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Benign Paroxysmal Positional Vertigo


Michael von Brevern,
^ Department for Neurology, Park-Klinik Weissensee, Berlin, Germany
^Vestibular Research Croup, Berlin, Germany

Address for correspondence Michael von Brevern, MD, Department of


Neurology, Park-Klinik Weissensee, Schnstrasse 80, 13085 Berlin,
Germany (e-mail: von.brevern@park-klinik.com).

Semin Neurol 2013:33:204-211.

Abstract

Keywords
- positional vertigo
- paroxysmal vertigo
- dizziness
- canalolithiasis
- cupulolithiasis

Benign paroxysmal positional vertigo (BPPV) is the most common and the most
effectively treated vestibular disorder. The prevailing pathomechanism is canalolithiasis, which is otoconia falling in one of the semicircular canals where they move in
response to changes of the head position, triggering excitation of the vestibular
receptors of the affected canal. In the majority of patients with BPPV, the posterior
canal is affected by canalolithiasis and there are two highly effective therapeutic
maneuvers for treatment. About 20% of patients present with lithiasis of the horizontal
or anterior canal. The author focuses on recent advances in diagnosis and treatment of
the more rare variants of BPPV.

Benign paroxysmal positional vertigo (BPPV) is characterized


by short attacks of vertigo that are provoked by certain head
movements such as turning over in bed, lying down, or tilting
the head backward when looking up. Typically, the first attack
of BPPV occurs in the morning in bed. A single attack usually
lasts 10 to 20 seconds and no longer than 1 minute. Beside
attacks of positional vertigo, patients may have prolonged
dizziness and mild imbalance. The time course of BPPV is
characterized by spontaneous remissions that occur typically
after days to weeks' and recurrences that occur in ^-50% of
patients.^

Epidemiology
Benign paroxysmal positional vertigo is the most common
cause for vertigo, particularly in the elderly. A populationbased survey in Germany found a lifetime prevalence of
3.2% in women and 1.6% in men and a cumulative incidence
reaching almost 10% at the age of 80 years.^ The point
prevalence of BPPV in unselected geriatric populations has
been found to be around 10%.'''^ In childhood, the disorder
is exceedingly rare. In dizziness clinics, approximately one
out of five patients presents with BPPV.
In idiopathic BPPV, women outnumber men by ~2:1. This
preponderance of female sex is less evident in BPPV after head
trauma and peripheral vestibular disorder.^

Issue Theme Neuro-Otology 2013:


Guest Editor, Terry D. Fife, MD

Pathophysiology
Benign paroxysmal positional vertigo is caused by otoliths
that are dislodged from the utricular macula and fall into a
semicircular canal. These particles are composed of calcium
carbonate and have a density higher than endolymph. In the
most common condition (canalolithiasis), the otoconia are
mobile within the canal and changes of the head position in
the plane of the affected semicircular canal causes them to
move to the most dependent point of the canal. The resulting
inappropriate endolymph flow activates hair cell receptors,
provoking attacks of positional vertigo and nystagmus.^ More
rarely, otoconia are attached to the cpula of a semicircular
canal and render it sensitive to gravity (cupulolithiasis).^ The
hypothesis of canalolithiasis and cupulolithiasis is supported
by an animal model.^ The most convincing proof for canalolithiasis is provided by the efficacy of positioning maneuvers, which aim to clear the affected canal from mobile
particles.

Diagnosis
Most patients present with the typical history of short attacks
of vertigo precipitated by turning in bed, lying down from
sitting, sitting up from lying, and extending the neck to look
up. Patients are usually aware that certain head movements
can provoke attacks of vertigo and often develop strategies to

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DOI http://dx.doi.org/
10.1055/S-0033-1354590.
ISSN 0271-8235.

Benign Paroxysmal Positional Vertigo

avoid these movements. A single attack usually lasts a few to


20 seconds and not longer than 1 minute. However, after a
flurry of attacks, patients may complain of prolonged nonspecific dizziness and imbalance lasting from hours to days.
Occasionally, patients with BPPV do not volunteer the typical
history. Diagnostic positioning is thus worthwhile in all
patients presenting with vertigo, dizziness, and unsteadiness,
even in the absence of a history of positional vertigo.
The confirmation of the diagnosis requires provocation
of vertigo by positioning testing and observation of positional nystagmus. The principle of diagnostic positioning is
to bring the head first into such a position that the affected
semicircular canal is spatially vertical and aligned with
gravity. In a second step, the head is then rotated in the
plane of this canal. As a rule-and in contrast to central
positional nystagmus-positional nystagmus in BPPV always beats in the plane of the affected canal and in the
expected direction for canal stimulation or inhibition.^ For
observation of positional nystagmus Frenzel goggles can be
helpful, particularly when the nystagmus is weak. In most
cases, however, nystagmus can be seen without special
equipment. Further vestibular and auditory testing is indicated only when a pre-existing disorder of the inner ear (e.
g., vestibular neuritis, Mnire's disease) is suspected.
Cerebral imaging with magnetic resonance imaging (MRI)
should be performed when symptoms or signs of concurrent brainstem or cerebellar dysfunction are present, or
when positional nystagmus presents with atypical features
or fails to resolve with repeated therapeutic positional
maneuvers.^ ^

von Brevern

Variants of Benign Paroxysmal Positional


Vertigo
Theoretically, each of the three semicircular canals of the
labyrinth can be affected by canalolithiasis or cupulolithiasis,
resulting in six variants. For anatomical reasons the posterior
canal is by far the most frequently affected canal, accounting
for 80 to 90% of cases. In 10 to 20% of patients, the horizontal
canal is involved. Benign paroxysmal positional vertigo of the
anterior canal is rare, accounting for only 1 to 2% of patients in
large case series,^^"^^ although some recent reports have
yielded a considerably higher incidence.^^'^^ Lithiasis of
multiple canals is not rare and occurs in up to 20% of patients
with BPPV, usually involving the posterior and the horizontal
canal of one labyrinth."'^^ Benign paroxysmal positional
vertigo can also affect both labyrinths simultaneously. Patients with head trauma are particularly vulnerable to develop bilateral and polycanalicular BPPV. 18
Benign Paroxysmal Positional Vertigo of the Posterior
Canal
The classic test to diagnose BPPV of the posterior semicircular
canal is the Dix-Hallpike maneuver (-Fig. 1). Alternatively,
the side-lying maneuver (Semont diagnostic maneuver) can
be performed; The sitting patient is tilted quickly to the side
to be tested with the head turned 45 degrees to the opposite
side. The positioning maneuver evokes positional vertigo and
nystagmus after a latency of one to a few seconds, beating in a
torsional and upward direction and lasting usually less than
30 seconds (-Table 1).

Fig. 1 (A,B) Dix-Hallpike maneuver for provocation of benign paroxysmal positional vertigo of the left posterior canal. Below, a model of the
undermost posterior semicircular canal is shown. When the patient lies down, the particles move away from the cpula within the canal. The
resulting endolymph flow causes an unphysiologic activation of the posterior canal and leads to paroxysmal vertigo and torsional-vertical
nystagmus. (UT, utricle; CU, cpula; OT, otoconial debris) (From von Brevern M, tempert T. Benign paroxysmal positional vertigo. Nervenarzt
2004;75:1027-1036. With permission of Springer + Business Media).
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Table 1 Characteristics of positional nystagmus in benign paroxysmal positional vertigo


Affected semicircular canal (diagnostic
maneuver)

Direction of nystagmus

Posterior canal (Dix-Hallpike maneuver)

Torsional with upper pole of the eye beating to the lower


ear + vertical upbeating to the forehead

Horizontal canal (Supine roll test)

Canalolithiasis:
Horizontal, geotropic, and transient in both lateral supine
positions
Cupulolithiasis:
Horizontal, apogeotropic, and persistent in both lateral
supine positions

Anterior canal (straight head-hanging position


or Dix-Hallpike maneuver)

Predominantly downbeating + minor torsional component


beating with the upper pole of the eye toward the affected
ear (may be missing)

Rarely, torsional-vertical positional nystagmus as described above, but lasting longer than 1 minute may be
observed in the Dix-Hallpike maneuver. In the absence of
other neurologic signs, this finding may indicate cupulolithiasis of the posterior canal. Typically, the intensity of
nystagmus is strongest with the head resting slightly raised
from supine ("half Dix-Hallpike maneuver"), thus with the
affected cpula in an earth-horizontal position to be maximally deflected by the gravitational
Benign Paroxysmal Positional Vertigo of the
Horizontal Canal

In BPPV of the horizontal canal, vertigo is typically provoked


by turning the head to either side in the supine position. Two
variants have been described, namely, the more common
canalolithiasis and the rarer cupulolithiasis of the horizontal
canal. For testing of the horizontal canal, the head of the
patient in the supine position is elevated ~30 degrees and
then turned quickly to either side.
With canalolithiasis of the horizontal canal, the supine
head roll test provokes transient horizontal nystagmus beating toward the ground (geotropic), regardless of whether the
head is turned to the right or left.^ Typically, the nystagmus
starts with no or minimal latency, beats horizontally with
respect to the head, lasts up to 1 minute, changes direction
depending on the direction of head turn, and shows no or
minimal fatigue with repetitive provocative maneuvers

(Table 1). For treatment, it is important to identify the


side of the affected ear. The response is stronger with the head
turned to the side of the affected horizontal canal. The net
angle and acceleration of the head rotation should be similar
for head turns to the right and left to allow for comparison of
nystagmus intensity. In addition, the change from sitting to
supine may provoke a transient horizontal nystagmus to the
healthy side (-Table 2).^^
Patients with cupulolithiasis of the horizontal canal have
positional vertigo and nystagmus that persist as long as the
precipitating head position is held ( -Table 1 ).^ A head turn to
either side in the supine position provokes persistent horizontal nystagmus beating away from the ground (apogeotropic). The nystagmus is more intense with the head turned
to the healthy side (-Table 2). When the patient lies in the
supine face-up position, a weak persistent nystagmus is
usually present that beats to the affected side and subsides
when the head is turned slightly to that side.^^ Apogeotropic
positional nystagmus is not synonymous with cupulolithiasis,
hut may also occur transiently with canalolithiasis of the
horizontal canal when otoconia are located in the anterior
part of the horizontal canal. In this case, conversion from
apogeotropic to geotropic positional nystagmus can be observed during repetitive performance of the supine head roll
test,^^"^^ whereas persistence of an apogeotropic positional
nystagmus is supportive of the diagnosis of cupulolithiasis of
the horizontal canal.

Table 2 Identification of the affected side in benign paroxysmal positional vertigo of the horizontal canal
Maneuver

Canalolithiasis

Cupulolithiasis

Supine head roll test

intensity of nystagmus stronger with


head turned to affected side

Intensity of nystagmus stronger with


head turned to healthy side

Lying backward from the


sitting position

Horizontal nystagmus beating to the


healthy ear

Horizontal nystagmus beating to the


affected ear

Bending the head forward in


the upright position

Horizontal nystagmus beating to the affected ear

Horizontal nystagmus beating to the


healthy ear

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Benign Paroxysmal Positional Vertigo


Benign Paroxysmal Positional Vertigo of the Anterior
Canal

The rarity of BPPV of the anterior canal is probably related to


the anatomical orientation of this canal, which is superior to
the utricle, making it unlikely that particles enter the anterior
canal. Benign paroxysmal positional vertigo of the anterior
canal can be provoked in the supine position with the head
hanging straight below the earth-horizontal and by DixHallpike positioning, which, no matter to which side the
head is turned, stimulates particle migration within the
affected anterior canal.^^'^^ In the Dix-Hallpike position,
nystagmus may be stronger or exclusively present with the
affected ear up^^ or down.^^'^^ The most sensitive diagnostic
test for BPPV of the anterior canal seems to be the straight
head-hanging position.^^^ Theoretically, the symptomatic
side can be recognized from the nystagmus direction as it is
downbeating with a small torsional component always pointing to the affected ear, irrespective of the side of the DixHallpike maneuver (-Table 1). However, the torsional component can be easily missed when three-dimensional (3D)

VOT Brevern

eye movement recording is not available, rendering identification of the affected side in clinical practice unreliable.^ ^^^^"^^ The positional downbeating nystagmus in
BPPV of the anterior canal may show no latency and no
crescendo-decrescendo time course, and rarely reverses
direction when sitting up from the provoking position.^^'^^
It is important to bear in mind that positional downbeat
nystagmus is most commonly due to central vestibular dysfunction, presenting usually with additional neurologic
signs.^^ Hence, the diagnosis of BPPV of the anterior canal
requires a meticulous neurologic examination. In addition,
brain MRI should be performed in those cases refractory to
treatment.

Treatment
Treatment of canalolithiasis aims to induce the displacement
of the otolithic debris through the open end of the affected
semicircular canal into the utricular cavity where they no
longer produce positional vertigo. The therapeutic aim in

V.

Fig. 2 (A-E) Epiey maneuver for treatment of benign paroxysmal positional vertigo of the left posterior canal, consisting of a set of five successive
head positions that are hand-guided by the therapist. The head rotations are performed rapidly and the time interval between each step is
30 seconds or until nystagmus subsides. The vertex of the head is kept tilted downwards throughout the rotations in the supine position. A
positional nystagmus appearing in the second and third head position and beating in the same direction with respect to the head indicates
successive movement of the particles toward the utricular cavity and predicts a favorable outcome. (From von Brevern M, Lempert T. Benign
paroxysmal positional vertigo. Nervenarzt 2004;75;1027-1036. With permission of Springer + Business Media).
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cupulolithiasis is detachment of otoconia from the cpula and


their subsequent removal from the canal. Patients with
persistent unsteadiness after resolution of positional nystagmus may benefit from vestibular rehabilitation.^^
Benign Paroxysmal Positional Vertigo of the Posterior
Canal
There are two highly effective conservative therapies for the
common BPPV of the posterior canal. The Epley maneuver
(also called canalith-repositioning procedure) consists of a
series of successive head positions each of ~90-degree displacement ("-Fig. 2). The Semont maneuver involves a rapid
180-degree swing of the head in the plane of the posterior
semicircular canal (-Fig. 3). Both maneuvers are similarly
effective with immediate resolution of symptoms in ~80% of
patients."'^^"^^ The maneuvers should be repeated at the
same session when positional vertigo and nystagmus are still
present, as this increases the success rate. When the Epley
maneuver fails, the Semont maneuver can be performed as an
alternative, or vice versa. Vibration of the mastoid during the
Epley maneuver has been recommended by some authors,
but it does not improve treatment outcome.^^
There is some evidence that therapeutic maneuvers can
be even useful in patients who despite a typical history,
complain of vertigo, but do not show nystagmus on DixHallpike positioning.^^'^^ The reason may be a limited
amount of otoconia in the affected posterior canal that is

sufficient to evoke vertigo, but not to stimulate vestibuloocular pathways.


For patients who do not respond immediately to the Epley
or Semont maneuver, as well as for those with frequent
recurrences, a modified Epley maneuver can be used for
self-treatment at home. This procedure has proved more
effective than Brandt-Daroff exercises,^^ but is less effective
than hand-guided treatment.
Benign Paroxysmal Positional Vertigo of the
Horizontal Canal
Canalolithiasis of the horizontal canal can be treated with the
head-roll maneuver (-Fig. 4). With this maneuver, response
rates varying widely from 50% to almost 100% have been
reported."'^^ Alternatively, the patient can be advised to
sleep with the head turned to the healthy side for one night
(forced prolonged positioning), as this provides relief from
positional vertigo in ~70% of patients."'^^ Compared with
both aforementioned maneuvers, two randomized trials have
shown that the Gufoni maneuver (Fig. 5) is similarly efficient with response rates as evaluated 1 hour after two
maneuvers of 61%"* and 89%."^^ Up to now, there is no
convincing evidence that one of these three maneuvers is
more effective than the others.
Cupulolithiasis of the horizontal canal is more refractory to
treatment than the canalolithiasis variant. A head-shaking
maneuver aims to remove otoconia from the cpula. Shaking

Fig. 3 Semont maneuver for treatment of benign paroxysmal positional vertigo of the left posterior canal. The maneuver is hand-guided by a
therapist who is not depicted for the sake of clarity. All movements are performed rapidly. The head is kept turned 45 degrees away from the
affected ear throughout the maneuver. (From von Brevern M. Lempert T. Benign paroxysmal positional vertigo. Nervenarzt 2004:75:1027-1036.
With permission of Springer + Business Media).
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Benign Paroxysmal Positional Vertigo

von Brevern

Fig. 4 (A-C) Roll maneuver for treatment of canaloiithiasis of the left horizontal canal. The supine patient is rotated 270 degrees in rapid steps of
90 degrees in the plane of the horizontal semicircular canal toward the healthy side. The time interval between each step is 30 seconds or until
nystagmus has subsided. (From von Brevern M, Lempert T. Benign paroxysmal positional vertigo. Nervenarzt 2004:75:1027-1036. With
permission of Springer + Business Media).

the head for 15 seconds at ~3 Hz with the head pitched


forward ~30 degrees resolved vertigo and nystagmus immediately in 37 to 47% of patients.^^'''^ A variant of the Gufoni
maneuver is similarly effective''^: The patient is quickly
brought from the sitting position into a side-lying position
on the affected ear. After 1 minute, the head is quickly turned
45 degrees upward. After another minute, the patient returns
to sitting. During both maneuvers, transition from geotropic
to apogeotropic positional nystagmus often occurs, indicating
transition from cupulolithiasis to canalolithiasis and requiring additional therapeutic maneuvers for the latter

Benign Paroxysmal Positional Vertigo of the Anterior


Canal
Treatment of this rare variant has not been thoroughly
evaluated. Probably, the simplest maneuver for treatment
of BPPV of the anterior canal is to move the patient from a
sitting to a lying position with the head straight and bent
backward as far as possible. In this position, the ampullary
segment of the anterior canal is turned upside-down and
particles can migrate out of the canal. After 1 minute, the

patient's head is quickly moved forward "chin to chest."


Finally, the patient sits up again.^^'^" This maneuver has
the advantage that identification of the affected canal, being
difficult in BPPV of the anterior canal, is not required. Those
rare patients that do not respond to positioning therapy can
be treated with plugging of the affected anterior canal.^^

Prognosis
Most recurrences occur in the first year after treatment with a
recurrence rate in canalolithiasis of the posterior canal of
15%peryear^''^
Patients often ask for strategies for the prevention of BPPV.
Postural restrictions for one to several days after successful
treatment of BPPV of the posterior canal (including not lying
on the affected ear, sleeping upright, and wearing a cervical
collar) slightly reduce the recurrence rate.^^ However, these
restrictions are unpleasant for patients and not recommended by many experts. A daily routine of Brandt-Daroff
exercises or self-application of the modified Epley maneuver
by the patient does not avoid recurrence of BPPV of the
posterior
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von Brevern

Fig. 5 Gufoni maneuver for treatment of canalolithiasis of the left


horizontal canal. The patient is brought quickly from a sitting position
with the head straight ahead into a side-lying position on the unaffected side and maintained there for 1 minute. Then the head of the
patient is quickly turned 45 degrees downwards. After 1 minute, the
patient returns to sitting.

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