KEY POINT:

PATHOPHYSIOLOGY OF A In response
to motion of

VESTIBULAR SYMPTOMS the head, two

phylogenetically

AND SIGNS: THE old reflexes, the

vestibulo-ocular
reflex and the

CLINICAL EXAMINATION vestibulospinal

reflex, act to
keep vision
David S. Zee clear and the
body upright.

ABSTRACT
A challenge for the clinical neurologist is to decide which of the myriad patients with
symptoms of dizziness, lightheadedness, or imbalance have a genuine vestibular
disorder, be it peripheral or central. The clinical examination is often the key. A series
of systematically applied, physiologically based maneuvers, designed to probe static
and dynamic function of the vestibulo-ocular reflexes and the individual labyrinthine
sensors, will almost always reveal the evidence of a vestibular system anomaly, which
either clarifies the diagnosis or points to a need for a further evaluation.
This chapter will describe these maneuvers and indicate their diagnostic usefulness.
They include dynamic visual acuity, occlusive ophthalmoscopy, head impulse
(rotational vestibulo-ocular reflex) and head heave (translational vestibulo-ocular
reflex) testing, mastoid vibration-induced nystagmus (equivalent of a hot-water
caloric stimulus in a patient with unilateral vestibular loss), hyperventilation-induced
nystagmus (abnormal in fistula, craniocervical junction anomalies, compressive and
demyelinating lesions, and cerebellar degenerations), Valsalva-induced nystagmus
(abnormal in fistula and craniocervical junction anomalies), head-shaking–induced
nystagmus (vertical nystagmus after horizontal head shaking points to a central
disorder), positional nystagmus (lateral canal, posterior canal, central) and sound-
induced nystagmus (superior canal dehiscence). When combined with a careful
examination of eye alignment, gaze holding, saccade accuracy and speed, and
smooth pursuit, a central or peripheral localization is usually possible.

BASIC PHYSIOLOGICAL generating the appropriate compensa- 13

PRINCIPLES AND ANATOMICAL
ORGANIZATION
The clinical evaluation of the patient
whose symptoms suggest vestibular
dysfunction relies upon a solid under-
standing of vestibular physiology and
anatomy and of the psychophysiologi-
cal aspects of normal and abnormal ves-
tibular sensation. The term vestibular
will be used here in its broadest sense
as the mechanism for detecting the atti-
tude of the head with respect to gravity
and to any movement of the head
within the environment and also for
tory motor responses that assure clear
vision and steady balance in response
to movements of the head and body.
Basic Reflexes
The vestibular sense is mediated by three
relatively simple reflexes. The vestibulo-
ocular reflex (VOR) ensures best vision
during head motion by stabilizing the
line of sight (also called gaze). The vesti-
bulospinal (VSR) and vestibulocollic (VCR)
reflexes help keep the head and body
upright. Two phylogenetically old sensors
within the labyrinth—the semicircular

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 " VESTIBULAR SYMPTOMS AND SIGNS
KEY POINT:
canals (SCCs) and the otolith organs—
A Two labyrinthine
respond to acceleration and thereby
sensors, the
semicircular transduce the motion and position of
canals and the the head.
otolith organs, The SCCs sense angular acceleration
transduce in order to determine head rotations.
angular Because of the mechanical properties
acceleration of the labyrinth, the position of the
(rotation) cupula within the ampulla (and hence
and linear the level of activity on primary vestibular
acceleration afferents) encodes head velocity. For
(tilt and
eye movements, the SCCs provide the
translation),
input for the compensatory slow phases
respectively.
of the rotational VOR in response to
head rotation. The otolith organs sense
linear acceleration to detect both head
translation and the position of the head
relative to the pull of gravity, ie, up-
rightness. They provide the input for
static ocular counterroll (torsion) in
response to sustained head-tilt and for
the compensatory slow phases of the
translational VOR in response to head
translation. Both the SCCs and the
otolith organs can detect motion in
six dimensions, ie, around any of the
three axes of rotation (pitch [vertical],
yaw [horizontal], and roll [torsion])
(Figure 1-1A), and in the three axes
of translation (fore and aft [surge], side
to side [heave], and up and down
[bob]) (Figure 1-1B).
A critical difference between the
vestibulo-ocular responses to head ro-
tation and head translation is that the
14 response to the latter depends upon
the direction of gaze. No compensatory
response to translation is needed when
viewing far targets, and for near targets
the compensatory response is scaled
and directed according to how close the
target is to the head and where the
target is relative to the head, ie, straight
ahead, to the side, or up or down. So,
for example, if one is moving forward
through the environment (fore and
aft translation or surge) and focusing
on a near target, the compensatory
vestibulo-ocular response is horizontal
when looking to the side, vertical when
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
looking up or down, and convergent
when looking straight ahead. This
normal dependence of the direction
and amplitude of the translational VOR
on where the eyes are in the orbit
may be reflected in the influence of
near viewing and eccentric viewing
on the intensity and direction of path-
ological nystagmus such as downbeat
nystagmus with craniocervical junction
anomalies.
A division of labor in the
labyrinth. The rotational VOR re-
sponds more faithfully to high-frequency
(rapidly changing) than to low-frequency
(slowly changing) head motion because
of the mechanical properties of the en-
dolymph and the cupula. This limiting
characteristic is reflected in the fading of
vestibular nystagmus during a constant-
velocity rotation in darkness as the
cupula returns toward its neutral posi-
tion (Figure 1-2). A constant-velocity
rotation is characterized by high-frequency
components near its onset; hence, ini-
tially the SCCs provide an accurate
measure of the speed of head rotation.
Low-frequency components predomi-
nate later in the rotation, so the per-
ception of rotation and nystagmus
dies out. In the light, of course, visual
(optokinetic and pursuit) signals can
be used to supplant the fading labyrin-
thine signal (see below).
The velocity-storage mechanism.
To improve the low-frequency response
of the rotational VOR, a central velocity-
storage mechanism maintains activity
coming from the vestibular periphery
by partially integrating the peripheral
SCC signal. Velocity storage increases
the time constant (the time for an ex-
ponential function to decay to 37% of its
initial value) of the VOR from a value of
about 6 seconds, based on activity in
peripheral afferents, to a value of 15 to
20 seconds, based upon the actual
nystagmus response (Figure 1-2). In
this way, the ability of the angular VOR
to reliably transduce head velocity to
 15
FIGURE 1-1 A, Conventions for describing head rotations: horizontal
(yaw), vertical (pitch), and torsion (roll). B, Conventions for
describing head translations: side to side (heave), fore
and aft (surge), and up and down (bob).
Courtesy of Aasef Shaikh, MD.

low-frequency stimuli is improved. An-
other equivalent measure of the time
constant can be calculated from the
difference in timing (or shift in the
phase relation) between maximal head
and maximal eye speeds during low-
frequency sinusoidal rotations. The
velocity-storage mechanism acts to mini-
mize this phase shift and thereby ensures
that the eye movement response has
the correct timing to compensate for
the head movement.
Visual (optokinetic and pursuit) and
somatosensory sensors also provide
signals for balance in a manner partially
redundant but also complementary to

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 " VESTIBULAR SYMPTOMS AND SIGNS

KEY POINT:
A Labyrinthine, visual,
somatosensory,
and
proprioceptive
signals converge
onto the same
neurons within
the vestibular
nuclei. This
multiplicity of
sensory inputs
allows for the
generation of FIGURE 1-2 Decay of nystagmus slow phase velocity after a step change in
angular velocity (solid line). The thin solid line indicates the
compensatory estimated time course of both the return of the cupula to its
vestibular initial position and the decay of activity on primary semicircular canal afferents.
responses over Note how the nystagmus outlasts the cupula and primary afferent response,
due to the central velocity-storage mechanism.
the entire range
Courtesy of Robert W. Baloh, MD, University of California, Los Angeles, Medical School.
of frequencies to
which the head
is subject during
both slow and those from the labyrinth. These differ- notes self-rotation in the direction op-
rapid motions. ent sensory inputs are used by the posite to the image motion. To deduce
vestibular system both for normal be- which labyrinthine input predominates,
havior and as a substrate for recovery in the patient should be asked to indicate
response to vestibular disease. For the the direction of head rotation with the
VOR, visual signals supplant labyrin- eyes closed to deduce the direction of
thine signals for the low-frequency, the slow phase of spontaneous nystag-
sustained components of head rotation. mus. The apparent direction of rotation
Somatosensory and visual signals help of the visual world should be reported
stabilize posture in a similar way. The by the patient with his or her eyes open.
critical importance of the interactions
among these different sensors is re- Vestibular Sensations From
flected in the fact that they converge on the Otolith Organs: The
the same vestibular neurons within the Tilt-Translation Ambiguity
vestibular nuclei. It is easy to envision When the otolith organs are excited by
how a disturbance of any of the sensory linear acceleration, the cause of the
16 inputs to the vestibular nuclei might stimulation cannot be distinguished on
lead to a sensory conflict and abnormal the basis of otolith activity alone. An in-
vestibular sensation. herent ambiguity exists about whether
the head is being tilted or translated.
Vestibular Sensations The shear forces on the macula of the
of Rotation utricle are the same in response to a
The direction in which the patients with sustained lateral head-tilt as to a lateral
spontaneous nystagmus and vertigo head translation. A sustained head-tilt,
think they are turning must be inter- however, requires a compensatory ocu-
preted carefully. An imbalance in laby- lar counterroll—that is, a torsional rota-
rinthine signals indicates a sense of tion of the globe—whereas a lateral
self-rotation in one direction, but any translation requires a compensatory hori-
spontaneous nystagmus leads to slow zontal slow phase of nystagmus. Similar
phase eye movements that cause retinal considerations apply when distinguish-
image motion that, if self-referred, con- ing between a static pitch of the head

Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.


down or up, which would require a
deviation of the eyes up or down in the
orbit, and a fore or aft translation of the
body, which would require the eyes to
converge or diverge. In practice, the
vestibulo-ocular responses to static tilts
of the head are rudimentary (in the
case of lateral head-tilt because the
orientation of the fovea is little affected
by head-tilt) or unnecessary (in the case
of pitch of the head up or down be-
cause saccades can be used to readjust
the line of sight).
Normally many clues are present to
help the brain put the patterns of oto-
lith activity in their appropriate context.
For example, if a corresponding change
in activity from the vertical SCCs occurs,
a head-tilt rather than a translation
would be inferred. Alternatively, the
visual sense can be used to judge
whether the patient is being translated
or tilted. If the uncertainty about the
position of the head relative to gravity is
not readily resolved (which is probably
more likely with an associated labyrin-
thine disturbance), the tilt–translation
ambiguity becomes a source of dis-
comfort and disorientation to patients.
Some of the vague and unusual subjec-
tive symptoms that patients describe
during standing and walking may arise
from their difficulty in deciphering the
source of a linear acceleration.
Disturbed Sensory Integration
In healthy subjects, any conflicts among
the various sensory inputs to the ves-
tibular nuclei are transient and readily
resolved, usually in favor of the labyrin-
thine sense. But even in some ‘‘natural’’
circumstances (eg, movie theaters with
ultrawide screens), the visual sense may
lead to illusions of motion. Patients who
have lost labyrinthine function or who
have distorted or spontaneous fluctua-
tions of labyrinthine signals may be-
come increasingly reliant on visual and
somatosensory cues for balance. Such
a substitution of sensory inputs is often
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
KEY POINT:
a useful, indeed necessary, adaptive
A Two important
strategy. Such a reordering of the pri-
sources of
orities of the sensory inputs relied on symptoms in
for balance, however, may lead to an patients with
exacerbation of symptoms when, for vestibular
example, inputs from the feet are mis- disorders are
leading (such as walking on sand) or the inability to
when visual inputs are misleading or resolve conflicts
unusually active or chaotic (such as among
watching movies or walking down aisles labyrinthine,
in supermarkets while trying to read visual, and
somatosensory
labels on store items).
inputs and
the inherent
ambiguity
Innervation of and Blood
between tilt
Supply to the Labyrinth
and translation.
The vestibular nerve is divided into two
branches: (1) a superior branch, which
runs with the facial nerve in the internal
auditory canal and supplies the anterior
and lateral SCCs and the utricle and (2)
an inferior branch, which runs with the
cochlear nerve and supplies the poste-
rior SCC and the saccule (Figure 1-3).
There is a comparable parallel blood
supply for these two parts of the
vestibular labyrinth, namely, the ante-
rior and posterior vestibular arteries,
respectively, which are branches of the
internal auditory artery (Figure 1-4).
Thus, lesions restricted to the superior
division of the vestibular nerve or to
the anterior vestibular artery affect the
lateral and anterior SCC and utricle
(with the appropriate abnormalities on
bedside and laboratory vestibular test- 17
ing) but spare the posterior SCC and
saccule. A clinical consequence of this
anatomical organization is that debris
from the affected utricle may settle in
the spared posterior SCC and lead to
benign paroxysmal positional vertigo
(BPPV) (see below).
Central projections from the SCCs
are predominantly to the rostral por-
tions of the vestibular nuclei complex
(medial and superior vestibular nuclei),
whereas those from the otolith organs
are predominantly to the caudal por-
tions of the vestibular nuclei complex
 " VESTIBULAR SYMPTOMS AND SIGNS

KEY POINTS:
A Semicircular
canal projections
are primarily to
the rostral
portions of the
vestibular
nuclei complex
(frequently
involved in
anterior inferior
cerebellar artery
distribution
infarcts). Otolith
projections are
primarily to the
caudal portions
of the vestibular
nuclei complex
(frequently
involved in
posterior inferior
FIGURE 1-3 Innervation of the labyrinth. Note that the superior division
cerebellar artery of the vestibular nerve (which runs with the facial nerve)
distribution innervates the anterior and lateral semicircular canal and
infarcts). the utricle, while the inferior division of the vestibular nerve innervates the
posterior semicircular canal and the saccule.
A Both the N, n = nerve.
otolith organs
Reprinted with permission from Baloh RW, Honrubia V. Clinical neurophysiology of the vestibular
and the system. 2nd ed. New York: Oxford University Press, 1990. Copyright # 1990, Oxford University
semicircular Press.
canals have
a direct
projection to
(lateral and inferior vestibular nuclei) vestibular projections probably underlie
the vestibulo-
(Figure 1-5). This explains the predomi- so-called tornado epilepsy (seizures in
cerebellum,
and small
nance of otolith symptoms and signs which vertigo is an important symptom)
cerebellar (tilt of the body and vertical eye mis- and vestibulogenic epilepsy (seizures
infarcts may alignment or skew deviation) in patients induced by caloric stimulation). Fur-
with infarction in the dorsolateral me- ther, patients with thalamic and cerebral
18 therefore mimic
a peripheral dulla in Wallenberg syndrome. There cortical lesions (probably involving the
labyrinthine are also projections from the labyrinth ‘‘vestibular cortex’’ in the insula) may
disturbance. directly to the vestibulocerebellum; have disturbed perception of the verti-
therefore, some focal cerebellar infarc- cal upright and inappropriate sensa-
tions may mimic a peripheral labyrin- tions of motion. The sometimes ill-
thine disturbance. defined sensations of tilt, imbalance,
The vestibular nuclei project to the dizziness, and disorientation experi-
motor centers for generating vestibulo- enced by some patients with lesions in
ocular and vestibulospinal responses as the cerebral hemispheres may reflect
well as more rostrally to the thalamus involvement of the areas in the cerebral
and thence to many areas in the cerebral cortex that receive vestibular projec-
cortex, including the so-called vestibular tions. A cerebral hemispheral asymme-
cortex within the insular cortex and try is also found in the influences of
other areas in the frontal and parietal vestibular stimulation on cerebral cor-
lobes. Disturbances in these rostral tex, with the nondominant hemisphere

Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.


playing a more prominent role as it does
for other aspects of spatial orientation.
THE HISTORY
The history from a patient with vestibu-
lar symptoms should try to answer the
following questions:
1. Do the patient’s symptoms of diz-
ziness reflect an abnormality in
the vestibular system per se, or do
they reflect some other general med-
ical cause such as anemia, hypoper-
fusion of the brain from postural
hypotension or cardiac arrhythmias,
endocrinedisorders(especially hypo-
thyroidism), hypoglycemia, or side
effects of medications (Table 1-1)?
2. Is the issue psychogenic (anxiety,
panic, depression, or phobia) as ei-
ther the primary cause or secondary
to the psychological burden of long-
standing vestibular dysfunction?
3. Do the patient’s symptoms reflect a
disordered processing of informa-
tion from the SCCs (rotation) or
from the otolith organs (diplopia,
tilt, or translation)?
4. Is the problem peripheral (labyrinth
or nerve) or central (ie, vestibular
nuclei, cerebellum, vestibular cortex)?
5. If a patient has ‘‘spells,’’ what are
the circumstances of onset, the
tempo, the duration, and the asso-
ciated symptoms? These variables
usually hold the key to the proper
diagnosis.
BEDSIDE EXAMINATION OF
VESTIBULO-OCULAR REFLEXES
Vestibular signs and symptoms may
reflect static (head still) or dynamic
(head moving) disturbances. These are
best considered separately (Table 1-2).
Static disturbances reflect the fact that
healthy subjects have balanced, tonic
levels of discharge in the vestibular
nerve and nuclei when the head is still.
Dynamic disturbances, on the other
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
KEY POINTS:
hand, come about because of a loss of
A Disturbances
the normal push-pull relationship—
in rostral
activity from one labyrinth increasing vestibular
as that from the other is decreasing— projections to
that produces compensatory responses the thalamus
to head motion. and cerebral
hemispheres
Static Balance may underlie
Spontaneous nystagmus (nystagmus complaints of
appearing with the head still) is the dizziness and
imbalance in
hallmark of an imbalance in the tonic
some patients.
levels of activity mediating the angular
VOR (Table 1-3). When peripheral in A The nondominant
origin, spontaneous nystagmus charac- hemisphere
teristically is damped by visual fixation plays the more
and increases or becomes apparent only important role
in spatial
when fixation is eliminated (Video
orientation,
Segment 1). Therefore, for the VOR,
using vestibular
one must do the equivalent of a as well as
Romberg test and search for spontane- visual and
ous nystagmus with the patient wearing somatosensory
Frenzel goggles (magnifying lenses that information.
prevent the patient from using visual
fixation to suppress spontaneous nys-
tagmus) or during ophthalmoscopy
19
FIGURE 1-4 Blood supply to the labyrinth. Note that the
anterior vestibular artery supplies the anterior
and horizontal semicircular canals and the
utricle, and the posterior vestibular artery supplies the
posterior semicircular canal and the saccule.
AICA = anterior inferior cerebellar artery; ASC = anterior semi-
circular canal; AVA = anterior vestibular artery; CCA = common
cochlear artery; HSC = horizontal semicircular canal; IAA =
internal auditory artery; MCA = main cochlear artery; PSC =
posterior semicircular canal; PVA = posterior vestibular artery.
Courtesy of Robert W. Baloh, MD, University of California, Los Angeles,
Medical School.
 " VESTIBULAR SYMPTOMS AND SIGNS

KEY POINT:
A Key questions
to be answered
by the history
and examination
are: Is it a
vestibular
problem, or
is there a general
medical cause?
Is it a psychiatric
problem?
If vestibular, does
the problem
emanate from
the otolith
organs, the
canals, or from
their respective
central
pathways?
Is the problem FIGURE 1-5 Vestibular nerve projection to the four vestibular nuclei. Note that the semicircular
canals tend to project to the more rostral portions of the vestibular nucleus
peripheral complex and the otolith organs to the more caudal portions of the vestibular
(labyrinth or nucleus complex.
nerve) or central? n = nerve; Post = Posterior.
If a patient has
From Stein BM, Carpenter MB. Central projections of portions of the vestibular ganglia
spells, what innervating specific parts of the labyrinth in the rhesus monkey. Am J Anat 1967;120:281–318.
provokes them, Copyright # 1967, Wiley-Liss, Inc. Reprinted with permission of Wiley-Liss, Inc., a subsidiary of
John Wiley & Sons, Inc.
how do they
begin, what is
the tempo, and
are associated (with the opposite eye occluded to only be evident when gaze is pointed
symptoms prevent fixation) (Video Segment 2). in the direction of the quick phase
present? The intensity of nystagmus with such (Alexander’s law). With central lesions,
maneuvers is compared with that ob- however, the opposite sometimes occurs.
served when the patient is fixing upon a The effect of convergence upon the
visual target. It should be noted that nystagmus should be noted; conver-
during ophthalmoscopy the direction of gence may expose, intensify, or alter
20 any horizontal or vertical slow phases direction of some central forms of ves-
will be opposite the direction of motion tibular nystagmus. Convergence may
of the optic disk because the retina is dampen congenital nystagmus.
behind the center of rotation of the If the anatomical arrangement of the
globe. With torsion, however, different SCCs is considered, one can localize and
parts of the retina will appear to be more accurately interpret certain pat-
moving in different directions depend- terns of nystagmus (Figure 1-6). Stimu-
ing upon where the examiner is looking lation of a single SCC leads to slow phase
relative to the fovea (through which eye movements that rotate the globe
passes the line of sight). in a plane parallel to that of the stim-
The intensity of nystagmus usually ulated canal. For the lateral SCC, this is
depends on the position of the eye in predominantly horizontal motion; for
the orbit. Nystagmus arising from pe- the vertical SCC, it is a mixed vertical-
ripheral lesions is more intense (slow torsional motion. For the vertical
phase velocity higher) or may in fact SCC, the relative amount of torsion or

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vertical rotation depends on the hori-
zontal position of the eyes in the orbit.
As an example, BPPV, which usually em-
anates from inappropriate stimulation
of the posterior SCC, is characterized by
a mixed vertical–torsional positioning
nystagmus elicited with the offending
ear down (Video Segment 3). The nys-
tagmus appears more vertical on gaze
away from the affected ear and more
torsional on gaze toward the affected
ear (Table 1-4).
Pure vertical or pure torsional nys-
tagmus almost always has a central
origin because it is rare for just both
anterior or both posterior SCCs (for a
pure vertical nystagmus) or for just the
anterior and posterior SCCs in one
labyrinth (for a pure torsional nystag-
mus) to be selectively involved. A mixed
horizontal–torsional nystagmus usually
indicates a peripheral lesion involving
the entire vestibular nerve or all the
SCCs within one labyrinth. One should
recall that, with spontaneous nystag-
mus, any torsional component will be
little damped by fixation (human beings
have a poor torsional fixation mecha-
nism), whereas vertical and horizontal
components will be better suppressed.
Therefore, the pattern of nystagmus (ie,
the axis of rotation of the slow phases)
may be different when fixation is allowed
from the pattern with no fixation. Only
without fixation can one see the effect
of the vestibular imbalance alone.
Skew deviation, a vertical misalign-
ment of the eyes that cannot be ex-
plained on the basis of an ocular muscle
palsy, is the hallmark of an imbalance in
the tonic levels of activity underlying
otolith-ocular reflexes. Skew deviation
is a natural component of the righting
reflex that occurs in lateral-eyed animals
in response to lateral tilt of the body
so that their eyes may remain aligned
along the horizontal meridian. In frontal-
eyed animals, this phylogenetically old
pattern of eye deviation presumably
emerges when otolith inputs become
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
KEY POINT:
unbalanced in a nonphysiological way.
A When a
Patients with skew deviation complain
spontaneous
of vertical and sometimes torsional nystagmus is
(one image tilted with respect to the purely vertical
other) diplopia. Cyclorotation (ocular or purely
counterroll) of both eyes associated torsional, a
with an illusion of tilt of the visual central lesion
world may appear. The head may also must be
be tilted, usually toward the side of excluded.
the lower eye. Together, skew devia- A mixed
tion, ocular counterrolling, and head- horizontal–
torsional
tilt constitute the ocular tilt reaction—
nystagmus
the vestibulo-ocular and vestibulocollic
usually has
components of the righting reaction in a peripheral
response to lateral tilt of the head and origin.
body (Figure 1-7) (Table 1-5).
Skew deviation is best detected using
the tools and techniques of ophthalmolo-
gists: objectively with cover testing or
subjectively with a red glass or Maddox
rod. With the alternate-cover test, one
looks for a vertical corrective movement
Symptoms That Point to a Vestibular
TABLE 1-1
Disturbance
" Sensations of tilt, translation (fore and aft, up and
down, right and left), imbalance, or rotation
(tumbling, cartwheeling, revolving)
" Symptoms induced by motion or repositioning of the
head, such as vertigo induced by turning over in bed
or getting up quickly (as with benign positional vertigo)
" Oscillopsia or body imbalance induced by head
movement (as occurs with bilateral labyrinthine loss
due to ototoxic antibiotics) 21
" Diplopia (looking for a skew deviation as a sign of
otolith–ocular imbalance)
" Valsalva-induced symptoms (eg, with an anomaly at
the craniocervical junction or with a perilymph fistula,
superior canal dehiscence, or disorders of the
ossicular chain)
" Vertigo or diplopia induced by noise (Tullio’s
phenomenon, eg, with a perilymph fistula, superior
canal dehiscence, or Ménière’s syndrome)
" Exercise- or heat-induced vertigo (ie, with demyelinating
lesions due to compression of the eighth cranial nerve,
as with an acoustic neuroma or cholesteatoma, or
central lesions such as multiple sclerosis)
 " VESTIBULAR SYMPTOMS AND SIGNS
TABLE 1-2 Vestibular
Abnormalities
" Static Vestibular Imbalance
Spontaneous nystagmus
(canal-ocular imbalance)
Skew deviation (otolith-
ocular imbalance)
" Dynamic Vestibular
Disturbances
Abnormal amplitude (gain),
incorrect direction (axis), and/
or incorrect timing (phase)
of the vestibulo-ocular reflex
to rotation (canal-ocular
reflex) or translation
(otolith-ocular reflex)
as an index of a vertical misalignment
when the cover is switched from one eye
to the other. Alternatively, one can use a
red glass or a Maddox rod to dissociate
the images seen by the two eyes; if the
patient sees one image above, this would
indicate a vertical misalignment. The
effect of the position of the eyes in the
orbit and of left and right head-tilt upon
the skew should also be recorded, be-
cause a fourth nerve palsy must be
excluded. The hallmark of a fourth
nerve palsy is a vertical misalignment
that is greatest with the affected eye
down and medial. The adducting eye is
22 higher, and the misalignment is greater
with the head tilted toward the side of
the higher eye. Skew deviation tends to
be relatively concomitant, ie, the de-
gree of misalignment changes little
with different directions of gaze, al-
though this is not always the case.
Ocular counterroll is difficult to detect
clinically without photographic means,
but if the amount of counterroll is
large, it can be appreciated by the tilt
of the imaginary line that connects the
macula and the optic disk. Skew devia-
tions may also occur only when gaze
is directed to the left and to the right.
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
Under these circumstances, the abduct-
ing eye is usually higher, and this pat-
tern is common with cerebellar disease.
The ocular tilt reaction can occur
with lesions anywhere in the otolith-
ocular pathway—peripheral labyrinth,
vestibular nerve, vestibular nucleus in
the medulla, medial longitudinal fascic-
ular in the pons or caudal midbrain, or
the interstitial nucleus of Cajal, rostral to
the oculomotor nucleus. With periph-
eral and vestibular nucleus lesions (eg,
immediately after a vestibular nerve
section or with Wallenberg syndrome),
the lower eye is on the side of the lesion.
TABLE 1-3 Evaluating
Spontaneous
Nystagmus
" Eliminate fixation (Frenzel
goggles, occlusive
ophthalmoscopy, Ganzfeld
[white featureless visual
background] eye closure)
" Note the direction (axis
around which the eye is
rotating) of the nystagmus
" Note the effect of eye
position and head position
on spontaneous nystagmus
" Note any dissociation of
the nystagmus between
the two eyes
" Note the effect of
convergence on nystagmus
" Provocative tests:
hyperventilation, mastoid
vibration, Valsalva maneuver,
noise, tragal compression,
jugular vein compression,
swallowing, pressure applied
in the external auditory
canal, lateral rotation of
the head, flexion or extension
of the head on the trunk
with the head kept upright
and the trunk pitched
backward or forward,
positional maneuvers.

The otolith-ocular pathway crosses at
the level of the vestibular nuclei, so that
with lesions above the decussation the
higher eye is on the side of the le-
sion (eg, with internuclear ophthalmo-
plegia). The head usually is tilting to-
ward the side of the lower eye.
Dynamic Disturbances
Disturbances of vestibular function pro-
voked by head motion or a change in
head position reflect abnormalities in
gain (amplitude), direction, or timing
(phase) of the VOR and VSR or occa-
sionally mechanical disruptions in the
labyrinth (eg, BPPV and perilymph fis-
tula including superior canal dehis-
cence). The angular VOR can be tested
at the bedside both by observing the
effect of head rotation on visual acuity
and by looking at the eye movements
themselves in response to head rotation
(Table 1-6).
Cervical Afferents
When the head is rotated on the body,
the possible influence of stimulation of
cervical afferents through the cervico-
ocular reflex (COR) must be considered.
In normal subjects, the COR is rudimen-
tary and can be ignored. In patients
with loss of function of the SCCs, how-
ever, potentiation of the COR may oc-
cur, or, alternatively, cervical afferents
may trigger preprogrammed compen-
satory slow phases or saccades inde-
pendent of inputs from the SCCs. One
must therefore interpret cautiously the
seeming presence of compensatory
eye movements during head-on-body
rotations in patients with a possible loss
of labyrinthine function. One way to
restrict stimulation to the labyrinth is to
elicit postrotatory nystagmus after ro-
tating the patient at a roughly constant
velocity on a swivel chair for 20 to 30
seconds with eyes closed. The chair is
then stopped and, using Frenzel gog-
gles, the eyes are observed for post-
rotatory nystagmus. The head can be
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
KEY POINT:
positioned to elicit horizontal (head
A The ocular tilt
upright), vertical (head on the side), or
reaction is a
torsional (head pointed to the ceiling) sensitive sign
nystagmus. of central
vestibular lesions
Dynamic Visual Acuity but may also
The patient’s best corrected visual acuity occur with
is measured using an acuity chart with peripheral lesions.
the head still (Video Segment 4). It is
then measured with the head passively
rotated, first horizontally, then vertically,
at a frequency of about 2 Hz to prevent
visual-following reflexes from helping to
stabilize the eyes. The rotation should
not stop at the turnaround point. Nor-
mal individuals may lose one line of
acuity during such head shaking, where-
as patients with a complete loss of laby-
rinthine function usually lose about five
lines. In contrast, during torsional head
23
FIGURE 1-6 Nystagmus slow-phase direction associated with
stimulation of individual canals (above) or combination
of canals (below). Note that a pure vertical nystagmus
can arise only from isolated, symmetrical involvement of two anterior or
two posterior semicircular canals and that a pure torsional nystagmus
can arise only from isolated, symmetrical involvement of an anterior and
a posterior semicircular canal in one labyrinth. Involvement of all three
semicircular canals in one labyrinth gives rise to a mixed horizontal-
torsional slow phase.
R = right; L = left; PC = posterior semicircular canal; HC = horizontal
semicircular canal; AC = anterior semicircular canal.
Reprinted with permission from Leigh RJ, Zee DS. The neurology of eye movements.
4th ed. New York: Oxford, 2006. Copyright # 2006, Oxford University Press.
 " VESTIBULAR SYMPTOMS AND SIGNS
TABLE 1-4 Typical Feature of
Benign Positional
Vertigo
" Mixed vertical-torsional
nystagmus: slow phases
downward and top of eyes
roll away from offending
(dependent) posterior
semicircular canal
" Nystagmus more vertical
when looking toward the
up ear and more torsional
when looking toward the
down ear
" Latency (up to 45 seconds),
transient (usually less than
(15 seconds), decreases with
repetitive testing)
" Reverses direction when
patient sits up
rotation, visual acuity drops much less,
even with complete loss of labyrinthine
function. This dissociation can some-
times be useful to detect malingering.
Head Rotations
The induced slow phases should be
measured when the patient’s head is
oscillated back and forth in yaw (hori-
zontal), pitch (vertical), and roll (tor-
sion) rotations. The subject should fix
upon the examiner’s nose. For yaw and
24 pitch rotations, the patient’s head is os-
cillated at a frequency of about 0.5 Hz
with an amplitude that produces a slow
phase crossing nearly the entire ocular
motor range. Corrective saccades (usu-
ally catch-up) appear as a sign of an
abnormal VOR. A superimposed gaze-
evoked nystagmus may make assess-
ment of the VOR difficult, especially
when the eye is away from the primary
position. For roll rotation, one usually
determines whether the torsional VOR
is present by observing the corrective
torsional quick phases, which can be
seen by focusing on the blood vessels in
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
the conjunctiva near the limbus. Quick
phases of torsional nystagmus may be
absent in one direction with unilateral
midbrain lesions that involve the rostral
interstitial nucleus of the medial longi-
tudinal fasciculus.
Next, brief, high-accelerated head
impulses should be applied with the
eyes initially about 15 degrees away
from primary position in the orbit and
the amplitude of the head movement
such that the eyes will end near the
primary position of gaze (Figure 1-8)
(Video Segment 5). The patient fixes
on the examiner’s nose. The usefulness
of head impulse testing is based upon
Ewald’s second law, which states that a
better response is elicited with excit-
atory than inhibitory stimulation, and
that this asymmetry is most marked at
high frequencies, accelerations, and
speeds of head rotation. Hence, if
there is a complete loss of function of
one SCC, this enduring defect will be
apparent whenever an impulse is ap-
plied so as to stimulate that particular
SCC. Head impulses should be applied
horizontally to probe the function of
the lateral SCC and then vertically in
the plane of an anterior canal in one
labyrinth and a posterior canal in the
other (so called RALP and LARP). The
head is slightly turned to the side about
30 degrees, and then the vertical im-
pulse is applied. A corrective, catch-up
saccade appears as a sign of an under-
active slow phase response. Occasion-
ally with central and usually cerebellar
lesions the corrective saccade will be
directed opposite to the slow phase
because the VOR is hyperactive. The
direction of the corrective saccade may
have a component orthogonal to the
direction of head rotation if there is
an inappropriately directed slow phase.
So, for example, if there is an upward
slow phase component to the horizon-
tal impulse response, the corrective
saccade will have a downward compo-
nent. This sign is commonly observed
with cerebellar lesions and reflects so-
called cross-coupling of the VOR.
Many normal subjects require a
catch-up saccade in the vertical plane
in response to upward head impulses
because downward slow phases may be
slightly hypoactive. Similarly, many oth-
erwise normal elderly individuals often
show a small degree of VOR hypometria
with head impulses, with a catch-up
saccade in both horizontal directions.
Patients with chronic complete bi-
lateral loss of labyrinthine function
superficially may appear to have a rela-
tively intact response to head im-
pulses because of information from
neck proprioceptors that triggers pre-
programmed compensatory eye move-
ments. Likewise, corrective saccades
can be generated so early in well-
adapted patients that they are em-
bedded in the response while the FIGURE 1-7 The ocular tilt reaction is composed of a
head is still rotating and so are hard head-tilt toward the side of the lower eye,
a skew deviation with one eye higher than
to discern. One should therefore make the other, and counterroll (torsion) of both eyes with the top
the head impulse somewhat unpre- poles rolling toward the side of the lower eye.
dictable in timing and amplitude. From Brandt T, Dieterich M. Vestibular syndromes in the roll plane:
topographic diagnosis from brain stem to cortex. Ann Neurol 1994,36:
The head heave test is used to eval- 337–347. Copyright # 1994, John Wiley & Sons, Inc. Reprinted with
uate the translational VOR. It is a variant permission of John Wiley & Sons, Inc.

of the head impulse test, although in this

case it is used to evaluate the function of
the utricle. As with the head impulse
test, the hands of the examiner are
applied over the side of the patient’s
head with the force largely being trans-
mitted through the bottom of the palms
over the patient’s temples. An abrupt,
high-acceleration lateral movement of
the head is imposed while the subject
is instructed to look at the examiner’s
nose. A corrective catch-up saccade in-
dicates the translational VOR is hy-
poactive. Because normal individuals
usually show a hypoactive translational
VOR and may require a corrective sac-
cade, the finding of an asymmetrical
response (as occurs in the first days after
a unilateral loss of function) is most
helpful.
Head-shaking nystagmus (HSN) pro-
vides another way to document imbal-
ance of dynamic vestibular function.
With Frenzel goggles in place, the
patient is instructed to shake the head
vigorously but carefully about 10 times,
side to side (Video Segment 6). Nys-
tagmus following the head shaking is
sought. Normal individuals have at most 25
a beat or two of HSN. With a unilateral
loss of labyrinthine function, a vigorous
nystagmus with slow phases directed
initially toward the lesioned side will
usually appear, followed by a reversal
phase with slow phases directed toward
the intact side (Table 1-7).
The initial phase of HSN reflects an
asymmetry of peripheral inputs during
high-velocity head rotations; according
to Ewald’s second law, a larger-amplitude
response is transmitted centrally dur-
ing rotation toward the intact side
than toward the affected side. This

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 " VESTIBULAR SYMPTOMS AND SIGNS

KEY POINT:
to properly interpret the presence
A Although
Evaluation of
TABLE 1-5 (or absence) and the direction of HSN.
several Skew Deviation
mechanisms In fact, when the primary-phase HSN
can lead to is very brief, the time constant of the VOR
" Best appreciated using is likely to be low, a common finding
head shaking alternate cover testing so
or mastoid that first one, then the other
with acute peripheral labyrinthine loss.
vibration– eye takes up fixation In Ménière’s syndrome, the direction of
induced the primary phase of HSN may be
nystagmus,
" Note the effect of the
direction of gaze on the
opposite that usually expected with a
their most peripheral lesion. With unilateral pe-
skew deviation
important ripheral lesions, vertical head shaking
clinical use " Note any spontaneous tilt
of the head and the effect
may lead to a small-amplitude horizontal
probably
of head-tilt on the skew nystagmus with slow phases directed
is as an
objective
deviation toward the intact ear.
sign of a " Note any static
Circular head shaking is another
vestibular counterrolling of the eyes form of head shaking that can simulate
imbalance, during ophthalmoscopy by constant-velocity rotational testing at
either observing the relationship
peripheral between the macula and
the optic nerve head
or central, in Evaluation of
TABLE 1-6
patients with Dynamic
no other Vestibular-Ocular
abnormalities asymmetry during the head shaking Reflex Function
on clinical
leads to an accumulation of activity with-
examination.
in the vestibular nuclei in the velocity-
" Visual acuity during head
oscillation
storage mechanism. The nystagmus after
head shaking in patients with a vestibular " Elicitation of slow phases
with slow head rotation
imbalance reflects the decay of activity in yaw (horizontal), pitch
within the velocity-storage mechanism. (vertical), and roll (torsion)
Accordingly, the amplitude and duration and with high accelerations
of the initial phase of HSN will depend, in in yaw and pitch (head
part, on the state of the velocity-storage impulse test) or in planes
that include the right
mechanism.
anterior and left posterior
The velocity-storage mechanism is semicircular canals (RALP)
disabled immediately after an acute uni- and the left anterior and
26 lateral vestibular loss. The primary phase right posterior semicircular
of HSN may be absent or attenuated in canals (LARP)
these circumstances. The reversal phase " Sustained rotation in a
of HSN, which reflects short-term adap- swivel chair or circular head
tation probably arising both in the shaking with Frenzel
vestibular nerve and in central struc- goggles to elicit postrotatory
nystagmus
tures, may still be present. Hence, there
may be an immediate ‘‘reversal phase’’ " Ophthalmoscopy during
of HSN. Unfortunately, it becomes head rotation to look for
instability of the optic disk
difficult to know whether one is observ-
ing the primary or the ‘‘secondary’’ re- " Bedside ‘‘mini-caloric’’ test
versal phase of HSN. Because the state " Head-shaking nystagmus
of the velocity-storage mechanism is
" Vibration-induced nystagmus
reflected in the time constant of the
VOR, rotational testing may be necessary

Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

FIGURE 1-8 The head impulse test for decreased labyrinthine function. Top, normal response to rotation to the left.
Bottom, abnormal response to rotation to the right. Note that the eyes are dragged in the orbit when
the head is rotated toward the affected side (right), and a catch-up, corrective saccade is required to maintain
fixation on the examiner’s nose.
Reprinted with permission from Halmagyi GM, Curhtoys IS. A clinical sign of canal paresis. Arch Neurol 1988;45:737–739.
Copyright # 1988, American Medical Association.

the bedside. When a subject rotates the Frenzel goggles or ophthalmoscopy

head in a circular fashion (tracing out
a circular path with the chin), the re-
sult is a torsional stimulus of constant
direction (ie, a step of torsional head
velocity). When the head is stopped,
there should be a torsional postrotatory
nystagmus. Patients with bilateral ves-
tibular loss have a reduced or absent
response.
Central lesions, for example with cere-
bellar dysfunction, may also lead to
HSN, often with vertical nystagmus ap-
pearing after horizontal head shaking
(so-called cross-coupled nystagmus).
with occlusion of the opposite eye
should be used to eliminate fixation
and make it easier to observe the nys-
tagmus. If no response is elicited with
the initial stimulus, larger volumes of ice
water can be used (up to 10 mL). In
patients with an acute unilateral loss of
TABLE 1-7 Nystagmus After 27
Horizontal
Head Shaking
" Peripheral Pattern

HSN probably can arise from mechan- Primary phase: slow phase to
ical disturbances in the labyrinth, eg, a hypoactive ear
perilymph fistula or an abnormality of Secondary reversal phase:
the cupula itself. slow phase opposite to
hypoactive ear

Bedside Caloric Testing " Central Pattern

A small amount of ice water (initially Cross-coupling: vertical

nystagmus after horizontal
about 0.3 mL), introduced with a 1-mL
head shaking
syringe can be used to assess the
function of the lateral SCC in each ear.

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 " VESTIBULAR SYMPTOMS AND SIGNS
KEY POINTS:
labyrinthine function, the caloric re-
A When a
sponse may actually be decreased on
positional
nystagmus
the intact side as well as lost on the
is horizontal, involved side. This is due to the initial
sustained, or attempts of adaptive mechanisms to
of high-enough rebalance the vestibular nuclei by sup-
intensity to be pressing activity in the vestibular nuclei
seen without on the intact side as well as by restoring
Frenzel goggles activity in the vestibular nuclei on the
and with its deafferented side.
direction
independent
of whichever Positional Testing
ear is down,
a central lesion
Nystagmus evoked by a change in head
must be position is a useful clinical sign. Posi-
excluded. tional (sustained) and positioning (tran-
sient) nystagmus are best elicited with
A When a
the patient wearing Frenzel goggles.
positional
First, perform the Dix-Hallpike maneu-
nystagmus is
ver (Video Segment 7). With the pa-
purely vertical
or purely tient sitting, the head is turned (not
torsional, and tilted) about 45 degrees toward one
especially if side. The examiner stands in front of
sustained, a the patient and holds the head at the
central lesion temples. The head, neck, and trunk
must be are moved en bloc to a head-hanging
excluded. position about 30 degrees below the
horizontal. After the initial positioning
of the head in the upright position,
there need be little change in the
position of the head on the body, apart
from making sure the head gets below
earth horizontal. A variant (‘‘side-lying’’)
in which the patient turns to the side
with the nose 45 degrees away from the
28 tested side and then lies on the side may
be easier to tolerate for older individ-
uals. Note the eye movements in primary
position and on left and right gaze.
After about 45 seconds or earlier if any
induced nystagmus has stopped, re-
turn the patient to the upright posi-
tion and observe the eye movements
again. Repeat the whole procedure with
the head rotated 45 degrees toward
the other shoulder. Transient mixed
vertical-torsional nystagmus induced by
these maneuvers is usually diagnostic
of BPPV emanating from the posterior
semicircular canal (Table 1-4) (Video
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
Segment 3). If nystagmus appears,
the same positioning maneuver should
be repeated to see whether the nystag-
mus becomes more difficult to elicit on
successive repetitions.
If Frenzel goggles are not used when
examining patients who develop po-
sitional nystagmus due to BPPV, the
torsional component may be more pro-
minent because the vertical (and any
small horizontal) component is more
easily suppressed by fixation mecha-
nisms. When the upright position is re-
assumed, nystagmus due to BPPV may
transiently reappear, but its direction
is now opposite that in the dependent
position. With successive repetitions,
the nystagmus of BPPV usually becomes
harder to elicit.
Testing for nystagmus with static
changes in head position (eg, with
the subject lying supine with the head
turned to the right and with the head
turned to the left) is useful in eliciting
the horizontal nystagmus associated
with the lateral canal variant of BPPV.
This nystagmus usually changes direc-
tion with lateral head turn (direction-
changing nystagmus), such that it is
either always beating toward the earth
(geotropic) or always beating away from
the earth (apogeotropic). Some normal
subjects show a weak horizontal nystag-
mus with positional testing in darkness.
It is usually in the same direction with
respect to the head (direction-fixed
nystagmus), whether the head is turned
to the left or to the right (Case 1-1).
Positional testing may also exacer-
bate a spontaneous nystagmus. With
an acute unilateral loss of labyrinthine
function, the horizontal component
of the spontaneous nystagmus is in-
creased with the patient lying with the
affected ear down and decreased with
the affected ear up. This effect of gravity
on the horizontal component of the
spontaneous nystagmus may be medi-
ated by an inappropriate otolith-driven
translational VOR.
 Case 1-1
A 46-year-old man developed transient vertigo upon turning over to
the right in bed. He also had slight imbalance and vertigo after getting
out of bed. His symptoms began the next day after spending a few hours
lying in the supine position while having dental work performed. On
examination, when placed in right Dix-Hallpike position, he has a mixed
vertical torsional nystagmus, with the vertical component upbeating and
the torsional component such that the top pole beats toward the
dependent (right) ear. After a particle repositioning maneuver, on repeat
testing he develops a horizontal positional nystagmus in both the right
ear and left ear down positions, which beats toward the side of the
dependent ear.
Comment. This patient has the typical history and findings for a right
posterior canal BPPV syndrome. After the particle repositioning maneuver,
the otoconia have moved from the posterior to the lateral SCC, causing
the horizontal positional nystagmus. This will usually resolve on its own
in 1 or 2 days but if necessary can be treated with a maneuver to dislodge
the otoconia from the lateral canals.

Miscellaneous Bedside impulses are transmitted relatively sym-

Vestibular-Ocular Tests
Vestibular suppression (cancellation
of a target moving with the head) is
another way for assessing the visual
tracking and fixation systems and is
comparable to fixation suppression of
caloric-induced nystagmus. The patient
is instructed to fix upon a target (eg, the
patient’s own outstretched hand) that
is moving en bloc with the patient’s
head. The interpretation of vestibular
suppression must consider the results
of direct testing of the VOR. Although
defects in fixation suppression of the
VOR usually parallel deficits in smooth
pursuit from central lesions, a patient
with a marked loss of vestibular function
might have perfectly normal vestibular
suppression (there is, of course, little to
suppress) even when pursuit is mark-
edly impaired.
Vibration applied to the mastoid tip
may also bring out nystagmus in pa-
tients with unilateral loss of function
and occasionally in other conditions
such as superior canal dehiscence or
other types of fistulae. In the case of a
unilateral loss of function, vibration of
either mastoid (because the vibration
metrically through the skull to both
labyrinths) elicits a nystagmus with a
slow phase toward the paretic ear, in
essence acting like a hot water caloric
stimulus to the intact labyrinth (Video
Segment 8). When vibration brings out
a vertical nystagmus, a central lesion
should be sought.
Valsalva maneuver–induced nys-
tagmus should always be searched for,
again with the subject wearing Frenzel
goggles or during ophthalmoscopy with
the opposite eye occluded. The Valsalva
maneuver should be performed both
with the nose pinched and against closed 29
glottis (Video Segment 9). Cranio-
cervical junction anomalies such as the
Arnold-Chiari malformation, perilymph
fistulas including superior canal dehis-
cence, and other abnormalities involving
the ossicles, oval window, and saccule
may be responsible for this sign. Jugular
vein compression may also induce nys-
tagmus with the same types of lesions.
Hyperventilation may induce a vari-
ety of symptoms in patients with anxiety
and phobic disorders but usually does
not produce nystagmus. Patients with
demyelinating lesions of the vestibular

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 " VESTIBULAR SYMPTOMS AND SIGNS

KEY POINT:
gence) and eye alignment should be
A Hyperventilation
Bedside Eye
TABLE 1-8 examined carefully because the locali-
is a useful
Movement zation of a vestibular abnormality to
provocative Testing
maneuver for central or peripheral structures often
producing depends on the demonstration of signs
" Alignment
of a central ocular motor abnormality
symptoms in
anxiety and " Range of motion (Table 1-8).
panic syndromes " Gaze holding: gaze-evoked
and for eliciting and rebound nystagmus BEDSIDE EXAMINATION OF
nystagmus with VESTIBULOSPINAL REFLEXES
compressive or
" Vergence
The examination of VSR follows the
inflammatory " Saccades (accuracy, speed,
same principles outlined for VOR test-
lesions that latency, presence of
extraneous saccades during ing (Table 1-9). A static imbalance in
produce
demyelination fixation) VSRs mediated by the lateral SCCs is
in peripheral sought by having the patient perform
" Pursuit (catch-up or back-up
or central saccades) tandem walking with eyes open and
vestibular eyes closed and in stepping tests (eyes
" Bedside optokinetic
closed, marching around a circle). In
pathways or
nystagmus (evaluate slow
with fistulas and quick phases) stepping tests, imbalance is reflected
when pressure in excessive turning. The stepping test
changes in the may be looked upon as the equivalent
subarachnoid test for the legs of past-pointing with the
space are nerve due to compression by a tumor arms. A static imbalance in vertical canal
transmitted
(acoustic neuroma or cholesteatoma) reflexes is searched for in the various
to the
or a small blood vessel or of central permutations of the Romberg test. The
membranous
pathways, as with multiple sclerosis, fundamental question is whether ex-
labyrinth.
may develop nystagmus with hyperven- cessive anterior or posterior (vertical
tilation. It is often an excitatory nys-
tagmus reflecting activation of the
abnormal nerve. Hyperventilation- TABLE 1-9 Vestibulospinal
induced nystagmus may also appear Testing
in patients with a history of a labyrin-
thitis (in which case it is often directed " Past-pointing with arms,
with eyes closed
with the slow phase toward the paretic
ear) or with a perilymph fistula (Video " Romberg feet apposed, in
30 Segment 10). tandem, in tandem on toes,
on one foot at a time,
Patients with perilymph fistulas such standing on compliant foam
as superior canal dehiscence or abnor- rubber
malities of the ossicles, oval window, and
" Fukuda stepping test or
saccule may develop nystagmus with walking around a circle
manipulation of the ossicular chain
or with changes in middle-ear pressure " Effect of Valsalva maneuver
and tragal compression on
due to noise, tragal compression, appli- postural stability with
cation of positive and negative pressure eyes closed
to the tympanic membrane, opening
" Tandem gait, forward and
and closing of the eustachian tube, and backward
so on.
In addition to bedside testing of the " Rapid turns with eyes open
and closed
VOR, each of the ocular motor sub-
systems (saccades, pursuit, and ver-

Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 KEY POINTS:
component) or lateral (torsional [roll] Dynamic vestibulospinal function
can be assessed by observing pos-
A Disorders of the
component) sway occurs when the pa-
semicircular canals
tient stands in tandem with eyes closed tural stability during rapid turns or in
(and their central
(to eliminate visual cues), on his or her response to external perturbation im- projections)
toes or on foam rubber (to minimize or posed by the examiner, eg, a gentle push relate to angular
distort somatosensory cues), or under forward, backward, or to the side. Just acceleration
both conditions together. It should be as for the VOR, tragal compression and (rotation). Vertigo
pointed out that static imbalance in the Valsalva maneuver can be used to (spinning of the
otolith-spinal reflexes also leads to ex- elicit an increase in postural sway. A environment
cessive sway (eg, lateral head and body complete examination of gait, strength, or the self),
tilt with utriculospinal imbalance) and reflexes, sensation in the legs and feet, nystagmus,
and cerebellar function is, of course, past-pointing of
sideways deviation on the stepping test.
the limbs, ataxia, a
Past-pointing of the arms (or feet) to essential for the proper interpreta-
positive Romberg
previously seen targets with eyes closed tion of postural instability and disequi-
sign, and turning
may also be a sign of vestibulospinal librium tests. during the
imbalance. For the arms, past-pointing In sum, physiologically based evalua- Fukuda (stepping-
is best elicited by having the patient tion of the VOR and VSR, with additional in-place) test
repetitively raise both arms over the careful attention to the ocular motor may be the
head with the index fingers extended system and other brain stem and cere- consequence.
and then bringing them down, with bellar functions, will help the clinician
A Disorders of the
eyes closed, toward the examiner’s localize the causes of dizziness in a
otolith organs
index fingers (without actually touching number of patients and guide the labo- (and their central
them), which are at waist level. ratory evaluation. projections)
relate to linear
acceleration
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Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.