Cupulolithiasis in BPPV:  Rare, or non-existent?

Newsflash: Read our science article, Cupulolithiasis: A Critical Reappraisal, at

BPPV is the best understood form of vertigo, and usually goes away promptly with simple maneuvers.  Sometimes, though, it can persist, and in those cases, a somewhat different and rare form is diagnosed, called cupulolithiasis.  This means “stones on the cupula”, the cupula being the main sensor of the inner ear.   In the time before we knew that ear stones—otoconia—moved freely in the ear canals causing vertigo, there was a theory that all the symptoms of BPPV were caused by otoconia that were attached to the sensor itself.  Does this really happen?

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A common malformation: Enlarged vestibular aqueduct syndrome (EVAS)

Since the inner ear is a pressure sensor, it is important to keep the pressures steady in the ear.  This is a challenge because the ear bridges the space in the skull between the outside-the ear canal—and the inside, housing the brain.  When you sneeze or strain, the pressure in the fluid around the brain rises.  When your ears pop, it means the pressure of the air outside the skull has changed. Somehow the inner ear has to deal with these changes and keep functioning smoothly.

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The inner ear can be malformed

The inner ear is a delicate and complex structure, so it is critical that it be formed properly.  Abnormalities in development arising prior to birth, called congenital malformations, can be associated with a complete absence of vestibular function from birth.  Milder malformations can at first be silent, only to cause progressive or sudden losses of function in childhood or later in life.   These malformations can involve the entire inner ear and affect both hearing and balance, or can be limited to just a portion of the inner ear.  They can arise because of an inherited gene defect, or can happen if the fetus is exposed to infections or toxins during the development of the ear during the first few months of pregnancy.  Often the development of the ear ceases when the infection or toxic exposure occurs, leaving the inner ear in a relatively unformed or “arrested” state.

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