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

1. Salt AN and Plontke SK. Endolymphatic hydrops: pathophysiology and experimental models. Otolaryngol

Clin North Am 2010;43(5):971-983.

2. Hallpike CS, Cairns HWB. Observations of the pathology of Meniere's syndrome. Proc Roy Soc Med.

1938;31:1317–1336.

3. Yamakawa K. Über die pathologische Veränderung beieinem Meniere-Kranken. J Otolaryngol Soc Japan.

1938: 2310–2312.

4. Merchant SN, Adams JC, Nadol JB Jr. Pathophysiology of Meniere's syndrome: are symptoms caused by

endolymphatic hydrops? Otol Neurotol. 2005;26:74– 81.

5. Gürkov R, Kantner C, Strupp M, Flatz W, Krause E, Ertl-Wagner B. Endolymphatic hydrops in patients with

vestibular migraine and auditory symptoms. Eur Arch Otorhinolaryngol. 2013 Oct 12.

6. Rassekh CH, Harker LA. The prevalence of migraine in Meniere's disease. Laryngoscope. 1992;102:135–

138.

7. Cha YH, Brodsky J, Ishiyama G, Sabatti C, Baloh RW. The relevance of migraine in patients with

Meniere’s disease. Acta otolaryngologica 2007 127 (12): 1241-1245.

8. Naganawa S, Nakashima T. Visualization of endolymphatic hydrops with MR imaging in patients with

Ménière's disease and related pathologies: current status of its methods and clinical significance. Jpn J

Radiol. 2014 Apr;32(4):191-204.

9. Shimono M, Teranishi M, Yoshida T, Kato M, Sano R, Otake H, Kato K, Sone M, Ohmiya N, Naganawa S,

Nakashima T. Endolymphatic hydrops revealed by magnetic resonance imaging in patients with acute low-

tone sensorineural hearing loss. Otol Neurotol. 2013 Sep;34(7):1241-6.

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.