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PART II  DIZZY SPELLS THAT OCCUR IN ATTACKS BUT WITH NO APPARENT TRIGGER This page intentionally left blank Ménière’s Disease You have severe episodes of vertigo lasting three to four hours. ­These episodes come on without any apparent cause, and they continue no­ matter what you do. The attacks are typically accompanied by nausea and vomiting and by decreased hearing, fullness, and tinnitus (a roaring sound) in one ear. Over time the hearing in that ear becomes progressively worse. Prosper Ménière was a French doctor who while working at a deaf mute institute in Paris was struck by the observation that vertigo and hearing loss often occurred together. In his initial report before the Imperial Acad­ emy of Medicine in 1861, he described a young man who had violent attacks of vertigo, nausea, and vomiting, without any apparent cause. ­ Later the young man noticed loud noises in the ears, along with decreased hearing. “I could not forget that beyond the ­ middle ear­ there exists an apparatus [the inner ear] which, mysterious as it is, has not revealed to us all the phenomena which takes place in it,” Ménière noted. Did the young man described by Ménière have what has become known as Ménière’s disease? The modern diagnostic criteria for Ménière’s disease include attacks of vertigo CHAPTER  3 Dizz y Spells with No Apparent Trigger 54 with one-­ sided ear fullness, roaring tinnitus, and hearing loss typically lasting hours. The key to the diagnosis is to document a decrease in hearing in the involved ear during an attack and a gradual progressive hearing loss between attacks. Ménière’s subject had vertigo attacks followed by tinnitus and fluctuating hearing loss, but apparently his hearing loss involved both ears. The hearing loss with Ménière’s disease typically begins in one ear and remains so for many years; only a small percentage of patients develop involvement of both ears, and then only ­ after many years, typically. It is pos­ si­ ble that the young man had some other inner ear disorder, such as autoimmune inner ear disease, but Ménière provided too few details about the case to allow modern diagnosticians to be sure. Ménière also described autopsy findings on a young girl who suddenly developed one-­ sided deafness and vertigo and died five days ­ later. The brain and spinal cord ­ were normal, but he noted blood filling the semicircular canals of the inner ear on the side of the deafness. In retrospect this was likely a case of acute leukemia with bleeding into the inner ear, but based on Ménière’s report, the concept that Ménière’s disease was caused by hemorrhage into the inner ear persisted well into the twentieth ­ century. Ménière ­ wasn’t suggesting that this young girl with sudden deafness and vertigo had the same disease as the young man with episodic vertigo, tinnitus, and fluctuating hearing loss. He was simply making the point that vertigo and hearing loss commonly occur together with inner ear disease. In the United States of the nineteenth ­ century, ­ there was­ little or no appreciation that the inner ear played a role in balance . For example, in 1890, Howard Ayers gave a lecture at the Marine Biological Laboratory at Woods Hole, Mas­ sa­ chu­ setts, titled “The Ear of Man: Its Past, Pres­ ent, and ­ Future.” He Ménière’s Disease 55 discussed the anatomy of the inner ear, including the semicircular canals and otolith organs, which we now know to be the anatomical sites where physical motion is sensed and transformed into signals passed on to the brain. Ayers thought­ these organs ­ were impor­ tant for hearing but not balance. “The function of audition [hearing] certainly belongs to the ear; not so, however the equilibrious [balance] function . . . ​­ there is no longer the slightest evidence in ­ favor of the theory [of the inner ear as a balance organ].” One prob­ lem that plagued Ménière and the other doctors who wanted to study the inner ear during autopsies in the­ middle of the nineteenth ­ century was the denseness of the bone that surrounds the inner ear. They could not make thin cuts through the tissue of the inner ear as was typically done to study other organs of the body with a microscope. Ménière sawed through the bone surrounding the inner ear and...


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