Cupulolithiasis
Harold F. Schuknecht, MD, Boston
This paper is dedicated to Julius Lempert
whose ingenuity, skill, and enthusiasm set otol¬
ogy in motion in this century. It was a privilege
and honor for me to have come momentarily
under the tutelage of this superb otologie sur¬
geon and teacher.
THE TERM cupulolithiasis is presented
for the first time to designate a vestibular
disorder which previously has been identi-
fied by several names including postural ver-
tigo, positional vertigo, and positional verti-
go of the benign paroxysmal type. Recent
pathological studies support the concept
that the disorder is caused by a deposit,
presumably composed of mineral, on the
cupula of the posterior semicircular canal
which renders this organ sensitive to gravi-
tational force and, therefore, subject to stim-
ulation with changes in head position. The
clinical features of cupulolithiasis are dis-
tinctive and serve to differentiate it from
positional vertigo caused by lesions of the
central nervous system. The diagnosis can
be made by inducing the characteristic ves-
tibular manifestations by provocative posi-
tional testing.
B\l=a'\r\l=a'\ny1 first described the disorder as he
observed it in a 27-year-old woman and he
wrote as follows:
The attacks only appeared when she lay on
her right side. When she did this, there ap-
Submitted for the Julius Lempert memorial issue
of the ARCHIVES.
From the Harvard Medical School and the Mas-
sachusetts Eye and Ear Infirmary, Boston.
Reprint requests to Massachusetts Eye and Ear
Infirmary, 243 Charles St, Boston 02114.
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peared a strong rotatory nystagmus to the
right. The attack lasted about thirty seconds
and was accompanied by violent vertigo and
nausea. If, immediately after the cessation of
the symptoms, the head was again turned to
the right, no attack occurred and in order to
evoke a new attack in this way, the patient had
to lie for some time on her back or on her left
side.
Barany and others originally attributed
this disorder to lesions in the semicircular
canals but, because the dizziness was precip¬
itated not by head movement but by head
position, they came to believe that the con¬
dition was due to a disorder of the otoliths.
Dix and Hallpike2 in 1952 made a notable
contribution to our understanding of posi¬
tional vertigo. In a study of 100 patients
with this symptom they found a high inci¬
dence of ear disorders (eg, otitis media,
acoustic trauma). They noted that the verti¬
ginous attacks occurred when the abnormal
ear was placed undermost in the testing
procedure. Their observations supported
Bárány's opinion that this type of vertigo is
of inner ear origin.
In 1956 Lindsay and Hemenway3 de¬
scribed for the first time a symptom complex
which they observed in seven patients. It
was characterized by a sudden, severe, and
prolonged vertiginous episode which subsid¬
ed after several weeks and was followed by
positional vertigo. All were in the fifth, sixth
or seventh decade of life and the positional
vertigo persisted for weeks to years. All had
impaired response to caloric testing and
some had hearing loss. These functional al¬
terations existed in the ear located under-
 most with the head in the provocative posi¬
tion.
Subsequently, I4 presented some additional
clinical observations, reported the results of
some pertinent animal experiments, and in¬
terpreted the pathological findings in three
sets of previously reported temporal bones
from patients with this disorder. One of the
sets of temporal bones resides in the collec¬
tion of John Lindsay in Chicago and the
other two in the collection of C.S. Hallpike
in London. It was also noted that ablation
of vestibular function in the involved ear—
that is, the ear located undermost with the
head in the provocative position—gave com¬
plete relief of positional vertigo in four pa¬
tients (three of Citron and Hallpike and one
of mine). The evidence from all sources
appeared to support the idea that positional
vertigo of the benign paroxysmal type, as it
was then termed, is caused by substances of
high specific gravity, possibly otoconia, act¬
ing upon the cúpula of the posterior semicir¬
cular canal.
The purpose of the present report is to
review the previous evidence once again and
to present new data from animal experi¬
ments as well as the pathological findings in
two additional sets of human temporal
bones, all of which may be interpreted to
support the thesis that this particular type
of positional vertigo is a clinical entity suit¬
ably termed cupulolithiasis.
Symptoms
The principal complaintof the patient is the
occurrence of sudden attacks of vertigo precipi¬
tated by certain head positions. Usually the
patient volunteers that the attack can be in¬
duced by rolling over in bed either to one side
or the other but not to both sides. Some state
that the attack is provoked by a sudden move¬
ment of the head to the right or left, or when
extending the neck, as in looking upward, and,
more rarely, upon stooping over. Characteristi¬
cally, there is a severe subjective sensation of
disequilibrium which many patients find in¬
tensely disagreeable. Those with good motor
function rarely fall because the onset, although
sudden, is not apoplectiform, so that there is
time to grasp for support during the short
period of disequilibrium. Aged patients experi¬
encing loss of muscle strength and control are
more in danger of falling.
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Many individuals experience a mild sensation
of nausea during and immediately following the
attack and a few have nausea and vomiting. In
rare cases nausea and vomiting may persist
for several hours and these patients often are
reluctant to undergo provocative testing.
The sensation of vertigo is always of short
duration, usually about five to ten seconds,
which helps to distinguish this condition from
other vestibular disorders. Because of the in¬
tensity of the vertiginous sensation the patients
commonly assign a longer duration to the at¬
tack and careful questioning may be required
to elicit a reliable history. At the onset of the
attack most patients quickly move from the
provocative head position and attribute to this
the quick termination of the attack; others,
however, will relate that if the head is main¬
tained in the provocative position the vertigo
will subside in a few seconds.
In many cases the disorder is self-limiting
and subsides within a few weeks or months; in
others there are remissions and recurrences
over time spans of weeks to years and in still
others it is persistent. Some patients report
that the attack is produced only occasionally by
the provocative position, a feature which is
confirmed by inconsistent responses to the test
procedure.
In an attempt to determine a possible etio¬
logic factor, the historical record should include
information relative to recent head injury, ear
symptoms (tinnitus, hearing loss, recent or past
otorrhea, ear surgery), or a recent severe pro¬
longed vestibular upset.
Although the sex distribution has not been
studied, it is Barber's5 impression and also
mine that most patients with the disorder are
females.
Test Procedure
After acquiring the history, the diagnosis of
cupulolithiasis usually can be promptly con¬
firmed by the positional test procedure which is
performed as follows:
The patient is seated upon an examining
table with the head turned to one side and the
gaze fixed on the examiner's forehead. The
examiner then grasps the head in his hands and
briskly places the patient into the supine
head-hanging position. The head should be
placed below the level of the table and 30 to 40
degrees to one side. After the position has been
assumed there is a quiescent latent period of
one to six seconds (average of three or four
seconds). If the vertigo is induced the patient
usually demonstrates a sensation of distress
 variously manifested by closing the eyes, grasp¬
ing the examiner, crying out in alarm, and
making active efforts to sit up again. If the
nystagmus is to be observed the patient must
be instructed before the test to keep the eyes
open. With the eyes in the position of foreward
gaze the nystagmus is predominantly rotatory.
In the right-ear-down position the rotation is
counterclockwise. If the reaction occurs in the
left-ear-down position the nystagmus is oppo¬
site to that of the right-ear-down position. With
the eyes directed toward the uppermost ear the
nystagmus is vertical and upward beating. The
nystagmus increases in rapid crescendo for
three to ten seconds and then rapidly subsides
as the patient's distress is relieved. The patient
is brought back into the sitting position with
the head still grasped by the examiner's hands.
Almost immediately there is vertigo and nys¬
tagmus in reverse to that elicited in the pre¬
vious position, but less severe and for a shorter
period of time. Each time the patient is placed
into the provocative supine position the reac¬
tion is less severe, and often it cannot be
elicited more than two or three times. A rest
period is required before the phenomenon can
be produced again. In the complete test pro¬
cedure the patient is placed in turn into both
right and left head-hanging positions.
Etiologic Factors
Spontaneous Degenerative Change, Ves¬
tibular Labyrinth.—Many patients suffering
from this disorder have no history of antece¬
dent head injury, ear disorder, neurological
disease, or ear surgery. They are usually in
the fifth, sixth, or seventh decades of life
and usually demonstrate no unusual degen¬
erative changes of aging. It is possible that
the disorder in these patients is an expres¬
sion of spontaneous degenerative change in
the vestibular labyrinth, consisting specif¬
ically of the generation of substances of
high specific gravity (possibly loose otocon¬
ia) in the endolymph of the pars superior
(utricle and canals). The symptoms often
subside spontaneously after a few weeks or
months, but may recur, and may persist
indefinitely. Most patients learn to live with
the disorder but consider it an annoyance
and wish to be free of it. A few experience
disability of sufficient magnitude to impose
restrictions on social and occupational activ¬
ities.
Labyrinthine Concussion.—Positional ver¬
tigo probably is the most common type
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of vestibular disorder following head in¬
jury.2611 Barber5 found this type of posi¬
tional vertigo in 47% of 47 patients with
longitudinal fracture of the temporal bone
and in 20.8% of 77 head injuries of compa¬
rable severity but without skull fracture.
Other types of trauma to the vestibular
system which may be incurred from a head
blow are: (1) transverse fracture of the tem¬
poral bone, and (2) injury to the vestibular
neural pathways of the brain. Transverse
fracture results in disruption of the membra¬
nous labyrinth and severe loss of auditory
and vestibular function. Injury to the vestib¬
ular pathways in the brain may incite posi¬
tional vertigo but the nystagmus often is
direction-changing, and lacks the onset de¬
lay, rotatory movement, limited duration,
fatigability, and severe subjective sensation
which are so characteristic of the cupulolithi¬
asis syndrome.
It has been suggested previously411 that
the possible mechanism for the development
of positional vertigo following head injury is
disruption of the utricular otolithic mem¬
brane and release of otoconia into the endo¬
lymph of the pars superior. Theoretically
the otoconia, being free to respond to gravi¬
tational force, would settle into the ampulla
of the posterior canal and with change of
head position could forcibly displace the
cúpula. Postmortem studies of the temporal
bone in head-injured patients thus far have
failed to reveal dislodged otoconia, probably
because they are absorbed by the décalci¬
fication process during histological prepara¬
tion. Vyslonzil,12 utilizing a dissection pro¬
cedure, without prior décalcification, found
otoconia in the posterior semicircular canals
of patients receiving ototoxic doses of strep¬
tomycin. Presumably, the reason these pa¬
tients did not demonstrate positional vertigo
was that the function of the posterior canal
crista had been destroyed by the drug.
Otitis Media.—Dix and Hallpike2 were
the first to observe that many patients with
positional vertigo of the benign paroxysmal
type have evidence of previous or existing
ear infection. In a study of 100 cases of
positional vertigo evidence of otitis media
was found in 26 of which 11 were bilateral
and 15 were unilateral. All 15 patients with
unilateral evidence of otitis media elicited
the positional nystagmus with the diseased
 ear placed undermost during the test pro¬ positioned with the right undermost.
ear
cedure. The caloric responses normal. Histo¬
were
Ear Surgery.—Positional vertigo of the logical study was reported to show a normal
paroxysmal type occurs as an uncommon left labyrinth and, on the right, normal semi¬
complication of stapes surgery and usually circular canals but severe degenerative
can be related to traumatic manipulations changes in the utricle, saccule, and cochlea.
involving the structures within the vestibule. The sensory epithelium of the utricle ap¬
The probable pathogenesis of postoperative peared disorganized, the underlying stroma
positional vertigo in these cases is rupture of was thick and infiltrated with cells, and the
the utricular otolithic membrane associated otolithic membrane was absent.
with release of otoconia. Usually the symp¬ Lindsay and Hemenway3 described the
tom subsides within a few weeks but, rarely, findings in the temporal bones of the patient
may persist for months or years. The com¬ who experienced a complex of symptoms
plication is uncommon following other types which they attributed to occlusion of a ves¬
of temporal bone surgery (fenestration, mas¬ sel supplying the vestibular labyrinth. At
toidectomy, tympanoplasty) and does not the age of 65 this patient experienced the
occur after labyrinthectomy. sudden onset of severe vertigo associated
Occlusion of Anterior Vestibular Artery. with vomiting which gradually subsided
—Lindsay and Hemenway3 described in over a period of one month. Subsequently,
1956 a symptom complex observed in pa¬ she had vertiginous episodes whenever she
tients in the latter decades of life which they assumed the supine position with the head
suspected was caused by occlusion of a ves¬ turned to the right. The vertigo was readily
sel supplying the vestibular labyrinth. The demonstrated by positional testing. Caloric
first manifestation of the disorder was the response was absent in the right ear. The
sudden onset of severe vertigo without deaf¬ positional vertigo continued until her death
ness or signs of central nervous system dis¬ 13 years later. Histological study was re¬
ease, followed by gradual recovery during ported to show degeneration of the superior
the subsequent few weeks. The clinical pat¬ division of the vestibular nerve in the right
tern was consistent with that known to ear only (Fig 1).
result from sudden destruction of one laby¬ In 1957 Cawthorne and Hallpike13 report¬
rinth. A second manifestation of the syn¬ ed the histological findings in a 59-year-old
drome was the development of positional woman who for two years had not been able
vertigo, commencing after the original attack to lie on her right side for fear of precipitat¬
had subsided, and persisting for weeks or ing an attack of vertigo. Caloric response
years. They presented the pathological was decreased in the right ear and position¬
findings in the temporal bones from a pa¬ al testing produced the attack in the supine
tient with this syndrome which were consis¬ right-ear-down position. Histological study
tent with occlusion of the anterior vestibular was reported to show moderate degeneration
artery. This artery supplies the utricular of the utricular macula and nerve and the
macula and the cristae of the horizontal and crista of the horizontal semicircular canal.
superior semicircular canals. Presumably de¬ The remaining structures of the right ear
generation of the utricle and its otolithic and the entire left ear were reported as
membrane following devascularization of being normal. It was the authors' opinion
this structure would release otoconia which that the findings were consistent with ather-
would then be free to settle upon the cúpula omatous occlusion of the arterial supply to
of the posterior semicircular canal. the right utricle and horizontal canal crista.
After examining the histological prepara¬
Pathological Findings tions of these three previously reported
cases, I concur with the general interpreta¬
Dix and Hallpike2 reported the findings tions of the authors. The diseased ear in
in the temporal bones of a 40-year-old wom¬ each case shows degeneration of the macule
an who had severe right sensorineural deaf¬ of the utricle and cristae of the horizontal
ness and positional vertigo of 20 years' du¬ and superior canals and the superior vestib¬
ration. The attacks occurred with the head ular nerve. In each ear the crista and nerve

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 Fig 1.—Atrophy of the superior division of the vestibular nerve (N), utri¬
cle (U), and horizontal semicircular canal crista (C) in a patient with posi¬
tional vertigo of the paroxysmal type. Courtesy of Lindsay and Hemenway.

of the posterior canal appears to be intact.
I have recently acquired the temporal
bones from two patients who experienced
typical positional vertigo of the paroxysmal
type. The histological findings in the two
sets of temporal bones are nearly identical
and provide further evidence concerning the
unique pathophysiology of this disorder.
Report of Cases
CASE 1.—At the age of 69, the patient first
experienced paroxysmal attacks of vertigo pre¬
cipitated by stooping over, lying down, and, in
particular, when rolling onto the left side. They
did not occur when rolling onto the right side.
She noted a tendency to stagger and fall for¬
ward and to the left and occasionally had the
sensation that she was being pulled backward.
On two occasions she fell to the floor at the
onset of an attack. The episodes were of short
duration, could be precipitated at will, and
were not associated with auditory symptoms.
Examination at the age of 71 revealed normal
tympanic membranes. Caloric tests using 5 cc
of water at 80 F produced responses of 90
seconds on both sides which was considered to
be normal. There was no spontaneous nystag¬
mus. Tests for positional vertigo provoked a
severe vertiginous episode of short duration
associated with clockwise rotatory nystagmus
when she was placed in the supine left-ear-
down position. Details such as onset latency,
exact duration, and fatigability were not re-
corded. There was no reaction in the supine
right-ear-down position. Pure tone audiometrie
tests showed a mild bilateral sensorineural
hearing loss for frequencies above 2,000 cycles
per second (cps). Speech discrimination was
not tested.
These symptoms continued unchanged and
were noted in the medical record on several
occasions during the following three years, the
last mention being made when the patient was
74 years of age. She died at the age of 77 of
massive cerebral hemorrhage, and both tempor¬
al bones were removed for histological study.
Histological Study.—The temporal bones are
in an excellent state of histological preservation
and preparation. The middle ears and mastoids
are well pneumatized and appear normal. The
organs of Corti show normal hair cell popula¬
tions throughout. There is a diffuse uniform
loss of about 60% of the spiral ganglion cells in
both cochleae.
In both ears the utricular and saccular macu¬
lae have normal sensory epithelium and intact
otolithic membranes and the vestibular nerves
appear normal. All cristae show intact sensory
epithelium.
Attached to the posterior surface of the cúp¬
ula of the left posterior semicircular canal is a
basophilic staining homogenous deposit measur¬
ing 300/u in its greatest dimension (Fig 2 and
3).
CASE 2.—At the age of 64, the patient began
having attacks of vertigo precipitated by
changes in head position. The duration of each

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 Fig 2.—Dense deposit (D) on the cúpula (C) of the posterior semicircular canal of the left ear.
Tests for positional vertigo provoked a severe vertiginous episode of short duration associated with
clockwise rotatory nystagmus when she was placed in the supine left-ear-down position (case No. 1).

Fig 4.—There is a dense deposit (D) on the cúpula

(C) of the posterior semicircular canal of the left ear.
Tests for positional vertigo provoked a clockwise ro¬
Fig 3.—Normal crista and cúpula of the right pos¬ tatory nystagmus and subjective sensation of vertigo
terior semicircular canal (case No. 1). in the supine left-ear-down position (case No. 2).

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 Fig 5.—Normal crista and cúpula of the right posterior semicircular canal (case No. 2).

Fig 6.—There is a thin layer of granular deposit (D) on the membranous wall
of the left posterior semicircular canal in its most inferior portion (case No. 2).

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 attack was but a few seconds and it was not
associated with auditory symptoms or loss of
consciousness. On one occasion she fell to the
floor, striking the occiput but did not lose
consciousness. The attacks were most severe
when arising from bed, and she had to sit on
the edge of her bed for some seconds before she
could rise. Audiometrie tests revealed near-nor¬
mal pure tone thresholds, speech reception lev¬
els of 12 db, and discrimination scores of 92%
in each ear. Caloric tests (Kobrak minimal and
Hallpike bithermal) were interpreted to be
normal. Electroencephalogram (ENG), skull
films, and brain scan were normal. Tests for
positional vertigo with eyes in central position
provoked clockwise rotatory nystagmus and
subjective sensation of vertigo in the supine
left-ear-down position. The nystagmus had a
latency of six seconds, and a duration of seven
seconds. With eyes closed and recorded on
ENG the latency was 13 seconds and the dura¬
tion 33 seconds. Upon assuming the upright
position there was counterclockwise rotatory
nystagmus and subjective vertigo. The nystag¬
mus had a latency of seven seconds and a
duration of four seconds. When recording on
ENG with eyes closed the latency was five
seconds and the duration was 20 seconds. No
reaction occurred in the supine right-ear-down
position. The vertiginous symptoms continued
unchanged for four years when at the age of 68
she died of pulmonary embolus, secondary to
congestive heart failure. Both temporal bones
were removed for histological study.
Histological Study.—The temporal bones are
well pneumatized and the mastoids, middle
ears, ossicles, and tympanic membranes appear
normal. There is mild preparation artifad con¬
sisting of areas of slight fragmentation of the
bony structures, and scattered small tears in
the membranous structures. There is compres¬
sion artifact to the extent that Reissner's mem¬
brane is in contact with the organ of Corti in
some areas. The organs of Corti and spiral
ganglia are atrophied in the basal 3 MM of the
cochleae but are normal elsewhere. The utricu¬
lar and saccular maculae and the sensory epi¬
thelium of all cristae, as well as the vestibular
nerves, appear normal in both ears.
Attached to the cúpula of the left posterior
semicircular canal is a granular, basophilic
staining mass measuring 350/i in its greatest
dimension (Fig 4 and 5). A thin layer of this
material also is located on the membranous
wall of the posterior semicircular canal in its
most inferior location (Fig 6).
Comment.—Both of these patients de¬
veloped positional vertigo during the sev-
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enth decade of life and which persisted for
the remainder of their life spans—eight
years for one and four years for the other.
One patient had fallen to the floor twice and
the other once but neither were injured.
Both were mildly disabled but were able to
live moderately comfortably with slight re¬
striction in activities. Neither patient had
experienced a head injury, ear infection, ear
surgery,or symptoms suggestive of occlusion
of the anterior vestibular artery, and the
etiology presumably was spontaneous degen¬
erative change of the vestibular labyrinth.
The significant pathological finding in
each is a basophilic deposit, presumably of
high specific gravity, located on the cúpula
of the posterior canal of the ear which was
undermost when vertigo was induced on po¬
sitional testing. The origin of the deposit is
not obvious on histological study. It proba¬
bly was not generated from the cúpula r
substance as the cupulae appear identical to
the cupulae of the posterior canals of the
opposite ears which do not have this depos¬
it. The utricular otolithic membranes appear
normal as compared to the saccular otolithic
membranes of the same ears and to the
otolithic membranes of the opposite ears;
the integrity of the otoliths, however, can¬
not be evaluated because they have been
resorbed by the décalcification process to
which they were subjected during histologi¬
cal preparation.
It is possible that the cupular deposits
represent calcium carbonate derived from
otoconia, perhaps altered to some extent,
and imbedded in a type of matrix which is
stained by hematoxylin. It is known that in
some animal specimens prepared by intravi¬
tal preparation the region of the otolithic
membrane occupied by otoconia is stained
to show the outline of these crystals. It is
also possible that the deposits represent
products of degeneration from other sources.
In a study of 550 specimens at the Massa¬
chusetts Eye and Ear Infirmary Ruby (un¬
published data) found similar deposits on
the cupulae of the posterior canals in 15 for
an incidence of 207%. In another 53 there
were small amorphous granular deposits on
the cupulae of the posterior canal. No such
deposits were found on the cupulae of the
superior and horizontal canals. It is proba¬
ble that some of these deposits are the result
 of postmortem degeneration of the utricular
otolithic membrane.
Experimental Observations
The observations from several experi¬
ments have provided information which is
pertinent to the proposed concept regarding
the pathogenesis of positional vertigo of the
paroxysmal type.
Cutting the Anterior Vestibular Artery in
the Cat.—In an experiment previously re¬
ported by me,4 the superior division of the
vestibular nerve as well as the anterior ves¬
tibular artery were sectioned. It is not tech¬
nically feasible to cut only the artery. The
operation was performed on the left ears of
four cats by a surgical approach through the
superior surface of the petrous pyramid.
Thus, it was possible to cut these structures
within the internal acoustic canal.
Each animal experienced a postoperative
vestibular upset consisting of horizontal nys¬
tagmus to the right, ataxia, falling to the
left, and head tilt to the left. The acute signs
of vestibular upset subsided gradually over a
period of days, but postural abnormalities
such as slight ataxia and head tilt persisted.
Positional vertigo having the character of
the benign paroxysmal type developed in
one of the four cats. It was first elicited
three months after operation and persisted
until termination of the experiment at seven
months. A rotatory clockwise nystagmus oc¬
curred only in the supine position with the
left ear undermost. The period of delay
varied from one to five seconds and the
duration from 20 to 36 seconds. The inten¬
sity and duration of nystagmus diminished
rapidly on repeated testing but did not fa¬
tigue completely on repeated testing.
All four cats were killed seven months
after operation and the temporal bones pre¬
pared for histologie study. Each left ear
showed severe atrophy of the superior divi¬
sion of the vestibular nerve, the macule of
the utricle, and the cristae of the superior
and horizontal canals, as well as the dark
cell areas of these structures. The dark cell
areas of the utricle and ampullae contain
cells which are cytologically compatible with
secretory function.14 The utricular otolithic
membranes were present but degenerated in
two and were entirely missing in two (Fig
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7). The superior part of the macules of the
saccules were atrophied in two ears. It is
common for the superior part of the macule
of the saccule to be supplied by a branch of
the anterior vestibular artery; the main sup¬
ply, however, is from a branch of the poste¬
rior vestibular artery. The posterior canal
cristae and inferior divisions of the vestibu¬
lar nerves were intact in all four.
The vestibular membranous labyrinth was
distorted and partially collapsed in all four,
possibly because of decreased endolymph se¬
cretion resulting from atrophy of the dark
cell areas (Fig 8). This may explain why
only one of four animals developed position¬
al vertigo, for the collapsed ampullary wall
of the posterior canal would impair move¬
ment of the cúpula.
Postrotatory Stimulation of the Posterior
Semicircular Canal.—An experiment was
performed to determine whether isolated
stimulation of a single posterior semicircular
canal causes rotatory nystagmus on forward
gaze.
Two operative procedures, consisting of
a right labyrinthectomy followed in three
weeks by section of the superior division of
the left vestibular nerve, were performed on
a cat. This animal, therefore, retained ves¬
tibular function of only the left posterior
semicircular canal and saccule. A test for
postrotatory nystagmus was performed six
weeks later with the head positioned so that
the left posterior canal was in the plane of
rotation. The nystagmus which resulted was
purely rotatory. With ampullofugal flow the
nystagmus was clockwise and strong and
with ampullopetal flow it was counterclock¬
wise and weak.
Histological preparations of the temporal
bones revealed successful total ablation of
the right vestibular system and destruction
of the sense organs of the left utricle and
superior and horizontal canals as planned.
There was partial collapse of the membra¬
nous labyrinth including the ampullary wall
of the posterior canal as in the previous
experiment, and it is surprising that postro¬
tatory nystagmus occurred in spite of this
condition.
Dependency of Nystagmus on Direction
of Gaze.—Bárány1 first noted that when the
eyes were directed toward the uppermost ear
in the provocative test position the nystag-
 Fig 7.—Degeneration of the utricular macula (U) and section of the anterior vestibular artery. Otoconia have
its otolithic membrane and the crista of the horizontal separated from the gelatinous structure of the otolithic
semicircular canal (C) of the cat, one month following membrane. Inset shows high-power view of otoconia.

Fig 8.—Collapse of the ampullary wall of the anterior vestibular artery in the cat. Movement
posterior semicircular canal following cutting of of the cúpula (C) is impaired. The margin of the
the superior division of the vestibular nerve and crista neglecta (N) is seen at the left.

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 mus became purely vertical. Animal experi¬
ments by Szentagothai15 have shown that
ampullofugal flow in the posterior canal re¬
sults in contraction of the ipsilateral superi¬
or oblique and contralateral inferior rectus
muscles, and ampullopetal flow results in
inhibition in these same muscles. Fluur16
confirmed these relationships by directly
stimulating the ampullary nerves of cats. On
the basis of this information and the direc¬
tion of pull of the ipsilateral superior ob¬
lique and the contralateral inferior rectus
muscles in different positions of gaze,
Harbert17 has demonstrated that the nystag¬
mus characteristic of positional vertigo of
the paroxysmal type is the result of activity
of the posterior semicircular canal. Further¬
more, he has postulated that the direction of
flow is ampullofugal in the posterior canal
being stimulated.
Intense Mechanical Stimulation.—It was
first shown by Wittmaack18 in 1909 and later
by Hasegawa19 in 1931 and de Kleyn and
Versteegh20 in 1933 that intense linear accel¬
eration by centrifugation results in removal
of the otoconia and the gelatinous layer.
Parker et al21 demonstrated in an experiment
on guinea pigs that moderate loss of otocon¬
ia from the maculae may be observed follow¬
ing exposure to 12 to 25 g for 195 to 330
seconds. Moderate to severe displacement of
otoconia resulted from acceleration expo¬
sures of approximately 50 g for one minute
and accelerations of 100 g for 30 seconds
and of over 100 g for 15 to 20 seconds
produced severe otoconia loss from all macu¬
lae. They also demonstrated slight to moder¬
ate otoconia displacements following impact
decelerations of 240 to 314 g. Parker et al21
stated that the results of their experiments
"support the hypothesis that 'benign parox¬
ysmal positional nystagmus' results from
displacement of otoconia into the canal am¬
pullae."
Pathophysiologic Mechanism
The concept of cupulolithiasis provides a
reasonable explanation for most of the clini¬
cal features of positional vertigo of the par¬
oxysmal type. It assumes that substances
having a specific gravity greater than endo¬
lymph and thus subject to movement with
changes in the direction of gravitational
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force come into contact with the cúpula of
the posterior semicircular canal. Presumably
these particles may exist free in the endo¬
lymph or may become attached to the cúpu¬
la. With the head in the erect position the
posterior canal ampulla is located inferiorly,
whereas in the provocative test position
(supine, head-hanging, ear-down) the pos¬
terior canal assumes a superior position.
The change in position from erect to su¬
pine-head hanging-ear down apparently re¬
sults in a severe ampullofugal displacement
of the cúpula of the posterior canal.
The delay of onset, which is usually sever¬
al seconds, may be due to the period of time
required to get the mass in motion; the
severity of the sensation and nystagmus
may be due to the great magnitude of the
cupular displacement; the limited duration
of the vertiginous attack may be due to the
return of the cúpula to a normal position
after the particles have left it; the fatigabili-
ty may be due to dispersement of particles
in the endolymph of the pars superior occur¬
ring during repeated head positionings; and
the repeatability after rest may be due to
the time required for the gravity sensitive
particles to again settle into the posterior
canal ampulla so that they can again act en
masse when the pars superior is turned over.
Management
Establishing the Diagnosis.—It is com¬
mon for the patient suffering from positional
vertigo of the paroxysmal type to have had
extensive diagnostic laboratory tests in a
well-meant search for the cause of the symp¬
toms. The physician who is aware of the
existence of this disorder will, however, sus¬
pect its presence when recording the history
and can readily establish the diagnosis by
provocative positional testing. Some pa¬
tients are reluctant to submit to the test
experience because of the anticipated dis¬
comfort which, in exceptional cases, includes
nausea and vomiting. Some will require one
hour or more of rest before full recovery
occurs. All of the characteristic features of
the attack must be observed: that is, delay
of onset, rotatory nystagmus, limited dura¬
tion, intense subjective sensation, reversal of
nystagmus on sitting up, and fatigability on
repeat testing.
 Having observed the typical response to with a set of self-imposed restrictions, so
positional testing, the possible etiologic fac¬ that detailed counselling is unnecessary. A
tors should be considered. These have been further responsibility of the physician is to
listed previously as (1) degenerative change render a prognosis regarding the future
of the utricle; (2) labyrinthine concussion; course of the disorder. The prediction as to
(3) otitis media; (4) ear surgery; and (5) whether the disorder is self-limited, recur¬
occlusion of the anterior vestibular artery. rent, or persistent depends somewhat on the
The examination should include routine etiology. Experience has shown that when
audiometrie and vestibular fundion tests. positional vertigo follows head injury or ear
Ordinarily it is not necessary to acquire surgery it tends to subside within a few
Bekesy audiometrie tracings, electronystag¬ weeks or months, but when due to ear infec¬
mographic recordings, or roentgen films of tion, degeneration of aging, or to vascular
the skull. The reasoning used in attempting occlusion it tends to be more persistent. The
to define an etiology, based on our present precise symptoms and course probably are
state of knowledge, is simply this: if there is determined in a large degree by the physical
no evidence of related head trauma, ear characteristics of the cupular deposit—for
infection, surgical procedure, or the syn¬ example, the mass, specific gravity, solubili¬
drome characteristic of occlusion of the an¬ ty, and whether it is free or fixed to the
terior vestibular artery, the etiology is as¬ cúpula. Thus, the prognosis regarding dura¬
sumed to be spontaneous degeneration of tion should be guarded.
the utricle. Ablating Vestibular Function.—In rare
Counselling the Patient.—Patients are of¬ cases the disability may be sufficiently se¬
ten alarmed by the attacks, fear the worst, vere to justify consideration of some form of
and are not relieved of their anxieties until ablation of the vestibular labyrinth. Al¬
a reasonable explanation is offered. The im¬
though theoretically it should be adequate
mediate, deliberate, and methodical execu¬ to ablate function of only the posterior semi¬
tion of the provocative test is usually circular canal, this has not yet become tech¬
effective in gaining their confidence. The nically feasible. The following pertinent case
question as to how much information re¬ histories demonstrate the consistent success
garding the etiology and mechanism is to be of labyrinth ablation in the management of
offered is an individual consideration with severe positional vertigo of the paroxysmal
each patient. Above all, the patient must be type. In each case total ablation was per¬
reassured that the disorder, regardless of formed in the ear which was undermost in
how disturbing it may be, is essentially be¬ the provocative position. All of these pa¬
nign. tients experienced severe sensorineural hear¬
The most rational and most simple man¬ ing loss in the involved ear so that total
agement of the disorder is avoidance of the inner ear ablation was permissible.
provocative position, and most patients ac¬ Case 3.—Reported by Citron and Hallpike
complish this with little restriction of nor¬ (1956)22.—A woman, aged 50, experienced a
mal everyday activities. The extent to which sudden, prolonged vestibular upset and severe
activities must be restricted is determined left-sided sensorineural-type hearing loss in
by the characteristics of the attack, such as 1949, followed by positional vertigo with the
suddenness of onset, severity of disequili¬ left ear undermost. Caloric responses were ab¬
sent on the left side. A left-sided labyrinth
brium, associated nausea and vomiting, and destruction was performed in 1953 by a surgical
frequency of occurrence. Climbing, swim¬ procedure through the horizontal canal. The
ming, and athletic activities such as skiing patient was completely relieved of positional
may be contraindicated. The operation of vertigo. (Follow-up time was not given.)
mechanical equipment such as driving an Case 4.—Reported by Citron and Hallpike
automobile or tending industrial machinery (1956)22.—A woman, aged 60, had a left-sided
may have to be curtailed. The extent of radical mastoidectomy for chronic otorrhea in
occupational handicap will be determined 1953 and afterward experienced positional ver¬
by the requirements for motor activity, in tigo in assuming the supine position with the
particular for head movement. Most pa¬ left ear undermost. Caloric responses were ac¬
tients will have adjusted to the disorder tive but there was severe sensorineural hearing

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 loss in the left ear. In 1955, a left labyrinth Comment
destruction was performed by subluxating the
stapes and injecting 95% alcohol solution into When usable hearing is present a tech¬
the vestibule. Positional vertigo could not be nique is required which will selectively
elicited following ablation. (Follow-up period ablate the vestibular labyrinth. Among the
was three months.)
methods which have been used with varying
Case 5.—Reported by Citron and Hallpike
(1962)23._A woman, at the age of 31, in 1937, degrees of success are the following: (1)
experienced a severe left-sided head injury and selective section of the vestibular nerve root;
was unconscious for three days. In 1956, at the
(2) ultrasound application; (3) cryosurgery;
age of 50, she developed positional vertigo.
and (4) streptomycin sulfate treatment.
When tested at the age of 51 she demonstrated
positional vertigo with the head in the supine Summary
position and the left ear undermost. A moder¬
ately severe left-sided sensorineural hearing Cupulolithiasis is a peripheral vestibular
loss was present and caloric responses were disorder characterized by positional vertigo
greatly reduced on the left side. The left eighth of the paroxysmal type. The diagnosis is
cranial nerve was sectioned with an approach
confirmed by the positional test procedure
through the posterior cranial fossa in January which provokes a vertiginous attack with
1961, following which positional vertigo could
not be elicited. (Follow-up period was not giv¬ characteristic nystagmus. The features of
en.) the attack which confirm the diagnosis of
Case 6.—Reported by Schuknecht (1962)4.— cupulolithiasis and differentiate it from posi¬
A woman at the age of 72, in 1952, experienced tional vertigo due to lesions of the central
sudden partial loss of hearing in the left ear. nervous system are (1) delay of onset; (2)
Five years later (1956) at the age of 77 she limited duration; (3) severe subjective sen¬
developed short, severe attacks of vertigo occur¬ sation; (4) rotatory nystagmus; and (5)
ring when the head was in certain positions, fatigability. Histological study of the tem¬
particularly in the supine position with the poral bones from two patients provide evi¬
head turned so that the right ear was under¬ dence that the disorder is caused by the
most. Two attacks were characterized by a deposition of a substance on the cúpula of
sudden sensation of falling backward and, on the posterior semicircular canal. It is pre¬
one occasion, she fell, hitting her head. She had sumed that this substance is of high specific
experienced a progressive loss of hearing in her gravity and renders this organ gravity-sensi¬
right ear for many years. tive and subject to stimulation with changes
Audiometrie tests revealed bilateral sensori¬ in head position. It seems probable that in
neural hearing loss, profound on the right and most cases the
deposit takes origin from the
moderate on the left. There was no sponta¬ otoconia of the utricle.
neous nystagmus. Caloric responses were equal
Etiologic factors ap¬
pear to be (1) spontaneous degeneration of
and normal—that is, 70-second responses to 5 the
utricular otolithic membrane; (2) laby¬
cc of water at 80 F, observing the nystagmus
through illuminated Frenzel glasses in a dark¬ ear
rinthine concussion; (3) otitis media; (4)
surgery; and (5) occlusion of the ante¬
ened room. Positional tests revealed severe nys¬
tagmus and vertigo in the right-ear-down posi¬ rior vestibular artery. It has been demon¬
tion, characterized by onset delay of five to ten
strated that the diseased ear is located un¬
seconds, with duration of six seconds and fatig- dermost in the provocative head position.
able on repeated testing. Nystagmus was of the This unique pathophysiological mechanism
combined counterclockwise rotatory and right for positional vertigo is supported by animal
horizontal type and was associated with a vio¬ experiments which show that (1) utricular
lent sensation of turning. degeneration releases otoconia into the en¬
The right labyrinth was destroyed by a surgi¬ dolymph; and (2) stimulation of the poste¬
cal procedure through the oval window. The rior semicircular canal creates a purely rota¬
postoperative vestibular upset was less severe tory nystagmus on forward gaze. For most
than anticipated. Postural tests on the fourth patients the most rational management is
postoperative day, and subsequently, failed to avoidance of the provocative position. When
elicit vertigo or nystagmus (Follow-up period the disorder seriously restricts the activities
was ten years.) of the patient, some form of labyrinth abla-

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 tion may be performed. Among the methods
in current use are total labyrinthectomy,
vestibular nerve section, ultrasound or cry-
References
1.B\l=a'\r\l=a'\ny,R: Diagnose von Krankheitsercheinun-
gen im Bereiche des Otolithenapparates, Acta Oto-
laryng (Stockholm) 2:434-437 (Oct 12) 1921.
2. Dix, R., and Hallpike, CS.: The Pathology,
Symptomatology and Diagnosis of Certain Common
Disorders of the Vestibular System, Ann Otol
61:987-1016, 1952.
3. Lindsay, J.R., and Hemenway, W.G.: Postural
Vertigo Due to Unilateral Sudden Partial Loss of
Vestibular Function, Ann Otol 65:692-708, 1956.
4. Schuknecht, H.F.: Positional Vertigo: Clinical
and Experimental Observations, Trans Amer Acad
Ophthal Otolaryng 66:319-331, 1962.
5. Barber, H.O.: Positional Nystagmus, Especial-
ly After Head Injury, Laryngoscope 73:891-944, 1964.
6. Cawthorne, T.E.: Positional Nystagmus, Ann
Otol 63:481-490 (June) 1954.
7. Gordon, N.: Post-traumatic Vertigo, With Spe-
cial Reference to Positional Nystagmus, Lancet
1:1216-1218, 1954.
8. Preber, L., and Silfverski\l=o"\ld,B.P.: Paroxysmal
Positional Vertigo Following Head Injury, Acta
Otolaryng (Stockholm) 48:255-265, 1957.
9. Cope, S., and Ryan, G.M.S.: Cervical and
Otolith Vertigo, J Laryng 73:113-120, 1959.
10. Barber, H.O.: Positional Nystagmus: Testing
and Interpretation, Ann Otol 73:838-850, 1964.
11. Schuknecht, H.F.: Mechanism of Inner Ear
Injuries From Blows to the Head, Ann Otolaryng
78:253-262 (April) 1969.
12. Vyslonzil, E.: \l=U"\bereine umschriebene An-
sammlung von Otokonien im hinteren h\l=a"\utigenBo-
gengang, Mschr Ohrenheilk 97:63-68, 1963.
13. Cawthorne, T.E., and Hallpike, C.S.: A Study
of the Clinical Features and Pathological Changes
Within the Temporal Bones, Brain Stem and Cere-
Downloaded From: http://archotol.jamanetwork.com/ by a University of Manitoba User on 06/08/2015
osurgical application to the vestibular laby¬
rinth, and vestibular ablation with strepto¬
mycin.
bellum of an Early Case of Positional Nystagmus of
the So-called Benign Paroxysmal Type, Acta Oto-
laryng (Stockholm) 48:89-105 (July-Dec) 1957.
14. Kimura, R.: Distribution, Structure and Func-
tion of Dark Cells in the Vestibular Labyrinth, Ann
Otol 78:542-562, 1969.
15. Szentagothai, J.: The Elementary Vestibulo-
Ocular Reflex Arc, J Neurolphysiol 13:395-407 (Nov)
1950.
16. Fluur, E.: Influences of Semicircular Ducts on
Extraocular Muscles, Acta Otolaryng (Stockholm),
suppl 149, pp 1-46, 1959.
17. Harbert, F.: Benign Paroxysmal Positional
Nystagmus, to be published.
18. Wittmaack, K.: \l=U"\berdie Ver\l=a"\nderungenim
Innen Ohr nach Rotationen, Verh Deutsch Otol Ges
18:150-156, 1909.
19. Hasegawa, T.: Die Ver\l=a"\nderungder labyrin-
th\l=a"\renReflexe bei zentrifugiertem Merrschweinchen,
Pflueger Arch Ges Physiol 229:205-225, 1931.
20. de Kleyn, A., and Versteegh, C.: Labyrinthre-
flex nach Abschleuderung der Otolithen Membrane
bei Meerschweinchen, Pflueger Arch Ges Physiol
232:454-465, 1933.
21. Parker, D.E.; Covell, W.P.; and von Gierke,
H.E.: Exploration of Vestibular Damage in Guinea
Pigs Following Mechanical Stimulation, Acta Oto-
laryng (Stockholm), suppl 239, pp 1-59, 1968.
22. Citron, L., and Hallpike, C.S. Observations
Upon the Mechanism of Positional Nystagmus of
the So-called "Benign Paroxysmal Type," J Laryng
Otol 76:253-259, 1956.
23. Citron, L., and Hallpike, C.S.: A Case of
Positional Nystagmus of the So-called Benign Par-
oxysmal Type and the Effects of Treatment by
Intracranial Division of the VIIIth Nerve, J Laryng
76:28-33, 1962.