Meniere Disease (Idiopathic Endolymphatic Hydrops) Clinical Presentation

The American Academy of Otolaryngology–Head and Neck Surgery Foundation (AAO-HNS) Committee on Hearing and Equilibrium published guidelines on the clinical diagnosis of Ménière disease in 1972, 1985, and 1995. According to these guidelines, Ménière disease is defined as “recurrent, spontaneous episodic vertigo; hearing loss; aural fullness; and tinnitus. Either tinnitus or aural fullness (or both) must be present on the affected side to make the diagnosis.”^[15]

Accordingly, the typical history involves episodic attacks of true whirling vertigo, which usually are preceded by a variable sense of ear pressure and fullness, decreased hearing, and a low-tone roaring tinnitus. Fewer than one third of patients present with all of the diagnostic components of the disease at onset. Vertigo is the most common initial component, with additional components appearing after a delay of months to years.^{[12, 16]}

Vertigo

Vertigo is a subjective sensation of motion while motionless. Horizontal or rotatory nystagmus is always present during vertiginous attacks.^[15] The vertiginous attacks may last from minutes to hours and often are associated with severe nausea and vomiting. At least 2 definitive episodes of vertigo of at least 20 minutes duration must have occurred to make the diagnosis.^[15] In 10% of patients with the symptom of vertigo, Ménière disease is the cause.^[17]

Acute attacks of vertigo may be accompanied with sudden falls without loss of consciousness. These are termed crises of Tumarkin or drop attacks.^[18] Most studies find the incidence of drop attacks to be less than 10%. In one case series, self-reporting of drop attacks was 72% among patients with diagnosis of Ménière disease.^[19]

After the acute attack, patients generally feel tired, unsteady, and nauseated for hours to days. The timing and frequency of attacks are variable. Some patients can regularly predict an attack while others note a completely random pattern. Attacks may be linked to dietary triggers, the menstrual cycle, or psychosocial stresses.

Between episodes, some patients are completely symptom free. Many notice progressive deterioration of hearing and balance function with each successive attack.

Hearing loss

Sensorineural hearing loss must be documented audiometrically in the affected ear at least once during the course of the disease. There may be fluctuation in the degree of hearing loss superimposed on a gradual decrement in function. The hearing loss primarily affects low frequencies.

Tinnitus

Tinnitus is often nonpulsatile and may be described as whistling, although the classic description is that of low-tone, ocean-like roaring. It may be continuous or intermittent, usually corresponding to the loss of hearing during the attack.

Examination results vary, depending upon the phase of disease. During remission, physical examination findings may be completely normal, particularly if the patient is symptom free.

During an acute attack, the patient has severe vertigo. Patients are often in significant distress. Many present to the physician’s office clutching a bucket and towel with signs of recent vomiting. Patients are sometimes diaphoretic and pale. Vital signs may show elevated blood pressure, pulse, and respiration. Significant spontaneous nystagmus may be present.

Otoscopy findings are usually normal, although pneumo-otoscopy of the affected ear may elicit symptoms or cause nystagmus. The Romberg test and gait testing may show some instability. The Fukuda marching step test may show significant deviation (if the patient can stand with closed eyes). Hearing usually is diminished on gross examination.

A complete neurologic examination is necessary to differentiate Ménière’s disease from other conditions. New-onset vertigo might be an early sign of stroke, migraine, or brainstem compression that may require emergent evaluation and care.

Evaluation of vertigo

The Dix-Hallpike positional test (also known as the Nylen-Bárány maneuver) is performed.^[20] A positive test result may be indicates coexisting benign positional vertigo. The Dix Hallpike test is performed as described below.

The patient is positioned in the middle of the table so that the head extends past the head of the bed when he or she is supine. The patient is then rapidly moved backward so that the head hangs over the edge, and the eyes are observed for evidence of nystagmus. If no nystagmus is observed over a 20-second period, the patient is returned to the upright position. The next step is to bring the patient rapidly to the head-right supine position, again looking for nystagmus. The maneuver is then repeated for the head-left supine position.

Any nystagmus or symptoms, as well as the position the head was in when these symptoms or signs were elicited, should be noted. Nystagmus typically has a latency of 2-5 seconds. Nystagmus and vertigo associated with peripheral causes such as Ménière disease should be fatigable. Central lesions should have no latency and do not fatigue.

If classic findings of benign paroxysmal positional vertigo are found during Dix-Hallpike testing, then canalith-repositioning procedures may be performed.^[21] Care should be taken to ask about preexisting spine issues and/or neck issues before performing the Dix Hallpike test in order to prevent injury.

The Romberg test generally shows significant instability and worsening during acute attacks when the eyes are closed.

The Hennebert sign is nystagmus caused by positive and negative pressure in the external auditory canal.^[9]

The Tullio phenomenon is sound-induced vertigo, nystagmus, or both.^[22] It is historically associated with syphilis but has been described in Ménière disease.

Evaluation of hearing loss

Gross evaluation of the patient’s hearing can be performed by gently rubbing the examiner’s fingers near the patient’s ears.

The Rinne test is performed with a 512-MHz tuning fork. It usually indicates that air conduction remains better than bone conduction.

The Weber test is also performed with a 512-MHz tuning fork. Normally, sound should be heard equally on both sides. With middle ear disease or blockage of the external auditory canal, or any other types of conductive hearing loss, the sound is more pronounced on the affected side. The tuning fork is said to “lateralize” to the affected side in conductive loss. With cochlear nerve dysfunction, the sound is more pronounced on the unaffected side (the usual finding in these patients). The tuning fork is said to lateralize to the unaffected side (the better hearing ear) in sensorineural loss.

Audiologic testing is more accurate than either of these tests (see Workup).

Complications of Ménière disease may include the following:

• Injury due to falls
• Anxiety regarding symptoms
• Accidents due to vertigo spells
• Disability due to unpredictable vertigo
• Progressive imbalance and deafness
• Intractable tinnitus