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J. Gurberg et al.
Other Treatments
Although vestibular rehabilitation is generally considered to be the core treatment
for most pediatric vestibular disorders, many of the most common vestibular conditions in children may not benet from physical therapy (e.g., vestibular migraine) or
may require a multimodal approach that includes both physical therapy and other,
concurrent treatments.
Medication
Many pediatric vestibular disorders benet from pharmacological therapies, though
typically these are ideally combined with other treatments. The use of medications
to treat specic vestibular disorders in children are summarized in further detail
under the section for each respective condition above. In particular, vestibular
migraine is often treated with a combination of rescue medications, such as the
triptans, and daily, preventative medications, such as cyproheptadine, tricyclic antidepressants, SNRI medications, beta blockers, and topiramate. PPPD is most effectively treated with a multimodal approach that includes SSRI/SNRI therapy along
with vestibular rehabilitation and CBT.Hemodynamic intolerance also sometimes
benets from treatment with midodrine or udrocortisone, when response to optimizing hydration and electrolyte intake is unsuccessful.
Surgery
The need for surgical intervention with pediatric vestibular disorders is uncommon.
Balance impairment in the setting of chronic middle ear dysfunction or recurrent acute
otitis media can improve signicantly after tympanostomy tube placement [38, 39].
Superior semicircular canal dehiscence is effectively treated with semicircular canal
occlusion or resurfacing, either through a transmastoid or middle cranial fossa
approach [58, 59]. Traumatic perilymphatic stulas that do not require spontaneously
will require middle ear exploration to conrm the diagnose and to repair to leak, which
can typically be performed transcanal, often with an endoscopic approach [60].
Treatment resistant BPPV that does not resolve with repositioning maneuvers may
require transmastoid occlusion of the offending canal to resolve, particularly when
cupulolithiasis is suspected [61]. Although semicircular canal occlusion for treatment
resistant BPPV has not been described in the medical literature in the pediatric population, the senior author has had good success with this procedure in two pediatric
patients with treatment resistant BPPV as of the time of this writing. Rarely, Chiari
malformations may cause balance impairment or even dizziness that may improve
with surgical decompression by a neurosurgeon [11, 16]. Also, it has been

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demonstrated that children with severe vestibular impairment and poor balance in the
setting of bilateral congenital SNHL may benet greatly from cochlear implantation
not only from a hearing standpoint, but also in terms of balance function [62]. Lastly,
vestibular implants are currently showing great promise in adult human trials, and they
will hopefully soon be an option for children with vestibular areexia, as well [63].
195
Alternative Therapies
Numerous alternative therapies play a vital role in the management of many pediatric vestibular disorders, as covered in further detail under the sections for each of
these specic conditions above. Vestibular migraine can benet greatly from
“migraine hygiene,” including trigger identication/avoidance and optimizing
sleep, hydration, diet and stress management. Hydration and electrolyte optimization are also the rst line management strategies for hemodynamic intolerance.
Magnesium supplements may also be helpful for patients with vestibular migraine,
though this has not yet been well studied in the pediatric population [64]. CBT plays
a central role in the management of PPPD and panic disorder, while it may also be
benecial for many patients with migraine [65, 66].
Accommodations andFollow-Up
Many children with chronic vestibular disorders may benet from accommodations
in the classroom in order to optimize their ability to learn effectively. Children with
VOR impairments may struggle with reading and with seeing activities at the front
of the classroom. Children with balance impairment may have difculty with navigating around the classroom or between classes, particularly in busy hallways.
Children with PPPD or vestibular migraine may have frequent dizziness ares in the
classroom and require breaks for recovery or even partial home schooling initially
with gradual upward titration of in-person learning, as tolerated. Some examples of
accommodations that may be benecial for children with vestibular disorders are
summarized in Table10.7.
Although the pediatric otolaryngologist often functions in a consultative role for
the child with dizziness or imbalance, it is important to ensure adequate follow-up
with either the otolaryngologist or an alternative appropriate provider (e.g., primary
care physician or neurologist) to conrm that ongoing improvements are made and
that medications are being tolerated appropriately. Although BPPV may resolve
with maneuvers, many children may experience recurrences or may not resolve with
initial maneuver attempts. Their symptoms also could persist after successful
maneuvers if concurrent conditions, such as PPPD, vestibular migraine, or peripheral vestibular losses, are present. Vestibular migraine is a chronic condition that
consistently requires long-term management and support. PPPD will often reach a

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J. Gurberg et al.
Table 10.7
disorders
• Allowing use of a laptop or tablet or printed materials to convey materials presented at front
of class
• Sitting in the middle or back of classroom to minimize arc of head movements between desk
and front of class (unless concurrent hearing loss warrants front row seating)
• Providing extra time for taking tests and for completing assignments, including standardized
exams
• Allowing brief breaks on a regular basis, if needed
• Minimizing excess sensory stimulation in the classroom (noise, unnecessary visual stimuli,
etc.), whenever possible
• Minimizing need to participate in classroom activities that require moving about the
classroom, whenever possible
• Maintaining a structured classroom setting with predictable routines and minimal distraction
• Using an enlarged font [16–18] and increased space between printed lines, when feasible
• Providing extra time for moving between classes
• Permitting transition between classes a few minutes early and/or late relative to classmates to
walk next class, as busy/high trafc hallways can exacerbate dizziness and imbalance
• Facilitating transition between classes with a trusted friend and/or staff member, if needed
and appropriate
School accommodations that may be benecial for children with chronic vestibular
point of complete recovery, but this can sometimes takes months or even years to
occurs, so it is best managed like a chronic condition with relatively frequent follow- up with a vestibular specialist to conrm that appropriate treatment strategies
are being adhered to.
Conclusion
Vestibular symptoms are surprisingly common in the pediatric population. Pediatric
dizziness and imbalance are often multifactorial, so a multidisciplinary approach is
often warranted. Although migraine is the most common cause of dizziness in children, many other conditions should also be considered. Vestibular testing can be
helpful in some cases, but a careful history and physical examination alone is usually adequate to arrive at an accurate diagnosis. Vestibular disorders in children and
adolescents can be effectively treated once diagnoses are reliably determined.
Vestibular physical therapy is the core management strategy for most pediatric vestibular conditions, though medications, surgery, and/or alternative therapies are
often warranted, as well.
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48. Kim HA, Bisdorff A, Bronstein AM, etal. Hemodynamic orthostatic dizziness/vertigo: diagnostic criteria. J Vestibular Res. 2019;29(2–3):45–56.
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59. Weinreich HM, Carey JP.Perilymphatic stulas and superior semi-circular canal dehiscence
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60. Rawal RZX, Lipson S, Brodsky JR.Endoscopic repair of traumatic perilymphatic stula in
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62. Wolter NE, Gordon KA, Campos JL, etal. BalanCI: head-referenced cochlear implant stimulation improves balance in children with bilateral cochleovestibular loss. Audiol Neurootol.
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Vestibular Migraine
DanielleM.Gillard andJeffreyD.Sharon
History
The rst possible description of vestibular migraine has been attributed to Aretaeus
of Cappadocia, who connected vertigo, headache, and tinnitus. Writing in the rst
century CE, he noted “… if the head be whirled round with dizziness, and the ears
ring as from the sound of rivers rolling along with a great noise, or like the wind
when it roars among the sails, or like the clang of pipes or reeds, or like the rattling
of a carriage, we call the affection Scotoma (or Vertigo); a bad complaint indeed, if
a symptom of the head, but bad likewise if the sequela of cephalæa….”
In 1961, Bickerstaff associated migraine with vertigo with a proposal for “basilar
artery migraine” [1]. At the time, it was thought that migraine predominantly
resulted from alterations in intracranial vasculature, causing ischemia followed by
arterial dilation. Bickerstaff noted that if the basilar artery was affected, then reversible brainstem dysfunction would occur, marked by visual loss, vertigo, dysarthria,
tinnitus, unsteadiness of gait, alterations in consciousness, and paresthesias. In the
current version of the International Classication of Headache Disorders (ICHD-3),
this entity is referred to as “migraine with brainstem aura,” and includes a migraine
with at least two transient brainstem symptoms, including dysarthria, vertigo, tinnitus, decreased hearing, diplopia, ataxia (not attributable to a sensory decit), and/
or decreased level of consciousness. While they share similarities, migraine with
brainstem aura and vestibular migraine are separate entities. The vast majority of
patients with vestibular migraine don’t t the denition for migraine with brainstem
aura. We prefer the term vestibular migraine, unless clear transient neurologic decits referable to the brainstem, such as dysarthria, are present during attacks.
D. M. Gillard · J. D. Sharon (*)
Department of Otolaryngology/Head and Neck Surgery, University of California,
San Francisco, CA, USA
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
B. T. Crane et al. (eds.), Disorders of the Vestibular System,
https://doi.org/10.1007/978-3-031-40524-2_11
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In 1917, Boenheim rst coined the term “vestibular migraine” [2]. Associations
between vertigo and migraine were highlighted by Heveroch [3], Symonds [4],
Richter [5], Levy [6], and Friedman [7]. In 1960, Shelby and Lance [8] published a
large case series of patients with migraine, and noted that one-third of the time vertigo
was present during the headache phase. Kayan and Hood published a study in 1984,
nding that 59% of patients with migraine had vestibulocochlear symptoms, and that
half had a history of motion sickness [9]. In 1992, Cutrer and Baloh published a paper
on “migraine associated dizziness,” which described a cohort of patients with dizziness, who had a history of migraine, and whose dizziness could not be ascribed to
another disorder [10]. In the late 90s, other authors used similar terms, including
“migraine associated vertigo” [11], and “migraine-related vestibulopathy” [12]. In
1999, Dieterich and Brandt [13] published a case series of 90 patients with episodic
vertigo, thought to be a variant of migraine. They noted that only 7.8% of patients in
their cohort t the denition of basilar artery migraine, and therefore argued that vestibular migraine is a more inclusive diagnostic entity. In 2001, Neuhauser etal. proposed the rst widely used diagnostic criteria for vestibular migraine [14]. However,
the disorder was not included in the 2004 International Classication of Headache
Disorders (ICDH-2), but was included as a migraine disorder in the ICDH-3 published in 2018. The Barany Society, an international consortium of vestibular specialists, published diagnostic criteria for vestibular migraine in 2012 [15].
D. M. Gillard and J. D. Sharon
Epidemiology
Migraine headaches are one of the most commonly diagnosed diseases in the world.
Roughly 33% of women and 13% of men will suffer from migraine at some point in
their life [16]. Migraine without aura is dened by the International classication of
Headache Disorders third edition (ICDH-3) as a headache lasting 4–72h that has
two of the four following characteristics: unilateral, pulsating, moderate or severe
intensity, and aggravated by activity. It also has either nausea/vomiting and/or photophobia or phonophobia [17]. Migraines can be accompanied by an aura that is
usually visual in nature, often described as scotoma (area of visual loss) or perceptual disturbance, such as seeing ashing zig zag lines or other iridescent patterns
that usually move across the visual eld. Migraine without aura is far more common
than migraine with aura. In a 2001 study of patients presenting to both dizziness and
migraine clinics, there was a large overlap discovered between patients [18]. In the
dizziness clinic 38% of patients met the criteria for migraine, and in the migraine
clinic 16.5% reported episodic vertigo.
Vestibular migraine (VM) has an estimated prevalence is between 1% and 2.7%
of adults [19, 20]. Depending on the study population, VM is the rst or second
most common cause of dizziness after benign positional paroxysmal vertigo
(BPPV). Using the 2008 National Health Interview Survey data, Formeister etal.
found that 11.9% of US adults had a problem with dizziness or imbalance in the
prior year. Of those, 23% met a case denition for vestibular migraine, representing

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2.7% of the adult US population. Sixty-four percent were female. Of those with
VM, 60% had missed work or school, and 52% had experienced a fall in the prior
year (both higher than population averages, and also averages for subjects with dizziness without VM). Remarkably, of those given a diagnosis, only 10% were told
that vestibular migraine was the cause of their symptoms, suggesting that underdiagnosis is common. Vestibular migraine is most likely to present between the third
to sixth [18, 21] decade of life, and it can present either as the initial migraine manifestation or after a prior diagnosis of migraine headache [13, 21].
Dizziness, like migraine, is also a common symptom. Up to 4% of ER visits [22]
and 5% of primary care provider visits [23] every year are for dizziness as the chief
complaint. Dizziness represents a large economic and quality of life burden in the
United States. The total annual medical expenditure for vertigo and dizziness is
almost $50 billion [24]. Costs for the workup of dizziness make up roughly 4% of
Emergency Department (ED) costs per year and exceed $4 billion with $360 million
for the cost of CT scans and $110 million for the cost of MRI in the ED alone [25].
Dizziness has also been shown to increase missed work days which leads to
decreased productivity, an additional economic burden [25, 26]. Vertigo and dizziness also increase the risk of falls, which can lead to resulting injury, especially in
an aging population. In individuals over 40, those with vestibular complaints had
12-times higher odds of falling compared to older individuals without vestibular
complaints [27]. Dizziness, especially in the elderly, is linked to increased disability
and lower quality of life independent of other comorbidities [28].
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Migraine Variants
While migraine is predominantly associated with headaches, it’s important to note
that there are several migraine variants that don’t involve a headache and have predominant symptoms that differ from the classical migrainous features. Typical aura
without headache, or acephalgic migraine, is a migraine variant that presents with
the usual migraine aura without associated headache [29]. So, for example one
could experience a visual aura by itself, without any other symptoms. Hemiplegic
migraine is a type of migraine with aura that presents with motor weakness symptoms [30]. Basilar artery migraine presents with symptoms of brainstem dysfunction including dysarthria, vertigo, tinnitus, hyperacusis, diplopia, ataxia and altered
consciousness [31]. Therefore, it’s clear that while headache is a major feature of
migraine, it’s only one of numerous neurologic manifestations of the disease.
There are also several episodic syndromes in children that are now thought to
be migrainous symptoms or precursors to classical migraine. This includes
abdominal migraine, which presents as recurrent abdominal pain in children [32]
and cyclical vomiting syndrome [33]. Furthermore, it’s now understood that both
benign paroxysmal vertigo of childhood and benign paroxysmal torticollis of
childhood are migraine variants [34]. In benign paroxysmal torticollis of infancy
children present in the morning with direction-varying head tilt that improves at

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night-time [35]. Benign paroxysmal vertigo of childhood is a pediatric migraine
syndrome that has predominantly vertiginous symptoms. Benign paroxysmal vertigo of childhood generally presents between ages 2 and 4, with discrete episodes
of vertigo that last seconds to minutes in otherwise healthy children. This disorder
is usually self- limited over the course of several years and usually resolves by age
7 or 8 [36]. It was rst described by Basser in 1954 [37] and was rst associated
with migraine headache in 1967 by Fenichel [37, 38]. These pediatric disorders
are now well recognized as a migraine variants and it has been shown that these
children are at an increased risk of developing typical migraine later in life [37–
39]. They are categorized in the ICHD-3 as “episodic syndromes that may be
associated with migraine.”
Furthermore, migraines can have a variety of associated otolaryngologic symptoms including neck pain, sinus pressure, nasal congestion, lacrimation, conjunctival infection, eyelid swelling, facial ushing and aural pressure. Many patients with
“rhinosinusitis”—especially those with clear CT scans—have been shown to suffer
instead from a migraine variant [40]. Additionally, the majority of individuals with
“sinus headache” meet the criteria for migraine or probable migraine [41]. These
patients frequently report facial pain, nasal congestion and rhinorrhea, which could
be confused with a primary sinus etiology. Interestingly, these patients have been
shown to have symptomatic improvement with classic migraine treatments, including response to empiric treatment for migraine with triptans [42]. It is clear that
there are a variety of atypical migraine syndromes and there are many that present
with otolaryngologic symptoms and ndings, underscoring the fact that one must be
familiar with migraine to understand many otolaryngologic symptoms.
D. M. Gillard and J. D. Sharon
Association ofMigraine andVestibular Migraine withOther
Vestibular Diseases
VM and migraine are commonly associated with a variety of other causes for vertigo. Those who suffer from BPPV, the most common cause of dizziness in the
general population, have between 38% and 56% incidence of concomitant migraine
symptoms [18, 43, 44]. In a cohort of posterior canal benign positional paroxysmal
vertigo (BPPV) patients at UCSF, those with migraine presented with BPPV about
5years earlier than the non-migraine cohort [45]. However, there was no difference
in severity of dizziness symptoms based on DHI score and no signicant differences
in the rate of self-reported falls or BPPV recurrence in the migraine versus nonmigraine group. In Meniere’s, another common otolaryngologic disorder that causes
symptoms of vertigo, 38%–56% of patients also have a history of migraine [46].
Migraine history has also been shown to be associated with bilateral vestibular loss;
up to 50% of cases of idiopathic bilateral vestibular loss have a positive migraine
history [47]. The number of vestibular diseases associated with migraine is quite
remarkable, and highlights the need to understand how migraine affects the vestibular periphery.
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