Review

Cephalalgia
2020, Vol. 40(1) 107–121
Vestibular migraine: An update on current ! International Headache Society 2019
Article reuse guidelines:

understanding and future directions sagepub.com/journals-permissions

DOI: 10.1177/0333102419869317
journals.sagepub.com/home/cep

Tzu-Chou Huang1,2 , Shuu-Jiun Wang3,4 and

Amir Kheradmand1,5

Abstract
Background: Vestibular migraine is among the most common causes of recurrent vertigo in the general population.
Despite its prevalence and high impact on healthcare cost and utilization, it has remained an under-recognized condition
with largely unknown pathophysiology. In the present article, we aim to provide an overview of the current understand-
ing of vestibular migraine.
Methods: We undertook a narrative literature review on the epidemiology, presentations, clinical and laboratory
findings, pathophysiology, and treatments of vestibular migraine.
Results: Currently, the diagnosis of vestibular migraine relies solely on clinical symptoms since clinical tests of vestibular
function are typically normal, or difficult to interpret based on inconsistent results reported in earlier studies. The
challenges related to diagnosis of vestibular migraine lie in its relatively broad spectrum of manifestations, the absence of
typical migraine headaches with vestibular symptoms, and its very recent definition as a distinct entity. Here, we highlight
these challenges, discuss common vestibular symptoms and clinical presentations in vestibular migraine, and review the
current aspects of its clinical diagnosis and evaluation. The concepts related to the pathophysiology and treatment of
vestibular migraine are also discussed.
Conclusion: Vestibular migraine is still underdiagnosed clinically. Future studies are needed to address the pathophysio-
logical mechanisms and investigate effective treatment regimens.

Keywords
Vestibular migraine, vestibular, migraine, dizziness, vertigo, headache
Date received: 18 March 2019; revised: 30 June 2019; accepted: 7 July 2019

Introduction
Clinicians are no strangers to patients with migraine
symptoms and frequent dizziness. As a common neuro-
logical disorder, migraine affects about 15% of the
general population (1). Dizziness is also a common
symptom, accounting for up to 15% of visits in front-
line healthcare settings (2,3). Considering the preva-
lence of both conditions, an overlap in the clinical
presentations of vestibular symptoms and migraine
may not seem surprising at first. Several studies, how-
ever, have found a close association between dizziness
and migraine beyond coincidence (4–15). Dizziness is
more common in migraineurs compared with those suf-
fering from other headache subtypes such as tension-
type headache, suggesting a pathological link between
migraine and dizziness (9,16,17). In some migraine
patients, dizziness or vertigo is even more prominent
and debilitating than the headache (12). The link
between the vestibular symptoms and migraine is also
reflected by a higher incidence of migraine in patients
with recurrent dizziness who do not fulfill the criteria
for other vestibular disorders (18–23). Such clinical
1
Department of Neurology, The Johns Hopkins University School of
Medicine, Baltimore, MD, USA
2
Living Water Neurological Clinic, Tainan, Taiwan
3
Neurological Institute, Taipei-Veterans General Hospital, Taipei, Taiwan
4
Brain Research Center and School of Medicine, National Yang-Ming
University, Taipei, Taiwan
5
Department of Otolaryngology-Head and Neck Surgery, The Johns
Hopkins University School of Medicine, Baltimore, MD, USA
Corresponding author:
Shuu-Jiun Wang, Neurological Institute, Taipei Veterans General Hospital,
#201, Shi-Pai Road, Section 2, Taipei 112, Taiwan.
Email: sjwang@vghtpe.gov.tw
108
findings have prompted efforts to recognize and classify
migraine-associated dizziness under a distinct diagnos-
tic entity.
Historically, different criteria have been applied to
define a clinical entity that can incorporate vestibular
and migraine symptoms and, along the way, various
terms such as migraine-associated dizziness, migraine-
related vestibulopathy, migrainous vertigo, benign
recurrent vertigo, and vestibular migraine have been
proposed (5,7,8,11–13,15). Among these terms, vestibu-
lar migraine (VM) has become widely accepted as it
links vestibular and migraine symptoms, and it is also
compatible with the terminology of the International
Classification of Headache Disorders (ICHD). The
operational criteria for VM diagnosis were proposed
in 2004 by Neuhauser et al. (24). These criteria were
later validated and revised with the consensus of the
International Headache Society (IHS) and the commit-
tee for the International Classification of Vestibular
Disorders (ICVD) of the Bárány Society (Table 1)
(25–27). As included in the VM diagnostic criteria,
the presence or history of migraine is essential for its
diagnosis, but the headache and vestibular symptoms
do not need to temporally coincide.
Table 1. Diagnostic criteria for vestibular migraine based on
the International Classification of Headache Disorders, 3rd
edition (ICHD-3) (25) and the International Classification of
Vestibular Disorders (ICVD) of the Bárány Society (26).
Vestibular migraine (ICHD-3 and ICVD)
A. At least five episodes fulfilling criteria C and D
B. A current or past history of 1.1 Migraine without aura or 1.2
Migraine with aura
C. Vestibular symptoms of moderate or severe intensity,
lasting between 5 minutes and 72 hours
D. At least 50% of episodes are associated with at least one of
the following three migrainous features:
1. Headache with at least two of the following four charac-
teristics:
a) Unilateral location
b) Pulsating quality
c) Moderate or severe intensity
d) Aggravation by routine physical activity
2. Photophobia and phonophobia
3. Visual aura
E. Not better accounted for by another ICHD-3 diagnosis or
by another vestibular disorder
Probable vestibular migraine (ICVD)
A. At least five episodes with vestibular symptoms of moder-
ate or severe intensity, lasting 5 min to 72 hours
B. Only one of the criteria B and D for vestibular migraine is
fulfilled (migraine history or migraine features during the
episode)
C. Not better accounted for another vestibular or ICHD
diagnosis
Cephalalgia 40(1)
Patients with VM symptoms frequently report sen-
sitivity to head motion and visual surroundings or dis-
abling misperceptions such as a sudden feeling of
imbalance or tilt. When it comes to characterizing ves-
tibular symptoms, dizziness or vertigo are often loosely
used terms that may convey different symptom experi-
ences in different people. According to the ICVD (28),
vertigo is a sensation of self-motion when no self-
motion is occurring or a sensation of distorted self-
motion during an otherwise normal head movement.
Dizziness is defined as a sensation of impaired spatial
orientation without a distorted sense of motion. In fact,
dizziness is a broader term that encompasses false spin-
ning sensations (i.e. spinning vertigo) and also other
false sensations such as swaying, tilting, or veering
(i.e. non-spinning vertigo). In this classification,
having dizziness does not preempt inclusion of vertigo
if both symptoms are present as defined by their cri-
teria. Vestibular symptoms do not include a pure sen-
sation of impending fainting (presyncope), disordered
thinking (mental confusion), or detachment from real-
ity (depersonalization). Likewise, the term dizziness
should not be applied if the patient experiences a gen-
eralized weakness or a non-specific sense of malaise or
fatigue.
Unlike a typical migraine aura, which can last
5–60 minutes, vestibular symptoms in VM sufferers
can persist for hours, leading to severe impairment of
daily activities (25,26,29,30). This is a common clinical
scenario in VM patients, which often becomes challen-
ging for clinicians and costly for patients to treat, indi-
cating an unmet need to delineate VM pathophysiology
(31). In this article, we review common VM symptoms
and clinical presentations. The current concepts related
to VM pathophysiology and its treatment are dis-
cussed. Also, some of the barriers in addressing the
neural mechanisms of dizziness, vertigo, and visuo-
spatial symptoms in VM patients are highlighted.
Epidemiology and demographic factors
Vestibular migraine is a common cause of spontaneous
vertigo in both children and adults (32–34). In children,
benign paroxysmal vertigo of childhood is considered
an early manifestation of migraine affecting about 3%
of children between ages 6 and 12 (35,36). A recent
nationwide study in the United States based on the
ICHD-3 criteria found a VM prevalence of 2.7% in
adults (37). In a large population study in Germany,
VM lifetime prevalence was estimated at about 1%,
and its one-year prevalence was about 1% (12). In a
community-based study of middle-aged women, the
one-year VM prevalence was higher, at about
5% (38). Despite classification efforts in recent years,
VM has remained clinically underdiagnosed.
Huang et al.
Vestibular migraine is reported to account for 4–10%
of diagnoses in specialized dizziness and headache
clinics, although these numbers reflect different inclu-
sion criteria among various studies (11,39–41).
According to a study that examined data from a ter-
tiary dizziness center, only 2% of patients were sus-
pected to have VM by referring doctors, whereas
20% were later diagnosed as VM by specialists (42).
In a Korean multicenter study, about 10% of migrain-
eurs were diagnosed with VM in their first visit to neur-
ology clinics (43). Another study found that only 10%
of patients who met the VM diagnostic criteria were
told that migraine was the cause of their vestibular
symptoms (37).
Similar to other subtypes of migraine, VM has a
female preponderance, with a reported female to male
ratio of 1.5–5 to 1 (5,7,8,11,12,44,45). The reported age
of onset for VM symptoms is between 8 and 50 years
old or even older (median ages being the mid-30s
to 40s) (12,21,39). Usually, migraine headache tends
to present first, and patients may be headache-free
for years before the onset of vestibular symptoms. In
one study, the mean duration between the onset of
headache and vestibular symptoms was about eight
years (46). In women, vestibular symptoms can
become more pronounced around the time of meno-
pause (47).
In keeping with the heritability and genetic back-
ground of migraine disorders, familial occurrence has
been reported in some VM patients, with an autosomal
dominant pattern of inheritance and a decreased pene-
trance in men (48). Within a family, there could be
multiple affected individuals, some with VM and
some with other migraine variants. In a survey of
family members in patients with chronic vertigo
(and no auditory or neurologic disorders), half of the
biological relatives who reported vertigo met the
diagnostic criteria for migraine, as opposed to a small
percentage of unrelated spouses who also reported
vertigo (13).
Clinical presentation: Basis for VM
diagnostic criteria
Common VM features: Vestibular migraine patients
may have a history of migraine earlier on in their
lives, and their migraine symptoms are often variable
in nature, duration, or temporal relation to their ves-
tibular symptoms (5,11,49). The core migraine features
in VM patients are similar to those of migraine with or
without aura. VM patients also report similar migraine
triggers such as sleep disruption, menstruation, stress,
or specific foods (e.g. aged cheese, red wine, or mono-
sodium glutamate) (11,50,51). Not uncommonly, VM
patients develop vestibular symptoms after the
109
headache subsides, and in some patients headache
and vestibular symptoms never occur together. Also,
a minority of VM patients may experience vestibular
symptoms in the time frame of 5–60 minutes, as defined
for the duration of aura, and even fewer patients have
these symptoms immediately before the headache
starts. Apart from the headache and vestibular symp-
toms, VM patients may experience photophobia, pho-
nophobia, or visual aura. In a case series, 60% of VM
patients reported phonophobia, 70% photophobia, and
36% migraine auras (11). In another study, 87% of
VM patients had photophobia, 86% phonophobia,
64% headache, and 13% migraine auras (52).
Phonophobia refers to a sound-induced discomfort,
which is a bilateral, transient phenomenon distinct
from unilateral or persistent aural symptoms. Other
intermittent auditory symptoms, such as a sense of
pressure or fullness in the ears or tinnitus, have been
reported in up to 40% of VM patients (5,8,9,52–55).
However, since these symptoms are non-specific and
also common in other vestibular disorders, they were
not included in the VM diagnostic criteria (26). Of note,
migraine features must be present at least half of the
time along with vestibular symptoms in order to fulfill
the criteria for VM diagnosis. At least one migraine
feature is needed along with the vestibular symptoms,
but different migraine features may be present at differ-
ent times. Although the co-occurrence of migraine and
vestibular symptoms is required by the ICHD-3 criteria
for VM diagnosis, the Bárány classification ICVD
has recognized the heterogeneity of migraine presen-
tation in VM patients, and those patients who only
have a history of migraine without co-occurrence of
migraine features and vestibular symptoms are included
under a subcategory of probable vestibular migraine
(Table 1) (26).
Vestibular symptoms: In VM patients, vestibular
symptoms are generally triggered or aggravated by
changing position, self-motion, or visual motion
within the surrounding environment. These patients
may describe their symptoms as a sense of spinning,
floating, rocking, tilting, swaying, or as being off-
balance, off-kilter, lightheaded, foggy, or unsteady
(Table 2). These various terms may reflect different
internally experienced phenomena. Analogies such as
feeling drunk or sea-sick, getting off a roller-coaster
or merry-go-round, or even walking on air or pillows
are also commonly used by the patients. Such diverse
nature of vestibular symptoms is recognized in the VM
diagnostic criteria as different forms of vertigo or
dizziness, including spontaneous vertigo, positional
vertigo, visually induced vertigo, and head motion-
induced vertigo or dizziness (Table 2). Spontaneous
vertigo is defined as a false sense of motion, either
as internal vertigo with primarily a sensation of
110
Table 2. Vestibular symptoms in vestibular migraine patients
and relevant terminology defined by the International
Classification of Vestibular Disorders (ICVD).
Terms usually used by patients
Spinning, rocking, tilting or swaying
Unsteady, off-balance or off-kilter
Lightheaded
Foggy, clouded or disoriented
Drunk or seasick
Floating sensation, or walking on air or pillow
Coming off a roller-coaster or merry-go-round
Vestibular symptoms defined by ICVD
Spontaneous vertigo as a false motion sensation of self or
surrounding
Visually induced vertigo
Positional vertigo (after a change of head position)
Head motion-induced vertigo (during head motion)
Head motion-induced dizziness (sensation of disturbed spatial
orientation)
self-motion, or as external vertigo with primarily a
visual sensation of motion within surroundings.
Positional vertigo refers to a false sense of motion
after changing head position, and visually induced ver-
tigo is characterized as a false sense of self-motion trig-
gered by complex or large moving visual stimuli. Head
motion-induced vertigo refers to a distorted sense of
motion during head movement. This is different from
positional vertigo that occurs after a new head position
is adopted. Head motion-induced vertigo is also distin-
guished from motion sickness, in which the dominant
symptom is nausea, and not vertigo. Various studies
have reported spontaneous vertigo in 20–85% of VM
episodes, positional vertigo in 18–60%, and head
motion intolerance in 30–80% of the episodes
(5,6,9,36,39,44,52,53,56). These symptoms may vary
in individual patients, but spontaneous vertigo is the
most common reported vestibular symptom, followed
by positional vertigo (12,57), head motion-induced ver-
tigo, and visually induced vertigo (5,58,59). The term
‘‘dizziness’’ is also included in the VM diagnostic cri-
teria and is defined as a sense of disturbed spatial orien-
tation. However, it is not clear why the current VM
criteria only mention head motion-induced dizziness
with nausea, and other related terms such as spontan-
eous, positional, or visually induced dizziness are not
included.
The duration of vestibular symptoms in VM patients
is quite variable (9,11,39,52). About 30% of patients
have symptoms lasting for minutes, 30% lasting for
hours, and 30% lasting for several days at a time.
The other 10% often report fluctuating daily symp-
toms. Nausea, vomiting, and susceptibility to motion
sickness are also frequent in VM patients; however,
Cephalalgia 40(1)
these symptoms are non-specific and may also
occur with other vestibular disorders (9,10,60–62).
Vestibular symptoms are considered moderate when
they interfere with daily activities and considered
severe when they prevent daily activities.
Clinical overlap in VM presentations: Any vestibular
disorder can be complicated by superimposed migraine
attacks, in which cases VM is not the primary culprit.
For example, migraine is more common in patients
with benign paroxysmal positional vertigo (BPPV)
than in age- and sex-matched controls (11,44,63–65),
and having migraine could be associated with an
increased risk of developing BPPV (66). Vertigo can
also be a feature of migraine with brainstem aura, but
other symptoms related to brainstem involvement (e.g.
dysarthria) are typically not seen in VM patients.
Thus, with such a wide range of clinical presentations,
the co-occurrence of migraine and vestibular symp-
toms is not specific to VM patients, and other dis-
orders with similar symptoms should always be
considered in the differential diagnosis (Figure 1). In
this scheme, the distinction between VM and other
vestibular disorders such as Ménière’s disease can
become unclear, especially since the auditory and ves-
tibular signs of labyrinthine pathology may be absent
in the early stages of Ménière’s disease (67,68).
Therefore, it is not surprising that both conditions
are often reported to coincide (55,68–71). Migraine
is more commonly reported in patients with
Ménière’s disease than in otherwise healthy individ-
uals, and about 13% of the patients meet the diagnos-
tic criteria for both VM and Ménière’s disease (72).
Such association has prompted theories about a link
between these two conditions versus those that attri-
bute the overlap to the secondary migraine symptoms
provoked by vestibular symptoms in Ménière’s dis-
ease. Whether there is a pathological link between
these two conditions remains unclear. Vestibular
symptoms in VM patients are also similar to those
of another clinical entity that was recently defined as
persistent postural-perceptual dizziness (PPPD)
(73,74). Similar to VM, PPPD patients have symptoms
consistent with perceptual dysfunction in spatial orien-
tation and also experience fluctuating dizziness or
unsteadiness, provoked by postural changes or visual
motion. Both VM and PPPD patients may have
comorbid psychiatric conditions such as anxiety and
depression (44,75–78). Also, migraine is one of the
most common conditions associated with chronic
visuospatial symptoms in PPPD patients (about
15–20% of cases) (44,73). Since VM and PPPD are
both defined only by clinical symptoms and have
unknown pathogenesis, it is not clear whether the
overlap in their clinical presentations represents a
pathological link between these two conditions.
Huang et al. 111

Right head impulse Left head impulse

300 300
Covert Covert

Velocity [°/s]

Velocity [°/s]
saccades saccades
200 200

100 100

0 0

0 100 200 300 0 100 200 300

Time [ms] Time [ms]

Figure 1. Video head impulse testing (vHIT) shows bilateral vestibulopathy in a patient with clinical presentation consistent with
vestibular migraine (VM): 37 year old Navy man with 2-year history of fluctuating dizziness, described as feeling of being drunk lasting
for hours, and attacks with spinning sensation lasting for minutes without headaches. He reported frequent headaches, twice a month,
associated with dizziness, nausea, photophobia and phonophobia since age 25. Bedside neurological and vestibular examinations were
only remarkable for difficulty in tandem gait. The vHIT shows reduced vestibular gains in the horizontal canal planes at 0.7, calculated
as eye velocity (black traces) divided by head velocity (red traces: Right side and blue traces: Left side). There are also corrective
saccades in the eye traces. These ‘‘covert’’ saccades are embedded in the deficient vestibulo-ocular responses during head rotation and
are difficult to discern with the naked eye. Cases like this show that vestibular dysfunctions can be complicated by superimposed
migraine attacks; however, VM is not the primary culprit.

under Frenzel goggles). The nystagmus was low-velo-

Clinical finding and laboratory testing
Vestibular migraine is a diagnosis primarily based on
clinical history, with currently no pathognomonic clin-
ical sign or laboratory test that can verify its diagnosis.
Vestibular laboratory abnormalities are quite variable
among VM patients, which may reflect inconsistent
findings regarding the existence of a peripheral vestibu-
lar component. Nevertheless, vestibular testing is still
helpful to rule out other disorders considered in the
differential diagnosis (Figure 1). Neurological examin-
ation is usually normal in VM patients during symp-
tom-free periods. However, some mild, non-specific
vestibulo-ocular abnormalities have been reported in
association with VM. Pursuit abnormality is reported
in up to 48% of VM patients, spontaneous nystagmus
in about 10% of patients, and central positional
or gaze-evoked nystagmus in up to 28% of patients
(5–7,45,59,79–84). These non-specific ocular motor
abnormalities increased over time, from 16% to 41%
of patients during a follow-up after 5.5 to 11 years (85).
Apart from pursuit dysfunction, the most frequent
ocular motor abnormality is central positional nystag-
mus. In a small study, about 70% of patients were
found to have pathological nystagmus during their
VM attacks (86). Another study found nystagmus was
provoked by horizontal headshaking in 35% of VM
patients, and spontaneous nystagmus in 19% of the
patients during vestibular attacks (87). Head shaking-
induced nystagmus is also reported in between the
attacks (79,81). Vestibular migraine patients were also
found to have positional nystagmus after removal of
visual fixation (e.g. vestibulo-ocular examination
city and sustained during the vestibular attack, but it
dissipated during the symptom-free period.
While some studies have found a higher incidence of
central vestibular dysfunction in VM patients (39,86),
others have found no significant central vestibulo-
ocular findings (76,88), or have reported a higher inci-
dence of peripheral vestibular dysfunction in VM
patients (6,23,80,89). Such variability could be related
to lack of standard diagnostic criteria among earlier
studies or a sole reliance on clinical symptoms by the
current VM criteria, which does not prevent inclusion
of patients with other vestibular disorders who may
have superimposed migraine symptoms. In this context,
findings such as asymmetry in caloric responses
(reported in up to 20% of VM patients), or vestibular
loss with video head impulse testing (vHIT) cannot be
specific to VM and may represent a secondary migraine
syndrome triggered by uncompensated vestibular dys-
function (Figure 1) (5,6,39,80,85,90–92).
The cervical and ocular vestibular evoked myogenic
potentials (cVEMP/oVEMP), the widely used labora-
tory tests of otolith function, have also shown conflict-
ing results in VM patients. Some studies have reported
reduced amplitude or delayed latencies of VEMP
responses (93–98), whereas other studies have found
asymmetrical VEMP responses with normal latencies
and amplitudes (99,100). Three recent studies using
ICHD criteria have reported abnormal oVEMP but
normal cVEMP responses in VM patients, which dif-
fered from the pattern of VEMP responses in normal
controls (101) or patients with Ménière’s disease. A sig-
nificant vestibulo-ocular finding that could be related to
112 Cephalalgia 40(1)

Vestibular migraine Healthy control

Right
Right
80 80

Eye velocity °/sec

Eye velocity °/sec
0 0

Left
Left
–80 –80
0 10 20 30 0 10 20 30
Time Time
TC Right: 30 sec Gain right: 0.8 TC Right: 7 sec Gain right: 0.8
TC Left: 33 sec Gain left: 0.8 TC Left: 7 sec Gain left: 0.8

Figure 2. Vestibulo-ocular responses during step velocity rotations (60 /sec) in a VM patient versus a healthy control. Data points
show the slow phase velocity of vestibular nystagmus induced by rotation around the body axis to the right (positive values) and left
(negative values). The gains of vestibular responses (maximum eye velocity/60 /sec step velocity) are normal in both the VM patient
and the healthy control; however, the durations of nystagmus measured as time constant (TC) are longer in the VM patient compared
to the healthy control (30–33 sec versus 7 sec). See reference 106 for rotational chair testing.

VM pathophysiology is a longer duration of post-rota-
tory nystagmus in VM patients compared with healthy
controls or migraine patients without dizziness (i.e.
increased time constant of the vestibulo-ocular reflex)
(Figure 2) (81). VM patients were also found to have
quicker modulation of the otolith-ocular response
during off-axis rotation (i.e. body centrifugation) (76).
These findings suggest an innate hypersensitivity of the
vestibular system in these patients. Balance impairment
is also commonly reported in VM patients, including
abnormal Romberg and sensory organization test
(SOT) (76,79,86,89,91,92). Studies using static posturo-
graphy have found increased sway in VM patients com-
pared with healthy controls (80,104). Hearing
impairment is reported in about 8% of VM patients,
which is often mild and non-progressive, as opposed to
progressive low frequency hearing loss in Ménière’s
patients (40,85,105).
Pathophysiology
To date, VM pathogenesis remains obscure as the wide
ranges of clinical and laboratory findings do not seem
consistent with a uniform pathology. Such variability
comes from sole reliance on clinical symptoms and lack
of objective measures for diagnosis, which have ham-
pered efforts toward understanding VM pathophysi-
ology. Various mechanisms have been put forward to
explain VM symptoms and some are broadly inferred
from other migraine disorders (Figure 3). In this con-
text, altered neural activity within the trigeminovascu-
lar system (TVS) is considered the primary mechanism
for headache in migraine patients (107). The TVS
neuropeptides, such as substance P and calcitonin
gene-related peptide (CGRP), can cause vasodilation
and neurogenic inflammation, leading to throbbing
pain or ‘‘central sensitization’’ that often presents as
allodynia (108–110). Some of these neurotransmitters
are also expressed in the vestibular system (e.g.
CGRP and serotonin) and might be involved in VM
pathophysiology (108,111,112). The trigeminal nucleus
is connected to the contralateral thalamus, which in
turn sends projections to the temporal, parietal, insular,
and cingulate cortical regions. The nociceptive brain-
stem centers such as the nucleus raphe magnus,
periaqueductal gray and hypothalamic areas are
also connected with the TVS and vestibular nuclei
(113,114,114). These reciprocal connections can modu-
late neural activity within both the TVS and the
vestibular system (112,113,115–118). For example, tri-
geminal stimulation produced nystagmus in migraine
patients, suggesting increased vestibular excitability in
these patients compared with healthy controls
(116,119). Similarly, spontaneous nystagmus during
VM attacks or prolonged vestibular responses in
some VM patients (i.e. long time constant of vestibular
nystagmus) could be linked to vestibular hyperexcit-
ability in these patients (76,81,120). It is, however, not
clear whether such hyperexcitability is at the level of the
peripheral vestibular system or the brainstem, or
whether it could be related to the modulating effects
of the cerebellum or cerebral hemispheres on the ves-
tibular system.
The phenomenon referred to as cortical spreading
depression has been proposed as the neural correlate
of migraine aura (107). This is a depolarization wave
that slowly spreads across the cerebral cortex, followed
by a prolonged suppression of cortical activity. The
theory of cortical spreading depression cannot explain
chronic or fluctuating symptoms in migraineurs.
However, according to its proposed mechanism, symp-
toms such as hypersensitivity to light and sound could
Huang et al. 113

Thalamocortical Sensory dysmodulation

network Spatial disorientation

Nociceptive brainstem Trigemino-vascular

centers system Headache

Vestibular Vestibulo-ocular dysfunction

system Vestibular hypersensitivity

Figure 3. Possible mechanisms involved in the pathogenesis of vestibular migraine. Abnormal sensory modulation or integration
within the thalamo-cortical network could result in dizziness and spatial disorientation. Hyperactivity within the trigeminovascular
system (TVS) and nociceptive brainstem centers could result in headache. Altered activity in the vestibular system could lead to
transient vestibulo-ocular dysfunction or vestibular hypersensitivity associated with migraine features.

(a) (b) Dynamic roll tilt

VM patients 2
Control Normal
1.6 Migraine
Head upright Threshold
VM
1.2

0.8
*
0.4
Right head tilt
0
0.1 1
–4 0 4
Frequency (Hz)
L SVV R

Figure 4. (a) Spatial orientation measured as subjective visual vertical (SVV) errors in a group of VM patients versus healthy controls
(127). In the upright head position, there is no significant difference between VM patients and controls. During static lateral head tilt to
the right (20 ), SVV errors in VM patients are in the opposite direction of head tilt, consistent with overestimation of the head tilt
position for spatial orientation. (b) During low-frequency dynamic head tilt, VM patients show a lower motion detection threshold
compared with migraine patients without dizziness and healthy controls (modified with permission from Lewis et al. (125)).

be linked to a generally hyperexcitable brain state in
migraine patients (121). This has led to the concept of
sensory dysmodulation, in which deficient habituation
and potentiation of sensory responses are implicated in
migraine pathogenesis (122,123). Accordingly, expos-
ure to one sensory stimulus results in hypersensitivity
that can extend to other sensory stimuli. VM patients,
likewise, show higher sensitivity to motion in the roll
plane (e.g. lateral head tilt with respect to gravity)
(Figure 4) (120,124,125). Perception of tilt during
whole body centrifugation is also altered in VM
patients, suggesting that the integration of semicircular
canal and otolith inputs is affected in these patients.
During whole-body yaw rotations, VM patients were
found to have altered perceptual thresholds while
reporting direction of rotation (126). During lateral
head tilts, VM patients had larger errors in spatial
orientation compared with healthy controls (Figure 4)
(127). When the head is tilted, the brain must integrate
vestibular signals that encode head position with visual
inputs in order to maintain spatial orientation. The
larger errors of spatial perception in VM patients
were in the opposite direction of the head tilt, consist-
ent with overestimation of the head position in the pro-
cess of sensory integration for spatial orientation. In
keeping with this result, VM patients reported dizziness
mostly in the same head direction that induced larger
errors of spatial orientation. VM patients were also
found to have high variability in spatial orientation
with the head in the upright position (128,129).
114
Altogether, these findings suggest that visuospatial
symptoms in VM patients are related to altered sensory
processing and integration that contribute to the per-
ception of spatial orientation. Regarding the effect of
visual motion, VM patients had larger visual-induced
errors in spatial orientation during and after optoki-
netic stimulation compared with healthy controls
(126,130,131). These errors also increased significantly
in migraine patients with the duration of visual
stimulation (132). In addition, VM patients had larger
postural sway with visual motion compared with
healthy controls (76,131,133). Regarding other sensory
modalities, there is evidence for sensory dysmodulation
affecting the auditory system in VM patients. In the
presence of noise, otoacoustic emissions were less sup-
pressed in VM patients compared with healthy con-
trols, suggesting a link to phonophobia in these
patients (134).
Imaging studies suggest structural and functional
changes within the temporo-parietal regions in VM
patients (117,135,136). The higher-order neural mech-
anisms within these cortical regions are involved in
sensory integration for coherent spatial perception
(137). In addition, VM patients were also found to
have abnormal thalamic activities (117,138). These
patients showed increased thalamic activations with
cold water ear irrigation during fMRI studies, in com-
parison with migraine patients without aura or healthy
controls (138). The magnitude of thalamic activation
was positively correlated with the frequency of migraine
attacks in VM patients. Also, increased ictal PET activ-
ities during vertigo attacks in VM patients were noted
in the temporo-parieto-insular areas and bilateral tha-
lami (117). These findings are in agreement with the
studies that show the role of the thalamus as a major
sensory relay within the vestibular pathways, and its
involvement in multisensory processing and integration
including vestibular, visual, and somatosensory inputs
(139,140). However, little is known about these sensory
processes and how these higher-level networks could be
affected in VM patients.
The familial occurrence of VM suggests a genetic
component in its pathogenesis. Although there are
some familial migraine syndromes such as hemiplegic
migraine or episodic ataxia, similar mutations (e.g.
CACNA1A or ATP1A2) are not found in VM patients
(141,142). The CACNA1A gene encodes a subunit of
the voltage-gated P/Q-type calcium channels, and its
mutation can cause at least three neurological disorders
linked to calcium channelopathy: Episodic ataxia type
2, familial hemiplegic migraine type 1, and spinocere-
bellar ataxia type 6 (143). Vertigo and migraine symp-
toms are also common in patients with episodic ataxia
type 2 (144). Despite the lack of evidence for mono-
genic inheritance, whether polygenic inheritance plays
Cephalalgia 40(1)
a role in VM patients warrants further research
(48,145–150).
Treatment
The uncertainties surrounding VM diagnosis over the
last few decades have also limited advances in treat-
ment. In general, VM patients can be managed with
lifestyle modification, dietary adjustments, medications,
vestibular physical therapy, and activities that can
enhance perception of spatial orientation, such as
ping-pong or dancing. Given the considerable psycho-
social impact of symptoms in VM patients, it is import-
ant to adjust treatment strategies based on each
patient’s needs. Those who are not significantly
impaired by their symptoms may benefit from reassur-
ance to allay their fear and prevent unnecessary medical
costs. This is especially important for patients with
prominent vestibular symptoms. Those who are signifi-
cantly impaired by their symptoms often benefit from
combined interventions. In this process, identifying
triggers and implementing measures to circumvent
them can be used as the first line of treatment. As a
key intervention, dietary adjustment and eliminating
triggers such as red wine, aged cheeses, artificial sweet-
eners, processed meats, chocolate, caffeine, MSG, and
alcohol are found to be effective in reducing symptoms
(14,151,152).
Like other migraine subtypes, medical treatment in
VM patients aims to reduce frequency and severity of
symptoms. Most medications used for VM treatment
are aimed at prophylactic therapy. These medications
are mainly antihypertensives, antidepressants, and anti-
epileptics with general applications in migraine preven-
tion. Their use in VM treatment is supported by data
mainly from small studies, and there is insufficient evi-
dence from randomized controlled trials that can verify
the effect of these medications for VM prevention
(Table 3) (153). Beta-adrenergic inhibitors such as pro-
pranolol, calcium channel blockers such as verapamil,
and anti-epileptic medications such as topiramate
and lamotrigine are sometimes effective in migraine
prophylaxis, but their role in VM treatment is not
clear (51,151,154–161). A prospective randomized
non-placebo-controlled study in VM patients suggested
flunarizine is effective in decreasing the severity and
frequency of vertigo attacks (159). A retrospective
study found that cinnarizine decreased vertigo fre-
quency in VM patients (160). Tricyclic antidepressants
(TCAs), which have a broad mechanism of action, are
shown to be effective in reducing episodic migraine
symptoms (154). Selective serotonin reuptake inhibitors
(SSRIs) or serotonin-norepinephrine reuptake inhibi-
tors (SNRIs) have also been tried for VM prevention
(154,161). These antidepressants may provide benefit in
Huang et al. 115

Table 3. Summary of studies on preventive treatments for vestibular migraine defined based on the ICHD-3 criteria.

Drug Daily dose Study design Duration Outcome

Propranolol 40–160 mg 33 patients, prospective, 4 months Severity: DHI 55.8  2.7 (Salviz et al. 2016)
randomized, controlled to 31.3  3.7* (158)
VSS 7.3  0.3 to 2.1  0.4*
Attacks per month: 12.6  1.8
to 1.9  0.7*
Venlafaxine 37.5–150 mg 31 patients, prospective, 4 months Severity: DHI 50.9  2.5 (Salviz et al. 2016)
randomized, controlled to 19.9  2.9* (158)
VSS 7.1  0.3 to 1.8  0.5*
Attacks per month: 12.2  1.8
to 2.6  1.1*
Venlafaxine 37.5 mg 25 patients, prospective 3 months Severity: DHI 41.7  16.9 (Liu et al. 2017)
to 31.3  14.1* (157)
VSS 5.9  1.7 to 3.8  1.2*
Flunarizine 10 mg 25 patients, prospective 3 months Severity: DHI 46.6  15.1 (Liu et al. 2017)
to 39.8  16.3* (157)
VSS 6.4  1.9 to 5.9  1.6*
Valproic acid 1000 mg 25 patients, prospective 3 months Severity: DHI 46.8  13.5 (Liu et al. 2017)
to 38.7  13.6* (157)
VSS 5.8  1.8 to 5.3  1.0
Cinnarizine 75 mg 24 patients, retrospective 3 months Attacks per month: 3.8  1.1 (Taghdiri et al. 2014)
open-label to 0.4  0.6* (160)
*p < 0.05.
DHI: dizziness handicap inventory (175); VSS: vertigo severity score (157,158).

cases with psychiatric comorbidities such as anxiety and
depression. Recently, monoclonal anti-CGRP antibo-
dies have been approved for migraine prevention.
Serum CGRP levels are elevated interictally in chronic
migraine patients and to a lesser extent in those with
episodic migraine (162). CGRP is also detected in
human cochlear and vestibular end organs, and thus
it may also play a role in vestibular physiology
(163,164). However, it is unknown whether CGRP
has a role in VM pathogenesis and whether the
CGRP antagonists or monoclonal antibodies can be
effective for VM treatment.
There are only a few randomized controlled studies
on acute treatment for VM attacks. One small double-
blind, placebo-controlled study showed 38% of VM
patients improved from severe or moderate vertigo to
mild or no vertigo in two hours after taking zolmitrip-
tan, compared to 22% with placebo (165). However,
due to the limited power of the study, the difference
did not reach statistical significance. Rizatriptan
reduced vestibular-induced motion sickness in migrain-
eurs compared to the placebo (166). The authors sug-
gested that rizatriptan may reduce motion sickness by
influencing serotonergic vestibulo-autonomic path-
ways. Sumatriptan was also effective in improving
both headache and vertigo regardless of their temporal
relation (155). Steroid injections were reported to be
effective in four patients with prolonged or frequent
vertigo attacks in VM patients (167), similar to the
effect of steroids on patients with status migrainosus
(168).
Vestibular rehabilitation has been used to alleviate
symptoms and promote recovery in VM patients, as in
other vestibular disorders (169). Although most studies
show that focused vestibular rehabilitation benefits
patients, no randomized control study has evaluated
the efficacy of vestibular physical therapy in VM
patients (170). One early study compared the effect
of vestibular rehabilitation in 14 VM patients to
25 patients with migraine who had unrelated vestibular
dysfunction, and showed both groups improved signifi-
cantly on subjective and objective outcome measures
within four months (84). This finding was supported
by another study of 34 VM patients (14). A prospective
study in which 20 VM patients received a nine-week
customized vestibular rehabilitation program also
showed benefits, regardless of their medication regimen
(171). In another study, 28 VM patients based on
ICHD-3 beta criteria and 79 patients with tension-
type headache and dizziness were recruited (172).
Both groups were trained in vestibular therapy exer-
cises during a five-day admission process and were
assessed 6 months later. The results showed that ves-
tibular rehabilitation contributed more prominently to
116
clinical improvement in the VM group compared with
the tension-type headache group. In practice, vestibular
rehabilitation can be particularly beneficial to patients
if secondary complications such as deconditioning or
visual dependence have developed. VM patients often
experience dizziness and disorientation with changes in
head and body positions, which raises the possibility of
a ‘‘higher level’’ dysfunction in multisensory integration
for spatial orientation (i.e. visual, vestibular, and som-
atosensory inputs). Therefore, natural activities that
can enhance spatial perception and body coordination,
such as ping-pong and dancing, can be helpful to alle-
viate symptoms in these patients. Such an approach is
supported by the effects of long-term training in discip-
lines such as ballet dancing and yoga. Experts in these
fields are better at processing and weighting body per-
ceptual information and are less visually dependent in
their perception of spatial orientation (173,174).
Conclusions
Vestibular migraine is among the most common causes
of recurrent vertigo in the general population, with lar-
gely unknown pathophysiology. Patients with VM
experience disabling spatial misperceptions including
unusual sensitivity to head motion or visual stimuli,
or sudden feelings of imbalance or tilt. These symptoms
often become chronic and lead to severe impairment of
daily activities in VM sufferers. Unlike typical migraine
aura, vestibular symptoms in VM patients are not tem-
porary symptoms that occur with headaches, and their
duration can last from hours to several days. Currently,
the VM diagnosis is entirely based on clinical his-
tory, and according to the recent consensus of the
International Headache and the Bárány Societies, the
presence of episodic vestibular symptoms associated
with at least one migraine feature can fulfill the VM
Clinical implications
. VM is among the most common causes of recurrent vertigo. In addition to vertigo, patients may experience
disabling spatial misperceptions.
. The diagnosis of VM is entirely based on clinical history, and no laboratory test is pathognomonic.
. The pathophysiology of VM remains obscure, but recent studies suggest sensory dysfunction is related to
processing vestibular inputs.
. The treatment of VM requires lifestyle adjustment, trigger avoidance and vestibular rehabilitation as well as
abortive and preventive medications, although solid evidence is still lacking.
Declaration of conflicting interests
The authors declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of this
article.
Cephalalgia 40(1)
diagnostic criteria. Neurological and ocular motor
examination is usually normal in VM patients during
symptom-free periods. However, some mild, nonspeci-
fic vestibulo-ocular abnormalities such as positional
nystagmus have been reported, especially during VM
attacks. So far, no pathognomonic clinical or labora-
tory finding exists for VM; however, vestibular testing
is still helpful to rule out other disorders considered in
the differential diagnosis. Vestibular laboratory
abnormalities are quite variable among VM studies.
Such variability could be related to the lack of standard
diagnostic criteria used in earlier studies or the reliance
on clinical symptoms alone in the current VM criteria,
which does not prevent inclusion of patients with other
vestibular disorders who may have superimposed, sec-
ondary migraine symptoms. Like other types of
migraine, both abortive and preventive medications
have been used in VM patients. However, the efficacy
of these medications has not been verified by large-
scale randomized placebo-controlled clinical trials.
Non-pharmacological treatment including life-style
adjustment, trigger avoidance and vestibular rehabilita-
tion are also shown to be beneficial in VM patients. The
pathophysiology of VM remains largely unknown;
however, recent findings have provided some prelimin-
ary insights. These include reports of abnormal motion-
detection thresholds during roll tilt or yaw rotation, or
larger errors of spatial orientation during head tilt,
which suggest high-level dysfunctions related to ves-
tibular processing in these patients. So far there is no
information about the plausible role of central neural
processes in VM spatial disorientation and their link to
visuospatial symptoms in these patients. Therefore,
gaining insight into these processes and how they
result in dizziness, vertigo, and spatial disorientation
in VM patients is an important step towards devising
effective treatment strategies.
Funding
The authors disclosed receipt of the following financial sup-
port for the research, authorship, and/or publication of this
article: This study was supported in part by the following
Huang et al.
grants: Ministry of Science and Technology of Taiwan
[MOST 107-2321-B-010-001], Brain Research Center,
National Yang-Ming University from The Featured Areas
Research Center Program within the framework of the
Higher Education Sprout Project by the Ministry of
Education (MOE) in Taiwan, as well as the United States
National Institute of Deafness and Other Communication
Disorders (NIDCD); K23DC013552.
ORCID iDs
Tzu-Chou Huang https://orcid.org/0000-0003-4674-7882
Shuu-Jiun Wang https://orcid.org/0000-0001-5179-5358
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