BACKGROUND: Auditory and vestibular symptoms and signs are common in patients 
with migraine, yet little is known about the pathogenesis of these symptoms and 
signs.
OBJECTIVE: To perform clinicopathological correlation in a patient with 
migraine, sudden deafness, and delayed endolymphatic hydrops.
METHODS: A patient with long-standing migraine with aura developed sudden 
hearing loss in the left ear at the age of 50 years and Ménière disease on the 
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 brain 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 showed 
prominent fibrosis consistent with an old infarction. The right inner ear showed 
hydrops, with relatively good preservation of the hair cells in the cochlea, 
saccular macule, and cristae of the semicircular canals. However, 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 
suffered only minimal damage when the patient was 50 years old, but this damage 
later led to the development of delayed endolymphatic hydrops on the right. 
Otolithic crises are thought to result from pressure changes across the 
utricular macule. We speculate that loss of hair cells in the utricular macule 
resulted from a collapse of the utricular membrane onto the macule.