Dinesh Kumar Sharma

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/66486

#### **Abstract**

Meniere's disease is a progressive disorder characterized by recurrent episodes of spontaneous vertigo, sensorineural hearing loss and tinnitus, often with a feeling of fullness in the ear. The exact ethology is not known. In 1972, a diagnostic criterion for Meniere's disease was proposed by American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS), and till date, it has been revised twice in the years 1985 and 1995. The principal audiological investigation is pure tone audiometry combined with a glycerol test. Speech audiometry and otoacoustic emissions also play a limited role. The value of electrocochleography is limited.

**Keywords:** pure tone audiometry, glycerol test, speech audiometry, OAE, electrocochleography

## **1. Introduction**

Meniere's disease named after an Italian scientist, Prosper Meniere, is a progressive disorder characterized by recurrent episodes of spontaneous vertigo, sensorineural hearing loss and tinnitus, often with a feeling of fullness in the ear [1]. The characteristic of the disease is that it is an unpredictable, fluctuating illness with noteworthy hidden disability [2].

The precise cause of Meniere's disease is still being investigated. It is believed to be associated with endolymphatic hydrops, that is, raised endolymph pressure in the membranous labyrinth of the inner ear, which gets dilated like a balloon when pressure increases and drainage is blocked. This results in swelling of the endolymphatic sac and other tissues in the vestibular system (responsible for the body's sense of balance), creating an acute vestibular imbalance, resulting in vertigo and fluctuating hearing loss [3].

Another suggested etiology is the autoimmune nature of the disease. The idea of autoimmunity was brought forward when improvement in bilateral progressive sensorineural hearing

loss was recorded following immunosuppressive therapy. The studies of the human endolymphatic sac have also suggested that it is the primary immunocompetent structure in the inner ear, which is capable of processing antigen, synthesizing antibodies and raising a cellular immune response [4].

No single test that makes the diagnosis of Meniere's disease with conformity has been established. Complete history, including a detailed description of the pattern of disease presentation supported by quantitative testing, helps in arriving at a diagnosis [5].

A number of international interdisciplinary organizations in a consensus paper have drawn diagnostic criteria for Meniere's disease. The paper suggests two categories of the Meniere's disease, definite and probable. The definition of definite variety incorporates a clinical criteria and observation of episodes of vertigo associated with audiometric findings of sensorineural hearing loss involving low and middle frequencies and triad of symptoms that include fluctuant hearing loss, tinnitus and/or fullness of the involved ear. The criteria limit the duration of vertigo from 20 min to 12 h. The definition of probable variety of Meniere's disease encompasses vertigo or dizziness and extends to episodic ear-related symptoms, which may occur for a variable period time between 20 min and 24 h [2].

The natural history of Meniere's disease is inconstant but usually progressive. The classical triad of tinnitus or aural fullness with episodic vertigo and hearing impairment is often not seen at the beginning of the disease. The disease starts as a single symptom entity, and only cochlear symptoms occur at the first stage. The period between the primary symptoms and the manifestation of other symptoms varies from months to several years with an estimated average of 6–18 months. After this period of variable duration, the complete triad of symptoms will appear [6].

Episodic attacks of vertigo (so-called Meniere's attack) are the most troublesome of the symptoms to the patient, and it is usually the symptom that causes the patient to seek medical treatment. The vertigo patient perceives either that the world is spinning around them or that they themselves are spinning. Typically, it occurs in the form of a series of attacks over a period of weeks or months, interspersed by periods of remission of variable duration [7].

When the patient experiences a feeling of rotation, the sign is nystagmus, which has been described as a condition of involuntary movements of eyeball. This is accompanied by other symptoms such as giddiness and sweating [8].

On most occasions, patients experience a heaviness or fullness of the involved ear, which is accompanied by impairment of hearing and ringing sensation. Often the beginning of symptoms is precipitous, which reaches its zenith within a span of minutes to hours. The entire episode persists for an hour so before it wanes. The patient may experience unsteadiness for a couple of hours or days after the attack subsides. In between episodes of the disease, subjects may suffer from positional vertigo [9].

Vertigo is the most disabling one among the cardinal symptoms of the disease. It adversely affects almost every aspect of life disturbing the normal lifestyle of the patient. The vertigo is made worse especially when movement is involved. Patient's ability to lead a normal way life is hampered by risks of fall. The chances of such events are made worse by small head movements, which make the patient subjectively very "ill." Vertigo can completely undermine the individual. This leads the patients to confine themselves to bed until the symptoms improve [10].

loss was recorded following immunosuppressive therapy. The studies of the human endolymphatic sac have also suggested that it is the primary immunocompetent structure in the inner ear, which is capable of processing antigen, synthesizing antibodies and raising a cel-

No single test that makes the diagnosis of Meniere's disease with conformity has been established. Complete history, including a detailed description of the pattern of disease presenta-

A number of international interdisciplinary organizations in a consensus paper have drawn diagnostic criteria for Meniere's disease. The paper suggests two categories of the Meniere's disease, definite and probable. The definition of definite variety incorporates a clinical criteria and observation of episodes of vertigo associated with audiometric findings of sensorineural hearing loss involving low and middle frequencies and triad of symptoms that include fluctuant hearing loss, tinnitus and/or fullness of the involved ear. The criteria limit the duration of vertigo from 20 min to 12 h. The definition of probable variety of Meniere's disease encompasses vertigo or dizziness and extends to episodic ear-related symptoms, which may occur

The natural history of Meniere's disease is inconstant but usually progressive. The classical triad of tinnitus or aural fullness with episodic vertigo and hearing impairment is often not seen at the beginning of the disease. The disease starts as a single symptom entity, and only cochlear symptoms occur at the first stage. The period between the primary symptoms and the manifestation of other symptoms varies from months to several years with an estimated average of 6–18 months. After this period of variable duration, the complete triad of symp-

Episodic attacks of vertigo (so-called Meniere's attack) are the most troublesome of the symptoms to the patient, and it is usually the symptom that causes the patient to seek medical treatment. The vertigo patient perceives either that the world is spinning around them or that they themselves are spinning. Typically, it occurs in the form of a series of attacks over a period of

When the patient experiences a feeling of rotation, the sign is nystagmus, which has been described as a condition of involuntary movements of eyeball. This is accompanied by other

On most occasions, patients experience a heaviness or fullness of the involved ear, which is accompanied by impairment of hearing and ringing sensation. Often the beginning of symptoms is precipitous, which reaches its zenith within a span of minutes to hours. The entire episode persists for an hour so before it wanes. The patient may experience unsteadiness for a couple of hours or days after the attack subsides. In between episodes of the disease, subjects

Vertigo is the most disabling one among the cardinal symptoms of the disease. It adversely affects almost every aspect of life disturbing the normal lifestyle of the patient. The vertigo is made worse especially when movement is involved. Patient's ability to lead a normal way

weeks or months, interspersed by periods of remission of variable duration [7].

tion supported by quantitative testing, helps in arriving at a diagnosis [5].

for a variable period time between 20 min and 24 h [2].

symptoms such as giddiness and sweating [8].

may suffer from positional vertigo [9].

lular immune response [4].

72 Up to Date on Meniere's Disease

toms will appear [6].

Some sufferers experience "drop attacks," which are sudden, severe unexplained falls without loss of consciousness or associated vertigo. These drop attacks are due to acute utriculosaccular dysfunction and are triggered by changes in inner ear pressure affecting otolith function [11].

Another unusual pattern of clinical presentation has been described, known as Lermoyez attacks. As opposed to typical spells in which tinnitus and hearing loss precede and worsen with the onset of vertigo, in Lermoyez attacks increased tinnitus and hearing loss precede the vertiginous episode and dramatically resolve with onset of vertigo [12].

Tinnitus experienced by Meniere's patients is continual and does not abate with time, although its intensity may vary. In addition, it may be heard more as a loud roaring or buzzing sensation, rather than a whistling, and is most commonly non-pulsatile and of the lowfrequency type. The pitch tends to be related to the region of the most severe hearing loss and the magnitude of tinnitus roughly proportional to the severity of hearing loss [13].

A sensation of aural fullness that may precede a definite vertiginous spell, is considered a symptom alternative to tinnitus in the criteria of AAO-HNS (1985, 1995) and is experienced by 74.1% of the patients [14].

The hearing loss usually affects one ear, which typically loses sensitivity to low-frequency sounds and is of sensorineural type. As the hearing thresholds rise, dynamic range decreases, the sounds are typically described as "tinny" by the patient, the quality of sounds becomes poor, and loudness of loud sounds rises rapidly due to a phenomenon known as recruitment. The patients become intolerant to such loud sounds. During the early days of the disease, the hearing loss tends to return to within normal thresholds, and however, later in course of disease, hearing loss persists and even deteriorates over the course of following episodes. Even in terms of frequency involvement, the hearing loss spreads to involve all the frequencies showing a flat line on the audiogram. The sensorineural hearing loss in Meniere's disease involves low frequencies giving a flat audiometric pattern, but sometimes we get peak audiograms that are nearly normal hearing at around 2 kHz and decreased sensorineural hearing at lower and higher frequencies. This type of pattern is considered to be diagnostic of Meniere's disease and is more commonly seen in patients with disease of short duration. Over time, the hearing loss flattens and becomes less variable [15].

Patients become profoundly deaf rarely in 1–2% of severely affected patients [2].

Additional features are diplacusis that is unusual sensitivity to noises, sounds can seem tinny or distorted known as dysacusis, a difference in the perception of pitch between the ears (43.6%) and recruitment (56%) [16].
