have failed to find benefit [111,128].

•Botulinum toxin – Botulinum toxin is used for patients with chronic migraine, but the benefit for patients with episodic or vestibular migraine is less certain. In a nonrandomized study of patients with vestibular migraine taking an oral preventive medication, the addition of botulinum toxin A was associated with a greater reduction in headache disability, a reduced vertigo attack frequency, and a similar improvement in dizziness handicap compared with an oral preventive medication alone [136]. A retrospective review of 22 patients with vestibular migraine receiving onabotulinumtoxinA reported significant improvements in migraine disability, dizziness handicap, and vertigo attack frequency compared with symptoms prior to treatment [137].

•CGRP antagonists – Calcitonin gene-related peptide antagonists (CGRP) include agents for acute and preventive treatment of migraine. One small chart review found that anti-CGRP medications were associated with moderate or significant improvement in vestibular and migraine symptoms in 15 of 25 patients [138]. In a prospective observational study of 50 patients with vestibular migraine and symptoms refractory to at least three other preventive medications, use of a CGRP antagonist (erenumab, fremanezumab, or galcanezumab) was associated with a 50 percent or greater reduction in vertigo and headache frequency in 90 and 86 percent, respectively, by 12-month follow-up [139]. Additional data are warranted to better define the role of CGRP antagonists for patients with vestibular migraine [140].

•Other treatments – Hormonal treatment for menstrually associated symptoms has been reported to be helpful in isolated cases [12]. (See "Estrogen-associated migraine headache, including menstrual migraine" and "Preventive treatment of episodic migraine in adults".)

Therapies of uncertain or limited benefit

●Vestibular therapy – Vestibular rehabilitation is frequently used for symptomatic patients with vertigo. However, the benefit of vestibular therapy exercises for patients who have solely unprovoked episodes of vertigo and no chronic dizziness or unsteadiness between attacks has not been established [141]. Studies of vestibular rehabilitation therapy for patients with episodic vestibular migraine have mostly been before-and-after studies without a control group. Most report improvement in a variety of measures, including balance tests, disability measures, vertigo severity, and even headache burden. However, the possibility of confounding conditions such as PPPD is often not reported [142,143].

Vestibular therapy may be especially helpful if there are optokinetic or other visual- or self-motion triggers present or if there are forms of chronic nonvertiginous dizziness between episodes of vestibular migraine, in which habituation-type vestibular therapy can be beneficial [144,145]. Therapy exercises are tailored to the patient based on vestibular testing, but may include balance exercises, gaze stability training, and habituation exercises to help desensitize the patient to certain triggers of dizziness. (See "Treatment of vertigo", section on 'Vestibular rehabilitation'.)

●Benzodiazepines – This class of medications has been used as acute vestibular suppressants in patients with vertigo, including vestibular migraine [12,110]. However, we generally limit benzodiazepines to use for acute symptomatic treatment of vertigo, unless such significant anxiety exists that the patient requires a daily benzodiazepine until a better alternative agent can be titrated to a therapeutic dose. (See 'Initial therapies' above and "Treatment of vertigo", section on 'Symptomatic treatment'.)

●Acetazolamide – Acetazolamide is effective in preventing attacks in episodic ataxia type 2 (EA2) but not widely used in the treatment of either migraine or vertigo. However, two studies have explored its use in vestibular migraine, though poor tolerance and high discontinuation rates were seen [124,146]. (See "Overview of the hereditary ataxias", section on 'Episodic ataxias'.)

PROGNOSIS — The natural history of vestibular migraine has not been well studied. In one long-term (median nine years) follow-up of 61 patients with vestibular migraine, 87 percent of patients continued to have recurrent vertigo, although the frequency of episodes was reduced in more than half of patients [147]. Cochlear symptoms (tinnitus, hearing loss) had increased in prevalence from 15 to 49 percent.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Migraine and other primary headache disorders".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Beyond the Basics topic (see "Patient education: Vertigo (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

●Definition – Vestibular migraine is a term used to describe episodic vertigo or other vestibular symptoms attributed to migraine. (See 'Introduction' above.)

●Epidemiology – Vestibular migraine is a common cause of episodic vertigo. As with other forms of migraine, vestibular migraine can affect children as well as adults, and females appear to be more often affected than males. (See 'Epidemiology' above.)

●Clinical features

•Vertigo – Vestibular migraine is characterized by recurrent episodes of transient vertigo. Most episodes last minutes to hours and may be spontaneous or triggered by visual or motion stimuli (eg, fluorescent lights, watching trains, busy patterns, crowds). Vertigo can be internal (a false sensation of self-motion) or external (a false sensation that the visual surround is spinning or flowing). (See 'Episodic vertigo' above.)

•Headache – Migrainous headaches are associated with most episodes of vestibular migraine. However, vertigo may occur without headache. (See 'Migraine headache' above.)

•Interictal symptoms – Patients with vestibular migraine may report a high prevalence of mild vestibular symptoms between attacks. A susceptibility to motion sickness is common. (See 'Interictal symptoms' above.)

●Diagnosis – The diagnosis of vestibular migraine is made clinically in patients who fulfill diagnostic criteria, after excluding alternative causes of episodic vestibular symptoms. (See 'Diagnostic criteria' above.)

●Diagnostic evaluation – There are no diagnostic tests specific for vestibular migraine. We perform diagnostic testing to exclude alternative causes of episodic vertigo for patients with new-onset symptoms that do not fulfill diagnostic criteria. In addition, diagnostic testing is warranted for those with clinical features that are atypical. (See 'Evaluation to exclude alternative conditions' above.)

•Neuroimaging – We suggest neuroimaging to evaluate for transient ischemic attack (TIA) or stroke when acute vestibular episodes are either new onset, associated with other abnormal neurologic findings on examination, or occur in patients with risk factors for stroke. (See 'Neuroimaging' above.)

•Audiometry – We suggest audiometry for patients with prominent or monaural auditory symptoms during episodes and those with symptoms that persist after episodes resolve. (See 'Audiometry' above.)

●Differential diagnosis – The differential diagnosis of vestibular migraine includes disorders that produce episodic vertigo (table 3). These most often include Meniere disease, other migraine variants, benign paroxysmal positional vertigo (BPPV), and vertebrobasilar TIAs. (See 'Differential diagnosis' above.)

●Treatment

•Acute attacks – For most patients with disabling symptoms during acute attacks, we suggest a treatment trial with vestibular suppressants rather than triptans at the time of attacks (Grade 2C). Options include antihistamines, antiemetics, or benzodiazepines. Triptans are a reasonable alternative when headache symptoms accompany vertigo attacks or when vertigo acts as a migraine aura. (See 'Acute treatment' above.)

•Preventive treatment – We suggest preventive treatment for patients with vestibular migraine episodes that are frequent (eg, three to six per month or more) or if acute treatments are ineffective (Grade 2B). For most patients, we prefer beta-blockers, antiseizure medications, or antidepressants rather than other medications used for migraine prophylaxis, unless specific medical comorbidities or contraindications exist. Options include (see 'Prophylactic migraine medications' above):

-Propranolol or metoprolol

-Topiramate, valproate, or lamotrigine

-Venlafaxine or amitriptyline

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges David F Black, MD, who contributed to earlier versions of this topic review.