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Meniere disease

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Increase in endolymph associated with episodic vertigo, hearing loss, tinnitus and aural fullness.

Also called idiopathic endolymphatic hydrops.

Reflex idiopathic inner ear degeneration.

One of the most common causes of dizziness originating in the inner ear.

A set of episodic symptoms including vertigo, hearing loss, tinnitus, and a sensation of fullness in the affected ear.

Episodes last from 20 minutes up to 4 hours.

Attacks of rotational vertigo that can be severe, incapacitating, and last anywhere from minutes to hours, but generally no longer than 24 hours.

It is defined by prolonged episodes of vertigo and fluctuating, asymmetric hearing loss that involves pure tone acuity for low pitched sound.

Audiometry is critical for diagnosis.

For some prolonged attacks can occur, lasting from several days to several weeks, often causing severe incapacitation.

Loss of hearing is often intermittent, occurring mainly at the time of the attacks of vertigo.

Hearing loss involves mainly the lower pitches, but over time may affect tones of all pitches.

Loud sounds may be distorted.

After months or years hearing loss often becomes permanent.

A disorder of the inner ear that can affect hearing and balance to a varying degree.

Vertigo may induce nystagmus, usually in the horizontal plane, reflecting the essential role of non-visual balance in coordinating eye movements.

Hearing loss fluctuates then becomes permanently impaired.

Process ranges in intensity from a mild irritation to a chronic, lifelong disability.

Meniere’s disease symptoms are episodic and last hours.

Process often begins with one symptom, and gradually progresses.

Differential diagnosis includes: syphilis, autoimmune disease of the inner ear, dysautonomia, perilymph fistula, multiple sclerosis, Cogan’s syndrome, acoustic neuroma, and both hypothyroidism and hyperthyroidism.

Tinnitus and full sensation of the ear may be transient and be associated with changes in hearing, occur during or before attacks, or may be constant in nature.

Patients may experience an increase in volume of tinnitus.

In most cases only one ear is involved, but both ears may be affected in about 15 percent of patients.

Typically starts between the ages of 20 and 50 years.

Genders affected in equal numbers.

Cause is unknown, but probably results from an abnormality in the volume of fluid in the inner ear, which may accumulate either due to excess production or inadequate absorption.

In some individuals allergies or autoimmune disorders may play a role in producing Meniere’s disease.

Some patients are susceptible to fatigue and stress, and that may influence the frequency of attacks.

Nausea, vomiting, and sweating sometimes accompany vertigo.

Audiometric examination typically indicates a sensory type of hearing loss in the affected ear.

Speech discrimination is often diminished in the affected ear.

An ENG (electronystagmogram) to evaluate balance function finds about 50% of affected patients have reduced function in the affected ear.

Electrocochleography (ECoG) may indicate increased inner ear fluid pressure in some cases.

The intensity of the process can be reduced by lying flat and focusing on a non-moving object.

Patients should avoid excessive salt intake, caffeine, smoking, alcolhol, fatigue and stress.

Therapy includes: a low salt diet, diuretic usage, antivertigo medications, intralymphatic gentimicin or dexamethasone, air pressor pulse generator, and surgery.

Air pressure pulse generator is a mechanical pump that is applied to the ear canal after a ventilating tube is inserted through the eardrum, for five minutes three times a day.

The pressure produced by the air pressure pulse generator is transmitted across the round window membrane and changes the pressure in the inner ear.

While surgery is required in only a minority of cases the procedural choices include: Endolymphatic sac shunt or decompression procedure, selective vestibular neurectomy, and labyrinthectomy and eighth nerve section.

Endolymphatic sac shunt often temporarily relieves attacks of vertigo in one-half to two-thirds of cases and the sensation of ear fullness is often improved.

Endolymphatic sac surgery does not improve hearing, and has a small risk of worsening it.

Selective vestibular neurectomy permanently cures a high percentage of vertigo attacks, but patients may continue to experience imbalance, while preserving hearing function.

Labryrinthectomy and eighth nerve section are procedures in which the balance and hearing mechanism in the inner ear are destroyed on one side, and are considered when the patient has poor hearing in the affected ear.

Labryrinthectomy and eighth nerve section result in the highest rates for control of vertigo attacks.

Sudden, severe attacks of dizziness or vertigo, known informally as “drop attacks,” can cause someone who is standing to suddenly fall.

Drop attacks are likely to occur later in the disease, but can occur at any time.

Medical management includes salt restriction, diuretics, and vestibular suppressants.

Refractory cases includes endolymphatic sac surgery, labyrinthectomy, or vestibular nerve sectioning to control symptoms.

First-line treatment limits and smoothes dietary sodium consumption and includes adjunct treatments of diuretics, and intratympanic corticosteroid injections, and avoidance of dehydration.

In a study of 51 patients that failed medical treatment endolymphatic sac to mastoid shunts were performed and revision sac procedures were performed in patients who developed significant recurrent vertiginous spells 5 months or longer after the original procedure: 24 months after the primary sac surgery 53% has no vertigo and 24% exhibited 1-40% symptoms of baseline studies while 14 patients with revision surgery 36% had no vertigo and 29% has symptoms 1-40% of baseline-77% of patients with endolymphatic sac surgery had improvement in major vertigo spells at 24 months after surgery and with revision surgery 65% improved, with the patients that developed symptoms after 24 months doing the best. (Wetmore S).

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