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Migraine Associated Vertigo (MAV) -- Vestibular Migraine (VM)

Timothy C. Hain, MD Page last modified: July 20, 2014

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You may also be interested in our many other pages on migraine on this site

See also: Understanding your dizziness and balance disorder DVD (segment on migraine)

Dizziness and headache are individually very common human conditions and their combination is also a common symptom complex. Diagnostically, one must determine whether the dizziness and headaches are independent or related to each other, and in particular, whether they are a manifestation of migraine. Here we will review the association between vertigo and migraine. This subject has also been recently reviewed by Reploeg and Goebel (2002) as well as Radke et al (2002).

Epidemiology (how many people have MAV)

MAV venn

Migraine is one of the major causes of vertigo (with BPPV being the only one causing more).

About 14% of the adult population of the United States has migraine. The distribution differs between male and females. At all ages, about 5% of men have migraine (Stewart, 1994; Lipton et al, 2002). Women of childbearing age have a much higher prevalence, jumping up to roughly 10% at the onset of menstruation, and increasing to nearly 30% at the peak age of 35 years. At menopause, rates of migraine abruptly decline in women back to roughly 10%.

The figure below shows the distribution of migraine with dizziness in a subset of patients from the dizziness practice of the author. Although the populations being drawn from is different, and in particular includes very few children, it illustrates that migraine with dizziness is most commonly encountered in persons between 40 and 70, and that the female:male ratio is about 3:1.

MAV age

 

Migraine headaches are often misdiagnosed by patients themselves as sinus headaches. Schreiber suggested that 88% of 2991 patients who had diagnosed themselves as having sinus headache, actually had migraine (Schreiber et al, 2004).

Mav MEN VENN

Migraine occurs frequently with several other causes of dizziness. The prevalence of migraine (13-14%) is far higher than that of Meniere's disease, which occurs in only 0.05% (1/2000) of the US population (Wladislavosky-Waserman et al, 1984).

In a small study of persons with Menieres disease, the prevalence of Migraine was about 50%, compared with a figure of about 25% in the non-Meniere's population (Radke et al, 2002). This is shown on the diagram above, where there is an overlap between Meniere's disease and Migraine.

Other studies have shown different results however. There have also been recent studies showing that there is a higher frequency of BPPV in persons with Migraine, as well as vice versa (Ishiyama et al, 2000; Uneri 2004), and about half of persons with BPPV onset before the age of 50 meet criteria for migraine. Occasionally, of course, patients with migraine will have other disorders such as brain tumors. Go HERE to see an example of this rare situation.

Table 1: Patients with Migraine having Vertigo
Percent of migraine patients with vertigo Comment Authors
26.5 % Unsolicited migraine (n=200) Kayan and Hood (1984)
33 %   Selby and Lance (1960)
42 % Migraine with aura Kuritzky et al (1981)

In practices focused on treating migraine, 27-42 % of patients report episodic vertigo (See table 1). A large number (about 36%) of these patients experienced vertigo during headache-free periods. The remainder experienced vertigo either just before or during the headache. The incidence of vertigo during the headache period was higher in patients with aura as opposed to in those without aura.

In practices focused on treating vertigo, 16-32% of patients have migraine (Savundra et al, 1997). The prevalence of migraine in the general population is roughly 13-14% (Stewart et al, 1994). The prevalance of migraine with vertigo in Germany has recently reported to be 1% (Neuhauser, Radtke et al. 2006). This seems a bit low to us, but it seems reasonable to conclude that there is an immense amount of migraineous vertigo, and that this migraineous vertigo is certainly the most common cause of vertigo in the general population.

Table 2: Patients with Migraine having Motion Sickness
Percent of migraine patients with motion sickness Comment Authors
49% Children Bille (1962)
45% Children (60) Barabas et al (1983)
50.7% Unselected Kayan and Hood (1984)

Motion sickness is a common migraine accompaniment. Most studies report about 50% of patients with migraine have motion sickness, compared to about 5-20% for control groups.

Syncope can also accompany migraine, and this offers another entirely separate mechanism. In migraine, hypotension is likely hormonal, and is attributed mainly to vasopressin (Gupta, 1997).

Fortification spectra, as might be seen in Migraine with aura. Scotoma with aspects of a fortification.

What causes the vertigo in MAV ?

There are several theories -- 

There has also long been noted a close association between Meniere's disease and Migraine.

Diagnosing MAV

MAV is one of a group of conditions that encompasses many dizzy patients (including Meniere's disease, chronic subjective dizziness, cervical vertigo), that are diagnosed from the pattern of symptoms rather than from a "blood test" or "X-ray".  These type of conditions nearly always are at least somewhat controversial, and have proponents as well as detractors.  They always have the clause "not better accounted for by ...", which means that they are "wastebasket" diagnoses.

Recently the international headache society, a body that considers itself the final authority on any condition that includes subjective head pain, has released their viewpoint (2013).  As this is a committee, and presumably there was some discussion, it is a reasonable place to start although it is not all that reasonable (see below).

The IHS state in an appendix to their most recent diagnostic paper, state that the proper name for MAV is actually "Vestibular migraine", and that the terms "migraine-associated vertigo/dizziness", migraine-related vestibulopathy, and migrainous vertigo are "previously used terms:".  This seems very pretentious as the IHS cannot control what terms people use for symptom collections like this. 

Well anyway, the criteria according to the IHS are:

A. At least 5 episodes fulfilling criteria C and D.

B. A current or past history of migraine with or without aura, using the IHS criteria.

C. Vestibular symptoms of moderate or severe intensity, lasting between 5 minutes and 72 hours.   We find this criterion vague and also odd. What exactly is "moderate or severe" ?  Why stop at 72 hours. 

D. At least 50% of episodes are associated with at least one of the following 3 migrainous features:

1. headache with at least two of the following four characteristics:

As criteria 1c and 1d will always be met, this criterian boils down to 50% of episodes with a headache.

2. Photophobia AND phonophobia. We don't see why both are required.  There are also many more sensory exaggerations that are left out here too.

3. Visual Aura. This is so rare as to be nearly useless

E. Not better accounted for by another ICHD-3 diagnosis or another vestibular disorder.   This is the wastebasket clause.  One would wonder what if you are dealing with another wastebasket syndrome -- such as CSD or cervical vertigo. 

The author of this page, Dr. Hain, based on 1000's of patient care experiences, uses the following simpler criteria to assign the diagnosis of MAV.

Our thought is that a better and much more sophisticated method would be to use something like a very experienced physician uses -- record an array of symptoms/intensity and signs, assign a weight to each one, and use an algorithm (perhaps linear discriminant analysis) to compute the probability of a diagnosis.

Testing for MAV

There is no "test" for MAV that by itself, is specific and diagnostic. MAV is usually diagnosed from the clinical pattern, and by excluding alternatives. Logically, MAV should be detectable by identifying sensory hypersensitivity, in the context of dizziness and headache. However, so far little has been done to quantify sensory hypersensitivity(such as allodynia) in this population.

For the most part, ENG inner ear testing in MAV is normal, but a low-level positional nystagmus is very common (Polensek and Tusa, 2010).  We agree with this -- especially pure upbeating supine. We also think there is more downbeating upright.

Hearing testing in MAV is generally normal, but in our large MAV clinic population we sometimes see bilateral reduction of hearing at low frequencies. It resembles early Meniere's disease, but is bilateral.

On rotational chair testing, there is often an increased VOR time constant (Jeong et al, 2010). We have not noticed this in our patients and we are dubious that this is true. The same authors also felt that there were higher than normal motion sensitivity susceptibility scores (seems reasonable), and often (21%), perverted head-shaking nystagmus (we are dubious). As pHSN is unusual in other syndromes, finding it in a patient who otherwise fits the criteria for migraine is helpful.

MAV syndromes

Migraine without aura (about 80%) and migraine with aura (about 15-20%) are the most prevalent forms of migraine and also are the most prevalent types of migraine associated with dizziness and vertigo. Symptoms include true vertigo with or without nausea and vomiting, and motion intolerance. Headache is usual but not required (see following). Auditory symptoms are common but usually bilateral (see following).

Sensory amplification is very common -- Hyperacusis is common in migraine, which may differentiate it from most ear disorders. Sensitivity to light (photophobia) is also commonly present. Photophobia is not at all specific to migraine however, and can also accompany migraine imitators such as meningitis, and vertigo imitators such as Cogan's syndrome. Other sensory amplifications which are common in persons with migraine include allodynia (pain from stimuli that are not painful in most people), sensitivity to weather changes, motion sensitivity, and medication sensitivity.

When patients are examined acutely when vertiginous, there is usually minimal or no spontaneous nystagmus. This provides a differential feature from most peripheral vestibular syndromes. When nystagmus is present, it is often directed vertically (e.g. upbeating or downbeating). Vertically directed spontaneous nystagmus is unusual in other contexts, providing another differential point.

Timing. Cutrer and Baloh (1992) found a bimodal distribution of duration of vertigo with 31% of individuals having spells that typically lasted a few minutes to 2 hours and 49% having spells that lasted longer than 24 hours. Symptoms lasting months are possible (Waterson, 2004). Therefore by duration, these episodes could be confused with those due to BPPV, Menieres, or even vestibular neuritis.

Although migraines are usually episodic, they can be chronic too.  Chronic migraine is the most severe of all migraine syndromes, with headaches averaging grater than 15 days/month.  Each year, about 2.5% of those with episodic migraine develop chronic migraine (Manack et al, 2011). In our practice in Chicago, we encounter many persons who are extremely motion sensitive, have visual sensitivity, and sound sensitivity, lasting months ! These persons usually respond to migraine prevention medication. Similar patients with chronic symptoms, even with few headaches, have been reported by others (e.g. Waterson, 2004)

***** NOTE that Headache is not required to make the diagnosis of MAV.

As in migraine, occasionally aura may occur without headache (acephalgic migraine), it also follows that vertigo may occur without headache. Examples are as follows:

Benign recurrent vertigo of adults, essentially a vertiginous migraine aura without headache, was described first by Slater (1979) but his observations have been confirmed by others (e.g. Lee et al, 2002; Cha et al, 2009). It consists of spells of vertigo, which can include tinnitus, but without hearing loss (were hearing loss allowed, this disorder would become very difficult to distinguish from Menieres disease ). Vertigo lasts from minutes to hours. According to Cha et al, sensory amplifications such as photophobia or auditory symptoms were uncommon in patients without headache, but were common in patients with headache. Not all authors agree that BRV is caused by migraine however, and Leliever and Barber suggested that it is caused by peripheral vestibular lesions (Leliever and Barber, 1981). At this writing (2012), this idea seems to have been discarded.

Another example of a similar recurrent vertigo, without headache, attributed to migraine is the Benign Paroxysmal Vertigo syndrome of children, as described below under the heading of familial syndromes, where headache does not occur. With respect to timing, Cutrer and Baloh (1992) also observed that dizziness and headaches are not necessarily closely associated. In fact, in their 91 patients, only 5 had a consistent recurring dizziness with headache. In 30%, dizziness was consistently independent of headache. In most, spells sometimes occurred with and sometimes independently.

Basilar Migraine or Basilar Artery Migraine (BAM )

Basilar Migraine, also known as Bickerstaff ‘s syndrome(1961), consists of two or more symptoms (vertigo, tinnitus, decreased hearing, ataxia, dysarthria, visual symptoms in both hemifields of both eyes, diplopia, bilateral paresthesias or paresis, decreased LOC) followed by a throbbing headache. Vertigo typically lasts between 5 minutes and one hour. In the author’s practice, the typical patient is a woman of about 35 years of age, who attacks of vertigo combined with headache. The family history is often positive. In the differential are TIAs and paroxysmal vestibular disorders accompanied by headache. Patients usually respond to diet or the usual migraine prophylactic drugs.

Auditory symptoms are rare compared to vestibular symptoms (Battista, 2004) but nevertheless there is good evidence that hearing loss and tinnitus do occur. Olsson (1991) in a study of 50 patients with basilar migraine (which is rare) documented a fluctuating low-tone sensorineural hearing loss in more than 50% of his patients, and about 50% of his patients noticed a change in hearing immediately prior to their migraine headaches. Virre and Baloh (1996) suggested that sudden hearing loss may also be caused by migraine. Hearing loss in migraine rarely progresses (Battista, 2004).  Only minor changes of no significance are found in formal tests of auditory function in persons with migraine (Hamed et al, 2011).

Tinnitus is also common in migraine (Kayan and Hood, 1984; Olsson, 1991). Because the formal criteria for Menieres disease (audiometrically documented hearing loss (not fluctuation), episodic tinnitus and/or fullness, episodic vertigo) are a subset of the documented spectrum of basilar migraine, there is the possibility for diagnostic ambiguity (Harker, 1996). Boismier and Disher reported that 6% of 770 patients who presented with vertigo fell into an ambiguous diagnostic situation between Meniere's and Migraine (2002). When headache is not prominent, features such as bilateral hearing fluctuation (according to Harker (1996) auditory symptoms are rarely unilateral), family history of migraine and perimenstrual exacerbations are used to decide whether Menieres or migraine is the more likely diagnosis.

Benign Paroxysmal Vertigo of Childhood

This is a disorder of uncertain origin, possibly migrainous. It's initials (BPV) are easily confused with those of Benign Paroxysmal Positional Vertigo (BPPV), but it is not caused by the same mechanisms. This disorder consists of spells of vertigo and disequlibrium without hearing loss or tinnitus (Basser, 1964). The majority of reported cases occur between 1 and 4 years of age, but this syndrome seems indistinguishable from benign recurrent vertigo (BRV, see following) in adults which is presently attributed to migraine, or so-called "vestibular Menieres", which is also attributed to migraine. The differential diagnosis includes Menieres disease, vestibular epilepsy, perilymphatic fistula, posterior fossa tumors, and psychogenic disorders.

Cyclic Vomiting

This is a very disturbing disorder in which persons suddenly develop vomiting, generally without headache or hearing symptoms. It usually responds to migraine prevention medications. Vertigo is common (especially positional resembling lateral canal BPPV). It overlaps with migraine and Menieres, but pretty much by definition there is no headache or hearing loss (if it did, it wouldn't be called cyclic vomiting -- it would be called migraine or menieres). See this page for more.

Familial syndromes:

There has recently been a report of a familial vestibulopathy, called familial Benign Recurrent Vertigo (BRV) consisting of episodic vertigo with or without migraine headache. Presumably there are both familial and nonfamilial forms -- fBRV and BRV. The non-familial form is sometimes also called recurrent vestibular neuritis as well as vestibular menieres.

Vestibular testing in the familial form can document profound bilateral vestibular loss. The familial syndrome responds to acetazolamide (Baloh et al, 1994). It is not associated with a mutation on the calcium channel gene (Oh et al, 2001). Also reported by Baloh and associates, a form exists with episodic vertigo and essential tremor. This form is also responsive to acetazolamide. (Baloh et al, 1996). Familial hemiplegic migraine has been linked to mutations in the calcium channel gene (Ophoff et al, 1996). French-Canadian intermittent ataxia syndrome also may present similarly. While no mutations have been identified in the common form of migraine, calcium channels could be functionally impaired by subtle gene changes such as polymorphisms.

Antiphospholipid antibodies. There are some reports that individuals with severe migraine headaches are more likely to have antiphospholipid antibodies. In the authors experience, these patients may present with transient monocular visual loss, and some also have fetal wastage and complicated migraines as well as a reticular rash on the legs. (Donders et al, 1998). Recent authors have suggested that the association of migraine with APA is not a valid one.

Treatment of MAV.

For treatment of migraine in general see this page. A flowchart is given here. Because of the possibility for serious injury associated with vertigo, prevention is the advised treatment for most types of MAV. Eliminating dietary triggers and prophylactic medication treatment are the modalities used most frequently. Patients are initially told to abstain from foods such as chocolate, strong cheese, alcohol (especially red wine) and MSG containing preparations (such as oyster sauce). We also suggest magnesium supplements (500 mg/day).

If this is not successful, after a month, patients are started on one of the following -- topiramate, verapamil, a long-acting beta-blocker such as long acting propranolol, or an antidepressant such as amitriptyline or venlafaxine depending on gender and medicalsituation. Verapamil and amitriptyline are particularly useful because of their anticholinergic properties may help control vertigo independently of whether they are useful for migraine per se.

Migraine prevention

 


References:

Adapted from lecture handout given for the seminar "Recent advances in the treatment of Dizziness", American Academy of Neurology, 1997 and "Migraine Vs Meniere's", at the American Academy of Otolaryngology meeting, 1999-2001.

Copyright July 20, 2014 , Timothy C. Hain, M.D. All rights reserved. Last saved on July 20, 2014