The cause of Ménière's disease is not known, but it may be related to a fluid imbalance in the inner ear.

The inner ear contains a fluid called endolymph. It is thought that, in Ménière's disease, too much of this fluid builds up in the inner ear. The resulting pressure affects the sensory systems in the inner ear that help maintain balance. This leads to the symptoms of tinnitus (ringing in the ears), hearing loss, vertigo (spinning sensation), and pressure or fullness in the ear.

Little is known about the cause of endolymph fluid buildup. It may be that too much fluid is produced or that the fluid does not properly drain from the inner ear, or it may be a combination of the two.