A1.6.6 Vestibular migraine
Previously used terms:
Migraine-associated vertigo/dizziness; migraine-related vestibulopathy; migrainous vertigo.
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 aura1
C. Vestibular symptoms2 of moderate or severe intensity3, lasting between 5 min and 72 hr4
D. At least 50% of episodes are associated with at least one of the following three migrainous features5:
1. headache with at least two of the following four characteristics:
a) unilateral location
b) pulsating quality
c) moderate or severe intensity
d) aggravation by routine physical activity
2. photophobia and phonophobia6
3. visual aura7
E. Not better accounted for by another ICHD-3 diagnosis or by another vestibular disorder8.
1. Code also for the underlying migraine diagnosis.
2. Vestibular symptoms, as defined by the Bárány Society’s Classification of Vestibular Symptoms and qualifying for a diagnosis of A1.6.5 Vestibular migraine, include:
a) spontaneous vertigo:
i. internal vertigo (a false sensation of self-motion);
ii. external vertigo ( a false sensation that the visual surround is spinning or flowing);
b) positional vertigo, occurring after a change of head position;
c) visually-induced vertigo, triggered by a complex or large moving visual stimulus;
d) head motion-induced vertigo, occurring during head motion;
e) head motion-induced dizziness with nausea (dizziness is characterized by a sensation of disturbed spatial orientation; other forms of dizziness are currently not included in the classification of vestibular migraine).
3. Vestibular symptoms are rated moderate when they interfere with but do not prevent daily activities and severe when daily activities cannot be continued.
4. Duration of episodes is highly variable. About 30% of patients have episodes lasting minutes, 30% have attacks for hours and another 30% have attacks over several days. The remaining 10% have attacks lasting seconds only, which tend to occur repeatedly during head motion, visual stimulation or after changes of head position. In these patients, episode duration is defined as the total period during which short attacks recur. At the other end of the spectrum, there are patients who may take four weeks to recover fully from an episode. However, the core episode rarely exceeds 72 hours.
5. One symptom is sufficient during a single episode. Different symptoms may occur during different episodes. Associated symptoms may occur before, during or after the vestibular symptoms.
6. Phonophobia is defined as sound-induced discomfort. It is a transient and bilateral phenomenon that must be differentiated from recruitment, which is often unilateral and persistent. Recruitment leads to an enhanced perception and often distortion of loud sounds in an ear with decreased hearing.
7. Visual auras are characterized by bright scintillating lights or zigzag lines, often with a scotoma that interferes with reading. Visual auras typically expand over 5-20 minutes and last for less than 60 minutes. They are often, but not always restricted to one hemifield. Other types of migraine aura, e.g., somatosensory or dysphasic aura, are not included as diagnostic criteria because their phenomenology is less specific and most patients also have visual auras.
8. History and physical examinations do not suggest another vestibular disorder or such a disorder has been considered but ruled out by appropriate investigations or such a disorder is present as a comorbid or independent condition, but episodes can be clearly differentiated. Migraine attacks may be induced by vestibular stimulation. Therefore, the differential diagnosis should include other vestibular disorders complicated by superimposed migraine attacks.
Transient auditory symptoms, nausea, vomiting, prostration and susceptibility to motion sickness may be associated with A1.6.5 Vestibular migraine. However, since they also occur with various other vestibular disorders, they are not included as diagnostic criteria.
Relation to migraine aura and migraine with brainstem aura
Both migraine aura and migraine with brainstem aura (formerly: basilar-type migraine) are terms defined by ICHD-3 (beta). Only a minority of patients with A1.6.5 Vestibular migraine experience their vertigo in the time frame of 5-60 minutes as defined for an aura symptom. Even fewer have their vertigo immediately before headache starts, as required for 126.96.36.199 Typical aura with headache. Therefore, episodes of A1.6.5 Vestibular migraine cannot be regarded as migraine auras.
Although vertigo is reported by more than 60% of patients with 1.2.2 Migraine with brainstem aura, ICHD-3 (beta) requires at least two brainstem symptoms in addition to visual, sensory or dysphasic aura symptoms for this diagnosis. Fewer than 10% of patients with A1.6.5 Vestibular migraine fulfil these criteria. Therefore, A1.6.5 Vestibular migraine and 1.2.2 Migraine with brainstem aura are not synonymous, although individual patients may meet the diagnostic criteria for both disorders.
Relation to benign paroxysmal vertigo
While A1.6.5 Vestibular migraine may start at any age, ICHD-3 (beta) specifically recognizes a childhood disorder, 1.6.2 Benign paroxysmal vertigo. The diagnosis requires five episodes of vertigo, occurring without warning and resolving spontaneously after minutes to hours. Between episodes, neurological examination, audiometry, vestibular functions and EEG must be normal. A unilateral throbbing headache may occur during attacks but is not a mandatory criterion. 1.6.2 Benign paroxysmal vertigo is regarded as one of the precursor syndromes of migraine. Therefore, previous migraine headaches are not required for diagnosis. Since the classification of A1.6.5 Vestibular migraine does not involve any age limit, the diagnosis can be applied in children when the respective criteria are met. Only children with different types of vertigo attacks, eg, short-duration episodes of less than five minutes and longer-lasting ones of more than five minutes, should receive both these diagnoses.
Overlap with Menière’s disease
1. Migraine is more common in patients with Menière’s disease than in healthy controls. Many patients with features of both Menière’s disease and A1.6.5 Vestibular migraine have been reported. In fact, migraine and Menière’s disease can be inherited as a symptom cluster. Fluctuating hearing loss, tinnitus and aural pressure may occur in A1.6.5 Vestibular migraine, but hearing loss does not progress to profound levels. Similarly, migraine headaches, photophobia and even migraine auras are common during Menière attacks. The pathophysiological relationship between A1.6.5 Vestibular migraine and Menière’s disease remains uncertain. In the first year after onset of symptoms, differentiation between them may be challenging, since Menière’s disease can be monosymptomatic with only vestibular symptoms in the early stages of the disease.
When the criteria for Menière’s disease are met, particularly hearing loss as documented by audiometry, Menière’s disease should be diagnosed, even when migraine symptoms occur during the vestibular attacks. Only patients who have two different types of attacks, one fulfilling the criteria for A1.6.5 Vestibular migraine and the other for Menière’s disease, should be diagnosed with both disorders. A future revision of ICHD may include a vestibular migraine/Menière’s disease overlap syndrome.
Migraine has a brain stem generator in the periaqueductal grey part of the mesencephalon near where labyrinthine fibers from the inner ear interconnect. Migraine and vertigo have had a long, somewhat circumspect, and private relationship. Neurology textbooks for the last 50 years have all listed vertigo under the long list of symptoms associated with migraine.[i] There is a large body of literature about the subject.
Vertigo is a hallucination of movement that comes from the Latin root word which means “to turn” and is likened to the feeling one has after spinning around a number of times and then stopping. It is what one feels on the playground after getting off of a merry go round. Although usually rotary in motion with the patient at the center, vertigo in its broadest definition can be expanded to be “the perception of motion on the part of the patient.” Thus, vertigo can be non-rotary, and include a sense of linear or up and down movement. Vertigo may originate in the vestibular branch of the eighth cranial nerve in the “inner ear” where it is called “peripheral” or “end organ” or in the brain--usually in the cerebellum or brain stem--where it is referred to as being “central.”
Dizziness is a much less precise medical term that has multiple causes and does not localize to a specific area of the brain. Dizziness may be described by the patient as “light-headed, giddiness, near faintness, swimmy headed, or unsteadiness.” Dizziness has a large differential diagnosis and may be due to inner ear inflammation, hypoglycemia, electrolyte imbalance, low blood pressure, decreased heart output, anemia, anxiety or panic disorder, hyperventilation, medication effect, or many other causes. On close questioning, most patients can differentiate between vertigo and migraine. Complicating this discussion is the fact that the occurrence of dizziness in the general population is over 20%. However, patients who have migraine with aura have significantly more dizzy spells than non-headache subjects.[ii]
Another interesting and well-known link to migraine is motion sickness which usually comes on in childhood and improves somewhat with ageing. The symptoms of motion sickness may be nausea, dizziness, vertigo, sweating, or headache. Commonly children experience this in the back of the car when riding. It also occurs with reading in the car, participating in amusement park rides--especially rides with fast circular motion--and on boats and airplanes. Most studies report that about 60% of patients with migraine have motion sickness, while only 5-20% of persons without migraine get motion sickness.[iii]
Medical practices that specialize in migraine find that 27-42 % of patients report episodic vertigo. About 36% of these migraineurs get vertigo when they have no headache, while many others get vertigo either just before or during the headache. Migraine with aura patients have a higher incidence of vertigo during the headache period than those who have migraine without aura.[iv] The converse of this is that practices that specialize in vertigo find 16-32% of their patients have migraine.
Literature Review of Vestibular Migraine and Benign Paroxysmal Vertigo
Vertigo that related to a newly recognized type of migraine was first described by Edwin Bickerstaff[v] in 1961 in his article on “Basilar Artery Migraine.” This syndrome will be discussed next in this chapter as a separate entity.
Fenichel[vi] writing in the Journal of Pediatrics in 1967 reported 2 siblings who suffered over several years from brief attacks of nausea and vertigo lasting from seconds to minutes. Later these children developed migraine with aura. Fenichel stated:
The proband of this report first presented at age two with symptoms of “benign paroxysmal vertigo.” The attacks insidiously changed in character from isolated vertigo to fairly typical migraine. Symptoms of benign paroxysmal vertigo developed in a younger brother who is now two years old. Family history reveals a high incidence for migraine in the maternal line.
In 1979 Slater[vii] writing in Journal of Neurology, Neurosurgery, and Psychiatry introduced a term, “Benign Recurrent Vertigo” which consisted of episodic attacks of vertigo, no cochlear symptoms, a history of migraine, and nystagmus. Stress, lack of sleep, alcohol use, and a strong family history were also found with this group which Slater considered a migraine variant. In 1980 Koehler[viii] presented eight young children with benign paroxysmal vertigo, a symptom complex comprised of attacks of vertigo, nystagmus, and ataxia. Follow-up studies revealed a close relationship to autonomic nervous system instability, particularly to migraine. Moretti, at al,[ix] reported five more cases of benign paroxysmal vertigo in 1980 stressing the connection of benign recurrent vertigo to migraine. They also commented on its prevalence in women, occurrence during menstruation, but felt that there was “no time relationship between vertigo and migraine attacks.”
Kuritzky, et al,[x] reported in 1981 on “Vertigo, Motion Sickness and Migraine.” They found that patients with classical migraine (ICH--migraine with aura):
reported significantly more vestibular symptoms than controls. Specifically they had more dizzy spells and vertigo episodes not associated with the headache. They also had more frequent motion sickness spells.
Kayan and Hood[xi] writing in Brain in 1984 on “Neuro-otological manifestations of migraine” studied vestibulocochlear derangements in 3 groups of patients: 200 migraine patients, 80 migrainous patients referred for neuro-otological examination because of their symptoms, and 116 tension headache patients who served as controls. Significant differences were established among these groups. Migraine patients had vestibulococlear disturbances as an aura, during headache-free times, or, with highest incidence, during the headache. Fifty-nine percent of the 200 migraine patients reported vestibular and/or cochlear symptoms which were disabling for 5%. Also 50% of the migraine patients had a history of motion sickness and 81% developed phonophobia during the headache. Persisting vestibulocochlear derangements were found in 77.5% of the 80 patients referred for neuro-otological examination. Kayan and Hood discussed “possible links between Meniere’s disease, benign paroxysmal vertigo, and migraine.”
In 1995 Abu-Arafeh and Russell[xii] wrote in Cephalalgia on “Paroxsmal vertigo as a migraine equivalent in children: a population study.” They studied the prevalence, causes, and clinical features of paroxysmal vertigo (PV) in the City of Aberdeen in 2165 children utilizing a screening questionnaire. Children with a history of 3 episodes of vertigo were invited for clinical interview and exam. Forty-five children fulfilled the criteria for PV (prevalence rate 2.6%). These children were noted to have features common to children with migraine along with abdominal pain, cyclical vomiting, atopy, and motion sickness. These children had a two fold increase in the prevalence of migraine (24%) compared with the general childhood population. The authors concluded that migraine and PV were related and that it was “reasonable to continue to regard PV as a migraine equivalent."
Buchholz and Reich[xiii] writing in 1996 in Seminars in Neurology on “The menagerie of migraine” reported that migraine may have hearing loss and vestibular dysfunction. Lindskog in 1999[xiv] writing in 1999 in Headache on “Benign Paroxysmal Vertigo in Childhood: A Long-Term Follow-up” found no relationship between childhood Benign Positional Vertigo (BPV) and migraine in a long term follow-up. These researchers followed 19 children aged 5 months to 8 years diagnosed in 1975-1981 with BPV. Follow-up was performed 13-20 years after diagnosis and 21% developed migraine which is more than expected in a normal population of that age. None of the patients had trouble with balance or vertigo at follow-up. The authors concluded that BPV has a good outcome and is “not a general precursor of migraine.” However, most of the published articles on the subject do not agree.
Baloh[xv] writing in 1997 in Headache on “Neurotology of Migraine” stated that “Neurotology symptoms are common with migraine, yet relatively little is known about the pathophysiology of such symptoms.” Baloh found motion sensitivity with motion sickness in 2/3 and vertigo in 1/4 of patients with migraine. He thought that sensitivity to sound (phonophobia) was the most common migraine auditory symptom but fluctuating and permanent hearing loss may rarely occur. Baloh noted that migraine can imitate Meniere’s disease and that so-called “vestibular Meniere’s disease” is usually associated with migraine.
Dieterich and Brandt[xvi] writing in 1999 in Journal of Neurology on “Episodic vertigo related to migraine (90 cases): vestibular migraine?” performed a retrospective study on 90 patients with episodic vertigo that could be related to migraine but that did not fulfill IHS criteria for basilar migraine. The following features were noted:
occurrence anytime in life with a peak in the 4th decade in men and a plateau between the 3rd and 5th decade in women; duration of rotational (78%) and/or to-and fro vertigo (38%) lasting from seconds to several hours, or less frequently even days. Monosymptomatic audiovestibular attacks (78%) occurred as vertigo associated with auditory symptoms in only 16%. Vertigo was not associated with headache in 32% of the patients. In the symptom-free interval 66% of the patients showed mild central ocular motor signs such as vertical (48%) and/or horizontal (22%) saccadic pursuit, gaze-evoked nystagmus (27%), moderate positional nystagmus (11%), and spontaneous nystagmus (11%). Combinations with other forms of migraine were found in 52%.
Dieterich and Brandt stated: “migraine is a relevant differential diagnosis for episodic vertigo.” They proposed using the more appropriate term “vestibular migraine.”
Radtke, et al,[xvii] writing in Neurology in 2002 on “Migraine and Meniere’s disease: is there a link?” commented that Prosper Meniere had suggested a relationship between the two episodic clinical syndromes in Paris in 1861 when he discussed the illness that bears his name. Radtke, et al, determined the lifetime prevalence of migraine in patients with Meniere’s disease (MD) compared to sex- and age-matched controls. They studied 78 patients with idiopathic unilateral or bilateral MD according to the criteria of the American Academy of Otolaryngology. Migraine was diagnosed by phone interviews using ICHD criteria. Information concerning the concurrence of vertigo and migrainous symptoms during Meniere attacks was also collected. The lifetime prevalence of migraine with and without aura was higher in the MD group (56%) compared to controls (25%: p<0.001). Forty-five percent of the patients with MD always experienced at least one migrainous symptom (migrainous headache, photophobia, aura symptoms) with Meniere attacks. Radtke, et al, concluded:
The lifetime prevalence of migraine is increased in patients with MD when strict diagnostic criteria for both conditions are applied. The frequent occurrence of migrainous symptoms during Ménière attacks suggests a pathophysiologic link between the two diseases. Alternatively, because migraine itself is a frequent cause of audio-vestibular symptoms, current diagnostic criteria may not differentiate between MD and migrainous vertigo.
Neuhauser and Lempert,[xviii] in Germany reported in 2004 in Cephalalgia on “Vertigo and dizziness related to migraine: a diagnostic challenge.” These authors stated:
Migrainous vertigo (MV) is a vestibular syndrome caused by migraine and presents with attacks of spontaneous or positional vertigo lasting seconds to days and migrainous symptoms during the attack. MV is the most common cause of spontaneous recurrent vertigo and is presently not included in the International Headache Society classification of migraine. Benign paroxysmal positional vertigo (BPPV) and Ménière's disease (MD) are statistically related to migraine, but the possible pathogenetic links have not been established. Moreover, migraineurs suffer from motion sickness more often than controls.
Neuhauser, et al,[xix] writing in 2006 in Neurology on “Migrainous vertigo: Prevalence and impact on quality of life,” studied the epidemiology of migrainous vertigo (MV) in the general population by assessing prevalence, clinical features, comorbid conditions, quality of life, and health care utilization. They screened 4,869 adults for dizziness and vertigo and then followed up with validated neurotologic telephone interviews. They used the diagnostic criteria for benign recurrent vertigo and migraine according to the IHS. They reported a lifetime prevalence of MV of 0.98% and a 12-month prevalence of 0.89%. Spontaneous rotational vertigo was reported by 67% of patients with MV while 24% had positional vertigo. Twenty-four percent always experienced headaches with their vertigo. Neuhauser, et al, concluded:
Migrainous vertigo is relatively common but under diagnosed in the general population and has considerable personal and healthcare impact.
Overview of Vestibular Migraine
Migraine causes more vertigo than any other condition. The incidence of migraine in the United States is 12% while the incidence of Meniere’s disease is 0.2%.[xx] About 50% of patients with Meniere’s disease have migraine.[xxi] Neurologic practices focusing on headache report episodic vertigo in 27-42% of migraineurs.[xxii] Dizziness, vertigo, tinnitus, photophobia, hearing loss, and nystagmus may accompany migraine. Vestibular Migraine may occur without headache. Typical acute and preventive treatment for migraine may improve migrainous vertigo.[xxiii]
The differential diagnosis of Vestibular Migraine includes ear infection, brain stem infarct, autoimmune inner ear disease, multiple sclerosis, early Meniere’s disease, vertebrobasilar ischemia, cerebellar tumor, and Arnold-Chiari malformation.
Treatment of Vestibular Migraine
To date there are no other options than using triptans such as sumatriptan and rizatriptan for the headache. Nausea can be treated with Antivert (meclizine) 25/50 mg tablets every 4-6 hours when necessary or the Transderm scope patch which is placed behind the ear and changed every 3 days. Nausea can be treated with Phenergan (promethazine) or Zofran (odansetron) 4/8 mg sublingual every 8 hours when necessary. Typical migraine preventive drugs such as topiramate, amitriptyline, beta blockers, and Depakote (for infertile women) could be tried.
[i] Merritt HH. A Textbook Of Neurology. 1973.Fifth Edition. Lea & Febiger.
[ii] Kuritzky A, Ziegler DK, Hassanein R. Vertigo, Motion Sickness and Migraine. Headache. 1981;21:227-231.
[iii] Hain TC. Migraine Associated Vertigo (MAV) Adapted from a 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.
[iv] Hain TC. Migraine Associated Vertigo (MAV) Adapted from a 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.
[v] Bickerstaff ER. Basilar Artery Migraine. Lancet. 1961;1:15-17.
[vi] Fenichel GM. Migraine as a cause of benign paroxysmal vertigo of childhood. J Pediatr. 1967;71:114-115.
[vii] Slater R. Benign recurrent vertigo. Journal of Neurology, Neurosurgery, and Psychiatry. 1979;42:363.
[viii] Koehler B. Benign paroxysmal vertigo of childhood: A migraine equivalent. European Journal of Pediatrics. 1980;134(2):149-151.
[ix] Moretti G, Manzoni G.C. Caffarra P, Parma M. "Benign Recurrent Vertigo" and Its Connection with Migraine. Headache: The Journal of Head and Face Pain. 1980;20(6):344–346.
[x] Kuritzky A, Ziegler DK, Hassanein R. Vertigo, Motion Sickness and Migraine. Headache. 1981;21:227-231.
[xi] Kayan A, Hood JD. Neuro-otological manifestations of migraine. Brain. 1984;107(Pt4):1123-1142.
[xii] Abu-Arafeh I, Russell G. Paroxysmal vertigo as a migraine equivalent in children: a population-based study. Cephalalgia. 1995;15(1):22–25.
[xiii] Buchholz DW, Reich SG. The menagerie of migraine. Seminars in Neurolgy. 1996;16(1):83-93.
[xiv] Lindskog U, Ödkvist L, Noaksson L, Wallquist J. Benign Paroxysmal Vertigo in Childhood: A Long-term Follow-up. Headache: The Journal of Head and Face Pain. 1999;39(1),33–37.
[xv] Baloh RW. Neurotology of Migraine. Headache: The Journal of Head and Face Pain. 1997; 37 (10), 615–621.
[xvi] Dieterich M, Brandt T. Episodic vertigo related to migraine (90 cases): vestibular migraine? Journal of Neurology. 1999;246(10):883-892.
[xvii] Radtke R, Lempert T, Gresty MA, Brookes GB, Bronstein AM, Neuhauser H. Migraine and Meniere’s disease. Is there a link? Neurology. 2002;59:1700-1704.
[xviii] Neuhauser H, Lempert T. Vertigo and dizziness related to migraine: a diagnostic challenge. Cephalalgia. 2004;24 (2):83–91.
[xix] Neuhauser HK, Radtke A, von Brevern M, Feldmann M, Lezius F, Ziese T, Lempert T. Migrainous vertigo: Prevalence and impact on quality of life. Neurology. 2006;67:1028-1033.
[xx] Wladislavosky-Waserman P, Facer G, et al. Meniere's disease: a 30-year epidemiologic and clinical study in Rochester, MN, 1951-1980. 1996. Laryngoscope 94:1098-1102.
[xxi] Radtke R, Lempert T, Gresty MA, Brookes GB, Bronstein AM, Neuhauser H. Migraine and Meniere’s disease. Is there a link? Neurology. 2002;59:1700-1704.