Background: Auditory and vestibular symptoms and signs are common in patien
ts with migraine, yet little is known about the pathogenesis of these sympt
oms and signs.
Objective: To perform clinicopathological correlation in a patient with mig
raine, sudden deafness, and delayed endolymphatic hydrops.
Methods: A patient with long-standing migraine with aura developed sudden h
earing loss in the left ear at the age of 50 years and Meniere disease on t
he right side at age 73. At age 76, he had a flurry of sudden drop attacks
typical of otolithic crisis. He died of unrelated causes at age 81. The bra
in and temporal bones were removed approximately 24 hours after death. The
cochlea and vestibular end organs were dissected after the surrounding bone
was carefully removed.
Results: The brain and cerebrovasculature were normal. The left cochlea sho
wed prominent fibrosis consistent with an old infarction. The right inner e
ar showed hydrops, with relatively good preservation of the hair cells in t
he cochlea, saccular macule, and cristae of the semicircular canals. Howeve
r, the utricular macule was denuded of hair cells.
Conclusions: The sudden left-sided deafness likely resulted from ischemia,
possibly due to migraine-associated vasospasm. Presumably, the right ear su
ffered only minimal damage when the patient was 50 years old, but this dama
ge later led to the development of delayed endolymphatic hydrops on the rig
ht. Otolithic crises are thought to result from pressure changes across the
utricular macule. We speculate that loss of hair cells in the utricular ma
cule resulted from a collapse of the utricular membrane onto the macule.