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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 benet 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 benet from pharmacological therapies, though typically these are ideally combined with other treatments. The use of medications to treat specic 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 benets 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 signicantly 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 conrm 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
10 Balance andVestibular Disorders inChildren andAdolescents
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demonstrated that children with severe vestibular impairment and poor balance in the setting of bilateral congenital SNHL may benet 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 areexia, as well [63].
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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 specic conditions above. Vestibular migraine can benet greatly from “migraine hygiene,” including trigger identication/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 benecial for many patients with migraine [65, 66].
Accommodations andFollow-Up
Many children with chronic vestibular disorders may benet 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 difculty 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 benecial for children with vestibular disorders are summarized in Table10.7.
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 conrm 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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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 trafc 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 benecial 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 conrm 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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26. Brodsky JR, Cusick BA, Kawai K, Kenna M, Zhou G.Peripheral vestibular loss detected in pediatric patients using a smartphone-based test of the subjective visual vertical. Int J Pediatr Otorhinolaryngol. 2015;79(12):2094–8.
27. Cushing SL, Gordon KA, Rutka JA, James AL, Papsin BC.Vestibular end-organ dysfunction in children with sensorineural hearing loss and cochlear implants: an expanded cohort and etiologic assessment. Otol Neurotol. 2013;34(3):422–8.
28. Cushing SL, Papsin BC, Rutka JA, James AL, Gordon KA.Evidence of vestibular and bal­ance dysfunction in children with profound sensorineural hearing loss using cochlear implants. Laryngoscope. 2008;118(10):1814–23.
29. Rine RM, Braswell J, Fisher D, Joyce K, Kalar K, Shaffer M.Improvement of motor develop­ment and postural control following intervention in children with sensorineural hearing loss and vestibular impairment. Int J Pediatr Otorhinolaryngol. 2004;68(9):1141–8.
30. Rine RM, Cornwall G, Gan K, etal. Evidence of progressive delay of motor development in children with sensorineural hearing loss and concurrent vestibular dysfunction. Percept Mot Skills. 2000;90(3 Pt 2):1101–12.
31. De Kegel A, Maes L, Van Waelvelde H, Dhooge I.Examining the impact of cochlear implan­tation on the early gross motor development of children with a hearing loss. Ear Hear. 2015;36(3):e113–21.
32. Jang JH, Kim H, Choo OS, Park HY, Choung YH.Changes in vestibular symptoms and func­tion after cochlear implantation: relevant factors and correlations with residual hearing. Clin Exp Otorhinolaryngol. 2020;14:69.
33. Gopen Q, Zhou G, Whittemore K, Kenna M.Enlarged vestibular aqueduct: review of contro­versial aspects. Laryngoscope. 2011;121(9):1971–8.
34. Tsang SH, Aycinena ARP, Sharma T. Ciliopathy: usher syndrome. Adv Exp Med Biol. 2018;1085:167–70.
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36. Goderis J, De Leenheer E, Smets K, Van Hoecke H, Keymeulen A, Dhooge I.Hearing loss and congenital CMV infection: a systematic review. Pediatrics. 2014;134(5):972–82.
37. Shamriz O, Tal Y, Gross M. Autoimmune inner ear disease: immune biomarkers, audio­vestibular aspects, and therapeutic modalities of Cogan’s syndrome. J Immunol Res. 2018;2018:1498640.
38. Casselbrant ML, Villardo RJ, Mandel EM.Balance and otitis media with effusion. Int J Audiol. 2008;47(9):584–9.
39. Cohen MS, Mandel EM, Furman JM, Sparto PJ, Casselbrant ML.Tympanostomy tube place­ment and vestibular function in children. Otolaryngol Head Neck Surg. 2011;145(4):666–72.
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47. Brodsky JR, Kaur K, Shoshany T, etal. Torticollis in children with enlarged vestibular aque­ducts. Int J Pediatr Otorhinolaryngol. 2020;131:109862.
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48. Kim HA, Bisdorff A, Bronstein AM, etal. Hemodynamic orthostatic dizziness/vertigo: diag­nostic criteria. J Vestibular Res. 2019;29(2–3):45–56.
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54. McCaslin DL, Jacobson GP, Lambert W, English LN, Kemph AJ. The development of the Vanderbilt pediatric dizziness handicap inventory for patient caregivers (DHI-PC). Int J Pediatr Otorhinolaryngol. 2015;79(10):1662–6.
55. Pavlou M, Whitney SL, Alkathiry AA, etal. Visually induced dizziness in children and valida­tion of the pediatric visually induced dizziness questionnaire. Front Neurol. 2017;8:656.
56. Almutairi AB, Christy JB, Vogtle L. Psychometric properties of clinical tests of bal­ance and vestibular- related function in children with cerebral palsy. Pediatr Phys Ther. 2020;32(2):144–50.
57. Christy JB, Payne J, Azuero A, Formby C.Reliability and diagnostic accuracy of clinical tests of vestibular function for children. Pediatr Phys Ther. 2014;26(2):180–9.
58. Lee GS, Zhou G, Poe D, etal. Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children. Laryngoscope. 2011;121(10):2256–61.
59. Weinreich HM, Carey JP.Perilymphatic stulas and superior semi-circular canal dehiscence syndrome. Adv Otorhinolaryngol. 2019;82:93–100.
60. Rawal RZX, Lipson S, Brodsky JR.Endoscopic repair of traumatic perilymphatic stula in children: a case series. J Adv Otol. 2021;17:182–5.
61. Beyea JA, Agrawal SK, Parnes LS.Transmastoid semicircular canal occlusion: a safe and highly effective treatment for benign paroxysmal positional vertigo and superior canal dehis­cence. Laryngoscope. 2012;122(8):1862–6.
62. Wolter NE, Gordon KA, Campos JL, etal. BalanCI: head-referenced cochlear implant stimu­lation improves balance in children with bilateral cochleovestibular loss. Audiol Neurootol. 2020;25(1–2):60–71.
63. Azevedo YJ, Ledesma ALL, Pereira LV, Oliveira CA, Bahmad F Jr. Vestibular implant: does it really work? A systematic review. Braz J Otorhinolaryngol. 2019;85(6):788–98.
64. Avery J, Etheridge L.Is high-dose magnesium supplementation helpful in adolescents with migraine? Arch Dis Child. 2021;106(10):1027–30.
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67. Headache Classication Committee of the International Headache Society (IHS). The interna­tional classication of headache disorders, 3rd edition. Cephalalgia. 2018;38(1):1–211.
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Chapter 11
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Vestibular Migraine
DanielleM.Gillard andJeffreyD.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 Classication 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 decit), 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 denition for migraine with brainstem aura. We prefer the term vestibular migraine, unless clear transient neurologic de­cits 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 denition of basilar artery migraine, and therefore argued that ves­tibular migraine is a more inclusive diagnostic entity. In 2001, Neuhauser etal. pro­posed the rst widely used diagnostic criteria for vestibular migraine [14]. However, the disorder was not included in the 2004 International Classication 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 dened by the International classication of Headache Disorders third edition (ICDH-3) as a headache lasting 4–72h 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 etal. found that 11.9% of US adults had a problem with dizziness or imbalance in the prior year. Of those, 23% met a case denition 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 ofMigraine andVestibular Migraine withOther 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 5years earlier than the non-migraine cohort [45]. However, there was no difference in severity of dizziness symptoms based on DHI score and no signicant 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.