This article reports vestibular laboratory findings from the most common disorders known to cause dizziness and vertigo in children. Specific information regarding migraine, trauma, benign paroxysmal vertigo of childhood, vestibular neuritis, and otitis media is reviewed, along with indications for balance function testing in children.
There are many disorders that can cause dizziness in the pediatric population. In 1999, Russell and Abu-Arafeh published an epidemiologic study showing that 15% of school-age children had experienced at least one episode of vertigo in the previous year. Numerous investigators have also reported the most common disorders causing vertigo and imbalance in children in their clinics. Even though these reports originate from different clinics and regions of the world, there is surprisingly good agreement regarding the primary causes of dizziness in the pediatric population. Regardless of this fact, children suffering from vertigo and imbalance have received less attention in the literature than their adult counterparts, most likely due to the difficulty that young children have in describing their symptoms, coupled with the challenges that exist for clinicians who work through the differential diagnostic process with children. Despite these limitations, it is now known that the most common disorders that cause dizziness in children may manifest themselves as abnormalities on quantitative balance function testing (ie, rotational testing, electronystagmography [ENG], and vestibular evoked myogenic potential testing). The purpose of this brief report is to describe techniques for determining which children may benefit from quantitative balance function testing and what the reported findings are for the 5 most common disorders accounting for vertigo and imbalance in children.
Background: children with vertigo
Assessment of the vestibular system in the pediatric population is gaining interest for several reasons. First, determining the integrity of the vestibular system can assist physicians in the diagnosis of the impairment and in defining the most appropriate course of treatment. Second, some children with dizziness/vertigo have serious health problems, and vestibular system assessment can help identify patients whose dizziness/vertigo stems from a significant neurologic impairment (eg, a brain tumor). However, unlike in adults, episodic vertigo occurs rarely in children. Further, dizziness in children can manifest itself in many forms. For example, a child with an acute vestibular system impairment may present with many of the same symptoms as adults (eg, vomiting, nystagmus, hearing loss, or ataxia). As with adults, children with vestibular disorders may also present with a loss of vestibular function that is progressive or chronic, thereby affecting the development of postural control. Identifying whether the pediatric dizziness is of vestibular origin requires a team approach beginning with an assessment by a physician. During this initial visit a detailed case history should be obtained along with a comprehensive neurologic and otologic examination. Following these examinations, if neurologic impairments can be excluded, quantitative balance function testing may help identify both peripheral and central vestibular system impairments.
Although dizziness and vertigo do occur in children, few dizziness clinics have expressed an interest in this population. One explanation for this is the challenge of extracting clinical and laboratory information from children. According to Wiener-Vacher, children often cannot report vertiginous symptoms due to their inability to verbalize any abnormal sensations they are experiencing. Vestibular disorders in young children often are dismissed by professionals and caregivers alike, and the symptoms are consequently attributed to behavioral problems (eg, finding ways to attract attention) or simply being “clumsy.” In addition, diseases that affect the vestibular system in adults have a different prevalence than the pediatric population. For example, benign paroxysmal positional vertigo (BPPV) is quoted as the most common form of vertigo in adults. Its prevalence has been estimated at 2.4% in the general adult population. In children, BPPV has been reported to occur in up to 6% of those presenting with symptoms of dizziness. This finding means the provider must approach the diagnosis of childhood dizziness/vertigo with a very different background of knowledge. Finally, while there continues to be interest in the development of techniques for assessing the vestibular system in adults, the same energy has not been devoted to adapting these techniques for application to children. One of the pioneers in the assessment of vestibular function in the pediatric population was Dr David Cyr of the Boys Town Institute. Much of his work in the 1980s focused on adapting existing adult protocols for use in children. Many of these adaptations are still in use today. While interest in the assessment of the vestibular system in children has led manufacturers to develop both age-adjusted pupil tracking algorithms and age-appropriate visual targets, the majority of manufacturers at the time of this report do not offer videonystagmography goggles that are small and lightweight enough to accommodate small children. Having equipment that allows for the accurate assessment of children is critical because many of the disorders causing dizziness and vertigo in children have a vestibular origin. While the clinical utility of quantitative balance function testing in children is well documented, continued research in and development of balance assessment techniques will further our understanding of pediatric dizziness/vertigo.
Differential diagnosis
The medical diagnosis of the patient with vestibular dysfunction is the responsibility of the otolaryngologist, otologist, neurotologist, or otoneurologist. The differential diagnostic process is more complex in children than adults, due to several reasons. First, symptoms of dizziness can manifest differently in children than in adults. Also, young children have limited verbal skills, and often the clinician must rely on the caregiver’s observations for the case history. Accordingly, determining which pediatric patients should receive vestibular testing can be difficult. In this regard, several investigators have designed structured case histories to be used for the evaluation of children reporting dizziness. One example set forth by Ravid and colleagues consists of a set of structured questions ( Fig. 1 ; also in the Appendix of this issue) coupled with a computer algorithm designed to aid in the differential diagnosis of the dizzy child ( Fig. 2 ).
To validate the effectiveness of the questionnaire, Ravid and colleagues performed a retrospective analysis of data collected from all children presenting with dizziness to their clinic over a 2-year period. The structured questionnaire was pitted against the computer algorithm and both of those against the final diagnosis. A total of 62 medical records were reviewed and in 57 (92%) of the patients, the questionnaire-derived diagnoses matched the medical record diagnoses. For 52 patients (84%) the result of the computer-assisted algorithm was identical to the diagnosis given to the patient as stated in the medical record. In a similar vein, Niemensivu and colleagues evaluated a structured case history for the diagnosis of dizzy children. Included in the sample were 24 vertiginous children with a history of episodic vertigo of unknown etiology. These children were age-matched and gender-matched, and underwent an otoneurologic examination, audiometry, and ENG. The most common disorders identified using this approach were otitis media–related vertigo, migraine-associated dizziness (MAD), and benign paroxysmal vertigo of childhood (BPVC).
In the authors’ experience, using a structured case history coupled with a decision tree has worked well in identifying those patients who will benefit from vestibular testing. For instance, if the algorithm suggests the patient may have labyrinthitis, then quantitative testing can be ordered to determine whether the child has a peripheral impairment, whether it is unilateral or bilateral, if unilateral, how severe, and whether the child is compensating centrally for the impairment. Conversely, if a child presents with chronic dizziness, no hearing loss, and specific neurologic deficits, balance function testing would most likely not be indicated, and other issues would need to be ruled out (eg, degenerative disease or posterior fossa tumor) using different techniques (ie, imaging). This information is useful to both the physician and the pediatric physical therapist for prescribing treatment. This approach also streamlines the differential diagnostic process, and affords the physician the ability to be selective in determining which tests will be beneficial to the diagnosis and which will not.
Differential diagnosis
The medical diagnosis of the patient with vestibular dysfunction is the responsibility of the otolaryngologist, otologist, neurotologist, or otoneurologist. The differential diagnostic process is more complex in children than adults, due to several reasons. First, symptoms of dizziness can manifest differently in children than in adults. Also, young children have limited verbal skills, and often the clinician must rely on the caregiver’s observations for the case history. Accordingly, determining which pediatric patients should receive vestibular testing can be difficult. In this regard, several investigators have designed structured case histories to be used for the evaluation of children reporting dizziness. One example set forth by Ravid and colleagues consists of a set of structured questions ( Fig. 1 ; also in the Appendix of this issue) coupled with a computer algorithm designed to aid in the differential diagnosis of the dizzy child ( Fig. 2 ).
To validate the effectiveness of the questionnaire, Ravid and colleagues performed a retrospective analysis of data collected from all children presenting with dizziness to their clinic over a 2-year period. The structured questionnaire was pitted against the computer algorithm and both of those against the final diagnosis. A total of 62 medical records were reviewed and in 57 (92%) of the patients, the questionnaire-derived diagnoses matched the medical record diagnoses. For 52 patients (84%) the result of the computer-assisted algorithm was identical to the diagnosis given to the patient as stated in the medical record. In a similar vein, Niemensivu and colleagues evaluated a structured case history for the diagnosis of dizzy children. Included in the sample were 24 vertiginous children with a history of episodic vertigo of unknown etiology. These children were age-matched and gender-matched, and underwent an otoneurologic examination, audiometry, and ENG. The most common disorders identified using this approach were otitis media–related vertigo, migraine-associated dizziness (MAD), and benign paroxysmal vertigo of childhood (BPVC).
In the authors’ experience, using a structured case history coupled with a decision tree has worked well in identifying those patients who will benefit from vestibular testing. For instance, if the algorithm suggests the patient may have labyrinthitis, then quantitative testing can be ordered to determine whether the child has a peripheral impairment, whether it is unilateral or bilateral, if unilateral, how severe, and whether the child is compensating centrally for the impairment. Conversely, if a child presents with chronic dizziness, no hearing loss, and specific neurologic deficits, balance function testing would most likely not be indicated, and other issues would need to be ruled out (eg, degenerative disease or posterior fossa tumor) using different techniques (ie, imaging). This information is useful to both the physician and the pediatric physical therapist for prescribing treatment. This approach also streamlines the differential diagnostic process, and affords the physician the ability to be selective in determining which tests will be beneficial to the diagnosis and which will not.
The most common disorders
Migraine headache, BPVC, and otitis media are commonly reported in the literature as associated with dizziness/vertigo in children. Trauma and vestibular neuritis also are cited as common causes of dizziness in children. Of note, all of these disorders can result in patients having abnormal findings on balance function testing. This fact reinforces the importance of having vestibular testing adapted for use with children, as well as pediatric normative response data available in the vestibular clinic. Two recent studies illustrate this argument well. First, a recent report by Szirmai evaluated vestibular function in children (ie, younger than age 14 years, N = 66) and adolescents (ie, age 14–18 years, N = 79). Szirmai reported that migrainous vertigo (MV) was the most common disorder causing dizziness in the younger children, followed by extravestibular disease, and then labyrinthitis. Only 36% of the patients in this cohort demonstrated normal vestibular system function. In the group of adolescents, extravestibular disease was the most common cause of vertigo followed by migraine. Only 39% of the adolescents demonstrated normal vestibular results.
Another recent report by Wiener-Vacher reviewed the most common vestibular disorders in more than 2000 children over a 14-year period. Patient records were examined retrospectively to determine the most common diagnoses in children presenting with dizziness and vertigo. Consistent with many earlier reports, the most commonly diagnosed vestibular disease was MV, which was responsible for nearly 25% of cases. BPVC represented 20% of the diagnoses. Cranial trauma and ophthalmological disorders each accounted for 10% of the dizziness cases. In this cohort, vestibular neuritis (5%) and posterior fossa tumors (<1%) were less often encountered.
Table 1 is a summary of findings from a series of studies each describing the most frequent causes of pediatric dizziness/vertigo. The characteristics of these disorders and diseases (ie, pathophysiology and balance function test findings) are now described.
| Balatsouras et al, 2007 | Bower & Cotton, 1995 | Choung et al, 2003 | D’Agostino, 1997 | Erbek et al, 2006 | Ravid et al, 2003 | Riina et al, 2005 | Weisleder & Fife, 2001 | Wiener-Vacher, 2008 | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Total subjects | 687 | % | 54 | 34 | 55 | 282 | 50 | 62 | 119 | 31 | >2000 | |
| Migraine | n (%) | 116 | 16.89 | 11 (20.4) | 4 (11.8) | 17 (30.9) | 15 (5.4) | 17 (34) | 24 (39) | 17 (14.3) | 11 (35.5) | 25% |
| BPVC | n (%) | 133 | 19.36 | 9 (16.7) | 5 (14.7) | 14 (25.5) | 60 (21) | 6 (12) | 10 (16) | 23 (19.3) | 6 (19.4) | 20% |
| Otitis media | n (%) | 22 | 3.20 | 5 (9.2) | 5 (14.7) | x | x | 12 (10.1) | ||||
| Viral infection | n (%) | 98 | 14.26 | 15 (27.7) | 4 (11.8) | 1 (1.8) | 53 (18.8) | 2 (4) | 9 (14) | 14 (11.8) | 5% | |
| Trauma | n (%) | 103 | 14.99 | 3 (5.5) | 3 (8.8) | 4 (7.3) | 85 (30.3) | 2 (3) | 6 (5) | 10% |
Migraine-associated vertigo in children
As mentioned previously, the most common diagnosis in children with vertigo and dizziness is migraine headache (ie, migraine equivalent), although the temporal relationship has been reported to be variable. That is, headache may precede, follow, or occur simultaneously with dizziness/vertigo, and often there are accompanying symptoms such as nausea and/or photophobia. However, the features can also be different from those seen in adults. In children, migraines often are localized to the frontal or periorbital region, last less than 2 hours, and may not manifest themselves as the typical throbbing pain often described by adults. Approximately 20% of children with migraine have associated dizziness. There are 3 commonly reported migraine variants that can produce abnormal findings on quantitative vestibular system testing : basilar migraine (BM), MV, and BPVC. BM occurs in about 3% to 19% of children with migraine and usually occurs at age 7 years. Many migraines present with an aura consisting of different sensory sensations (eg, olfactory, visual, or vestibular). BM has been described in the literature as presenting with an aura consisting of audio-vestibular manifestations such as tinnitus, loss of hearing, acute imbalance, and vertigo. Usually pediatric patients with BM demonstrate normal neurologic examinations. According to Eggers, only a very small proportion of patients with MV meet the criteria for BM.
Etiology and Pathophysiology of Migraine
The pathophysiology of dizziness/vertigo in these migraine variants is currently unresolved. In basilar migraine, the root cause has been suggested to be asymmetric activation of brainstem vestibular nuclei or defective Ca 2+ channels that are shared by the brain and the inner ear. MV is considered by most its own entity, and like BM, its pathophysiology is still not completely understood. Investigators have set forth hypotheses, which include cortical spreading depression affecting the parietoinsular vestibular cortex and changes in activity between the parietal cortex and the vestibular nuclei.
Balance Function Findings in Children with Migraine
Laboratory findings in patients with MV can consist of both central and peripheral impairment, and have been well documented in adults. In 1991 Olsson evaluated 50 patients with BM, 49 of whom demonstrated abnormal ocular motor testing. When caloric test findings are combined across studies, the frequency with which unilateral impairments were observed ranged from 8% to 60% of patients. Marcelli and colleagues reported vestibular findings in 22 children diagnosed with migraine. Of this sample, 73% of the participants with MV demonstrated either peripheral or central vestibular abnormalities. The vestibular manifestations varied and included spontaneous-positional nystagmus, post head-shaking nystagmus, BPPV, vibration-induced nystagmus, absence of vestibular evoked myogenic potentials (VEMPs), and unilateral or bilateral caloric reductions. Vestibular system testing has been demonstrated to provide useful information in the differential diagnosis of children with migraine, especially in those children in whom the headaches are associated with vertigo or dizziness.
Benign paroxysmal vertigo of childhood
BPVC was initially reported by Basser in 1964, describing the clinical presentation of BPVC in 17 children aged 4 years or younger. Primary symptoms included episodic attacks of vertigo lasting from seconds to minutes, resulting in the child being unable to stand without support. Additional symptoms included nystagmus, tinnitus, pallor, diaphoresis, and vomiting. BPVC is a pediatric migraine equivalent recognized by the International Headache Society (IHS) classification system. There is no loss of consciousness during attacks of BPVC, and complete recovery follows an attack. A child who is capable will describe a sensation of spinning. The age of onset of BPVC has been reported to typically occur before 4 years of age and is rarely seen after 8 years. In an epidemiologic study by Russell and Abu-Arafeh, 2% of school-age children met the criteria for BPVC (ie, at least 3 transient episodes of the sensation of rotation, either of the child or of the surrounding environment, severe enough to interfere with normal activity, and not associated with loss of consciousness or any neurologic auditory abnormality).
Etiology and Pathophysiology of Benign Paroxysmal Vertigo of Childhood
While the pathophysiology of BPVC is currently unknown, there is strong supporting evidence that BPVC is a migraine headache variant. Many children with BPVC will go on to develop migraine later in life. At present, the etiology of BPVC remains unknown but there is mounting evidence suggesting that in many cases it is vascular in origin. For example, episodic vasospasm could result in ischemia to the inner ear culminating in an end-organ impairment and vertigo. A proposed central mechanism that has been postulated to be responsible is interruption in blood flow to the vestibular nuclei and associated pathways. Regardless of the etiology, the variable nature of laboratory findings makes diagnosing this disorder difficult. At present, the diagnosis of BPVC is primarily dependent on a reliable and characteristic history.
Balance Function Findings in Children with Benign Paroxysmal Vertigo of Childhood
There has been great variability in the quantitative vestibular test results obtained from patients with BPVC. Several studies have reported that children with BPVC commonly present with significant bithermal caloric asymmetry. However, other investigators have found no such relationship. Mierzwinski and colleagues evaluated 124 children with vertigo, 13 of whom presented with characteristics commonly associated with BPVC. Results showed that of this subset of 13 patients, normal ENG test results occurred for 5 patients. In addition, 1 patient was found to have a significant unilateral weakness, and the other 7 patients had either central vestibular impairments or a mixture of peripheral and central vestibular system impairments.
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