3.6 Vertigo
3.6.1 Balance Assessment
Key Features
Electronystagmography (ENG)
Rotational chair
Dynamic post urography
Vestibular evoked myogenic potential (VEMP)
Balance involves three components: (1) sensory input from the visual, vestibular, and proprioceptive systems; (2) central nervous system integration of these sensory signals with the subsequent generation of appropriate motor commands; and (3) adequate musculoskeletal abilities to perform the motor tasks. Proper assessment of balance/vertigo issues involves looking at all three of these components.
Epidemiology
Dizziness is a common symptom in ~30% of people over the age of 65. Approximately 615,000 persons in the United States have been diagnosed with Ménière′s disease. Between 10 and 64 persons per 100,000 are affected by benign paroxysmal positional vertigo (BPPV) each year. Hundreds of thousands of hospital days are incurred every year in the United States due to vertiginous symptoms.
Clinical
Persons with vestibular disorders report a variety of symptoms. They can include, but are not limited to, vertigo (i.e., spinning sensation), dysequilibrium (i.e., subjective sense of falling), imbalance (i.e., observable unsteadiness), lightheadedness (i.e., feeling of faintness), oscillopsia (i.e., vision instability with head movement), nausea, and vomiting. Dizziness can also be accompanied by hearing loss, tinnitus, and hyperacusis (sound sensitivity). Dizziness can be brief (lasting seconds to minutes) or prolonged (lasting hours to days). Patients may report episodic or positional dizziness. Precipitating factors such as recent upper respiratory tract infection, head trauma, vestibulotoxic drug exposure, and migraine can be ascertained.
Evaluation
Physical Exam
A full head and neck with otologic and cranial nerve exam is performed. Neurologic exam can also give insight into central causes of dizziness. Use of Frenzel goggles can help accentuate nystagmus on exam by removing visual fixation. Physical exam should include evaluation of spontaneous, gaze, and headshake nystagmus. Gait is assessed for ataxia and wide stance. Romberg and Fukuda stepping tests are done to evaluate for sway. A Dix-Hallpike test will assess presence of posterior canalolithiasis. Cerebellar testing (such as finger-to-nose) can test for dysmetria and tremor. A head impulse test can demonstrate catch-up saccades. Proprioception of upper and lower extremities can be assessed with a tuning fork. Mobility of the neck (e.g., cervicogenic dizziness) and vertebral artery (e.g., vertebral artery stenosis) should be tested as well.
Basic Balance Testing
ENG/VNG
Electronystagmography (ENG) and videonystagmography (VNG) test peripheral and central vestibular function. During ENG, electrodes are placed above and below the eyes, at the outside corner of the eye, and on the forehead. Electrical potentials during testing reflect direction and velocity of eye movements. VNG does not rely on electrical potentials but rather on video analysis of eye motion. VNG measures the movements of the eyes directly through infrared cameras and may be more accurate, more consistent, and more comfortable for the patient than traditional ENG. One advantage to VNG is the ability to test torsional nystagmus in addition to vertical and horizontal nystagmus. There are four main components to ENG/VNG:
Oculomotor activity: Smooth pursuit tracking, saccade analysis, gaze fixation, spontaneous nystagmus, and optokinetic stimulation
Positioning tests: Such as Dix-Hallpike
Positional tests: Nystagmus on head supine, head left and right, body left and right, and 30° incline
Caloric irrigation: Warm and cold air or water
Rotary Chair
Rotational chair testing expands the evaluation of the peripheral vestibular system beyond the frequency and intensity limitations of the ENG/VNG evaluation. The phase, gain, and symmetry of the vestibular ocular reflex are assessed. The patient is then subjected to various rotations through sinusoidal harmonic acceleration tests and step tests with the head restrained to a chair with a computer-controlled motor in a darkened enclosure. Off-axis rotation provides ear-specific information and tests the utricle and superior vestibular nerve function. Standard rotational chair tests the horizontal semicircular canal function and stimulates the otoliths. Utricular function can specifically be assessed through subjective visual vertical or horizontal testing. The patient attempts to set an illuminated light to true vertical or horizontal in the absence of a visual reference and ambient light.
Posturography
Dynamic posturography assesses the functional relative use of vision, vestibular, and somatosensory cues. Sensory organization is assessed by measuring a patient′s ability to maintain balance while disrupting somatosensory and/or visual input. The patient is exposed to six conditions using a combination of normal, eyes closed, and the tilting of the support surface and/or the visual surround. Instability or fall on conditions 5 (eyes closed, moving platform, stable visual surround) and 6 (eyes open, moving platform and visual surround) suggest peripheral vestibulopathy.
VEMP
Cervical vestibular evoked myogenic potentials (cVEMP) are recorded by electrodes placed on the ipsilateral sternocleidomastoid muscle in response to click stimuli. This assesses saccular function and the integrity of the inferior division of the vestibular portion of the eighth cranial nerve. Lower thresholds and increased peak-to-peak amplitudes suggest superior semicircular canal dehiscence. Asymmetry ratios > 40% suggest a saccular or inferior vestibular nerve weakness.
Ocular vestibular evoked potentials (oVEMP) are recorded by electrodes placed on the contralateral inferior oblique muscle in response to sound stimulus. This test assesses utricular function and the integrity of the superior division of the vestibular nerve. Lower thresholds and increased peak-to-peak amplitudes suggest superior semicircular canal dehiscence. Asymmetry ratios > 40% suggest a utricular or superior vestibular nerve weakness.
Other Tests
An audiologic evaluation should be conducted when a balance disorder is suspected to be vestibular in origin.
Pathology
Balance disorders can be caused by disorders of the peripheral vestibular system or the central vestibular system, systemic issues with parts of the body other than the head and brain, and vascular disorders. Peripheral vestibular system issues can be caused by Ménière′s disease, labyrinthitis, BPPV, perilymphatic fistula, vestibular neuritis (VN), ototoxicity, vestibular schwannomas, superior canal dehiscence syndrome (SCDS), autoimmune disease, and other disorders. Central vestibular system issues can be caused by migraine, hydrocephalus, brain tumors, multiple sclerosis, cerebrovascular disease including transient ischemic attack or stroke, stress, tension, fatigue, vision disturbances, and other disorders. Systemic issues can include peripheral neuropathies, hyperventilation, and dehydration. Vascular disorders can include orthostatic hypotension, arteriosclerosis, vertebral artery stenosis, and vasovagal syndrome. Cervicogenic dizziness can occur after neck injury or in association with neck pain.
Treatment Options
Treatment for the underlying cause of dizziness should be initiated. Factors that contribute to dizziness (e.g., migraine, visual disturbance) should be addressed and treated. In cases of BPPV, canalith repositioning is highly effective (e.g., the Epley maneuver for posterior canalolithiasis). Ablation of the vestibular organ through vestibulotoxic injections (e.g., intratympanic gentamicin) or surgery (e.g., labyrinthectomy or vestibular nerve section) may be appropriate in certain situations to allow symptom reduction through compensation. In cases of superior semicircular canal dehiscence or a perilymphatic fistula, surgery to repair the defect may alleviate the patient′s symptoms. Neurology consultation is ordered when central vestibulopathy or complex vestibular migraine is suspected.
Vestibular therapy is helpful in a number of vestibular disorders. Adaptation, habituation, substitution, and particle repositioning exercises are addressed during vestibular therapy. Vestibulosuppressants can be prescribed for severe vertigo; however, this may prolong time to recovery.
Complications
Vision problems and limitations in physical mobility can influence the ability to conduct accurately some components of ENG/VNG, rotary chair, posturography, and VEMP testing. Results can also be influenced by a variety of medications and alcohol.
3.6.2 Benign Paroxysmal Positional Vertigo
Key Features
Transient, episodic vertigo is induced by head position changes.
Benign paroxysmal positional vertigo (BPPV) is most commonly caused by otoliths displaced into the posterior semicircular canal.
Posterior canalolithiasis produces a latent geotropic upbeating torsional nystagmus that fatigues with time on Dix-Hallpike test.
Repositioning maneuvers depend on canal involved.
Benign paroxysmal positional vertigo (BPPV) is the most common form of vertigo reported by multidisciplinary balance centers. Displaced otoconia settle in the lowest part of the inner ear, the posterior semicircular canal. Head motion moves these crystalline masses, stimulating the neuroepithelium of the posterior semicircular canal. This causes patients to experience brief episodes of vertigo. Vertigo with latent and fatigable geotropic upbeating rotary nystagmus elicited by the Dix-Hallpike maneuver is diagnostic of posterior canalolithiasis, and most cases are treated successfully with the Epley repositioning maneuver. The horizontal canal is the second most commonly affected semicircular canal; anterior (or superior) canalolithiasis is rare.
Epidemiology
BPPV is the most common form of dizziness. Typically, it occurs spontaneously in elderly patients; in younger patients, BPPV often presents after head trauma or viral labyrinthitis. Symptoms can resolve without treatment.
Clinical
Signs
In posterior canalolithiais, upbeating, geotropic rotatory nystagmus is noted when a patient is placed in the Dix-Hallpike position. There may be a short latency (delay) in the onset of nystagmus, and the duration is brief (less than 1 minute). Nystagmus reverses upon sitting upright (i.e., downbeating, ageotropic rotary nystagmus). The response fatigues with repetition. In horizontal canalolithiasis, geotropic or ageotropic nystagmus is demonstrated on supine roll test. In anterior canalolithiasis, downbeating torsional nystagmus toward the affected ear can be seen during Dix-Hallpike; it is controversial whether anterior canalolithiasis is a true entity.
Symptoms
The patient experiences a spinning sensation that lasts less than a minute, which is associated with head movement, most often when bending forward, looking upward, or lying down and rolling from one side to another. Patients may also have mild, generalized imbalance. Symptoms may also include nausea and vomiting.
Differential Diagnosis
Most diagnoses can be eliminated on history alone. The differential diagnosis for dizziness lasting seconds includes perilymphatic fistula, BPPV, vertebrobasilar insufficiency, superior semicircular canal dehiscence, and cervicogenic dizziness. Dizziness lasting hours can suggest endolymphatic hydrops, Ménière′s disease, and vestibular migraine. When symptoms last for days, labyrinthine concussion, labyrinthitis, and vestibular neuritis are suspected. Dizziness lasting months may indicated a retrocochlear mass, vestibulotoxicity, or cerebellar disorder. Central etiologies such as stroke and multiple sclerosis can also present with dizziness.
Evaluation
Physical Exam
A full neurotologic exam should be performed. A basic vestibular exam is described in Chapter 3.6.1.
Imaging
Patients with positioning nystagmus suggestive of canalolithiasis do not typically require imaging. Computed tomography (CT) of the temporal bones may be useful in evaluating other causes of dizziness, such as superior semicircular canal dehiscence or temporal bone trauma. Magnetic resonance imaging (MRI) of the brain with and without gadolinium may be useful in ruling out intracranial process or retrocochlear mass.
Labs
Laboratory evaluation is not useful in the diagnosis of BPPV.
Other Tests
Other causes of vertigo may be evaluated with electronystagmography or videonystagmography, vestibular evoked myogenic potentials, rotary chair, and posturography. Tullio phenomenon and Hennebert sign for fistula can also be performed. Audiologic testing is obtained in vestibular symptoms other than BPPV.
Pathology
Cadaveric temporal bone dissections and labyrinthine operations performed in patients with symptoms of BPPV have demonstrated the presence of small calcium crystals (thought to be displaced otoconia from the saccule or utricle) within the posterior semicircular canal. Changing head position causes movement of otoliths within the canal, inducing motion of the cupula. There has also been speculation that cupulolithiasis (presence of otolithic crystals trapped on the cupula) plays a role in some cases. The most common canal affected is the posterior semicircular canal. The second most common canal is the horizontal canal.
Treatment Options
Medical
For posterior canal BPPV, the Epley repositioning maneuver is the mainstay of treatment, with success rates exceeding 90% ( Fig. 3.12 ). This maneuver entails performing a Dix-Hallpike maneuver to elicit vertigo. Following cessation of vertigo, the patient′s head is rotated 90° to the unaffected side. After 45 to 60 seconds, the head is then turned 90° further by having the patient lie on his or her side, such that the face is toward the floor. The patient is then brought back to the upright position with his or her chin tucked to the chest. Each of these positions can be kept for approximately one minute. The canaliths are ultimately repositioned in the vestibule away from sensory epithelium. This procedure may be repeated as needed for relapses or refractory cases. Following the Epley, patients are instructed not to lie flat or assume any provoking position for a few days. Habituation exercises such as Brandt-Daroff exercises can be prescribed for relapsing cases or patients who cannot tolerate the Epley maneuver in the office. The Semont maneuver is an alternative repositioning maneuver for posterior canal BPPV.
Lateral canal BPPV can be treated with the barbecue roll (logroll), Gufoni, or Vannucci maneuvers. Anterior canal BPPV can be treated with an Epley maneuver on the opposite ear or modifications of the Semont maneuver. Habituation exercises can also be prescribed. Referral to vestibular therapy may be necessary when maneuvers cannot be performed in the office (e.g., cervical disease or reduced mobility).