Bilateral endolymphatic hydrops in a patient with
migraine variant without vertigo Isabelle Y Liu, MD1; Ali R Sepahdari, MD2; Gail Ishiyama, MD3; Kevin Johnson, BA, RT(R)4; Akira Ishiyama, MD1
1Department of Head & Neck Surgery, UCLA David Geffen School of Medicine;
2Department of Radiology, UCLA David Geffen School of Medicine;
3Department of
Neurology, UCLA David Geffen School of Medicine; 4Siemens Healthcare
Objective: Endolymphatic hydrops (EH) has been well described in
patients with Meniere’s disease; However, there is no study to date, to our
knowledge, that examines for the presence of EH in a patient with migraine
and bilateral hearing loss. Here we present the MRI findings using a
sequence for detecting EH in a unique case of a patient experiencing
migraine headaches and auditory symptoms without vertigo for more than
20 years.
Study design: Case report
Methods: Magnetic resonance imaging sequences included
“cisternographic” three-dimensional T2, and delayed intravenous-
enhanced three-dimensional fluid-attenuation inversion recovery (DIVE-
3D-FLAIR) sequences, performed with 2350 ms (bright perilymph) and
2050 ms (bright endolymph) inversion times. The bright endolymph images
were subtracted from bright perilymph images to create a composite image
with bright perilymph, dark endolymph, and intermediate bone signals.
Results: A 40-year-old female presented with a left-sided sensorineural
hearing loss and severe migraine headaches that began at age 12. More
recently, she experienced severe migraines with right-sided fluctuating
sensorineural hearing loss, tinnitus, and aural fullness. Audiometry
confirmed increased right-sided hearing loss at times of symptom severity.
Vestibular testing was within normal limits. MRI demonstrated the presence
of severe bilateral vestibular and cochlear EH.
Conclusions: Endolymphatic hydrops of both the cochlea and vestibule
can be present in patients without Meniere’s or vertigo. The relationship
between migraine and Meniere’s disease may be more complex, with this
patient with migraine associated bilateral hearing loss demonstrating
bilateral EH. New imaging modalities allow for studies into the field of
inner ear pathology, with significant implications for future research.
Abstract
• The presence of endolymphatic hydrops does not always
correlate with Meniere’s disease. This has been previously
demonstrated in studies of patients with acute low frequency
sensorineural hearing loss, where a significant number of
patients had EH without vertiginous symptoms9. Temporal bone
studies also confirm the presence of “asymptomatic hydrops4.”
• Previous literature has shown a correlation between EH and
vestibular migraine5, as well as between MD and migraines6. In
this case we present a patient who has severe migraines with
acute hearing loss at age 12, and presentation consistent with
migraine variant without vertigo and MRI demonstrates
significant bilateral endolymphatic hydrops.
• The case is consistent with findings relating the coexistence of
migraine with Meniere’s disease is associated with higher
incidence of bilateral hearing loss and younger age of onset.
• This suggests that endolymphatic hydrops is not the causative
factor but a result of inner ear pathology.
• Migraine may be one causative factor of inner ear pathology that
leads to the common final pathway of hydrops.
• New, noninvasive imaging modalities are allowing for exciting
new opportunities to study EH in live patients, which will allow for
further elucidations into Meniere’s disease.
Discussion Introduction Endolymphatic hydrops (EH) is an anatomic finding in which the
structures bounding the endolymphatic space are distended by
enlargement of the endolymphatic volume1. This finding was first
correlated with Meniere’s disease (MD) in the 1930s2-3, leading
researchers to conclude that EH is causative of Meniere’s. However,
further research has shown that the connection between EH and MD is
not a simple correlation. There is a subset of patients who have EH
without the classical triad of symptoms that define Meniere’s disease:
vertigo, hearing loss, and tinnitus4. Additionally, MD has a significant
overlap with migraine, and patients with vestibular migraine have also
been found to have increased rates of EH5-6. Furthermore, the
comorbidity of migraine with Meniere’s disease is associated with
concurrent bilateral aural symptoms and hearing loss, earlier age of
onset, and a strong family history7. The advent of new imaging
techniques, allowing for the differentiation between perilymph and
endolymph in the inner ear, have allowed for evaluation for EH in live
patients8. Here we present a patient with bilateral endolymphatic
hydrops diagnosed by magnetic resonance imaging, who presents with
auditory symptoms without vertigo, and the coexistence of severe
migraine headaches.
Imaging was done on a 3-T scanner (Skyra, Siemens Healthcare,
Erlangen, Germany) using a 12-channel head coil, paired with a two-
piece 8-channel surface coil, 4 hours after administration of 0.2 mmol/kg
gadodiamide intravenous contrast (Magnevist, Bayer HealthCare).
Imaging consisted of three sequences: 1) “Cisternographic” 3D turbo
spin echo T2; 2) "Perilymph bright, endolymph dark" heavily T2-
weighted (hT2w)-3D-FLAIR, obtained with an inversion time of 2350
ms, 3) "Endolymph bright, perilymph dark" hT2w-3D-FLAIR, obtained
with inversion time of 2050 ms. All sequences were acquired in the axial
plane along the infraorbitomeatal line, as three dimensional volumetric
scans with 0.3 x 0.3 x 0.3 mm isotropic voxels. The endolymph bright
hT2w-3D-FLAIR images were subtracted from the perilymph bright
hT2w-3D-FLAIR images, in order to obtain an image with bright
perilymph, dark endolymph, and intermediate signal bone. The
cisternographic T2 was used to assist with anatomic reference.
Materials and Methods
Figure 3. DIVE-3D-FLAIR MR imaging of affected patient. A. Maximum
intensity projection image shows the vestibule completely blacked out
by the dilated endolymphatic space on both sides (right sided shown by
arrow). B (right) and C (left). Standard cisternographic T2 images show
all the fluid in the inner ear as bright, therefore showing no significant
difference with the normal patient. D (right) and E (left). Subtracted
images show the vestibule completely filled with the black signal of endolymph.
A
B C D E
Figure 1. DIVE-3D-FLAIR MR imaging of a normal patient. A. Maximum
intensity projection image shows the vestibular endolymph as a small
signal void surrounded by bright perilymph (arrow). B. Standard
cisternographic T2 image where all the fluid in the inner ear is bright,
including both endolymph and perilymph. C. Subtracted image shows black endolymph surrounded by white perilymph.
A
B C
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References
Results A 40-year-old female presents with severe migraine headaches and a
stable left-sided hearing loss since age 12. Over the past year, she has
developed right-sided fluctuating sensorineural hearing loss, tinnitus,
and aural fullness and associated severe migraine headaches. The
patient never experienced vertigo and a full battery of vestibular testing,
including electronystagmography (with calorics), quantitative rotational
testing, and vestibular evoked myogenic potentials were all within
normal limits
Figure 2. Standard audiometry in a patient with bilateral low frequency
hearing loss. The left-sided hearing loss is chronic and stable since
adolescence, while the right-sided hearing loss is recent and fluctuates
with symptom severity.