Meniere’s disease is a chronic illness that affects a substantial number of patients every year worldwide. The disease is characterised by intermittent episodes of vertigo lasting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure. Although there is currently no cure, more than 85% of patients with Meniere’s disease are helped by either changes in lifestyle and medical treatment, or minimally invasive surgical procedures such as intratympanic steroid therapy, intratympanic gentamicin therapy, and endolymphatic sac surgery. Vestibular neurectomy has a very high rate of vertigo control and is available for patients with good hearing who have failed all other treatments. Labyrinthectomy is undertaken as a last resort and is best reserved for patients with unilateral disease and deafness.
Tracking the prevalence and incidence of this disorder is difficult. It is diagnosed based on the presence of a cluster of symptoms which may not occur in unison, making it less likely for a physician to make the diagnosis. Meniere’s disease appears to predominantly affect adults around the age of 40 years, with a range of onset between 20-60 years. The disorder does affect a small number of children younger than 20 years, and these cases comprise around 3% of all Meniere’s cases. In addition, women seem to be slightly more likely to suffer from Meniere’s disease than men with a 1.3:1 preponderance. There is evidence that Meniere’s disease has a genetic component. Morrison found an autosomal dominant inheritance pattern with 61% penetrance for 41 families.
Familial Ménière's disease: clinical and genetic aspects
The symptoms of Ménière's are variable; not all sufferers experience the same symptoms. However, so-called "classic Ménière's" is considered to have the following four symptoms:
Periodic episodes of rotary vertigo or dizziness, but no longer than 24 hours.
Fluctuating, progressive, unilateral (in one ear) or bilateral (in both ears) hearing loss, usually in lower frequencies.
Unilateral or bilateral tinnitus.
A sensation of fullness or pressure in one or both ears.
Nausea, vomiting, and sweating sometimes accompany vertigo, but are symptoms of vertigo, and not of Ménière's.
Some sufferers also experience nystagmus, or uncontrollable rhythmical and jerky eye movements, usually in the horizontal plane, reflecting the essential role of non-visual balance in coordinating eye movements.
There is an increased prevalence of migraine in patients with Ménière’s disease.
Diagnosis is establish with complaints and medical history. However, a detailed otolaryngological examination, audiometry and head MRI scan should be performed to exclude a tumour of the eighth cranial nerve or superior canal dehiscence which would cause similar symptoms. There is no definitive test for Ménière's, it is only diagnosed when all other causes have been ruled out.
The American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium (AAO HNS CHE) set criteria for diagnosing Ménière's, as well as defining two sub categories of Ménière's: cochlear (without vertigo) and vestibular (without deafness).
Certain - Definite disease with histopathological confirmation
Definite - Requires two or more definitive episodes of vertigo with hearing loss plus tinnitus and/or aural fullness
Probable - Only one definitive episode of vertigo and the other symptoms and signs
Possible - Definitive vertigo with no associated hearing loss
Tests may include:
- Blood tests to check for an underlying cause
- Hearing test this is also called an audiometry
- Electronystagmogram a type of eye movement test
- Auditory brainstem response measures electrical activity in the hearing nerve and brain stem
- Electrocochleogram measures electrical response of the inner ear to sound
- MRI scan a test that uses magnetic waves to make pictures of structures inside the ear
The center of production for endolymph is a structure in the cochlea called the stria vascularis. Once the endolymph is created it is distributed throughout the scala media of the cochlea, and is then absorbed into the endolymphatic duct and sac. The endolymphatic sac of Meniere’s patients can malfunction, thus not allowing the endolymph to be reabsorbed. An endolymphatic sac blockage can cause the endolymph volume to increase along with the pressure within the cochlea. This could lead to the perception of aural fullness, hearing loss, and tinnitus. The natural biochemical mechanism for relieving this endolymphatic sac blockage is to release hormones which cause an increase in endolymph, creating even more hydraulic pressure in the cochlea. Eventually there is a release of this endolymphatic sac blockage, allowing the pressure of the cochlea as well as the endolymph volume to return to baseline. This pressure release is responsible for the vertigo symptoms that occur during a full-blown Meniere’s attack.
Although its pathogenesis has been proposed to be a disorder of water transport in the inner ear, subsequently, it remains unsolved, until now. A recent study revealed that both plasma stress hormone, vasopressin (pAVP) and its receptor, V2 (V2R) expression in the inner ear endolymphatic sac were significantly higher in Meniere's patients.
Expression and translocation of aquaporin-2 in the endolymphatic sac in patients with Meniere's disease.
Role of Coenzyme Q10, 2010
PATIENT RISK FACTORS
Ménière's disease is idiopathic, but it is believed to be related to endolymphatic hydrops or excess fluid in the inner ear.
There are some risk factors:
- Age: 20 to 60
- Race: Caucasian
- Sex: female
- Family history of Meniere's disease
- High-salt diet
- High-sugar diet
- Otitis media
- Recent viral illness
- Infection of Herpes Virus
- Excess noise
- Excess alcohol
- Use of drugs that can be toxic to the ear such aminoglycosides, aspirin, and quinine
- Use of PPI
Other possible conditions that may lead to Ménière's symptoms include syphilis, Cogan's syndrome, autoimmune disease of the inner ear, dysautonomia, perilymph fistula, multiple sclerosis, acoustic neuroma, and both hypo- and hyperthyroidism.
The unpredictable episodes of vertigo are usually the most debilitating problem of Meniere's disease. The episodes often force a person to lie down for several hours and lose time from work or leisure activities, and they can cause emotional stress.
Meniere's syndrome can also increase risk of:
- Accidents while driving a car or operating heavy machinery
- Depression or anxiety in dealing with the disease
- Permanent hearing loss
- Drugs to treat vertigo, such as meclizine or scopolamine
- Antiemetics—medications to help control nausea
- Other medications that may improve hearing, control inner ear swelling, or limit overall symptoms, including:
- Cortisone drugs for a short time
- Antidepressants or anti-anxiety medications
- Aminoglycoside therapy (such as streptomycin or gentamicin) to permanently destroy the part of the inner ear that deals with balance
Surgical procedures are not always helpful, and include:
- Endolymphatic sac decompression—removal of a portion of inner ear bone and placing a tube in the inner ear to drain excess fluid
- Labyrinthectomy—destruction or removal of the entire inner ear, which controls balance and hearing
- Vestibular nerve section
Vestibular Exercises (Vestibular Rehabilitation)
Dietary and Lifestyle Changes
These may help limit symptoms:
- Bed-rest during acute attacks of vertigo
- Avoid foods that are high in salt and high in sugar
- Drink adequate fluids
- Promptly begin replacing fluids lost to heat or exercise
- Avoid caffeine, aspirin, and smoking
- Minimize stress
- Avoid medications that seem to bring on or worsen symptoms
- Consider a hearing aid, if necessary
- Consider masking devices (white noise) to limit the effects of tinnitus
- Take safety measures to avoid falling
- Restrict chocolate consumption
- Reduce alcohol intake
- B6 supplementation
Vitamin B6 (Pyridoxine)
B6 supplementation may be effective in vertigo, including Meniere’s syndrome. One study found that 15 of 47 patients with Meniere’s disease who had failed to respond to previous treatments improved with B6 (pyridoxine). Three patients failed to improve and others gradually improved but it was uncertain whether pyridoxine supplementation was responsible. Furthermore, 23 patients with vertigo due to unknown causes received pyridoxine and many of them responded. Intravenous administration relieved symptoms more rapidly than oral administration
[Lewy A., Fox N. Clinical notes; New instruments and techniques: pyridoxine (B6) used in the treatment of vertigo. Arch Otolaryngol November, 1947, pp.681- 3]
Vitamin B3 (Niacin)
Individual reports have suggested that taking vitamin B3, especially as niacin, along with vitamin B6 improves their response. In addition, on bad days, using a B-complex several times a day instead of only once can help also.
History of Meniere's disease and its clinical presentation.
Incidence of Ménière's disease
Prevalence of Menière's disease in general population of Southern Finland
Meniere's disease and the use of proton pump inhibitors
National Institute on Deafness and Other Communication Disorders
Beatrice Elena e Onesti Federica