Acta Oto-Laryngologica, 2007; 127: 547
549

CASE REPORT

3D-FLAIR MRI findings in a patient with Ramsay Hunt syndrome

MAKOTO SUGIURA1, SHINJI NAGANAWA2, SEIICHI NAKATA1, SAWAKO KOJIMA1 &

TSUTOMU NAKASHIMA1
1
Department of Otorhinolaryngology and 2Department of Radiology, Nagoya University Graduate School of Medicine,
Nagoya, Japan

Abstract
Three-dimensional, fluid-attenuated inversion recovery (3D-FLAIR) of magnetic resonance imaging (MRI) has recently
been developed to detect hemorrhage or high concentrations of protein. We present a patient with Ramsay Hunt syndrome,
in whom high signals in the cochlear and vestibular apparatus were identified with 3D-FLAIR. The high signal areas in
3D-FLAIR were not detected by T1- and T2-weighted MRI in this case. This is the first report of high concentrations of
protein in the inner ear in Ramsay Hunt syndrome using 3D-FLAIR, and suggests that high concentrations of protein in the
inner ear are associated with hearing deterioration in some patients with Ramsay Hunt syndrome. 3D-FLAIR could be a
useful diagnostic tool in the early stages of Ramsay Hunt syndrome.

Keywords: Ramsay Hunt syndrome, fluid-attenuated inversion recovery (FLAIR), magnetic resonance imaging (MRI)

Introduction of the inner ear in Ramsay Hunt syndrome has been

The fluid-attenuated inversion recovery (FLAIR)
sequence is part of the routine protocol for brain
magnetic resonance imaging (MRI) [1]. Many
studies have noted subtle findings on FLAIR images.
Subtle high-signal areas in the cerebrospinal fluid
(CSF) can be a sign of subarachnoid hemorrhage,
meningitis, or acute infarction. The FLAIR se-
quence sometimes indicates hemorrhage or a high
concentration of protein, which is difficult to detect
by T1- or T2-weighted MRI. The two-dimensional
(2D)-FLAIR sequence shows flow-related artifacts
caused by the inflow of CSF from outside the slice
volume, which sometimes obscures the pathology.
We have reported previously that CSF-related flow
artifacts are significantly lower in the three-dimen-
sional (3D)-FLAIR images than in 2D-FLAIR
images [1]. The 3D-FLAIR sequence allows the
detection in serial thin slices of conditions that
mimic other pathologies of the inner ear, such as
inner ear hemorrhage and high concentrations of
protein. However, to our knowledge, no evaluation
described.
In this report, we describe a patient with Ramsay
Hunt syndrome in whom high signals in the coch-
lear and vestibular apparatus were identified with
3D-FLAIR.
Case report
A 54-year-old woman experienced left otalgia, vesi-
cular eruption of the vertex, eruption of the external
acoustic meatus, and intermittent rotatory vertigo
lasting for about 5 min. There were no other
symptoms or history of ear disease. Her medical
history was unremarkable. Five days later, she
developed left facial weakness. Examination revealed
facial palsy affecting the entire left side of her face of
severity 5 on the House and Brackmann grading
scale. Her tympanic membrane appeared normal
and mobile. Pure-tone audiometry showed sensor-
ineural hearing loss of 32 dB (the average of values at
500, 1000, and 2000 Hz) in the left ear. Her speech
discrimination scores were 100% at 50 dB in the

Correspondence: Makoto Sugiura, MD, PhD, Department of Otorhinolaryngology, Nagoya University Graduate School of Medicine 65, Tsurumai-cho,
Showa-ku, Nagoya 466-8550, Japan. Tel: /81 52 744 2323. Fax: /81 52 744 2325. E-mail: makotos@med.nagoya-u.ac.jp

(Received 24 March 2006; accepted 27 April 2006)

ISSN 0001-6489 print/ISSN 1651-2551 online # 2007 Taylor & Francis
DOI: 10.1080/00016480600801399
548 M. Sugiura et al.
Figure 1. Axial MRI. 3D-FLAIR before enhanced MRI at the
time of acute illness. Bright signals are visible in the left cochlea
(short arrow) and vestibule (long arrow). The high-signal areas on
3D-FLAIR were not enhanced.
right ear and 95% at 60 dB in the left ear. Distortion
product
otoacoustic emissions revealed weak re-
sponses at 1000 and 2000 Hz, and absence at
4000 and 6000 Hz in the left ear. No caloric
response was observed, even with ice-water irriga-
tion of the left ear after the eruption of the external
acoustic meatus had almost recovered (day 9). MRI
3D-FLAIR images before gadolinium enhancement
4 days after the onset of facial palsy revealed high
signals in the left cochlea and vestibule (Fig. 1). An
enhanced MRI showed enhancement at the genicu-
late ganglion. These high-intensity areas on the
3D-FLAIR images were not enhanced on a con-
trast-enhanced T1-weighted image. We could not
identify the area of increased signal intensity ob-
served on the 3D-FLAIR images on pre- or post-
contrast T1-weighted images. These MRI findings
imply that the hearing deterioration was associated
with high concentrations of protein in the cochlea
and vestibule.
The patient was considered to have high concen-
trations of protein in the left cochlea and vestibule,
associated with a varicella-zoster virus (VZV) infec-
tion. She was treated with valaciclovir (1000 mg
three times per day) from the first day of facial palsy.
She underwent additional treatment with steroids
4 days after the onset of facial palsy. Two weeks later,
her hearing loss had resolved completely. Six weeks
later, her facial weakness had resolved completely.
VZV IgG was 86.0 U/ml on day 5 and 60.0 U/ml on
day 19, but VZV IgM was not high on either day 5 or
day 19. A follow-up MRI 3D-FLAIR 2 months after
her acute illness revealed a reduction in the high-
signal areas in the left cochlea and vestibule. An MRI
examination at the same time showed residual
enhancement of the facial nerves.
Discussion
This is the first report of high signals in the affected
ear of a patient with Ramsay Hunt syndrome
examined with 3D-FLAIR. High signals on 3D-
FLAIR may reflect minor hemorrhage (because
these signals were not detected by T1- or T2-
weighted MRI) or an increased concentration of
protein in the inner ear. Our FLAIR findings suggest
that high concentrations of protein in the inner ear
are associated with hearing deterioration in some
patients with Ramsay Hunt syndrome.
Since the report by Daniels et al. in 1989 [2],
many investigators have reported the enhancement
on MRI of paralyzed facial nerves after the injection
of gadolinium (Gd)-diethylenetriaminepentaacetic
acid (DTPA). Jonsson et al. [3] reported that
ipsilateral facial nerve contrast enhancement is
frequently observed in cases of acute peripheral
facial palsy, predominantly in the meatal fundus. It
has been reported that no difference in Gd en-
hancement of the facial nerves is observed between
patients with Bell’s palsy and those with Ramsay
Hunt syndrome [3]. However, in some cases of
Ramsay Hunt syndrome, the vestibular and co-
chlear nerves, the labyrinth, and the sheets of the
internal and external auditory canal are enhanced
[3]. In this case, enhanced MRI showed enhance-
ment at the geniculate ganglion on the affected
side.
This case is unusual because 3D-FLAIR before
gadolinium enhancement showed diffuse, increased
signals throughout the vestibule and cochlea, which
are consistent with a hemorrhagic process or high
concentrations of protein. These 3D-FLAIR find-
ings may imply that the patient’s hearing deteriora-
tion was associated with high concentrations of
protein in the cochlea and vestibule. We could not
identify the area of increased signal intensity ob-
served on the 3D-FLAIR images on a pre-contrast
T1-weighted image. Therefore, 3D-FLAIR could be
a useful diagnostic tool in the early stages of Ramsay
Hunt syndrome.
Reactivation of VZV is a known cause of Ramsay
Hunt syndrome. VZV can infect a wide variety of cell
types in the central and peripheral nervous system,
including neurons, oligodendrocytes, meningeal
cells, ependymal cells, and cells of the blood vessel
wall. This wide range of susceptible cells explains the
diversity of the clinical and pathological nervous
system manifestations of VZV [4]. Based on these
results, it is possible that inner ear hemorrhage fol-
lows a collapse of an artery and/or vein in the inner
ear, caused by the reactivation of VZV. Further
studies are required to clarify the relationship
between hearing deterioration and increased signals
throughout the vestibule and cochlea on 3D-FLAIR
images in Ramsay Hunt syndrome. We believe that
our study will further our understanding of the
pathophysiology of hearing deterioration in patients
with Ramsay Hunt syndrome.

Acknowledgement
This study was supported by research grants from
the Ministry of Health, Labour and Welfare and
from the Ministry of Education, Culture, Sports,
Science and Technology of Japan.
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