Clinical manifestations

Presentation and course

The ICHD-3 describes benign paroxysmal vertigo (1.6.2) under episodic syndromes that may be associated with migraine (1.6). The disorder is characterized by recurrent, brief attacks of vertigo occurring without warning and resolving spontaneously in otherwise healthy children, although adults may be also affected. The criteria require other conditions be ruled out including posterior fossa tumors, seizures, and other vestibular disorders. The diagnostic criteria are:

Benign paroxysmal vertigo is one of the most common causes of vertigo in children between 2 and 6 years of age (15; 21). An otherwise normal child suddenly, and without warning, becomes vertiginous. Vertigo is so profound that even a sitting posture cannot be maintained and often the child wishes to remain absolutely still. Neither the initiation nor the severity of the attack is influenced by head or body position. The child becomes pale, is clearly frightened, and may lie motionless on the floor. Infants indicate the need to be held by a parent. Consciousness is not altered. A headache is not usually present but may be reported. The prevalence of headache in benign paroxysmal vertigo of childhood has varied significantly in the literature, but a frequency as high as 85% has been reported (14). Motion sickness is also very common in this patient population. Nausea or other abdominal discomfort may follow but should not necessarily be present. Episodes usually last for seconds or minutes, but on rare occasions they may continue for hours to days (05). Nystagmus may accompany the vertigo. The neurologic examination is otherwise normal. The simple parental observation of unsteadiness is sufficient to infer vertigo in young children, especially because the majority of patients will have difficulty articulating vertigo symptomatology (29).

The attacks are stereotyped. Episodes, of which five are required to make the diagnosis, can recur at irregular intervals—sometimes happening in clusters over days to weeks and then subsiding spontaneously. The frequency of episodes may vary from 2 to 10 per month, with a mean duration between 1 and 5 minutes (range 1 minute to 2 days) (01; 33; 35; 36). More often, they recur once a month or less. With time, the attacks either cease completely or evolve into a more common migraine syndrome. The evolution of the condition may first include the additional feature of vomiting and then headache with the vertigo. The vertigo becomes progressively less severe and may disappear altogether.

Multiple video recordings created by a patient’s mother and published by Bertholon and colleagues demonstrated a spontaneous nystagmus occurring during the duration of the vertigo episodes in benign paroxysmal vertigo of childhood (07). The nystagmus was a strong, left horizontal nystagmus at fixation. The duration of the nystagmus was 30 to 90 seconds. The video also demonstrated a rightward deviation of the body. Other potential causes of episodic vertigo were ruled out in this particular patient by way of EEG during the episodes, normal MRI brain, normal audiometry and head impulse test, and normal neurologic and otoneurological evaluation between episodes of vertigo.

Prognosis and complications

There is a paucity of data regarding the long-term outcomes of these children, but overall, as the nomenclature implies, benign paroxysmal vertigo usually has a favorable course, with most cases undergoing remission within a 10-year period (27). However, up to 50% of these children can exhibit persistent symptoms into adolescence and adulthood (29). In one case series, about one-third of patients with benign paroxysmal vertigo went on to have migraine as adults. Interestingly, the cases that evolved into migraine as adults show a longer course of vertigo during childhood (06).

The condition usually disappears spontaneously (range 2 to 16 years; ie, after 3 months to 8 years) based on a study of 54 patients (03). A small series of 13 patients found that five patients later developed headache, meeting diagnostic criteria for migraine without aura, and four met criteria for probable migraine without aura (29). No patients developed headache fulfilling criteria for migraine with aura. Another series demonstrated a two-fold increased risk of developing migraine in the future (24%) when compared to the general childhood population (10.6%) (01).

Significant complications are not common, though severe vomiting, if untreated, may lead to dehydration and electrolyte imbalance. Depending how frequent and disabling the attacks are, both patient and family may be significantly distressed. Patients should receive careful clinical follow-up given the possible evolution into migraine (with or without aura).

Clinical vignette

A 4-year-old boy started having sudden attacks where he dropped to the ground, closed his eyes, looked pale, and curled up in a ball. The episodes began approximately two months prior to presentation. The patient would experience significant nausea, vomiting, and vertigo once or twice per week. The episodes would last until the patient was able to fall asleep and resolve on awakening. He did not complain of any visual changes, weakness, head pain, or numbness with the episodes. There was no reported alteration in his level of consciousness. He had no medical illness at the onset of these episodes, and his past medical history was noncontributory. MRI of the brain without contrast with attention to the posterior fossa and routine 30 min sleep deprivation electroencephalography were reported as normal. Family history was significant for episodic migraine without aura in his mother. There was no family history of hearing loss, Ménière disease, or neurofibromatosis. After one year, the onset of symptoms frequency was every 10 to 14 days, and they slowly tapered to once a month until age 7. At age 7, the patient began having headaches diagnosed as migraine.

The 4-year-old boy described above presented with a history that is consistent with benign paroxysmal vertigo. Normal workup was also reassuring. A family history of migraine may provide a clue to the diagnosis.

Biological basis

Etiology and pathogenesis

The pathogenesis of benign paroxysmal vertigo is still unclear. Symptoms may be related to abnormal activation of brainstem vestibular nuclei and vestibulocerebellar pathways, which are also implicated in migraine pathophysiology, according to functional neuroimaging studies (09). A disruption in the integration between visual, vestibular, somatosensory systems, all of which are regulated by the cerebellum, can lead to a sensation of imbalance or vertigo (48).

In a case series of 79 patients with benign paroxysmal vertigo, 75.5% of patients had family members with migraine (14). The prominent familial prevalence implies that genetic factors play a role, although the mode of inheritance of benign paroxysmal vertigo is still not well understood.

One case report of familial hemiplegic migraine that evolved from sequential benign periodic torticollis and benign paroxysmal vertigo was found to have the CACNA1A gene mutation. Further genetic studies are required to prove a consistent genetic link to this and other known familial hemiplegic migraine mutations (10).

Epidemiology

The prevalence of episodic vertigo and imbalance in children has been found to vary from 0.4% to 5.3% (38; 32). The child’s inability to describe his or her vestibular complaints may account for underdiagnosis. The etiologies of childhood vertigo are vast but fortunately usually benign, ranging from middle ear-related conditions to migraine (31).

Among patients presenting with a complaint of vertigo, benign paroxysmal vertigo of childhood has a prevalence ranging from 13.7% to 18.7% (20; 12). Other common etiologies of childhood vertigo were found to be vestibular migraine (17.6% to 23.8%), labyrinthitis/vestibular neuronitis (8.47%), and posttraumatic vertigo (8.4% to 14%).

The most common age of onset of benign paroxysmal vertigo is less than six years old (01; 33; 13; 29; 05; 42; 34). As age increases during childhood, the frequency of patients developing benign paroxysmal vertigo decreases. In a series of 144 patients with vertigo, benign paroxysmal vertigo was determined to be the etiology of vertigo in 0% of patients greater than 12 years of age (34).

With regard to gender differences, data are controversial, with some stating equality between genders (11) and others documenting a higher prevalence in females (54% to 67%) (06; 14).

Prevention

In a case series of 117 pediatric patients with vestibular symptoms, of which the most common diagnosis was benign paroxysmal vertigo (41.9%), two-thirds of patients reported poor eating habits described as either high carbohydrate intake, prolonged fasting, or habitual high caffeine consumption (14). Symptom improvement was observed in 50% of patients after only dietary alterations. On review of the literature, it is not clear whether benign paroxysmal vertigo has triggers, like in migraine, such as stress, weather changes, or specific food items. The episodes of vertigo, by definition, occur without warning (22).

Differential diagnosis

The differential diagnosis of vertigo in a pediatric patient is broad and consists of both benign and malignant processes. As mentioned above, the most common causes of vertigo in the pediatric population are vestibular migraine, benign paroxysmal vertigo, vestibular neuritis, and posttraumatic vertigo (20; 12). Other less common etiologies are exposure to drugs, toxins, or infectious causes, such as otitis media, motion sickness, Ménière disease, metabolic disorders, posterior fossa tumors, cervical spine abnormalities, and psychogenic causes (20).

Vestibular migraine can be differentiated from benign paroxysmal vertigo by the presence of headache. Although vestibular migraine requires the presence of headache associated with vertigo on at least half of the episodes, patients with benign paroxysmal vertigo do not necessarily experience any headache around the time of their vertiginous symptoms. Vestibular symptoms in vestibular migraine also tend to last for a longer duration when compared to benign paroxysmal vertigo.

Posttraumatic vertigo can result from blunt trauma to the neck, head, and craniocervical junction or from “whiplash” injury. It may present in various patterns of vertigo, but most frequently as benign paroxysmal positional vertigo (25). Posttraumatic vertigo should, by history, have onset following a physically traumatic event, which is not commonly described in benign paroxysmal vertigo. Posttraumatic vertigo, if due to mechanical damage or disruption of the vestibular structures, often has onset very soon after the injury (17).

Vestibular neuritis typically occurs as a single episode with acute onset after an identified viral illness in less than half of patients (40). Symptoms typically last for several days until spontaneous recovery. Physical examination commonly demonstrates unsteady gait and signs of cranial nerve eight dysfunction, such as unilateral and horizontal nystagmus with the fast phase beating away from the affected side. Head impulse test is typically abnormal. Benign paroxysmal vertigo presents with discrete episodes of vertigo lasting seconds to minutes, rarely hours at a time.

Table 1. The Most Common Causes of Vertigo in Childhood with their Associated Features

Benign paroxysmal vertigo of childhood should also be distinguished from benign paroxysmal positional vertigo, which manifests as brief attacks of vertigo in the setting of head position change such as turning in bed or lying down, which is thought to be caused by canalithiasis in the inner ear semicircular canals of the vestibular system. Benign paroxysmal vertigo of childhood can occur at any age but has its peak incidence in the fifth and sixth decades of life (28). Diagnosis of benign paroxysmal positional vertigo of childhood is often confirmed by performing the Dix-Hallpike maneuver and can be treated with canalith repositioning techniques, such as the Epley maneuver. Benign paroxysmal positional vertigo and Ménière disease, which present with symptoms and evidence of hearing changes or hearing loss, are rare in children (02; 37).

Patients presenting with vertigo along with symptoms and signs suggestive of increased intracranial pressure, brainstem compression, or cerebellar compression should alert the examiner to the possibility of a space-occupying lesion, a posterior fossa tumor. Physical examination may demonstrate bulbar palsies due to dysfunction of cranial nerves, long tract signs with hemiparesis, and truncal or limb ataxia. Papilledema may be present. A complaint of headache, worst in the morning and in recumbency, is the most frequently reported symptom associated with posterior fossa tumors (41). Any of the above signs or symptoms should prompt urgent magnetic resonance imaging of the brain.

As mentioned in the introduction, benign paroxysmal vertigo is listed under the “episodic syndromes that may be associated with migraine” section along with cyclical vomiting syndrome, abdominal migraine, and benign paroxysmal torticollis.

Benign paroxysmal torticollis is characterized by spontaneous, recurrent episodes of head tilting in infants and toddlers and may evolve into benign paroxysmal vertigo. Patients with the above-mentioned conditions are believed to have an increased likelihood to develop migraine later in life (43). In fact, studies show a possible progression from benign paroxysmal torticollis to benign paroxysmal vertigo and from benign paroxysmal vertigo to vestibular migraine, a phenomenon termed by Brodsky and colleagues as “vestibular march” (08).

Cyclical vomiting syndrome and abdominal migraine were brought under a new umbrella termed “recurrent gastrointestinal disturbance.” Although vomiting may be a symptom featured in patients with benign paroxysmal vertigo, cyclical vomiting syndrome does not include vertigo, by definition, and the episodes of vomiting should occur at least a week apart from one another. The severity of vomiting also tends to be more severe with at least four vomiting episodes per hour lasting at least an hour and up to 10 days. Similarly, abdominal migraine lacks the presence of vertiginous symptoms despite sharing similarities in symptoms, such as pallor and vomiting.

Benign paroxysmal positional vertigo, though frequently confused with benign paroxysmal vertigo, differs in its age of onset as well as its triggering by position changes. Benign paroxysmal positional vertigo is characterized by the sudden and transient sensation of vertigo, is defined as the subjective perception of rotational or translational movement without external stimulus, and is provoked by positional changes of the head. Benign paroxysmal positional vertigo can occur at any age but has its peak incidence in the fifth and sixth decade of life (28). The pathophysiology will not be discussed here. Diagnosis of benign paroxysmal positional vertigo is often confirmed by performing the Dix-Hallpike maneuver and can be treated with canalith repositioning techniques, such as the Epley maneuver.

Associated or underlying disorders

Other differential diagnosis includes exposure to drugs, toxins and infectious causes, such as otitis media, motion sickness, postinfectious complications such as vestibular neuritis and middle ear effusion, Ménière disease, metabolic disorders, posterior fossa tumors, cervical spine abnormalities, trauma, and psychogenic causes (20). Acute episodic attacks of vertigo in children, not induced by motion, are most often caused by migraine or epilepsy. Benign paroxysmal vertigo of childhood must be distinguished from benign paroxysmal positional vertigo, which manifests as brief attacks of vertigo in the setting of head position change such as turning in bed or lying down, which is thought to be caused by free-floating particles in the inner ear vestibular system. Benign paroxysmal positional vertigo, vestibular neuronitis, and Ménière disease, the usual paroxysmal vertigo syndromes of adults, are rare in children (02; 37).

Diagnostic workup

The diagnosis of benign paroxysmal vertigo is primarily a clinical one; laboratory tests are useful only to exclude other possibilities. A family history of migraine or motion sickness may be present and is helpful in making the diagnosis.

Marcelli and colleagues describe a prospective cohort of 15 patients who met criteria of benign paroxysmal vertigo in which patients were followed over time with regular cochlear and neuro-otological testing (36). Cochlear testing was performed with pure tone audiometry with air and bone conduction. They utilized frequencies of 125 to 8000 Hz and 250 to 4000 Hz for air and bone conduction, respectively. Neuro-otological testing was performed with video nystagmography. ICHD-3 criteria require “normal neurological examination and audiometric and vestibular functions between attacks,” thus, to confirm a diagnosis of benign paroxysmal vertigo, audiometry, and vestibular function should be tested and confirmed as normal. To exclude secondary causes, especially in patients who present with signs or symptoms of elevated intracranial pressure, brainstem compression, cerebellar compression, hemiparesis, or ataxia, an MRI of the brain with and without contrast and an MRI of the cervical spine with and without contrast should be performed. Patients presenting with episodes of vertigo associated with alterations in their level of consciousness, frequent aura-like symptoms, automatisms, or stereotyped motor or sensory symptoms should undergo an electroencephalogram (EEG) to evaluate the possibility of seizures.

Testing of vestibular evoked myogenic potentials (VEMPs) performed in children with benign paroxysmal vertigo of childhood or recurrent vertigo of childhood have been compared to normal controls (18). VEMP testing reflects the function of the utricle and the saccule and consist of cervical vestibular evoked myogenic potentials (cVEMPs) and ocular vestibular evoked myogenic potentials (oVEMPs). Several differences were found between the two groups during the VEMP testing, including increased thresholds for the recurrent vertigo of childhood group. The N1 latency and interval of cVEMP were increased in the recurrent vertigo of childhood group, as well. Their conclusion was that a degree of impairment may exist in the inferior vestibular nerve and its conduction pathway in recurrent vertigo of childhood.

Auditory brainstem response testing may help in supporting the diagnosis of benign paroxysmal vertigo (49). Zhang and his colleagues demonstrated that 37 of their 56 patients (66%) with benign paroxysmal vertigo had abnormal high stimulus auditory brainstem responses.

Management

Acute treatment is rarely needed because the paroxysms of vertigo are usually so brief that the child has recovered before treatment can be initiated. If attacks are frequent, symptomatic medications, such as antiemetics, can be helpful. Prolonged attacks are best treated by sedating the child to induce sleep. Ibuprofen at 10 mg/kg or promethazine with hydrating fluids may be useful to treat acute attacks lasting 15 minutes or longer (46; 23). On review of the literature, no reports of using typical migraine acute therapy, like triptans, has been performed in benign paroxysmal vertigo. It may be reasonable to consider such therapy in refractory cases if no contraindications exist in the patient.

If the episodes become more frequent or are longer-lasting, preventive medications commonly used for migraine can be considered. Data on preventive treatment of migraine with and without aura on the pediatric population are limited. The most frequently used pharmacological agent is topiramate, the only FDA-approved medication for migraine prophylaxis in children 12 years and older. Other pharmaceutical agents include propranolol and cyproheptadine (19). Amitriptyline, valproic acid, levetiracetam, zonisamide, gabapentin, nortriptyline, verapamil, and flunarizine require further evaluation (30). Calcitonin gene-related peptide (CGRP) monoclonal antibodies and onabotulinumtoxinA, although demonstrated to be effective in migraine, have not been tested in benign paroxysmal vertigo.

Behavioral measures such as lifestyle modifications including good sleep hygiene, regular exercise, and a well-balanced diet are usually recommended, although their effectiveness also needs further investigation (24). Duarte and colleagues found dietary alterations favoring low carbohydrate intake, maintenance of regular meal intervals, and lower caffeine intake resulted in symptom improvement (14).