Auris Nasus Larynx 36 (2009) 698701

www.elsevier.com/locate/anl

Positional and positioning down-beating nystagmus

without central nervous system findings
Yasuo Ogawa *, Mamoru Suzuki, Koji Otsuka, Shigetaka Shimizu,
Taro Inagaki, Mami Hayashi, Akira Hagiwara, Naoharu Kitajima
Department of Otorhinolaryngology, Tokyo Medical University, Japan
Received 7 June 2008; accepted 1 April 2009
Available online 26 April 2009

Abstract
We report the clinical features of 4 cases with positional or positioning down-beating nystagmus in a head-hanging or supine position without
any obvious central nervous system disorder. The 4 cases had some findings in common. There were no abnormal findings on neurological tests or
brain MRI. They did not have gaze nystagmus. Their nystagmus was observed only in a supine or head-hanging position and it was never observed
upon returning to a sitting position and never reversed. The nystagmus had no or little torsional component, had latency and tended to decrease
with time. The positional DBN (p-DBN) is known to be indicative of a central nervous system disorder. Recently there were some reports that
canalithiasis of the anterior semicircular canal (ASC) causes p-DBN and that patients who have p-DBN without obvious CNS dysfunction are
dealt with anterior semicircular canal (ASC) benign paroxysmal positional vertigo (BPPV). There are some doubts as to the validity of making a
diagnosis of ASC-BPPV in a case of p-DBN without CNS findings. It is hard to determine the cause of p-DBN in these cases.
# 2009 Elsevier Ireland Ltd. All rights reserved.
Keywords: BPPV; Down-beating; Nystagmus; ASC; Positional nystagmus; Positioning nystagmus

1. Introduction
Positional down-beat nystagmus (p-DBN) is a clinical sign
of central nervous system (CNS) involvement. It occurs with
lesions in the vestibulocerebellum, the craniocervical junction
and with drug intoxication [1]. Experimental extirpation of
the nodulus in the cat causes postural down-beat nystagmus
[2]. Occasionally, p-DBN is seen in patients without CNS
involvement [1]. The p-DBN with or without slight positional
vertigo in the head-hanging position, is a frequent, and often
the only clinical sign in neurological patients and the p-DBN
may spontaneously recover or persist. In these cases, a central
disorder is most commonly suspected, but examination of
these patients does not always disclose a CNS abnormality.
The cause of p-DBN is not clear yet.

* Corresponding author at: Nishishinjuku 6-7-1 Shinjuku-ku, Tokyo 1600023, Japan. Tel.: +81 3 3342 6111; fax: +81 3 3346 9275.
E-mail address: y-ogawa8@tokyo-med.ac.jp (Y. Ogawa).

Benign paroxysmal positional vertigo (BPPV) is the most

common ear-related cause of vertigo. BPPV is defined as
transient vertigo induced by a rapid change in head position,
associated with a characteristic paroxysmal positional
nystagmus. BPPV is usually caused by involvement of the
posterior semicircular canal (PSC). The horizontal semicircular canal (HSC) type BPPV has been recognized.
Whereas PSC and HSC BPPV are well-described entities,
anterior semicircular canal (ASC) BPPV is very rare. The
incidence of ASC BPPV has been described in between 1%
and 11% of all BPPV [3,4]. The ASC BPPV is characterized
by a predominant down-beating nystagmus with a small
torsional component [3,4].
Recently, there have been some reports that canalithiasis
of the ASC causes the p-DBN, and that patients who have pDBN without any obvious CNS dysfunction are considered
to have ASC BPPV [36]. We encountered 4 cases with
positional or positioning DBN without CNS abnormalities.
In this report, we describe the features of neuro-otological
findings including nystagmus, and their clinical course.

0385-8146/$ see front matter # 2009 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.anl.2009.04.001

Y. Ogawa et al. / Auris Nasus Larynx 36 (2009) 698701

2. Patients and methods

2.1. Patients
From November 2000 to May 2007, we treated 4 patients
(1 man and 3 women; ages ranging from 21 to 38 years) with
p-DBN. The patients were referred for a neuro-otological
work-up because of vertigo or dizziness. Examinations
included pure tone audiometry and basic neuro-otologic
examinations, such as cranial nerve examination, Romberg
test, eye tracking test (ETT), saccades, and optokinetic
nystagmus (OKN) test. Nystagmus tests including gaze,
positional and positioning tests were performed. Positional
and positioning nystagmus were recorded using an infrared
CCD camera. All the patients underwent MRI.
2.2. Case reports (Table 1)
Case 1 was a 35-year-old woman, who complained of 4
years of brief positional vertigo mainly on lying down. She
first presented at our clinic on November 7, 2000.
There was no gaze nystagmus or positional nystagmus.
The DBN was triggered by the positioning test and it was
observed when she assumed a head hanging posture. The
torsional component was very weak and it was hard to
discriminate its direction. The duration of the nystagmus
was about 20 s and the frequency was about 3 Hz. No
nystagmus was evoked with a posture change from a head
hanging to a sitting position. She showed no hearing loss in
either ears or other neuro-otological dysfunction. Vestibular
evoked myogenic potentials (VEMP) were normal bilaterally. The brain MRI was normal. The nystagmus remained
for 8 months but became hardly noticeable afterwards.
Case 2 was a 34-year-old man. He complained of brief
positional vertigo. He had experienced severe vertigo,
nausea and vomiting twice during the last 1 year. Positional
vertigo developed 2 weeks before evaluation. He had his first
check up at our hospital on December 5, 2003. He had no
difficulty in hearing, headache or other neurological
abnormalities. Pure tone audiogram, neurological and eye
movement examinations including ETT and OKP were
normal. Brain MRI was normal. Positional test revealed pDBN in the head-hanging position and in the left ear down
position. The positioning test also evoked brief and short
DBN upon straight head-hanging position. The frequency of
the p-DBN was about 2 Hz. No nystagmus was evoked by

699

changing from the head-hanging to the sitting position. At

re-examination 2 weeks later, the p-DBN in the headhanging position became weak. His nystagmus lasted for 2
weeks, but faded away afterwards.
Case 3 was a 21-year-old woman. Her chief complaint
was dizziness and positional vertigo. She was aware of
dizziness with nausea and vomiting for the first time when
she woke up on January 9, 2005, but thereafter it was
repetitive. She visited our hospital on January 18, 2005. She
showed no hearing loss in either ear and no other neurootological dysfunction. The positional test revealed DBN in
the supine position, head-hanging position and left ear down
and right ear down positions. The frequency of p-DBN was
about 1 Hz. The positioning test evoked brisk and short pDBN upon straight head hanging position. No nystagmus
was observed by changing from head-hanging to sitting
position. There were very weak horizontal and torsional
components. She was hospitalized, because of her intense
dizzy sensation. At first a CNS disorder was suspected, but
MRI revealed no abnormal findings. Eye movement
examinations including ETT and OKP were normal. Caloric
test with cold water at 10 8C was normal. Her dizziness
gradually reduced and she was discharged on January 30, but
p-DBN persisted until October, 2005.
Case 4 was a 38-year-old woman. Her chief complaint
was dizziness and positional vertigo. She was first aware of
vertigo when she turned her head down on May 5, 2007. She
had a history of migraine. She visited our hospital on May
16, 2007. p-DBN was observed in the supine position and
bilateral ear down position and the p-DBN contained
torsional components. p-DBN was not observed when
changing from a head-hanging to a sitting position. Pure
tone audiogram, neurological and eye movement tests
including ETT and OKP were normal. A CNS disorder was
suspected, but MRI was normal. The electronystamograph
showed p-DBN in the head-hanging position with a
horizontal component. Nystagmus was not observed in
the sitting position (Fig. 1). The nystagmus persisted for 3
months.

3. Discussion
DBN in primary gaze is a sign of central nervous system
dysfunction [5]. p-DBN has also been reported as a sign of
CNS dysfunction [1]. Brandt [1] reported p-DBN in the

Table 1
Clinical summary of patients with positional or positioning down beating nyastagmus and no CNS findings.
Patients number

1
2
3
4

Sex

f
m
f
f

Age

35
34
21
39

DBN

Symptom

Positioning test

Positioning test
(sitting position)

No
Supine, head left
Supine, head left, head right, head-hanging
Supine, head left, head right, head-hanging

Head-hanging
Head-hanging
Head-hanging
Head-hanging

Vomiting
No
No
No
No

Time course
Headache

+
+
+

8
2
9
3

months
weeks
months
months

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Y. Ogawa et al. / Auris Nasus Larynx 36 (2009) 698701

Fig. 1. Electronystamograph of p-DBN in case 4, during the positioning nystagmus test. p-DBN was induced by the head-hanging maneuver and was accompanied
with leftward horizontal component. The evoked p-DBN gradually reduced. The vertical nystagmus was not observed upon returning to a sitting position.

head-hanging position is indicative of a cerebellar nodulus

lesion. Experimental extirpation of the nodulus in the cat
caused postural DBN [2]. This has also been confirmed by
clinical observation [7]. In our 4 cases with p-DBN, there
were no abnormal CNS findings, but there were several
common findings. The patients were all relatively young and
complained of brief positional vertigo, mainly on lying
down. Some of these nystagmus was called positioning
rather than positional nystagmus. There were no abnormal
findings in neurological tests and brain MRI. They did not
have any gaze nystagmus. The nystagmus was observed only
in the supine position and was never observed in sitting
position. The nystagmus had little or no torsional
component. The nystagmus had latency and was fatigable.
Their nystagmus faded away in 2 weeks to 10 months.
Recently, there are some reports that canalithiasis of the
ASC causes p-DBN [3,5].
Bertholon et al. [5] reviewed 50 patients with p-DBN and
75% had clear evidence of CNS dysfunction, but in 25%

there were no such abnormalities. They reported that these

patients of p-DBN without CNS possibly have refractory
ASC canalithiasis [5]. The ASC BPPV is very rare
comprising only 1% and 11% of all BPPV. The ASC
neuron projects to the ipsilateral superior rectus muscle and
to the contralateral inferior oblique muscle. The induced
nystagmus is down beating and torsional during the DixHallpike (DH) maneuver. The torsional component is
smaller for the ASC than PSC because PSC is at 568 from
the sagittal plane whereas the anterior canals are at 418.
Thus, more vertical component is expected from the ASC
BPPV and more torsional component from PSC BPPV [5].

4. Questionable points with diagnosis of ASC BPPV

Herdman and Tusa [8] summarized the features of the
nystagmus with regard to various canal involvements. The
head-hanging procedure produces a down-beating and

Fig. 2. Diagram of the representation of the canalithiasis of the ASC during the positioning nystagmus test. (A) The location of otoliths in the ASC lumen in a
sitting position. (B) The patient is in a head-hanging position. The otoliths move in ampullofugal direction in the lumen of ASC by gravitational force, thus
producing the excitatory ampullofugal stimulation of the crista. (C) When the patient returns to a sitting position, the otoliths go out to the utriculus. Arrows
(solid line) indicate the head movement. A dotted line arrows indicate the direction of the otolith displacement.

Y. Ogawa et al. / Auris Nasus Larynx 36 (2009) 698701

torsional nystagmus and the DH reversal procedure produces

an up-beating nystagmus. The nystagmus of the posterior
type and horizontal type BPPV are characterized by a
direction changing positional or positioning nystagmus. If
the cause of p-DBN is ASC-BPPV, up-beating nystagmus
should occur by positioning change from a head hanging to a
sitting position.
Recent reports concerning ASC-BPPV referred to pDBN in a head-hanging position, but did not take up the
question of up-beating nystagmus by positioning change
from a head hanging position to a sitting position [3,5,6].
Furthermore, even if ASC-BPPV occurred, the particles
most likely drop into the utriculus spontaneously by
positional changing (Fig. 2). Any pathology of BPPV, such
as canalithiasis or cupulolithiasis are also not likely, since
the nystagmus never reversed. There are some reservations
about making a diagnosis of ASC-BPPV in a patient of pDBN without CNS findings.

5. Possible etiology
It is hard to determine the cause of p-DBN in these cases.
P-DBN may be caused by multiple sclerosis, ischemia,
intoxication, crania-cervical malformation or cerebella
degeneration. However, there was no identifiable etiology
in our 4 cases. Vascular insufficiency is not likely, since all 4
subjects were relatively young. The duration of their
nystagmus varies from 2 weeks to 10 months, and it might
be due to the diversity of the etiology. We speculate that the
possible cause of p-DBN in our 4 cases was a variant of

701

ASC-BPPV or otolithic dysfunction. A localized lesion is

probable, but the pathology may be elucidated by further
accumulation of case documentation.

Acknowledgement
The authors are indebted to Prof. J. Patrick Barron of the
International Medical Communications Center of Tokyo
Medical University for his review of this manuscript.

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