Chapter 24 Meniere’s disease can present in a variety of ways. The symptom constellation consists of episodic vertigo, fluctuating or progressive sensorineural hearing loss, aural fullness, and tinnitus. The vertigo is usually a hallucination of violent spinning that lasts 30 minutes to many hours, but not more than a day. In advanced cases, attacks of vertigo may be replaced by drop attacks (also known as crises of Tumarkin). The hearing loss is generally asymmetric, with the worse-hearing ear or ear with the most hearing fluctuation more often being responsible for the vestibular complaints. Changes in hearing, tinnitus, and aural fullness are often temporally associated with the onset of vertigo. Clinical examination of patients with Meniere’s disease is generally unremarkable, as most patients do not present during an acute flare. In such unusual situations, patients may manifest spontaneous nystagmus, diaphoresis, and pallor. Tuning fork testing reveals a sensorineural hearing loss in the affected ear. Between attacks, spontaneous or positional nystagmus is not commonly seen. The sensorineural hearing loss may also resolve completely during quiescent intervals. Audiometry can be very helpful in the diagnosis of Meniere’s disease. The classic audiometric presentation involves a low-frequency sensorineural loss, occasionally associated with a very mild “inner ear conductive” component. With time, all frequencies may be affected. The pattern of hearing loss is highly variable. Speech discrimination scores are in the expected range for a given sensori-neural threshold. Electrocochleography has been hailed as a means of detecting subclinical Meniere’s disease (i.e., when audiometry is normal) by measuring an increased summating potential to action potential ratio (e.g., greater than 0.30). Electrocochleography has failed to gain widespread support because of its unclear sensitivity and specificity and the ability to diagnose Meniere’s disease on the basis of history and audiometry alone. Meniere’s disease is widely believed to result from a buildup of pressure within the membranous labyrinth (also called endolymphatic hydrops). Inner ear fluid homeostasis remains poorly understood, so it is not clear whether Meniere’s disease results from overproduction, underabsorption of endo-lymph, or both. Autoimmune and virally mediated etiologies have also found support. As the pathogenesis of Meniere’s disease has been attributed to a variety of pathologies, it might be better considered a syndrome. Most cases of Meniere’s disease can be controlled nonsurgically, such as with a low-sodium diet and administration of thiazide diuretics. Intractable Meniere’s disease may cause disability as a result of profound hearing loss or, more commonly, recurrent vertigo. Most of the surgery for intractable Meniere’s disease is directed at controlling the episodic vertigo. Surgery for Meniere’s disease should be considered only after maximal medical therapy, directed at the root cause and symptom control, has been exhausted (Table 24–1). The use of corticosteroids, either systemically or intratympanically, has yielded anecdotal benefits, but remains controversial. Corticosteroid administration should be more strongly considered in patients with possible bilateral Meniere’s disease, as surgical inter-vention may render the vestibular and auditory systems nonfunctional.
ENDOLYMPHATIC SAC SURGERY
| Therapy |
| Low-sodium diet |
| Thiazide diuretic |
| Methazolamide |
| Corticosteroid administration (systemically or intratympanically) |
| Vestibular suppressants and antiemetics |
SURGICAL TREATMENT OF MENIERE‘S DISEASE
Surgery is indicated when medical treatment fails to relieve the acute attacks of vertigo and the frequency and severity of the vertigo is disabling. None of the surgical techniques for Meniere’s disease has demonstrated any substantive benefit on hearing or tinnitus.
A variety of surgical therapies are used to treat the vertigo of intractable Meniere’s disease. These include, in order of increasing invasiveness, aminoglycoside perfusion, endolymphatic sac shunt or decompression, and labyrinthectomy or vestibular neurectomy. The procedure selected depends on the individual’s age, functional level, and degree of hearing impairment in both the affected ear and the opposite ear. Every effort should be made to preserve functional hearing.
Surgery is successful in relieving acute attacks of vertigo or drop attacks in most patients but varies according to the procedure used. Unsteadiness, however, may persist for a period of several weeks to months, until the central nervous system compensates for the loss of vestibular function in the operated ear. Compensation following surgery for Meniere’s disease is dependent on other factors such as age, visual impairment, and other neurologic dysfunction.
AMINOGLYCOSIDE MIDDLE EAR PERFUSION
The ototoxic-vestibular more so than cochlear properties of gentamicin are used to chemically ablate the vestibular system. The drug is injected either through a needle or through a catheter that has been inserted through a myringotomy in the posterior aspect of the tympanic membrane. The drug is administered until the affected labyrinth is sufficiently suppressed or destroyed. Treatment may be stopped if the hearing is affected. Vertigo is controlled in 80 to 90% of patients, and hearing is lost in up to 30%.1 Some persistent imbalance, usually mild, is common after the treatment. Many patients choose this treatment option because it is the least invasive, it is done on an outpatient basis, it entails little risk, and it has a relatively high chance of successful control of vertigo.
ENDOLYMPHATIC SAC SURGERY
This procedure is thought to work by relieving endolymph pressure, that is, relief of hydrops. Most cases are done under general anesthesia on an outpatient basis. A more complete description of technique is given below. Endolymphatic sac decompression and sac shunting have been reported to be successful in 65 to 90% of patients.2,3
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