Patients explain a sensation of motion when no motion is taking place or an altered sensation with normal motion to define vertigo. In a vestibular migraine, the sensation is often described as a "to-and-fro" sensation, and this can complicate diagnosis. Simultaneous headaches do not always accompany the vestibular symptoms, thus making an in-depth history and multi-specialty evaluation pivotal in order to make a diagnosis .
The International Headache Society in the International Classification of Headache Disorders, Second Edition classified a migraine into distinct diagnoses; however, the only benign paroxysmal vertigo of childhood-type and basilar-type migraine included vertigo . The Neuheuser criteria were historically the most widely accepted classifier of dizziness related to a migraine. It divided a vestibular migraine into "definite" versus "probable" disease .
Definite vestibular migraine criteria included:
Probable vestibular migraine diagnostic criteria included:
The currently accepted vestibular migraine diagnostic criteria consist of the following:
Due to the relatively new diagnostic criteria, much of the reviewed literature is based on the Neuheuser criteria.
There is limited concrete evidence regarding the absolute etiology of a vestibular migraine. However, genetics may play a role in vestibular migraines as displayed by a recent study showing an autosomal dominant inheritance trait linked to gene locus 5q35 in a four-generation family line. The majority of this family demonstrated headaches followed by vertigo within 15 to 20 years after the onset of migraine headaches . Other studies have reported links to a region on chromosome 11q  or 22q12 .
Many peripheral vestibular disorders are seemingly associated with a vestibular migraine, some of which include: Meniere syndrome and benign paroxysmal positional vertigo.
While it seems Meniere syndrome is associated with a vestibular migraine; the relationship appears complicated. Clinical evaluation of dizzy or vertiginous patients is commonplace at many tertiary otolaryngology centers. With the understanding of some selection bias, a study from one of these centers demonstrated nearly 30% of Meniere syndrome patients may also have vestibular migraine, while an additional 30% did not meet vestibular migraine criteria but did demonstrate some migraine-like qualities .
An additional study showed some fascinating associations between Meniere syndrome and vestibular migraine. Thirteen percent to 18% of those initially diagnosed with probable or definite vestibular migraine developed bilateral, low-frequency sensorineural hearing loss within 9 years. Interestingly, 7% to 11% developed cochlear symptoms that met the American Academy of Otolaryngology, Head and Neck Surgery (AAO-HNS) criteria for bilateral Meniere syndrome  .
In 2013, Wise and Sargent presented that those who suffer from simultaneous migraine headaches with intractable Meniere syndrome experienced worse quality of life-related surgical outcomes compared to intractable Meniere syndrome alone. These findings were obtained after undergoing endolymphatic sac decompression for intractable Meniere syndrome.
The idea that one may suffer from both diseases cannot be discounted, and therefore ought to be considered in the workup and treatment. Additionally, due to similar symptomatology diagnostic uncertainty may confuse the patient regarding the actual disease with which they suffer .
Benign Paroxysmal Positional Vertigo (BPPV)
The association between BPPV and vestibular migraine is another complicated one as the relationship is not well understood. It has been shown that those with BPPV who are successfully treated with the Epley maneuver have a higher prevalence of motion sickness or migraine history compared to the general public .
Further, a conglomerate of the symptoms of vertigo, nausea, and headaches accompanied by sustained nystagmus on positional testing that dissipates when the patient is symptom-free suggests a vestibular migraine . Additionally, another study has suggested an association between the diagnoses of probable or definite a vestibular migraine and atypical positional nystagmus and/or vertigo . Despite these suspected associations, singular canal or multiple canal cupulolithiasis ought to be excluded, as this may be a driving inducer for a vertigo-associated migraine.
Migraine Anxiety-Related Dizziness (MARD)
MARD is a condition that must be considered as recurrent dizziness can cause debilitating anxiety. This disease is diagnosed with symptoms of anxiety, migraine, and vestibular symptoms. Treating anxiety as well as the migraine and balance disorder is a must .
Vestibular migraine is most commonly noted in midlife with a predilection for females. There is a 5:1 female-to-male preponderance with the mean age of onset of 37 years for females and 42 years for males. Migraine typically predates the vertiginous symptoms. Mean age of onset of headaches is 28 years old and mean age of dizziness onset is 49 years old .
Little is known or understood regarding the pathophysiology of the disease, or at least there is the little consensus of agreement. However, there are theories that vestibular symptoms may be an aura, an associated symptom to a headache, or due to actual peripheral vestibulopathy. In 2011, Eggers et al. reported vertigo in a vestibular migraine as an associated phenomenon rather than an aura .
Another theory involves the idea of "kindling." Just as epileptic seizures may lower the threshold for future seizures, a similar thought process exists for vestibular migraines. The possibility that vestibular migraineurs become sensitized to stimuli such as motion in the visual field, and this may lead to recurrent vestibular migraine symptoms .
Finally, one study showed that caloric testing triggers a migraine in some individuals. In this study, 39 individuals with a history of a migraine underwent caloric testing. Within 24 hours, 19 of these individuals developed their typical migraine symptoms. 47% of those that developed a migraine within 24 hours actually developed a migraine during testing. This study design used a matched control group and only one of 21 from this group developed a migraine within the initial 24-hour period after testing . This finding is perplexing, leading one to believe that peripheral vertigo can be a trigger of a migraine or migraine-related symptoms.
Thirty percent of those diagnosed with vestibular migraine may present without history of headache . Common complaints include acute episodes of vertigo lasting seconds to days, dizziness, imbalance, and spatial disorientation. Other symptoms may include lightheadedness, swimming sensation, heavy headedness, a rising sensation, tingling sensation, a rocking sensation, excessive motion sickness susceptibility; additionally, transient fluctuating hearing loss, aural fullness, tinnitus, and mild sensorineural hearing loss on audiogram are not uncommon. To define vertigo, one explains a sensation of motion when no motion is taking place or an altered sensation with normal motion. In a vestibular migraine, the sensation can oft be described as a "to-and-fro" sensation, and this can complicate diagnosis. Based on the variable duration, this disease can mimic other causes of vertigo. Vertigo and headache do not always present synchronously, in fact, it is rare for both symptoms to present simultaneously. There does not seem to be a consistent pattern of vestibular symptoms during headache presentation .
A vestibular migraine does not seem to go away. Episodes can be associated with women’s menstrual cycles. Studies have shown that nearly 50% of those diagnosed with probable vestibular migraine progress to definite a vestibular migraine according to the previously mentioned Neuheuser criteria. Another study showed that nearly 30% experienced increased frequency of attacks over a 9-year observation period, although nearly 50% experienced a reduced frequency of attacks. Nearly 90% of vestibular migraineurs still report vertiginous attacks after nine years. Additionally, cochlear complaints such as aural fullness, tinnitus and/or hearing loss during vertigo spells seem to become more common as time progresses .
A thorough history is imperative as there are no pathognomonic imaging studies or available testing consistent with a vestibular migraine. Despite this, there are nonspecific findings noted with certain studies. One such example comes with magnetic resonance imaging (MRI). MRI has shown an associated finding consistent with a general migraine. White matter hyperintensities are four times more likely in this group as compared to age and gender-matched controls . Some functional neuroimaging lends some light on general migraine pathophysiology; however, few such reports are available regarding a vestibular migraine and may be an area of future exploration and discovery.
Vestibular testing and neurotologic exams typically yield nondiagnostic abnormalities in those with isolated probable or definite vestibular migraine. It must be recognized, that if an individual has Meniere syndrome or BPPV association, they are more likely to have abnormal testing .
Another study has shown an increased incidence of unilateral canal paresis on caloric testing and vestibular hyporesponsiveness or hyperresponsiveness in vestibular migraine patients . One study suggests abnormal sacculocollic pathway (cervical vestibular evoked myogenic potentials (cVEMP)) and normal ocular VEMP (oVEMP) findings in a vestibular migraine compared to healthy controls; however, these findings have not been duplicated . These findings are not unique to a vestibular migraine.
Current management of a vestibular migraine consists of conventional migraine management as there is currently no accepted specific treatment for this disease . Additionally, convincing a patient of the diagnosis can prove difficult and lead to a delay in treatment or absence of treatment. This is understandable based on the fact that vertiginous symptoms are often asynchronous with a headache and maybe a difficult connection for the practitioner or patient to grasp .
Sargent’s experience suggests that triptan therapy is rarely of benefit in the acute treatment of a vestibular migraine  despite small reports suggesting modest benefit in acute vertigo attacks  . Triptans and calcium-channel-blocking medications have proven ineffective in shortening aura or preventing headaches in general migraine ; however, they have not been tested in vestibular migraine. Thus, for acute vestibular migraine only antiemetic and antivertigo therapies are currently recommended .
Current recommendations for vestibular migraine prevention include lifestyle changes such as avoidance of dietary, behavioral or sleep hygiene triggers . In one retrospective study, caffeine cessation was associated with symptomatic improvement in 15% of the vestibular migraine population tested. This reduction in symptomatology was incomplete, however, and many required further medical therapy. From this study, 75% of those with a vestibular migraine experienced ample relief from lifestyle changes in addition to treatment with nortriptyline or topiramate and did not require further treatment. Nortriptyline gave relief in 46% while topiramate provided relief in 25% in this retrospective study . In a separate prospective study, topiramate (50 to 100 mg per day) was shown to reduce the frequency and severity of vertigo and headaches .
A 2017 randomized controlled trial by Liu, et al. demonstrated the efficacy and safety of venlafaxine, flunarizine, and valproic acid in the prophylaxis of VM. Each treatment demonstrated unique treatment properties such as venlafaxine which showed an advantage in the treatment of emotional symptoms. Venlafaxine and valproic acid also were shown to be preferable to flunarizine in decreasing the frequency of vertiginous attacks. Valproic acid seemed to be less effective than venlafaxine and flunarizine in decreasing vertigo severity .
The American Headache Society, American Academy of Neurology recommend the following therapies as possibilities for an episodic migraine: butterbur, divalproate, metoprolol, propranolol, timolol, and topiramate . Aside from topiramate, none of these have been evaluated for the treatment of a vestibular migraine specifically; however, it seems that one could consider these as potential treatment options. Other possibilities include amitriptyline and calcium channel blockers . One could consider other existing comorbidities in conjunct with a vestibular migraine and tailor therapies to treat both or multiple illnesses, for example, nortriptyline for concomitant anxiety, topiramate or propranolol for associated headaches/hypertension).
Physiotherapy may play a role in treating some of the associated sequelae of vestibular migraine which include anxiety, visual dependence, and/or loss of confidence .
Those with interictal symptoms of imbalance could consider vestibular rehabilitation therapy as a potential treatment modality .
Currently, no surgical management is recommended for vestibular migraine and may result in worsening of illness is associated with Meniere syndrome (Wise SR & Sargent EW; presentation 2013).
Meniere syndrome and BPPV were discussed previously.
It is anticipated that nearly 50% of those with childhood vertigo are related to a migraine. Benign paroxysmal vertigo of childhood is the most common cause of vertigo between the years of 2 and 6 with a prevalence estimated at 2.6% between the ages of 5 and 15 . This typically presents with seconds to minutes of vertigo/dizziness, nystagmus, postural imbalance, nausea or vomiting with the return to baseline between episodes. Children may outgrow this condition, as many are relieved from its burden after their sixth birthday.
Chronic subjective dizziness (CSD) is an entity that includes chronic unsteadiness or dizziness without vertigo. Additionally, it presents with hypersensitivity to motion stimuli, poor tolerance of visual stimuli or precision visual tasks. This condition must last three or more months to make the diagnosis. It has been found that CSD often coexists with other recurrent vertigo illnesses .
The following diagnoses commonly replace a vestibular migraine when the patient is followed for an extended period: Meniere syndrome, benign paroxysmal positional vertigo, psychogenic dizziness, spinocerebellar ataxia. The following must be ruled out before making the diagnosis: episodic ataxia type 2 and transient ischemic attacks .
No studies have been discovered that show any prognostic variables associated with vestibular migraine.
Clinical experience has shown varying levels of disability and responsiveness to treatments amongst the vestibular migraine population.
No operative therapy is currently recommended for a vestibular migraine and may actually worsen clinical state (Wise SR & Sargent EW at presentation given 2013).
Physiotherapy may play a role in treating some of the associated sequelae of a vestibular migraine which include anxiety, visual dependence, loss of confidence .
Neurotologic and/or neurology evaluation seems to be a reasonable recommendation.
Convincing a patient of the diagnosis of a vestibular migraine can prove difficult and lead to delay in treatment or absence of treatment. This is understandable based on the fact that vertiginous symptoms are often asynchronous with a headache and may be a difficult connection for the practitioner or patient to grasp .
A vestibular migraine is an elusive diagnosis with variable presentation and without pathognomonic testing despite significant associated morbidity
Familiarity with recently accepted Barany Society and the International Headache Society diagnostic criteria is important to make a diagnosis. See below:
A vestibular migraine can present at any age, although middle-aged women are most commonly affected
One theory exists that a vestibular migraine may be caused by vestibular end-organ disease; therefore, regular patient monitoring is merited to evaluate for possible contributing morbidity
A vestibular migraine may be associated with other illnesses such as Meniere syndrome or BPPV. The verdict is currently out regarding the chronology of events/triggers. Do Meniere attacks trigger a vestibular migraine, or does a vestibular migraine give rise to Meniere syndrome features?
The diagnosis and management of vestibular migraine is complex and best done with a multidisciplinary team. There are many treatments for vestibular migraine but there is no specific treatment that works in everyone. Current treatment options include lifestyle changes such as caffeine omission, avoidance of known dietary, behavioral or sleep hygiene triggers. Prophylactic medications such as topiramate, nortriptyline, propranolol may provide some benefit despite few controlled studies on the matter. Vestibular rehabilitation may play a role. Treatment of associated anxiety is a necessity.
The prognosis for these patients remains unknown but anecdotal reports indicate that recurrences are common.
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