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Vestibular Testing.5

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113 views

Vestibular Testing.5

Vestibular Testing - Continuum
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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REVIEW ARTICLE


Vestibular Testing
C O N T I N UU M A UD I O By Timothy C. Hain, MD; Marcello Cherchi, MD, PhD, FAAN
I NT E R V I E W A V AI L A B L E
ONLINE


VIDEO CONTENT
ABSTRACT
A VA I L A B L E O N L I N E PURPOSE OF REVIEW: Vestibular testing, both at the bedside and in the
laboratory, is often critical in diagnosing patients with symptoms of
vertigo, dizziness, unsteadiness, and oscillopsia. This article introduces
readers to core concepts, as well as recent advances, in bedside and
instrumented vestibular assessments.

RECENT FINDINGS: Vestibular testing has improved immensely in the past


2 decades. While history and bedside testing is still the primary method of
differential diagnosis in patients with dizziness, advances in technology
such as the ocular vestibular-evoked myogenic potential test for superior
canal dehiscence and the video head impulse test for vestibular neuritis
have capabilities that go far beyond the bedside examination. Current
vestibular testing now allows clinicians to test all five vestibular sensors in
the inner ear.

SUMMARY: Contemporary vestibular testing technology can now assess the


entire vestibular periphery. Relatively subtle conditions, such as superior
canal dehiscence or a subtle vestibular neuritis, can now be diagnosed
with far greater certainty.

INTRODUCTION

V
estibular testing is defined as the quantification of the function of
CITE AS:
CONTINUUM (MINNEAP MINN) the motion-sensing portions of the inner ear (semicircular canals
2021;27(2, NEURO-OTOLOGY): and otoliths). Vestibular testing is generally performed in the
330–347.
context of an evaluation of the symptom of dizziness, and such
Address correspondence to
evaluations often benefit from information about hearing.
Dr Marcello Cherchi, 645 N Accordingly, although this article mainly focuses on vestibular assessments, it also
Michigan Ave, Chicago, IL 60611, includes content about how hearing testing contributes to forming a diagnosis.
[email protected].
edu.
Vestibular testing has improved immensely over the past 30 years. Five
motion sensors are located in each inner ear: three semicircular canals and two
RELATIONSHIP DISCLOSURE: otolith organs (the utricle and saccule). Ideally, one should be able to quantify the
Dr Hain has served as an
associate editor for Audiology function of all five.
and Neurotology. Dr Cherchi In 1914, Robert Bárány was awarded the Nobel Prize in Physiology or
reports no disclosures.
Medicine for the development of a test of the lateral semicircular canal.1 Since
UNLABELED USE OF then, and especially in the past decade, new tests have come into clinical use that
PRODUCTS/INVESTIGATIONAL can quantify the remaining two canals (anterior/superior and posterior), as well
USE DISCLOSURE:
Drs Hain and Cherchi report
as both otolith organs.
no disclosures. The main goal of vestibular testing is to determine whether vestibular function
is normal or abnormal, testing in each sensor of the peripheral vestibular
© 2021 American Academy apparatus, which may identify when central vestibular and ocular motor
of Neurology. pathways exhibit dysfunction. For example, if examination identifies subtle

330 APRIL 2021

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spontaneous downbeating nystagmus but testing shows normal canal and otolith KEY POINTS
function, then this would suggest that peripheral vestibular function is intact and
● The function of all five
the observed nystagmus must therefore be arising from a central vestibular vestibular sensors in the
abnormality. inner ear, including the
otolith organs (saccule and
BEDSIDE TESTING utricle) and all three
semicircular canals, can now
Dizziness has numerous causes, many of which are unrelated to the peripheral
be tested.
vestibular system (eg, blood pressure fluctuations). A thorough neurologic
history and general neurologic evaluation are desirable. The approach to the ● The assessment of the
patient with dizziness when time does not permit a complete neurologic balance of a patient with
evaluation has been discussed elsewhere2 and is discussed in the article dizziness starts when the
patient is met in the waiting
“Approach to the History and Evaluation of Vertigo and Dizziness” by Terry D. room and walked to the
Fife, MD, FAAN, FANS,3 in this issue of Continuum. examination room.
In this section, the repertoire of relevant bedside tests is briefly described.

Balance Assessment
The goal of the assessment of balance is to quantify imbalance, look for
inconsistency, and to separate vestibular patterns of imbalance from other
neurologic problems, such as cerebellar ataxia, sensory loss in the feet,
movement disorders, and simulated unsteadiness.

GAIT OBSERVATION. The assessment of the balance of a patient with dizziness starts
when the patient is met in the waiting room and walked to the examination
room. Informal observations should be made concerning how the patients arise
from their chair, as well as how they lower themselves into the examination room
chair, their speed of locomotion, whether they swing their arms, and whether
they have a wide-based gait or use the wall or a caregiver’s arm to steady
themselves. To screen for a functional disorder, it is helpful to compare informal
observation and formal balance testing such as the Romberg test, which is
described in the following section. Most patients with acute vestibular problems
are unsteady, and most patients with chronic vestibular problems are not
unsteady. Inconsistencies should be noted.

EYES-CLOSED TANDEM ROMBERG TEST. The tandem Romberg test, also referred to as
sharpened Romberg, is quick and useful, albeit also nonspecific and somewhat
insensitive.4 It is a test for sensory ataxia. Borderline normal performance
consists of the ability to stand heel-to-toe, with eyes closed, for 6 to 30 seconds.
The test can be made easier and thus quantified to some extent by allowing the
eyes to be open or by allowing the feet to be in parallel but next to one another
(standard Romberg test) rather than in tandem. Variants of the test involve
standing on a foam pad, rather than in tandem, or on a narrow rail.5,6
High-normal performance, defined as the time before a step is required to
prevent a fall, is generally found in young adults, who can often perform the
eyes-closed tandem variant of the Romberg test for 30 seconds. Performance
declines greatly with age, especially in patients in their seventies and older.6
Many middle-aged patients with chronic inner ear disorders will have no
difficulty standing in tandem with their eyes open, but they may need to take a
step before 6 seconds passes with eyes closed.
It is helpful to develop a judgment of how much impairment of the Romberg
test is appropriate for a given degree of ear injury. Patients with bilateral

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VESTIBULAR TESTING

vestibular loss have moderate ataxia; they rely heavily on their vision and are
unsteady when their eyes are closed when standing with a narrow base, whether
together in parallel or tandem. Most patients with substantial bilateral vestibular
loss cannot stand in the eyes-closed tandem Romberg test for 6 seconds. Patients
with bilateral vestibular deficits with an additional superimposed position sense
deficit, such as peripheral neuropathy, lose balance when standing with a
narrowed base even with eyes open. Patients with chronic unilateral vestibular
loss are only mildly ataxic, and they usually perform normally on the eyes-closed
tandem Romberg test. Patients with acute unilateral vestibular hypofunction
with nystagmus may be much more off balance but can adapt in weeks to a few
months to show fairly normal balance.

THE FUKUDA STEPPING TEST. The Fukuda stepping test (FIGURE 2-1) and assessment
for past-pointing are measurements of vestibulospinal function. They are rarely
used in contemporary clinical practice. In the Fukuda stepping test, the patient is
asked to march in place with eyes closed for approximately 30 seconds; and the
clinician then notes rotation and translation on a calibrated mat.7 The Fukuda
stepping test fell from popularity after it was shown that it has very wide
variability in subjects without balance problems. Honaker and Shepard8
concluded, “Overall, the [Fukuda stepping test] provides little benefit to
clinicians when used in the vestibular bedside examination.”
The past-pointing test is also called the Quix test.9 During the test, the patient
and examiner assume mirror-image postures with outstretched hands so that the
fingers almost touch, and the examiner assesses whether the patient’s fingers drift
after their eyes are closed. The Quix test has not undergone rigorous scrutiny in the
literature, and it is rarely used. Practically, much stronger tools are available to
detect vestibular imbalance based on nystagmus, ie, Frenzel goggle testing.

Hearing Assessment at the Bedside


Because many vestibular conditions share
an underlying pathology with the hearing
apparatus, it is prudent to examine hearing
and evaluate the status of the external ear
and tympanic membrane. High-frequency
hearing can be screened quickly with the
rubbed-fingers test, during which
examiners use their own hearing as a
control. Young patients should be able to
perceive the finger rubbing at an arm’s
length, whereas many older patients
cannot hear the rubbing sound until it gets
as close as 0.3 m (1 ft). Tuning fork tests
can also be used for a similar purpose, but
formal audiometry is the preferred next
step if the patient fails the finger-rub test.
FIGURE 2-1 Low-frequency hearing loss is not well
The Fukuda test. Patients march in assessed with the finger-rub test, and
place for 30 seconds with their eyes
closed. The amount of rotation and
patients with Ménière disease, for
translation is documented and example, may pass this bedside test. In
interpreted from markings on the mat. other words, one cannot avoid ordering

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audiometry even if the finger-rub test is normal if Ménière disease is a possible KEY POINTS
diagnosis.
● Frenzel goggles are
Otoscopy can determine if the ear canal is occluded by cerumen, if a critical to the rapid and
perforation or scarring of the tympanic membrane has occurred, or if a mass is efficient evaluation of
present as may be seen with a cholesteatoma or glomus tumor. patients with dizziness
Finally, some bedside maneuvers are useful in patients with tinnitus, which because they improve the
clinician’s ability to detect
often accompanies dizziness. Rarely, objective tinnitus deriving from the tensor
vestibular nystagmus.
tympani muscle of the tympanic membrane can be diagnosed by careful
observation for intermittent dimpling of the eardrum during otoscopy. In ● Vestibular spontaneous
addition, rarely in patients with pulsatile tinnitus, the examiner will be able to nystagmus is suppressed by
hear the high-flow bruit of a dural arteriovenous malformation with a fixation.

stethoscope. For more information about tinnitus, refer to the article, “Tinnitus,
Hyperacusis, Otalgia, and Hearing Loss” by Terry D. Fife, MD, FAAN, FANS,
and Roksolyana Tourkevich, MD,10 in this issue of Continuum.

Nystagmus Testing
Evaluation of nystagmus is very useful in a patient with dizziness. Optimally, this
requires the use of Frenzel goggles (FIGURE 2-2), which are worn by the patient to
reduce fixation, as well as to magnify the examiner’s view of the patient’s eyes.
Frenzel goggles are useful because most inner ear causes of dizziness produce
nystagmus that can be suppressed by fixation. To see nystagmus roughly 1 week
after onset of an acute vestibular syndrome such as vestibular neuritis, the
patient’s eyes must not be allowed to fixate when being viewed.
Of the two available variants of Frenzel goggles (optical and infrared video),
the infrared video goggles are far superior, but the optical goggles are more
affordable. Without a method of viewing the eyes without fixation, some types
of nystagmus may not be
observable. The ophthalmoscope
can be used for making
inferences about spontaneous
nystagmus if Frenzel goggles are
not available; this is discussed in
more detail in the following
sections.

NYSTAGMUS ASSESSMENT TESTS


THAT DO NOT REQUIRE FRENZEL
GOGGLES. Some types of
nystagmus may be observable
even without Frenzel goggles.

SPONTANEOUS NYSTAGMUS. The


assessment for spontaneous
nystagmus is important for
diagnosing conditions
characterized by vestibular FIGURE 2-2
imbalance, such as vestibular Frenzel goggles. A, Optical Frenzel goggles, which
reduce fixation through the use of +20 diopter
neuritis. It should not be omitted lenses over the eyes. B, Video Frenzel goggles,
in the assessment of a patient which use infrared illumination to prevent fixation;
with dizziness. With either the image of the eye can be made very large.

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VESTIBULAR TESTING

KEY POINTS Frenzel goggles placed on the


patient or using the
● Congenital nystagmus is
enhanced by fixation.
ophthalmoscope, the eyes are
observed for spontaneous
● The ophthalmoscope can nystagmus. FIGURE 2-3
be used to assess illustrates the two most common
spontaneous nystagmus if
types of spontaneous
Frenzel goggles are not
available. nystagmus. The nystagmus
typical of inner ear dysfunction
● The Alexander law can be is jerk nystagmus in primary
used to assess for gaze in which the eyes deviate
spontaneous jerk
nystagmus.
slowly (slow phase) in one
direction followed by a rapid
correction (fast phase) in the
opposite direction. The direction
of nystagmus is named for the
fast phase (VIDEO 2-1). The
more rapidly the eyes deviate off
center (the slow phase), the
more frequently the corrective FIGURE 2-3
Illustration of how the eyes move from side to side.
jerks (the fast phase) occur, By convention, the upward direction on the tracing
making the nystagmus appear corresponds to rightward eye movement, while the
faster. Most nystagmus of other downward direction on the tracing corresponds to
patterns (eg, pendular or leftward eye movement. Pendular nystagmus is
less common but may be seen in some central
saccadic) are of central origin nervous system causes or congenital nystagmus.
(VIDEO 2-2). For the most part, The far more common jerk nystagmus (in this case,
if a jerk-type nystagmus can be to the left because it is named after the fast phase)
detected without the use of some is typical of vestibular nystagmus.
method of blocking fixation, the
patient has either acute dizziness
or a disorder that impairs fixation, such as a cerebellar or brainstem problem. These
central cases are relatively rare compared with inner ear types of dizziness.
An exception to the preceding general rule is congenital nystagmus. Unlike
vestibular nystagmus, congenital nystagmus is often reduced by removal of
fixation, and this is one of the ways it can be recognized. The most common
congenital nystagmus is latent nystagmus, generally found in people with
congenital strabismus (CASE 2-1). Latent nystagmus is a jerk nystagmus that
changes direction according to the viewing eye (VIDEO 2-3). Patients with latent
nystagmus also have no stereovision and will fail bedside tests for stereopsis,
such as the Titmus Fly test.
When Frenzel goggles are not available, the ophthalmoscopic examination can
help with obtaining some information about spontaneous nystagmus. The examiner
monitors movement of the back of the eye while obscuring vision by covering the
other eye. As the back of the eye moves in the direction opposite of the front of the
eye, horizontally and vertically, the examiner must be sure to invert the direction of
the nystagmus when making notes. If an ophthalmoscope is also not available, the
presence of a unidirectional nystagmus can sometimes be inferred from an
evaluation of the effect of gaze nystagmus. According to the Alexander law,
vestibular nystagmus nearly always increases when the patient looks in the
direction of the fast phase, so an asymmetric frequency or intensity of nystagmus

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will be seen depending on whether the patient looks to the right or left (although
the nystagmus does not change direction) in peripheral vestibular nystagmus.11

DIX-HALLPIKE TEST. Ideally, the Dix-Hallpike test is performed with Frenzel


goggles, optical or video (FIGURE 2-4), although in many cases, the nystagmus of
benign paroxysmal positional vertigo (BPPV) is seen readily with the naked eye.
The sensitivity of the test improves when performed with the goggles. For the
Dix-Hallpike test to the right (VIDEO 2-4), the patient’s head is turned 45 degrees
to the right and then the patient is moved quickly from the sitting position to this
head-hanging position. If the patient feels no dizziness or if nystagmus is not
appreciated after 15 seconds, then the patient is moved back up to the sitting
position. For the Dix-Hallpike test to the left (VIDEO 2-5), the patient’s head is
turned 45 degrees to the left and then the patient is quickly laid back to the
head-hanging left position. After 15 seconds, the patient is again moved to a
sitting position. This may be repeated if BPPV is still suspected based on history.
In a positive test, a burst of nystagmus is provoked by either the head-right or
the head-left position. Further information on BPPV is described in the article
“Episodic Positional Dizziness” by Kevin A. Kerber, MD, MS,12 in this issue
of Continuum.

A 50-year-old man was born with congenital esotropia; as a child, he CASE 2-1
frequently squinted and eventually was able to describe that he was
experiencing double vision, so at age 5 years he underwent surgery to
correct the esotropia.
Videonystagmography recordings (with the camera placed over the
right eye while the patient had initially viewed out of the left eye)
documented a spontaneous left-beating nystagmus; however, caloric
tests were normal. Because he had spontaneous nystagmus but no caloric
weakness, the audiologist concluded that the patient had a central
vestibular disorder.
In the office, the patient had no depth perception (stereopsis) as
determined by the Titmus Fly test in which the patient wore polarized
glasses while looking at a specially formatted picture of a housefly that
should appear to pop out from the page if depth perception is intact. The
patient also had amblyopia in the right eye and weak left-beating
horizontal nystagmus in the light. When the left eye was covered, the
nystagmus reversed direction and became right beating. The nystagmus
stopped in complete darkness (with the use of video Frenzel goggles).
Close inspection of the videonystagmography pursuit traces showed that
the eye actually tracked faster than the target to the left, and backup
saccades were present.

This case illustrates some of the examination findings in a patient with a COMMENT
type of congenital nystagmus called latent nystagmus, which often results
from congenital esotropia, and points out how some of the findings taken
in isolation can lead to an incorrect diagnosis.

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VESTIBULAR TESTING

The most common type of positional


nystagmus, posterior canal BPPV, beats
upward and has a rotatory component,
such that the top part of the eye beats
toward the undermost ear (VIDEO 2-4 and
VIDEO 2-5). The nystagmus typically has a
latency of 2 to 5 seconds and lasts 5 to
60 seconds, and an unwinding
downbeat/rotatory nystagmus may be
seen when the patient is sat up again
(VIDEO 2-6). Variations of BPPV have
different vectors. The lateral canal variant
of BPPV is typified by a strong horizontal
nystagmus that reverses direction between
head left and right (VIDEO 2-7 and
VIDEO 2-8). The rare anterior canal variant
is associated with a downbeating
nystagmus elicited by the Dix-Hallpike
test. The Dix-Hallpike test is a high-yield
portion of the dizziness bedside
assessment and should almost never be
omitted. For more information on
positional vertigo, refer to the article,
“Episodic Positional Dizziness” by
Kevin A. Kerber, MD, MS,12 in this issue of
Continuum.

NYSTAGMUS ASSESSMENT TESTS THAT


REQUIRE FRENZEL GOGGLES. In general, the
following additional maneuvers should
not be used if Frenzel goggles are not
available. More detail about these
maneuvers can be found elsewhere.13

NECK-VIBRATION TEST.
The neck-vibration
FIGURE 2-4
Dix-Hallpike positional maneuver. To test (FIGURE 2-5) is very useful as a
precipitate the characteristic bedside test because it is a robust and
nystagmus of benign paroxysmal durable test for unilateral vestibular
positional vertigo, the patient is rapidly
brought from sitting (A) into a
weakness.14 In the neck-vibration test, the
head-hanging position (B and C) that patient’s eyes are observed in complete
makes the posterior canal vertical and darkness (ie, with video, not optical,
brings it through a large angular Frenzel goggles) while vibration
displacement.
(typically from a massaging device) is
applied to the sternocleidomastoid muscle
for 10 seconds. The vibration is applied first on one side and then on the other
(VIDEO 2-9). One looks for direction-fixed nystagmus (that is, it does not change
direction with changes in the direction of gaze) with fast-phase beating to the
side opposite the ear with vestibular hypofunction. The neck-vibration test
requires less expertise than the head impulse test (see the Head Impulse Test
section), requires almost no subjective judgment, and is not greatly affected by

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the passage of time since the onset of the KEY POINT
lesion. Provided that video Frenzel goggle
● The neck-vibration test is
equipment is available, the neck- a sensitive and durable test
vibration test can be helpful in patients of unilateral vestibular
with suspected unilateral vestibular weakness.
hypofunction.

HEAD-SHAKE TEST. With the patient


wearing Frenzel goggles and sitting, the
patient's head is rotated by the examiner
in the horizontal plane for 20 cycles. The
examiner should aim for a 30- to
45-degree turn of the head to either side
(if the patient is safely able) and a
frequency of 2 cycles per second. A
nystagmus that lasts 5 seconds or longer
FIGURE 2-5 indicates an organic disorder of the ear or
Neck-vibration test. While the central nervous system and indicates
examiner applies vibration to the further investigation is warranted. The
sternocleidomastoid muscle, video
Frenzel goggles are used to detect
head-shake test is neither as reliable nor as
nystagmus. A positive test elicits strong durable as the neck-vibration test. False
unidirectional horizontal nystagmus positives are common.
from both sides.
VALSALVA TEST.
The Valsalva test is
optional and mainly performed if a
pressure or sound sensitivity symptom is elicited from the history. While
wearing the Frenzel goggles, the patient inhales a deep breath and strains for
10 seconds while the examiner observes for nystagmus. Nystagmus at the onset
and release of pressure indicates a positive test. The glottis can be open or closed.
The Valsalva test is used mainly to assess for superior canal dehiscence. Because
far more sensitive laboratory tests for superior canal dehiscence (ie,
vestibular-evoked myogenic potential) are available, performing this test at the
bedside has little benefit. It is also possible, in a small subset of patients with
superior canal dehiscence, to elicit nystagmus in response to sound. This is called
the Tullio test, but it is also highly insensitive and rarely performed.

HYPERVENTILATION TEST. The hyperventilation test is also optional. It is mainly


performed if the examination has been entirely normal or if a vestibular
schwannoma or other partial lesion of the vestibular nerve is suspected. The
patient takes 30 deep, forceful breaths. Immediately after hyperventilation and
with the use of the Frenzel goggles, the patient’s eyes are inspected for
nystagmus. Hyperventilation-induced nystagmus suggests a partially
conducting eighth nerve or central vestibular pathways caused by, for example,
a tumor of the eighth cranial nerve, gamma knife radiosurgery for vestibular
schwannoma, or multiple sclerosis. The hyperventilation test is both insensitive
and nonspecific.

VESTIBULO-OCULAR REFLEX GAIN TESTING


The vestibulo-ocular reflex mediates eye movements in response to head
rotation, such that eyes move equally in amplitude but in the direction opposite

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VESTIBULAR TESTING

of the head rotation, which serves to stabilize the image of a viewed target on the
fovea during movement.

Head Impulse Test


The head impulse test is a bedside vestibular test, first described in 1988.15 It is
useful for detecting vestibular damage and most helpful in documenting
vestibular neuritis. The test requires no special equipment. Standing directly
in front of the patient, the examiner holds the patient’s head firmly on each
side and instructs the patient to look at a fixed point, usually the examiner’s
nose. The examiner then abruptly rotates the patient’s head rapidly but only a
small distance to the left and right (approximately 10 degrees); this brisk
rotation (the impulse) should be in a pattern that is unpredictable in the
timing and direction of the head turn; several impulses toward each side
should be assessed. In a person with an intact vestibular system, the
vestibulo-ocular reflex will keep the eyes on target and the patient will still be
looking at the examiner’s nose after the impulse. In a patient with a recent
unilateral vestibular deficit, especially involving the vestibular nerve, the
eyes move with the head (due to an impaired vestibulo-ocular reflex on the
side to which the head was turned), and this is followed by a corrective rapid
eye movement to bring the eyes back to the target (the examiner’s nose)
(CASE 2-2). The head impulse test is most useful when video Frenzel goggles
are not available. The next two maneuvers are aimed at documenting bilateral
vestibular loss.

Dynamic Illegible E Test


This is a test of dynamic visual acuity. Using an eye chart positioned at least
3 meters (10 feet) from the patient, preferably calibrated in LogMAR units
(FIGURE 2-6), the examiner records visual acuity while the patient’s head is still.
Then, the examiner gently turns the patient’s head 30 degrees to the left and right
at approximately 1 to 2 Hz and again records the visual acuity. Patients with no
loss of vestibular function drop from 0 to 2 lines per LogMAR of acuity with
turning of the head. Patients with complete or partial bilateral loss of vestibular

CASE 2-2 A 40-year-old man reported acute dizziness and unsteady gait and had
been vomiting for several hours. Although he was ambulatory, he was
unsteady and preferred to hold onto his wife’s shoulder as he was taken
from the waiting room to the examination room.
On examination, he could not stand with his eyes closed in a tandem
Romberg stance. He had a left-beating spontaneous nystagmus readily
seen in both eyes. The nystagmus increased on left gaze and stopped on
right gaze. His head impulse test was positive to the right and normal to
the left.

COMMENT This is the presentation of acute right-sided vestibular neuritis. This case
illustrates how a unilateral vestibular weakness presents with spontaneous
unidirectional horizontal nystagmus that obeys the Alexander law.

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function drop from 3 to 7 lines
per LogMAR of acuity. Patients
with acute complete bilateral loss
generally can read only the top
line (ie, the F and P).

Ophthalmoscope Test
The ophthalmoscope test is used
to obtain objective corroboration
when a patient has a positive
dynamic illegible E test. The
examiner focuses on the optic
disk and then gently moves the
patient’s head as described for
the illegible E test. The vestibulo-
ocular reflex gain is abnormal if
the disk moves with the head, ie,
moves back and forth from the
examiner’s perspective.16 The
ophthalmoscope test is less
sensitive than the dynamic
FIGURE 2-6 illegible E test and should be
Dynamic illegible E test. This test of dynamic performed with the patient’s
visual acuity is performed with the examiner
spectacles on to avoid interaction
oscillating the patient’s head side to side about
the vertical axis at approximately 1 to 2 Hz; the with the effects of spectacles on
lowest line that can be read is ascertained. A vestibulo-ocular reflex gain.
decline in visual acuity before and during
movement of at least three lines is abnormal,
Hearing Testing
indicating bilateral vestibulopathy.
© 2007 Timothy C. Hain. TABLE 2-1 lists the indications for
common laboratory procedures
used for evaluating hearing in
patients with vertigo and dizziness. Not all of these tests are useful for every patient.
To be time- and cost-efficient, tests should be chosen according to each patient’s
specific set of symptoms (TABLE 2-2). For more information on hearing loss, refer to

Hearing-Related Laboratory Testing for Dizziness and Vertigo TABLE 2-1

Test Indications

Audiogram Hearing symptoms, dizziness, vertigo, Ménière disease suspected, superior canal
dehiscence suspected

Otoacoustic emissions Hearing symptoms, functional hearing loss

Brainstem auditory evoked Suspicion of vestibular schwannoma with no access to MRI


response

Electrocochleography Secondary test for Ménière disease

MRI = magnetic resonance imaging.

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VESTIBULAR TESTING

the article, “Tinnitus, Hyperacusis, Otalgia, and Hearing Loss” by Terry D. Fife,
MD, FAAN, FANS, and Roksolyana Tourkevich, MD,10 in this issue of Continuum.
When hearing symptoms are present or when a disorder such as Ménière
disease or vestibular schwannoma is reasonably suspected, then an audiogram is
the most useful initial test. An audiogram is recommended even for patients
who have few symptoms of hearing loss because some patterns of hearing loss
cannot be determined at the bedside (eg, low-frequency hearing loss) and may
not be readily noticed by the patient. TABLE 2-2 outlines four common hearing
patterns that may be documented on audiometry. FIGURE 2-7 shows the typical
low-tone hearing loss seen in early Ménière disease.
It is incumbent for the clinician seeing patients with dizziness to recognize
each of the abnormal patterns above because they should trigger important
actions on the part of the clinician.

AUDIOGRAM. The audiogram is a subjective test (requiring cooperation from the


patient) that measures hearing and mainly tests the cochlea. Certain
abnormalities suggest otologic vertigo (TABLE 2-2). It is nearly always indicated
in patients with dizziness. Hearing declines symmetrically in both ears with
age, mainly at high pitches. In some cases, it is helpful to combine the audiogram
(a subjective test) with the otoacoustic emissions test (an objective test) to
look for inconsistency when factitious hearing loss is a concern.

OTOACOUSTIC EMISSIONS. Otoacoustic emissions testing is an objective test based


on registration of sound elicited from the inner ear itself in response to an external
sound. Otoacoustic emissions are a quick and simple automated procedure. In
newborns or others who cannot cooperate with formal audiometry, otoacoustic
emissions are valuable because, when present, they show that cochlear function is
present. Otoacoustic emissions are usually not helpful in people older than
60 years old because otoacoustic emissions are reduced with age. In adults younger
than 60 years, otoacoustic emissions as objective tests are useful in detecting
functional hearing loss through the inconsistency between otoacoustic emissions
and audiometry. For example, if a patient claims to be deaf on one side but has a
robust otoacoustic emission in that ear, functional hearing loss is a reasonable
possibility. Otoacoustic emissions can also be normal in central hearing deficits,
such as a brainstem or cortical site of hearing loss, but these cases are very rare.
Regarding functional hearing loss, a large assortment of audiologic procedures can
detect psychogenic hearing loss, including the excellent Stenger test. The Stenger
effect refers to the psychoacoustic phenomenon in which a tone, presented
simultaneously to both ears but with a greater intensity in one ear, will only be
perceived in the ear receiving the louder stimulus; the Stenger effect is leveraged
by the Stenger test to detect functional unilateral hearing loss.

TABLE 2-2 Common Audiometric Findings in Selected Disorders Causing Dizziness

◆ Ménière disease: unilateral low-frequency sensorineural loss at the onset of disease


◆ Vestibular schwannoma: unilateral progressive high-frequency sensorineural loss
◆ Superior canal dehiscence (conductive hyperacusis): bone conduction better than air
conduction at 500 Hz

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BRAINSTEM AUDITORY EVOKED KEY POINTS
RESPONSE TESTING. Brainstem
● Hearing testing is critical
auditory evoked response, also to assess for Ménière
referred to as auditory brainstem disease and contributes
responses among audiologists, is an greatly to the diagnosis of a
evoked potential test measuring vestibular schwannoma.
brainstem responses to sound. In
● The combination of a
patients with dizziness, it is most subjective hearing test such
useful to detect vestibular as the audiogram with an
schwannomas. Brainstem auditory objective test such as
evoked response testing has fallen otoacoustic emissions can
help with the diagnosis of
out of favor because MRI testing functional hearing loss.
has far superior diagnostic
sensitivity and specificity.

ELECTROCOCHLEOGRAPHY.
FIGURE 2-7
An audiogram showing right-sided low-frequency Electrocochleography is an
sensorineural hearing loss. Low-frequency evoked potential test in which the
sensorineural hearing loss on the right side is recording electrode is positioned
depicted, which is fairly specific for right-sided
on the eardrum to get a better
Ménière disease. Here, X depicts hearing
definition of the cochlear potential
thresholds for the left ear, and O and the triangle
indicate thresholds for the right ear. from the inner ear. An abnormal
electrocochleogram may suggest
Ménière disease in patients with a
clinical history that is consistent with Ménière disease. Electrocochleography is
technically difficult and relatively unrewarding in diagnostic power and should
not be considered a useful screening test in all patients with vertigo. For these
reasons, electrocochleography has fallen out of favor and is not widely available.

Vestibular Laboratory Testing


Vestibular laboratory testing can now assess all five sensory organs in the inner
ear. Three tests—videonystagmography (VENG), rotary chair, and video head
impulse—assess the semicircular canals. VENG and rotary chair test only
horizontal canal function, whereas the video head impulse test can test all three
semicircular canals on each side. These tests are mainly helpful in patients
with dizziness when no clear diagnosis is evident after history and bedside
examination (TABLE 2-3). Two vestibular tests, cervical vestibular-evoked
myogenic potential and ocular vestibular-evoked myogenic potential, assess the
otolith organs (saccule and utricle, respectively). Vestibular tests are sometimes
unreliable and, to confirm abnormal results, it is often helpful to obtain several
independent measurements, usually entailing two different techniques (ie, video
head impulse test and VENG or rotary chair). One can also use a combination
of a good bedside examination (with video Frenzel goggles) and one or more
laboratory tests to increase reliability of the diagnostic process. More detail about
the older vestibular tests (ie, VENG and rotary chair) can be found in an
American Academy of Neurology (AAN) assessment of vestibular testing.17

VIDEONYSTAGMOGRAPHY. VENG is a battery of eye-movement recordings that can


identify vestibular asymmetry, such as that caused by vestibular neuritis, and
document spontaneous or positional nystagmus, such as that caused by BPPV.

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VESTIBULAR TESTING

VENG includes a caloric test, wherein the vestibular system is stimulated by


warming or cooling the eardrum with water or air. VENG is a long and difficult
test, both for the patient and the technician, and patients sometimes call it the
“water torture test” because part of the test may induce vertigo and, in some
people, motion sickness.
Laboratories sometimes attempt to avoid the strong dizziness that can be
encountered in the caloric test by replacing water irrigation with air, resulting in
less stimulating and messy procedures but lower-quality data. False-positive
interpretations, especially “bilateral vestibular weakness,” are common if the
person performing the air caloric testing does not point the stream of warm or cool
air directly at the eardrum (CASE 2-3). The interpretation of “central vestibular
disturbance,” is sometimes applied to patients with a VENG finding outside of the
usual distribution found in inner ear disorders. For example, patients with weak
but measurable spontaneous upbeating or downbeating nystagmus may be given
the diagnosis of central vestibular disturbance and referred for neurologic
evaluation. Another source of testing error can be a partial cerumen impaction of
the ear being tested, which results in the false-positive diagnosis of unilateral
vestibular weakness. VENG results are usually interpreted by audiologists, whose
training mainly covers inner ear disorders; consequently, some audiologists
characterize as a “central finding” any results they do not recognize deriving from
an inner ear disturbance, leading to a neurology referral.
As is the case with other vestibular tests, an abnormal result that does not
fit the clinical picture should be supplemented by rotary chair testing or video
head impulse testing, ideally, in combination with vestibular-evoked myogenic
potential testing (see the following section). VENG remains the vestibular test of
choice but can be associated with some discomfort, including nausea and/or
headache, especially in patients with motion sickness or migraine. Similar clinical
information can be gained by combining the video head impulse test and bedside
nystagmus testing with video Frenzel goggles. It should be noted that, currently,
Centers for Medicare & Medicaid Services and many other insurance plans do
not cover video head impulse test procedures (TABLE 2-4).

VESTIBULAR-EVOKED MYOGENIC POTENTIAL. Vestibular-evoked myogenic potential


testing measures the function of the otolith organs (the utricle and saccule) and

TABLE 2-3 Vestibular Laboratory Tests (in Order of Usefulness)

Test Indication(s)
Videonystagmography (VENG) Vertigo

Video head impulse test (vHIT) Vertigo

Vestibular-evoked myogenic potential (cervical Vertigo, sound sensitivity, pulsatile tinnitus


[cVEMP] and ocular [oVEMP])

Rotary chair test (Rchair) Bilateral vestibular loss suspected, secondary test to confirm abnormal
caloric responses or video head impulse test suggesting unilateral or
bilateral vestibular loss

Posturography (CDP) Assess for functional disorder, assess fall risk

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was mentioned briefly in the 2000 AAN vestibular assessment,17 but now it is
mainstream and fairly widely available. Vestibular-evoked myogenic potential
testing is sensitive to the superior semicircular canal dehiscence syndrome18 and
has a minor role in supporting the diagnosis of bilateral vestibular loss, vestibular
neuritis, and conductive hearing loss.
The two variants of the vestibular-evoked myogenic potential test most
commonly used in clinical practice are the ocular vestibular-evoked myogenic
potential and cervical vestibular-evoked myogenic potential. The ocular
vestibular-evoked myogenic potential quantifies utricular function, whereas the
cervical vestibular-evoked myogenic potential quantifies saccular function.
Vestibular-evoked myogenic potential testing is sensitive to the superior
semicircular canal dehiscence syndrome. Either the ocular or cervical test may
show larger than normal responses and lower than normal sound thresholds on
the affected side(s). While thin-slice CT scans of the temporal bone images
document dehiscence, this procedure both exposes the patient to radiation as
well as may be falsely positive, as asymptomatic dehiscence is found in about 1%
of the population.19 The combination of a visible dehiscence (abnormal opening
of the bony canal) on CT of the temporal bone with abnormal vestibular-evoked
myogenic potential responses (either cervical or ocular) strongly points to the
diagnosis of superior semicircular canal dehiscence syndrome. For more
information on superior canal dehiscence syndrome, refer to the article,
“Selected Otologic Disorders Causing Dizziness” by Gail Ishiyama, MD,20 in this
issue of Continuum.

A 70-year-old woman underwent videonystagmography for evaluation of CASE 2-3


chronic dizziness. The air caloric testing technique was used. Warm air
caloric stimulation was applied in each ear sequentially, then cool air
caloric stimulation was applied in each ear sequentially; the total response
(sum of the peak slow-phase velocity responses from all four caloric
stimuli, warm and cool in each ear) was only 8 degrees per second whereas
normal is between 20 and 100 degrees per second. The patient was
referred for a neurologic evaluation for potential bilateral vestibular loss.
On examination, she could stand in the tandem Romberg stance with her
eyes closed and had no loss of visual acuity on the dynamic illegible E test.
Her ophthalmoscope and head impulse tests were normal.

This case illustrates a discrepancy between clinical examination and an COMMENT


inadequate vestibular test. On physical examination, the facts that the
patient could maintain a tandem Romberg stance with her eyes closed and
she had normal performance on dynamic illegible E testing are
incompatible with a diagnosis of bilateral vestibular loss. In contrast, air
caloric testing is very dependent on pointing the column of air directly at
the tympanic membrane to properly evoke good caloric vestibular
responses. The conclusion of “bilateral vestibular loss” was a false-positive
result.

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VESTIBULAR TESTING

Vestibular-evoked myogenic potential responses, like balance, normally


decline with advancing age but generally are repeatable and stable. Cervical
vestibular-evoked myogenic potentials (whose pathway travels through the
inferior division of the vestibular nerve) are often normal in patients with
vestibular neuritis (which more commonly affects the superior division of the
vestibular nerve), and thus can be helpful in making this diagnosis.21
Vestibular-evoked myogenic potential testing now has a Current Procedural
Terminology (CPT) code (TABLE 2-4).

ROTARY CHAIR. Rotary chair testing measures vestibular function of both inner ears
together. It is sensitive to bilateral loss of vestibular function and performs better
than VENG for this purpose.17 In unilateral vestibular loss, rotary chair testing is
sensitive but nonspecific because it is poor at identifying the side of the lesion.

TABLE 2-4 Common Vestibular and Auditory Tests and Their Corresponding CPT Codesa

Test CPT code


Comprehensive audiometry threshold evaluation and speech recognition 92557
(92553 and 92556 combined)

Auditory evoked potentials; screening of auditory potential with broadband 92635


stimuli, automated analysis neurodiagnostic, with interpretation and report

Caloric vestibular test with recording, bilateral; bithermal (ie, one warm and 92537
one cool irrigation in each ear for a total of four irrigations)

Electrocochleography 92584

Distortion product evoked otoacoustic emissions; limited evaluation (to 92587


confirm the presence or absence of hearing disorder, 3-6 frequencies) or
transient evoked otoacoustic emissions, with interpretation and report

Distortion product evoked otoacoustic emissions; comprehensive diagnostic 92588


evaluation (quantitative analysis of outer hair cell function by cochlear
mapping, minimum of 12 frequencies), with interpretation and report

Computerized dynamic posturography sensory organization test (CDP-SOT), 92548


6 conditions (ie, eyes open, eyes closed, visual sway, platform sway, eyes
closed platform sway, platform and visual sway), including interpretation
and report

Vestibular-evoked myogenic potential (VEMP) testing, with interpretation 92517


and report; cervical (cVEMP)

Vestibular-evoked myogenic potential (VEMP) testing, with interpretation 92518


and report; ocular (oVEMP)

Vestibular-evoked myogenic potential (VEMP) testing, with interpretation 92519


and report; cervical (cVEMP) and ocular (oVEMP)

Video head impulse test None; bill as 92700 and have patient
sign an advanced beneficiary notice

Sinusoidal vertical axis rotational testing 92546

a
Current Procedural Terminology (CPT) codes maintained by the American Medical Association and approved by the Centers for Medicare and
Medicaid Services in the United States as of January 1, 2021.
CPT © 2021 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

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Rotary chair testing is most useful when caloric vestibular testing done as part of KEY POINT
VENG falsely suggests that vestibular responses are reduced or absent on both sides
● False-positive
because of small ear canals, cerumen in the external canal, or inadequate irrigation videonystagmography
technique. Rotary chair testing can clarify matters because it does not determine findings of bilateral
vestibulo-ocular reflex function by caloric stimulation via the external ear canal vestibular weakness or
but rather by actual movement of the head. Rotary chair testing is almost always central vestibular
disturbance are common
covered by health insurance, but it requires costly equipment. In addition, rotary
sources of referrals to
chair testing requires considerable technician and patient time to perform the test. neurologists.

VIDEO HEAD IMPULSE TEST. This recently available vestibular test can quickly diagnose
both severe bilateral vestibular loss and complete unilateral vestibular loss, especially
when due to vestibular nerve injury. The video head impulse test is less sensitive
to vestibular damage due to hair cell disease, such as Ménière disease, whereas
VENG testing is more accurate. The video head impulse test is more resistant to
false-positive findings in patients with functional symptoms than tests of
nystagmus such as VENG and the rotary chair.
Variants of the video head impulse test purport to measure function of the
posterior and anterior semicircular canals, but they should not be relied on for
this because current commercially available eye-movement monitoring technology
is not able to quantify the entire three-dimensional vector of eye movement. As
a consequence, the results of video head impulse tests done for the anterior or
posterior canal planes are often puzzling. Despite the high utility of video head
impulse testing, many insurances do not currently cover it.

POSTUROGRAPHY. This discussion specifically refers to the computerized dynamic


posturography device. Posturography attempts to evaluate vestibular,
proprioceptive, and visual contributions to balance and is similar to the Romberg
test. It requires cooperation from the patient. Posturography involves a series of
six testing conditions, progressing through permutations of normal/absent/
misleading visual input and normal/misleading proprioceptive input. The series
of conditions become gradually more difficult, and a patient’s performance
should decline with progressively more difficult conditions. However, if the
conditions are presented in a random order, and if the patient performs normally
on difficult conditions but poorly in easy conditions, then such inconsistency22
may raise suspicion for a functional disorder. Posturography is also a method of
assessing fall risk and is usually reimbursed by Medicare insurance (TABLE 2-4),
but often it is not covered by other types of insurance.

SUMMARY OF THE ROLE OF VESTIBULAR TESTING. In patients with dizziness,


vestibular testing assists in the diagnostic process by supplementing the clinical
history and examination. Vestibular tests can usually identify unilateral
vestibular loss, bilateral vestibular loss, and superior canal dehiscence. When
combined with hearing testing, they can assist in the identification of Ménière
disease. When comprehensive vestibular testing is all normal, it goes a long way
toward excluding primary peripheral vestibular disorders that leave persisting
dysfunction. Most other conditions, including vestibular migraine, dizziness
related to anxiety, dizziness from cardiovascular disturbances (eg, orthostatic
hypotension), and conditions such as persistent postural perceptual dizziness
have normal results on vestibular assessments at the bedside and in the
laboratory. In these cases, vestibular tests may assist the clinician in avoiding

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VESTIBULAR TESTING

fruitless management strategies, such as vestibular-suppressant medication or


vestibular rehabilitation for patients who have no vestibular disturbance.

CODING OF VESTIBULAR TESTING. Bedside testing (without instrumentation and


recording), such as the Dix-Hallpike test for BPPV or the bedside head impulse
test for unilateral and bilateral vestibular loss, has no billing code that is
reimbursed in the United States. Instrumented testing, often involving the same
basic process as bedside testing, but with a recording, is usually reimbursed by
insurance (TABLE 2-4).

CONCLUSION
For a clinician to diagnose a patient with dizziness, a careful history and an
examination that includes specific bedside vestibular tests are crucial. In some
instances, this may need to be supplemented with audiometric tests and with
instrumented vestibular testing, the latter of which has seen significant advances
in the past decade as it is now possible to evaluate the entire labyrinth (all
semicircular canals and otolith organs).

VIDEO LEGENDS
VIDEO 2-1 VIDEO 2-6
Weak jerk nystagmus in a patient with resolving Left-posterior canal benign paroxysmal positional
vestibular neuritis. The eyes are first in primary vertigo (BPPV). The patient is in the left Dix-Hallpike
position of gaze, and modest spontaneous position, and upbeat and left-torsional nystagmus is
left-beat nystagmus is present. When the patient present. This pattern is consistent with left-posterior
directs gaze rightward, no nystagmus is present. canal BPPV. The patient then sits up and is put in the
When the patient directs gaze leftward, left-beat right Dix-Hallpike position, and downbeat and right-
nystagmus is more pronounced than it had been on torsional nystagmus is present. This pattern is
primary position of gaze. This pattern is in accordance compatible with “unwinding” of left-posterior canal BPPV.
with the Alexander law for a right-sided vestibular
weakness. © 2021 American Academy of Neurology

© 2021 American Academy of Neurology VIDEO 2-7


Geotropic direction-changing positional
VIDEO 2-2 nystagmus. When the patient is first in the left
Vertical pendular nystagmus in a patient with Dix-Hallpike position, left-beat nystagmus is
pontine bleed and palatal myoclonus (not shown). present. When the patient is moved to the right
Dix-Hallpike position, right-beat nystagmus is
© 2021 American Academy of Neurology present. This pattern is geotropic
direction-changing positional nystagmus.
VIDEO 2-3
Latent nystagmus, a variety of congenital © 2006 Timothy C. Hain, MD
nystagmus. At first, the camera is over the right eye,
the patient is viewing out of the left eye, and left-beat VIDEO 2-8
nystagmus is present. Then, the camera is switched Apogeotropic direction-changing positional
from the right eye to the left eye (noted as Switch nystagmus. When the patient is first in the left
Cover in the video), and the patient is viewing out of Dix-Hallpike position, right-beat nystagmus is
the right eye, and right-beat nystagmus is present. present. When the patient is moved to the right
The fast phase of the nystagmus is ipsiversive to the Dix-Hallpike position, left-beat nystagmus is
eye out of which the patient is viewing. This pattern is present. This pattern is apogeotropic
characteristic of latent nystagmus. direction-changing positional nystagmus.

© 2021 American Academy of Neurology © 2006 Timothy C. Hain, MD

VIDEO 2-4 VIDEO 2-9


Dix-Hallpike test and right benign paroxysmal Vibration-induced nystagmus in a patient with a
positional vertigo from the posterior canal. vestibular schwannoma that was removed 40 years
ago. Vibration is applied on the right mastoid
© 2021 American Academy of Neurology process and then on the left mastoid process.
Vibration on either side elicits left-beat nystagmus,
VIDEO 2-5 consisting of rightward drift (slow phase of
Dix-Hallpike test and nystagmus from left benign nystagmus toward the side of vestibular weakness)
paroxysmal positional vertigo from the posterior and leftward saccade (fast phase of nystagmus
canal. away from the side of vestibular weakness).

© 2021 American Academy of Neurology © 2005 Timothy C. Hain, MD

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