Bilateral Endolymphatic Hydrops in a Patient With Migraine Variant Without Vertigo: A Case Report

Abstract

To use modern high-resolution inner ear imaging modalities to evaluate for endolymphatic hydrops (EH) in a patient with migraine-associated fluctuating hearing loss without vertigo spells or dizziness. EH has been well described in patients with Meniere's disease on both human temporal bone studies and modern high-resolution imaging; however, there is no study to date, to our knowledge, that examines the presence of EH in a patient with migraine and bilateral hearing loss. We present the MRI findings using a sequence for detecting EH in a unique case of a patient experiencing migraine headaches accompanied by fluctuating hearing loss without vertigo. 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. A 40-year-old female presented with a left-sided sensorineural hearing loss and severe migraine headaches that began at age 12. For the past year, she experienced severe migraines with right-sided fluctuating sensorineural hearing loss, tinnitus, and aural fullness. Audiometry confirmed a drop of right-sided hearing at times of migraines and increased symptom severity. Vestibular testing was within normal limits. MRI demonstrated the presence of severe bilateral vestibular and cochlear EH. EH of both the cochlea and vestibule can be present in patients without Meniere's disease or vertigo. The relationship between migraine and Meniere's disease may be complex, as demonstrated in this patient with migraine-associated bilateral hearing loss with MRI documentation of severe bilateral EH. The fact that migraine can be associated with EH is important and demonstrates a potential relationship between the pathophysiology of migraine and that of EH. Given this patient's previous association of migraine and hearing loss at age 12, it appears that migrainous attacks occur simultaneously with the hearing loss, and may be potentially causative of the fluctuating hearing loss, mediated possibly through the development of EH. New imaging modalities allow for studies into the field of inner ear pathology, with significant implications for future research.