Otology & Neurotology
39:e39–e44 ß 2017, Otology & Neurotology, Inc.
High Resolution Three-Dimensional Delayed Contrast MRI
Detects Endolymphatic Hydrops in Patients With
Vertigo and Vestibular Schwannoma
Roxana Moayer, yGail P. Ishiyama, zStellios Karnezis,
zAli R. Sepahdari, and Akira Ishiyama
Department of Otolaryngology–Head and Neck Surgery; yDepartment of Neurology; and zDepartment of Radiology,
University of California, Los Angeles, California
Objective: Advances in high resolution magnetic resonance
imaging (MRI) have enabled the detection of endolymphatic
hydrops (EH), a pathological ballooning of the endolym-
phatic fluid system, known to be associated with Menière’s
disease. When a patient has a known diagnosis of vestibular
schwannoma and develops recurrent episodic vertigo spells,
many surgeons recommend surgical intervention, attributing
the vestibular symptoms to the vestibular schwannoma. The
aim of this study is to evaluate the clinical outcome in
patients with vestibular schwannoma and EH, treated medi-
cally, for recurrent spells of vertigo.
Patients: Two patients with EH and vestibular schwannoma
who presented with recurrent spells of vertigo are included.
Both had characteristic low frequency hearing loss ipsilateral
to the schwannoma.
Intervention: MRI sequences with 3T scanner (Skyra,
Siemens Healthcare, Erlangen, Germany) using high resolu-
tion three-dimensional delayed postcontrast protocol included
‘‘cisternographic’’ T2 and delayed intravenous-enhanced
three-dimensional fluid-attenuation inversion recovery
Vestibular schwannoma and Menière’s disease (MD)
have overlapping clinical presentations despite distinct
underlying pathogeneses. Temporal bone histopathology
studies have identified a high rate of endolymphatic
hydrops (EH) in patients with vestibular schwannoma,
with up to 60% with concomitant EH (1,2). Only recently,
and at few institutions, magnetic resonance imaging (MRI)
has provided resolution adequate for accurate detection of
EH. This presents a unique clinical challenge in settings
where high-resolution MRI is not readily available for
preoperative assessment of vestibular schwannomas.
While MRI has been the gold-standard for assessment
of vestibular schwannoma (3), newer high-resolution
Address correspondence and reprint requests to Roxana Moayer,
M.D., 10833 Le Conte Ave, CHS 62-237, Los Angeles, CA 90095;
E-mail: roxanamoayer@mednet.ucla.edu
The authors disclose no conflicts of interest.
DOI: 10.1097/MAO.0000000000001627
e39
Copyright © 2017 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
(DIVE-3D-FLAIR) sequences, performed with 2350 ms
(bright perilymph) and 2050 ms (bright endolymph) inversion
times and with subtracted images.
Main Outcome Measure: MRI FLAIR evaluation of EH
and presence or absence of vestibular symptoms.
Results: Both patients had resolution of the disabling vertigo
spells with a diuretic, and Patient 1 had unchanged EH,
while Patient 2 had partial resolution of the EH and the
FLAIR hyperintensity.
Conclusion: When EH coexists with vestibular schwannoma
in a patient presenting with recurrent vertigo spells, medical
treatments for EH may alleviate the vestibular symptoms.
We recommend that patients with small vestibular schwan-
nomas who present with vertigo spells undergo high resolu-
tion MRI to evaluate for EH and undergo a trial of medical
treatment with diuretics. Key Words: Acoustic
neuroma—Endolymphatic hydrops—Menière’s disease—
Vertigo—Vestibular imaging—Vestibular schwannoma.
Otol Neurotol 39:e39–e44, 2018.
three-dimensional MRI with delayed contrast enhance-
ment to simultaneously identify EH and localize the
schwannoma’s nerve of origin, is unique to the most
modern methodologies of visualization techniques.
Vestibular schwannomas are benign tumors of
Schwann cells arising from the vestibular nerve with
an incidence of 1 to 2/100,000 persons-per-year. Initial
clinical presentation includes tinnitus, dizziness, senso-
rineural hearing loss with disproportionate drop in word
recognition score (1).
MD is characterized by fluctuating hearing loss, aural
fullness, tinnitus, and recurrent episodic vertigo (4,5).
Temporal bone histopathology studies show that EH, a
ballooning of the endolymph, is the primary pathophysi-
ologic finding in MD (6). With high-resolution three-
dimensional MRI now able to detect EH (7–9), we
propose that MRI be used in patients with vestibular
schwannoma and vertigo spells to evaluate for EH before
making a surgical decision.
e40 R. MOAYER ET AL.

MATERIALS AND METHODS images were visualized to evaluate nerve of origin of

the schwannoma.
Imaging was performed on a Siemens 3-T Skyra unit using a
16-channel head and neck coil paired with two 4-channel RESULTS
surface coils positioned over the ears 4 hours following an
intravenous injection of 0.2 mmol/kg gadobutrol (Gadavist, Patient 1: 41-year-old man presenting with 1.5 years of
Bayer Health Care). Scanning consisted of ‘‘cisternographic’’ recurrent vertigo lasting 30 minutes to several hours,
heavily T2-weighted 3-D turbo spin echo sequence (sampling associated with right sided aural fullness, ocean-roar
perfection with application-optimized contrasts by using dif- tinnitus, and fluctuating hearing. He had noted salt
ferent flip angle evolutions: T2 SPACE), and heavily T2-
weighted 3-D FLAIR sequence (hT2w-FLAIR). The T2 sensitivity with onset of vertigo spells 2 hours from
SPACE sequence acquisition parameters included: slice thick- ingestion of salty foods. Audiograms revealed speech
ness: 0.27 mm, TR/TE: 1350/270 ms, number of averages: 2, reception threshold (SRT) of 35 dB on the right, and
echo train length: 75, flip angle: 155, matrix: 320  288, FOV: 20 dB on the left with 100% speech discrimination
140  140 mm. The hT2w-FLAIR sequence acquisition bilaterally, and a low frequency hearing loss (Fig. 1).
parameters included: slice thickness: 0.5 mm, TR/TE: MRI demonstrated 2.0  2.5  2.8 mm intracanalicular
9,000/534 ms, inversion time: 2350 ms, number of averages: right vestibular schwannoma. Vestibular testing by elec-
2, echo train length: 141, flip angle: 120, matrix: 320  277, tronystagmography (ENG) and cervical vestibular
FOV: 172  150 mm. A second 3-D FLAIR sequence was evoked myogenic potentials (cVEMPs) were symmetric
performed with identical parameters except for inversion time
and normal.
of 2050 ms, resulting in an image with bright endolymph and
relatively dark perilymph. This was termed ‘‘PEI-FLAIR’’ High-resolution specialized MRI revealed right saccu-
(positive endolymph image), in keeping with the original lar EH and slight cochlear duct EH with an intracana-
description of this technique by Naganawa et al. (7). A licular acoustic schwannoma. Ipsilateral increased
subtracted image was obtained (hT2w-FLAIR  PEI-FLAIR), FLAIR enhancement of the perilymph was noted
producing an image with bright perilymph, dark endolymph, (Fig. 2). Sagittal oblique reformatting imaging at high-
and intermediate signal bone. Sagittal oblique reformatting resolution revealed a small vestibular schwannoma

PURE TONE AUDIOGRAM

Name: Patient 1 Age: 41 Date: 14-Mar-16 Case No.: 1

-10 -10

0 0

10 10

20 20

30 30
Hearing Level (dB)

Hearing Level (dB)

40 40

50 50

60 60

70 70

80 80

90 90

100 100

110 110

120 120
125 250 500 1000 2000 4000 8000 125 250 500 1000 2000 4000 8000
Frequency (Hz) Frequency (Hz)

Masking Details:

Right Left

Air conduction, masked if necessary

Air conduction, not masked (shadow point)

Bone conduction, not masked

Bone conduction, masked

Uncomfortable loudness level

Remarks:

Speech Audiometry:

AD Threshold 35dB; Discrimination 100%

AS Threshold 20dB; Discrimination 100%

FIG. 1. Patient 1: Audiogram demonstrating low frequency sensorineural hearing loss.

Otology & Neurotology, Vol. 39, No. 1, 2018

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 HIGH RESOLUTION 3-D DELAYED CONTRAST MRI DETECTS EH IN PATIENTS e41

FIG. 2. Patient 1: Right inferior vestibular nerve schwannoma with right saccule hydrops. A, Sagittal oblique reformation of the
cisternographic T2-weighted sequence shows a small mass arising from the inferior vestibular nerve (arrow). The normal cochlear nerve
(CN), facial nerve (FN), and superior vestibular nerve (SVN) are seen as distinct from the lesion. B, Axial maximum intensity projection
image of the perilymph-bright 3D FLAIR sequence shows relatively higher signal in the right perilymph (short arrow) compared with the left.
There is also an enlarged signal void in the anterior portion of the right vestibule (long arrow) indicative of vestibular hydrops. C, Axial
subtracted image (perilymph bright, endolymph dark, bone intermediate) through the anterior, inferior portion of the right vestibule shows a
large area of dark signal reflective of a saccular hydrops (arrow) and minimal dilation of the cochlear duct (arrowhead). The ampullated end
of the posterior semicircular canal (PSC) and the cochlear basal turn are marked for reference. D, Axial subtracted image (perilymph bright,
endolymph dark, bone intermediate) through the anterior, inferior portion of the contralateral, normal inner ear shows bright perilymph
occupying this portion of the vestibule, which is reflective of the normal, very small size of the saccule. 3D FLAIR indicates three-dimensional
fluid-attenuation inversion recovery.

arising from the right inferior vestibular nerve, with the
medial margin of the tumor approximately 2 mm from the
cochlear aperture and the lateral margin 2 mm lateral to
the internal auditory meatus. He was started on low salt
diet and acetazolamide and had resolution of the vertigo
spells and aural fullness.
Patient 2: 42-year-old man presenting with 1-year of
recurrent, episodic severe rotational vertigo, associated
with nausea and vomiting, left sided fluctuating tinnitus
and aural fullness. He tried a low salt diet and avoidance
of caffeine without relief. Audiograms revealed SRT of
25 dB on the left and 10 dB on the right, 72% speech
discrimination on the left, and 100% speech discrimina-
tion on the right, and low frequency hearing loss on the
left (Fig. 3). The patient declined ENG testing.
High-resolution specialized MRI revealed left cochlear
and saccular hydrops. Sagittal oblique reformatting imag-
ing at high-resolution revealed a small acoustic schwan-
noma localized to the left cochlear nerve measuring
4.5  2  3 mm. The schwannoma was approximately
1.5 mm from the cochlear aperture. Increased FLAIR
enhancement of the perilymph was noted on the left side.
He was started on acetazolamide and noted improvement
of vertigo and tinnitus. Repeat MRI revealed resolution of
the FLAIR enhancement in the perilymph and partial
resolution of the hydrops, present in cochlear duct only
with resolution of the saccular EH after treatment (Fig. 4).
DISCUSSION
We present a case series of two patients with vestibular
schwannoma using high-resolution three-dimensional
MRI to both identify precise localization of the schwan-
noma with sagittal oblique reformatting (down the barrel)
views and regional identification of EH localization. This
is the first study demonstrating the use of sagittal oblique
reformatting to evaluate the precise localization of the
origin of a small schwannoma. This is also the first study
using high-resolution MRI to identify regional localiza-
tion of EH and to correlate with precise localization of the
schwannoma. In a study of 128 patients with vestibular
schwannoma, 49 patients (39%) had the full Menière’s
triad of recurrent vertigo, hearing loss and tinnitus (10).
Similarly, temporal bone studies have associated vestib-
ular schwannoma with EH (2). In this context, we present
this case series to demonstrate the use of medical thera-
pies usually used in MD to treat recurrent vertigo.
In both cases, the EH is ipsilateral to the vestibular
schwannoma, and in the same endorgan innervated by
the affected nerve. In case 1, the inferior vestibular
nerve, the origin of the vestibular schwannoma, is the
nerve innervating the saccule, and the localization of EH.
In case 2, the cochlear nerve, the origin of the schwan-
noma, is the nerve that innervates the cochlear duct, and
the localization of the EH that remained after diuretic.
The relationship between these disease processes is
under investigation.
In 2011, Naganawa retrospectively reviewed 13 cases
of vestibular schwannoma to understand whether vertigo
symptoms in patients with vestibular schwannoma cor-
related with EH. Ipsilateral cochlear hydrops was identi-
fied in one patient and vestibular hydrops was noted in
another four patients, however, there was no significant
correlation between vertigo and EH (11).

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e42 R. MOAYER ET AL.
PURE TONE AUDIOGRAM

Name: Patient 2 Age: 42 Date: 12-Nov-13 Case No.: 2

-10 -10

0 0

10 10

20 20

30 30
Hearing Level (dB)

Hearing Level (dB)

40 40

50 50

60 60

70 70

80 80

90 90

100 100

110 110

120 120
125 250 500 1000 2000 4000 8000 125 250 500 1000 2000 4000 8000
Frequency (Hz) Frequency (Hz)

Masking Details:

Right Left

Air conduction, masked if necessary

Air conduction, not masked (shadow point)

Bone conduction, not masked

Bone conduction, masked

Uncomfortable loudness level

Remarks:

Speech Audiometry:
AD Threshold 10dB; Word Recognition score 100%
AS Threshold 25dB; Word Recognition Score 72%

FIG. 3. Patient 2: Audiogram demonstrating low frequency sensorineural hearing loss.

Similarly, in a retrospective clinical review, five with increased FLAIR hyperintensity. However, given
patients with MRI-confirmed intralabyrinthine vestib- that in Patient 2, there was reversal of the enhancement
ular schwannoma were compared with five patients with treatment in addition to reversal of hydrops, we
with imaging-confirmed delayed EH. Similar to our think it is less likely due to proteinaceous deposition. We
results, they concluded that clinical course and audio- have previously reported reversal of endolymphatic
vestibular testing could not reliably distinguish vestib- hydrops in acetazolamide-responsive patients with
ular schwannoma from hydrops (12). In contrast to Menière’s disease, documented using high-resolution
their local contrast administration, we find intravenous three-dimensional MRI in a subset of patients responsive
(IV) contrast administration to be preferable. In com- to diuretics (16).
parison, IV contrast is more uniformly distributed In a series of four patients with small vestibular
throughout the inner ear (13) and no otologic procedure schwannomas and severe vertigo, all four patients had
is required. Furthermore, IV contrast MRI allows for significant improvement with intratympanic gentimicin
evaluation of increased permeability of the blood-lab- (17). The authors cite hearing preservation as an
yrinthine barrier on both the ipsilateral and contralat- advantage of gentimicin injection over alternative
eral side (14). treatments, however, gentimicin is not without risk
The pathophysiologic process resulting in EH remains of damage to cochlear function (18). Also, of note,
unknown in MD. In both patients, there was evidence of gentamicin is a permanent ablative therapy, and would
blood-labyrinthine barrier permeability increase on the not be warranted for patients responsive to low dose
ipsilateral side, a finding associated with MD (14). of diuretic.
Histopathologic findings of damage to the blood-laby- On a clinical note, both patients presented were
rinthine barrier have been demonstrated on electron recommended surgery as treatment for their vertigo
microscopy of the vasculature in vestibular endorgans spells when diagnosed with vestibular schwannoma.
of patients with MD (15). Vestibular schwannoma is also By evaluation with high-resolution three-dimensional
associated with increased proteinaceous deposition in the MRI, we were able to diagnose EH and treat with
endolymph and perilymph (10) which may be associated diuretics. Therefore, we propose that patients with

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 HIGH RESOLUTION 3-D DELAYED CONTRAST MRI DETECTS EH IN PATIENTS e43

FIG. 4. Patient 2: Left cochlear schwannoma with left saccule and cochlea hydrops. A, Axial perilymph-bright 3D FLAIR sequence
through the level of the cochlear basal turn shows increased intensity of perilymph signal in the left scala tympani compartment (long
arrow) compared with the right side (short arrow) and effacement of the left scala vestibuli (arrowhead) compared with the normal right
scala vestibuli (open arrowhead). B, Axial subtracted sequence (perilymph bright, endolymph dark, bone intermediate) through the
level of the pars inferior of the vestibule shows a dilated endolymphatic space of the left saccule (long arrow) and cochlear duct
(arrowhead). This region is filled with normal perilymph on the right side (short arrow). C, Axial cisternographic T2 and sagittal oblique
reformation show a small mass in the left internal auditory canal (arrow) arising from the cochlear nerve, in the anterior inferior
quadrant of the internal auditory canal. The facial nerve (FN) and superior vestibular nerve (SVN) are marked for reference. D,
Posttreatment 3D FLAIR (top) and subtracted (bottom) sequences demonstrate decreased perilymph signal asymmetry and normal
appearance of the vestibule with persistent dilation of the cochlear duct (arrowhead). 3D FLAIR indicates three-dimensional fluid-
attenuation inversion recovery.

small vestibular schwannoma who present with vertigo
spells should undergo high-resolution three-dimen-
sional MRI with delayed phase contrast and if there
is MRI evidence of endolymphatic hydrops, a trial of
medical treatment can be tried as the vertigo spells may
originate from the EH rather than from the vestibular
schwannoma.
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