Labyrinthine disorders are the most common causes of true vertigo. Ménière disease is characterized by paroxysmal attacks of vertigo associated with fluctuating tinnitus and deafness. One or the other of the latter two symptoms may be absent during the initial attacks of vertigo, but invariably they assert themselves as the disease progresses and increase in severity during acute attacks. Ménière disease affects the sexes about equally and has its onset most frequently in the fifth decade of life, although it may begin earlier or later. Cases of Ménière disease usually occur as a sporadic trait, but hereditary forms, both autosomal dominant and recessive, have been described (see reviews by Konigsmark). The main pathologic changes consist of an increase in the volume of endolymph and distention of the endolymphatic system (endolymphatic hydrops). It had been speculated several decades ago that the paroxysmal attacks of vertigo are related to ruptures of the membranous labyrinth and release of potassium-containing endolymph into the perilymph, changes that have a paralyzing effect on vestibular nerve fibers and lead to degeneration of the delicate cochlear hair cells (Friedmann). An immune pathogenesis has also been proposed, based tentatively on the presence of circulating antibodies putatively against heat shock protein in some patients.
In typical Ménière disease, the attacks of vertigo are abrupt and last for several minutes to an hour or longer. The vertigo is unmistakably whirling or rotational and usually so severe that the patient cannot stand or walk. Varying degrees of nausea and vomiting, low-pitched tinnitus, a feeling of fullness in one ear and a diminution in hearing are practically always associated. Nystagmus is present during the acute attack; it is horizontal in type, usually with a rotary component and with the slow phase to the side of the affected ear. On attempting to touch a target with the eyes closed, there is past pointing as well as a tendency to fall toward the affected ear when standing or walking. The patient prefers to lie with the faulty ear uppermost and is disinclined to look toward the normal side, which exaggerates the nystagmus and dizziness. As the attack subsides, hearing improves, as does the sensation of fullness in the ear; with further attacks, however, there is a progressive increase in deafness.
The attacks vary considerably in frequency and severity. They may recur several times weekly for many weeks on end, or there may be remissions of several years’ duration. Frequently recurring attacks may give rise to a mild chronic state of disequilibrium and a reluctance to move the head or to turn quickly. With milder forms of the disease, the patient may complain more of head discomfort and of difficulty in concentrating than of vertigo and then may be considered to signify anxiety. Symptoms of anxiety are common in patients with Ménière disease, particularly in those with frequent severe attacks.
A small proportion of patients with Ménière disease experience sudden, violent falling attacks. These episodes have been referred to by the quaint name “otolithic catastrophe of Tumarkin,” who attributed them to deformation of the otolithic membrane of the utricle and saccule. Patients characteristically describe a sensation of being pushed or knocked to the ground without warning, or there may be a sudden movement or tilt of the environment just before the fall. Consciousness is not lost, and vertigo of the usual type and its accompaniments are not part of the falling attack, although some patients become aware of these symptoms after falling. The attacks may occur early or late in the course of the disease. Typically, several attacks occur over a period of a year or less and remit spontaneously (Baloh et al). An initial attack must be distinguished from other types of drop attacks, but the occurrence of the more typical vertiginous attacks of Ménière disease, with deafness and tinnitus, clarifies the diagnosis.