1. Vestibular migraine
A.
At least five episodes with vestibular symptomsa of moderate or severe intensityb, lasting 5 min to 72 hc
B.
Current or previous history of migraine with or without aura according to the International Classification of Headache Disorders (ICHD)d
C.
One or more migraine features with at least 50 % of the vestibular episodese:
Headache with at least two of the following characteristics: one-sided location, pulsating quality, moderate or severe pain intensity, aggravation by routine physical activity
Photophobia and phonophobiaf
Visual aurag
D.
Not better accounted for by another vestibular or ICHD diagnosish
2. Probable vestibular migraine
A.
At least five episodes with vestibular symptomsa of moderate or severe intensityb, lasting 5 min to 72 hc
B.
Only one of the criteria B and C for vestibular migraine is fulfilled (migraine history or migraine features during the episode)
C.
Not better accounted for by another vestibular or ICHD diagnosish
6.3 Prevalence of Vestibular Migraine
Vestibular migraine was diagnosed in 7 % in a group of 200 dizziness clinic patients and in 9 % of 200 migraine clinic patients [8]. In a population-based study (n = 4,869) with screening interviews followed by expert telephone interviews, the lifetime prevalence of VM was estimated at 0.98 % (95 % CI 0.7–1.37) [18]. Of note, VM accounted for only a third of migraine patients with a history of vertigo, which indicates the need for a thorough neuro-otological workup for exclusion of other diagnoses [18]. In a community-based sample of middle-aged women in Taiwan, VM was identified in 5 % and in 30 % of those with migraine [19]. VM is still widely underdiagnosed, as shown by a study from a dizziness clinic in Switzerland, where VM accounted for 20 % of the diagnoses in young patients, but was suspected by the referring doctors in only 2 % [20] (for an extensive review on epidemiology of VM, see Chap. 5 in this volume).
6.3.1 Demographic Aspects
VM may occur at any age [4, 5, 7]. It is more common in women with a reported female to male ratio between 1.5 and 5 to 1 [5–8]. Familial clustering may occur, probably based on an autosomal dominant pattern of inheritance with decreased penetrance in men [21]. In most patients, migraine begins earlier in life than VM [7, 8]. Some patients have been free from migraine attacks for years when VM first manifests itself [7]. Not infrequently, vertigo attacks replace migraine headaches in women around menopause.
6.4 Vestibular Migraine in Children
Benign paroxysmal vertigo of childhood is an early manifestation of VM which is recognized by the ICHD [14]. It is characterized by brief attacks of vertigo or disequilibrium, anxiety and often nystagmus or vomiting, recurring for months or years in otherwise healthy young children [22]. Many of these children later develop migraine, often years after vertigo attacks have ceased [23]. A family history of migraine in first-degree relatives is twofold increased compared to controls [24]. The prevalence of recurrent vertigo probably related to migraine was estimated at 2.8 % in children between 6 and 12 years in a population-based study [24].
6.4.1 Symptoms
6.4.1.1 Types of Vertigo
Patients with VM typically report spontaneous or positional vertigo. Some experience a sequence of spontaneous vertigo transforming into positional vertigo after several hours or days. This positional vertigo is distinct from benign paroxysmal positional vertigo (BPPV) with regard to duration of individual attacks (often as long as the head position is maintained in VM versus seconds only in BPPV), duration of symptomatic episodes (minutes to days in VM versus weeks in BPPV) and nystagmus findings [25]. Altogether, 40–70 % of patients experience positional vertigo in the course of the disease, but not necessarily with every attack. A frequent additional symptom is head motion intolerance, i.e. imbalance, illusory motion and nausea aggravated or provoked by head movements [5, 26]. Visually induced vertigo, i.e. vertigo provoked by moving visual scenes such as traffic or movies, can be another prominent feature of VM [5, 27]. Nausea and imbalance are frequent but nonspecific accompaniments of acute VM. The combination of different types of vertigo distinguishes VM from other neuro-otological disorders such as benign paroxysmal positional vertigo or Menière’s disease, which typically present with monosymptomatic vertigo. Patients with VM are often affected by motion sensitivity even in-between attacks [28], which may lead to a chronic type of vestibular dizziness [25]. Another factor contributing to chronic vestibular migraine is secondary psychiatric morbidity, particularly anxiety disorders [29, 30]. These two components of interictal dizziness may not be easily discriminated in individual patients [31].
6.4.1.2 Relation to Headaches
VM often misses not only the duration criterion for an aura as defined by the ICHD but also the temporal relationship to migraine headaches: vertigo can precede headache as would be typical for an aura, may begin with headache or may appear late in the headache phase. Many patients experience attacks both with and without headache [4, 6, 7]. Quite frequently, patients have an attenuated headache with their vertigo as compared to their usual migraine [3, 6]. In some patients, vertigo and headache never occur together [4, 6, 8]. Misdiagnosis of VM as “cervical vertigo” may occur when accompanying pain is mainly or exclusively localized in the neck, which is quite common in patients with migraine [32].
6.4.1.3 Other Symptoms
Along with the vertigo, patients may experience photophobia, phonophobia, osmophobia and visual or other auras. These phenomena are of diagnostic importance, since they may represent the only apparent connection of vertigo and migraine. Patients need to be asked specifically about these migraine symptoms since they often do not volunteer them. A dizziness diary can be useful for prospective recording of associated features.
Auditory symptoms, including hearing loss, tinnitus and aural pressure, have been reported in up to 38 % of patients with VM [2, 5, 6, 33, 34]. Hearing loss is usually mild and transient, without or with only minor progression in the course of the disease [6]. About 20 % develop mild bilateral downsloping hearing loss over the years [35]. In contrast, unilateral moderate to severe hearing loss starting in the low-frequency range would rather favour a diagnosis of Menière’s disease.
6.4.1.4 Precipitating Factors
Asking for migraine-specific precipitants of vertigo attacks may provide valuable diagnostic information, e.g. provocation by menstruation, deficient sleep, excessive stress, skipped meals, lack of fluid and exposure to sensory stimuli, such as bright or scintillating lights, intense smells or noise. The influence of specific foods and weather conditions is probably overestimated. Sometimes, migraine accompaniments and typical precipitants may be missing, but VM is still considered the most likely diagnosis after other potential causes have been investigated and appear unlikely. In this case, a favourable response to antimigraine drugs may support the suspicion of an underlying migraine mechanism. However, apparent efficacy of a drug should not be regarded as a definite confirmation of the diagnosis, since spontaneous improvement, placebo response and additional drug effects (e.g. anxiolytic or antidepressant) have to be taken into account.
6.5 Findings on Clinical Examination
In most patients, the general neurologic and otologic examination is normal in the symptom-free interval [4]. Neuro-ophthalmological evaluation may reveal mild central ocular motor deficits such as persistent positional nystagmus and saccadic pursuit, particularly in patients with a long history of VM [7, 35, 36]. Interictal head-shaking nystagmus was observed in 50 % of VM patients [37]. In one study, patients with VM became nauseous after caloric testing four times more often than migraine patients with other vestibular disorders [38]. A neuro-otologic study of 20 patients during the acute phase of VM showed pathological nystagmus in 14 patients, mostly central spontaneous or positional nystagmus. Three patients had a peripheral type of spontaneous nystagmus and a unilateral deficit of the horizontal vestibuloocular reflex. Imbalance was observed in all patients except one [39]. Another study confirmed the high prevalence of persistent positional nystagmus, which was often horizontal and direction changing, but could also beat in the vertical or torsional plane [40].