Crystal VanDerHeyden, AuD, CCC-A, and Jaynee A. Handelsman, PhD, CCC-A
Ménière's disease is a chronic illness that is characterized by symptoms of episodic vertigo, aural fullness, tinnitus, and fluctuating sensorineural hearing loss. There are approximately 615,000 cases of Ménière's disease in the United States (National Institute on Deafness and Other Communication Disorders, 2008). The diagnosis is most common in adults during their 4th or 5th decade of life and has a slight female preponderance. There also appears to be a strong genetic component (Sajjadi & Paparella, 2008). Ménière's has been studied since 1861, when Prosper Ménière first described a condition with the symptoms as listed. Although there is still much to be understood about the underlying physiological mechanisms that cause Ménière's disease, research has shown that a majority of patients receive benefit either from a change in lifestyle or from medical or surgical intervention.
The pathophysiology of Ménière's disease is not clearly understood. It was previously thought that Ménière's was closely correlated with endolymphatic hydrops, a condition in which endolymph builds up due to an obstruction in the endolymphatic sac. Hormones such as saccin and glycoproteins are produced in excess, which may relieve the blockage and cause vertigo due to the sudden release of endolymph across the sac (Sajjadi & Paparella, 2008). However, histological studies of temporal bones have shown the presence of endolymphatic hydrops in patients without symptoms associated with Ménière's disease (McCall et al., 2009), raising the question of why some people with hydrops are symptomatic while others are not. Other possible origins of the disease are perisaccular fibrosis, atrophy of the endolymphatic sac and loss of epithelial integrity, hypoplasia of the vestibular aqueduct, and narrowing of the lumen of the endolymphatic duct (Sajjadi & Paparella, 2008). McCall and colleagues (2009) examined the vestibular end organs of patients with intractable Ménière's that were obtained during labyrinthectomy. They noted that the specimens showed "variable degrees of neuroepithelial degeneration including conversion of the sensory epithelium to a monolayer, [basement membrane] thickening, cellular vacuolization, absence of hair cell stereocilia, and increased intercellular stromal spaces" (McCall et al., 2009, p. 10). Further research is needed to replicate this study and obtain more information regarding the pathophysiological processes underlying Ménière's disease.
Ménière's disease is characterized primarily by its accompanying symptoms, which include tinnitus, hearing loss, aural fullness, and vertigo. The tinnitus is typically described as a "roaring" sound that becomes louder prior to the onset of vertigo. Hearing loss typically follows the configuration of low-frequency sensorineural hearing loss and commonly fluctuates. Many patients describe a sensation of aural fullness or pressure prior to the onset of vertigo, which may be accompanied by tinnitus. Episodes of vertigo typically last at least 20 minutes and may persist for up to several hours.
In 1995, the American Academy of Otolaryngology-Head and Neck Surgery established the following criteria for diagnosis of Ménière's disease (p. 182):
Certain Ménière's disease
Definite Ménière's disease
Probable Ménière's disease
Possible Ménière's disease
(Reprinted from Otolaryngology-Head and Neck Surgery, 113, American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium, "Committee on hearing and equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease," 181–185, copyright 1995, with permission from Elsevier.)
These criteria illustrate the importance of integrating case history and the patient's report of symptoms with objective test measures to reach a diagnosis of Ménière's disease.
Evaluation of patients with Ménière's symptoms is very important in determining a diagnosis. Before the patient is seen by an otolaryngologist, the physician may request a variety of clinical tests. The case history is critical. It is especially important to determine the onset of symptoms and to describe the frequency and duration of vertiginous episodes as well as identify any accompanying ear complaints. A comprehensive audiologic evaluation should be completed, including pure-tone air and bone conduction thresholds at standard audiometric frequencies, word recognition scores, and immittance testing. Audiometric thresholds should be closely monitored to document any fluctuations in hearing sensitivity. Formal vestibular testing should be done to assess the integrity of the vestibular system and may include dynamic posturography, rotary chair, and videonystagmography evaluations. Electrophysiological evaluations such as electrocochleography and vestibular evoked myogenic potentials may be completed as well. Finally, the patient will be seen by an otolaryngologist to determine a diagnosis and discuss treatment options.
Much research has been done to determine the efficacy of treatment options for patients with Ménière's disease. These treatment options range from changes in lifestyle to ablative surgery. A fairly high correlation of seasonal allergies exists in patients diagnosed with Ménière's disease, and studies have shown a significant decrease in vertigo symptoms for these patients after implementing allergy-avoidance behaviors and/or starting immunotherapy for allergies. Other lifestyle changes, such as limiting caffeine, chocolate, alcohol, and salt, have been effective in reducing vertigo attacks. Patients diagnosed with Ménière's disease are typically counseled to adopt a low-salt diet (1,500–2,000 mg per day), and some are also started on a diuretic.
If changes in lifestyle are not sufficient for the suppression of vertigo attacks, other treatments are considered. The Meniette device is a minimally invasive form of therapy in which pressure pulses are delivered to the ear through a small device following placement of a tympanostomy tube in the tympanic membrane. These pressure changes are thought to help stimulate the flow of endolymph, which can result in a reduction of vertigo symptoms. Studies have shown that up to 67% of patients report an improvement in symptoms at 2 years, while longer term studies have shown a success rate of up to 58% (Dornhoffer & King, 2008). The primary drawback to use of the Meniette device from the patients' perspective is the cost; third-party payers do not cover the device at this time.
Steroid therapy has also been utilized for treatment of symptoms associated with Ménière's disease, especially in patients with a sudden decrease in hearing. It is a nonablative form of treatment that does not require surgery. Steroids can be given orally or via intratympanic (IT) injection. A review of the literature in 2004 concluded that the strongest evidence of improvement was seen in patients with sudden, idiopathic hearing loss, while evidence of improvement in patients with Ménière's disease is weakly correlated (Doyle et al., 2004). A study completed by Boleas-Aguirre, Lin, Della Santina, Minor, and Carey (2008) reported a 91% success rate in Ménière's patients after 2 years following IT dexamethasone. Of the patients followed for longer than 2 years, 70% did not require any further treatment. It is important to consider other autoimmune inner ear disorders if a patient responds favorably to the steroid treatment.
Some patients elect to undergo endolymphatic sac decompression surgery, which has a high preservation rate for hearing and balance function. In this procedure, a Silastic sheet is inserted into the lumen of the endolymphatic sac. Efficacy of this surgery is fairly controversial. Some studies report improvement in vertigo symptoms, while others note no difference between the surgical and placebo groups (Sajjadi & Paparella, 2008).
If treatments aimed at restoring normal function of the inner ear are not successful, ablative forms of treatment are considered. One of these forms is IT gentamicin injection, in which gentamicin is injected directly into the middle ear space. This form of treatment has been found to successfully treat vertigo attacks in many patients, but potential secondary effects include disequilibrium and sensorineural hearing loss, and these must be explained to the patient.
Surgical options for patients with intractable Ménière's are vestibular nerve section or labyrinthectomy. Vestibular nerve section has been performed in cases where hearing preservation is desired. However, this form of surgery has become less popular since the widespread introduction of IT gentamicin. Labyrinthectomy is more desirable in patients who already have a substantial degree of hearing loss (Gacek & Gacek, 1996). Ablative procedures are generally effective in alleviating the disabling attack of vertigo, and patients typically recover quite well due to the principles of central compensation following a stable unilateral vestibular system weakness.
Although Ménière's disease affects a substantial number of people in the United States, control of vertigo attacks can often be achieved through a variety of lifestyle changes coupled with other medical or surgical interventions. The proper diagnosis of Ménière's depends on an accurate case history, completion of auditory and vestibular testing measures, and a thorough medical evaluation. A majority of patients benefit from dietary changes or minimally invasive medical treatments and procedures, while those who do not may explore options such as IT gentamicin, vestibular nerve section, and labyrinthectomy. Vestibular rehabilitation can be useful in patients who are no longer experiencing acute attacks of spontaneous vertigo but who may be left with symptoms of disequilibrium or unsteadiness. While much is known about the symptoms and treatments associated with Ménière's disease, the underlying pathophysiology of the disorder is poorly understood. Further research is necessary to obtain a better understanding of the disease process, which will likely increase treatment efficacy.
Crystal VanDerHeyden is an audiologist at the University of Michigan Health System in Ann Arbor, Michigan. Her clinical interests focus on the area of diagnostic testing, including vestibular assessments, auditory evoked potentials, and comprehensive pediatric and adult audiologic evaluations. She is actively involved with the training and mentorship of audiology graduate students and is a member of the 2010 Michigan Audiology Coalition conference planning committee. Contact her at email@example.com.
Jaynee A. Handelsman is Assistant Director of the Vestibular Testing Center in the Department of Otolaryngology Head and Neck Surgery in the University of Michigan Health System. Her clinical, teaching, and research areas of focus include the assessment and management of patients with dizziness and balance disorders, as well as the impact of potentially ototoxic medications on auditory and vestibular system function. Dr. Handelsman is ASHA Vice President for Audiology Practice (2010–2012) and an ASHA Fellow. Contact her at firstname.lastname@example.org.
American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium. (1995). Committee on hearing and equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease. Otolaryngology-Head and Neck Surgery, 113, 181–185.
Boleas-Aguirre, M. S., Lin, F. R., Della Santina, C. C., Minor, L. B., & Carey, J. P. (2008). Longitudinal results with intratympanic dexamethasone in the treatment of Meniere's disease. Otology & Neurotology, 29, 33–38.
Dornhoffer, J. L., & King, D. (2008). The effect of the Meniette device in patients with Meniere's disease: Long-term results. Otology & Neurotology, 29, 868–874.
Doyle, K. J., Bauch, C., Battista, R., Beatty, C., Hughes, G. B., Mason, J., ... & Musiek, F. L. (2004). Intratympanic steroid treatment: A review. Otology & Neurotology, 25, 1034–1039.
Gacek, R. R., & Gacek, M. R. (1996). Comparison of labyrinthectomy and vestibular neurectomy in the control of vertigo. Laryngoscope, 106, 225–230.
McCall, A. A., Ishiyama, G. P., Lopez, I. A., Bhuta, S., Vetter, S., & Ishiyama, A. (2009). Histopathological and ultrastructural analysis of vestibular endorgans in Meniere's disease reveals basement membrane pathology. BMCEar, Nose and Throat Disorders, 9(4) . Retrieved from www.biomedcentral.com/1472-6815/9/4.
National Institute on Deafness and Other Communication Disorders. (2008). Quick statistics. Retrieved from www.nidcd.nih.gov/health/statistics/quick.htm .
Sajjadi, H., & Paparella, M. M. (2008). Meniere's disease. Lancet, 372, 406–414.