A 9-year-old male, with no significant past medical history, presents to the office with dizziness over 3 days. He reports sudden onset of a “queasy” feeling at bedtime. He then awoke from sleep with a “spinning” sensation, vomited, and felt off balance. Family reports that he looked pale during the episode and was unable to open his eyes due to the spinning sensation. Family was unable to evaluate for nystagmus. He vomited several times and then fell asleep. The next day he stayed in bed all day and continued to complain of a constant spinning sensation. By the following day, patient was off balance, but returned to school.
Patient denied tinnitus, hearing loss, headaches, blurred vision, nystagmus, loss of consciousness, frequent falls, weakness of the arms or legs, chest pain, light sensitivity, or noise sensitivity. He also has no history of seizures, head trauma, concussion, heart disease, or Chiari malformation. He showed third-degree spontaneous nystagmus to the right, positive head impulse test to the left; post headshake revealed nystagmus to the right. He had normal strength and reflexes and no dysmetria or dysrhythmia on finger to nose or arm roll. He showed positive on a Fukuda stepping test to the right and unsteady Romberg’s test.
This is a patient with a classic presentation of left vestibular neuritis (VN). This disorder is believed to result from viral inflammation of the vestibular nerves sparing the labyrinth (cochlea and vestibular end organs). It is distinguished from labyrinthitis, which results in a vestibular deficit AND sensorineural hearing loss.
VN is typically fairly acute in onset and produces clinical findings indicative of an ablative lesion of vestibular function on the involved side. Interruption of the baseline neural firing from the diseased side causes a static imbalance in the vestibule-ocular reflex (VOR) resulting in spontaneous nystagmus (predominantly horizontal in nature) with the fast phase directed toward the intact ear. Initially this nystagmus is “third-degree,” occurring in all cardinal gazes and gradually extinguishes to nystagmus only visible in gaze toward the fast phase, with complete resolution of nystagmus once central compensation for the lesion has been achieved. Other clinical findings include normal otoscopic examination, a positive head impulse test directed toward the affected side, visible postheadshake nystagmus, and positive rotation on the Fukuda stepping test due to imbalance in the vestibulo-spinal reflex.
In the CHOP Balance and Vestibular Program, we were able to perform definitive, multidisciplinary testing. Patient demonstrated normal oculo-motor testing on the videonystagmography (VNG) but baseline spontaneous nystagmus with a 55% reduced left vestibular response on caloric testing. Rotational chair testing showed slightly reduced gain with abnormal asymmetry and abnormal phase angle, which are classic markers of an uncompensated acute vestibular lesion. His vestibular evoked myogenic potential (VEMP) was normal on the left side, pinpointing the lesion to the superior division of the vestibular nerve, since the inferior division innervates the saccule. The video head impulse test (vHIT) showed positive corrective saccade on left acceleration, corroborating the clinical findings of reduced horizontal semicircular canal function on that side.
Our vestibular physical therapists evaluated this patient and instituted a program of therapy to hasten central compensation for the lesion. Within 4 weeks of therapy, he had become asymptomatic and was back to full activity.
In children, the most common cause of dizziness is a vestibular aura associated with migraine, but a host of other disorders from congenital inner ear lesions to concussion and ototoxic vestibulopathy can cause balance problems.
Symptoms from vertigo to imbalance to delayed motor development and impaired dynamic visual acuity can all signify a disorder in the vestibular system. At the CHOP Balance and Vestibular Program, our specialists are equipped to evaluate and treat these problems. The core team includes specialists from Otolaryngology/Neurotology, Audiology, and Physical Therapy. Our multidisciplinary approach with a wide array of consulting, world-class specialists in Neurology, Physiatry, Neuro-Ophthalmology, Neurosurgery, Behavioral Health, and Orthopaedics make us a premier program able to deliver local diagnostics and care.
References and suggested readings
O’Reilly R, T Morlet T, Cushing S (eds). Manual of Pediatric Balance Disorders. Plural, San Diego, 2013.
O’Reilly R, Grindle C, Zwicky E, et al. Development of the vestibular system and balance function: differential diagnosis in the pediatric population. In Kesser BW, Gleason AT (eds): Otolaryngology Clinics of North America, Dizziness and Vertigo Across Lifespan. Elsevier, NY. April 2011; 44(2): 251-271.
O’Reilly R, Greywoode J, Morlet T, et al. Comprehensive vestibular and balance testing in the dizzy pediatric population. Otolaryngol Head Neck Surg. 2011;144 (2):142-148.
O’Reilly R, Morlet T, Nicholas B, et al. Prevalence of vestibular and balance disorders in children. Oto Neurotol. 2010;31(9):1441-1444.
Strupp M, Magnusson M. Acute unilateral vestibulopathy. Neurol Clin. 2015;33(3):669-685
Contributed by: Robert O’Reilly, MD, and Erin Field, PA-C