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Meniere's Disease

Meniere%27s Disease

Meniere's disease is a disorder of the inner ear affecting hearing and balance.


Presentation

Meniere's disease tends to present in episodes which can either be frequent or have long periods of remission. Between attacks, patients experience no symptoms [7]. The major clinical features are:

  • Vertigo: This is the most disturbing clinical feature of this condition. Patients experience a feeling that they or their surroundings are moving or spinning. Apart from a spinning sensation, they may also feel dizzy, uneasy and have an imbalance sensation. Nausea and vomiting may be present. Each episode lasts for about 20 minutes to a couple of hours. Drop attacks may occur. Attacks tend to disrupt the entire schedule and each episode ends in the patient feeling sleepy. A sensation of being disoriented maybe present for a few days after an attack.
  • Tinnitus: This is more prominent when the patient is exhausted or the surroundings are very quiet. They may hear noises and sounds coming from inside the body like buzzing, humming and hissing.
  • Hearing loss: In the early stages hearing loss is not noticeable, but with progression of disease, hearing loss for all frequencies becomes prominent and permanent. Initially, deafness maybe there only for low frequencies.

Ear fullness is perceived prior to an attack of vertigo and it feels as if the ear has been plugged and is full of pressure. Thus, a typical attack consists of vertigo, tinnitus and loss of hearing. Towards the later stages vertigo reduces while tinnitus and deafness remain permanent. The presentation may vary from individual to individual.

Falling
  • If you feel dizzy, sit or lay down to prevent falling. Also be aware that you may lose your balance, so take precautions against injuring yourself in a fall.[belmarrahealth.com]
  • Vertigo may be accompanied by: Nausea or vomiting Sweating Paleness of the skin Weakness or falling In some cases, headache or diarrhea Fluctuating hearing loss Tinnitus, a ringing in one or both ears Feeling of fullness or pressure in the ear Poor sense[uvahealth.com]
  • Some people with Meniere's disease have "drop attacks" during which the dizziness is so bad they lose their balance and fall. Scientists don't yet know the cause.[medlineplus.gov]
  • Vertigo may be accompanied by: Nausea or vomiting Sweating Paleness of the skin Weakness or falling In some cases, headache or diarrhea Fluctuating hearing loss Ringing in one or both ears—tinnitus Feeling of fullness or pressure in the ear Poor sense[lahey.org]
Pallor
  • Nausea, vomiting, diarrhea, pallor, and sweating may all accompany an acute attack. Hearing may return to normal after an attack, but repeated attacks will usually decrease its acuity.[docneuro.com]
Turkish
  • One of the intervention studies was published in Turkish and we sought help from the Cochrane ENT Group to translate this article ( Akkuzu 2006 ).[doi.org]
Nausea
  • Nausea, vomiting, and sweating often occur. Symptoms get worse with sudden movement. Often, you will need to lie down and close your eyes. You may feel dizzy and off-balance for anywhere from 20 minutes to 24 hours.[medlineplus.gov]
  • […] habituation of nystagmus and the vertigo, nausea, and malaise symptoms with repeated maneuvers.[docneuro.com]
  • A 51-year-old woman, with complete remission after optimal maximal debulking and chemotherapy for an International Federation of Gynecology and Obstetrics IIIc primary ovarian carcinoma, presented with nausea, vomiting, vertigo and headache 18 months[ncbi.nlm.nih.gov]
  • Both anti-vertigo and anti- nausea medications may cause drowsiness.[medicinenet.com]
Vomiting
  • Nausea, vomiting, and sweating often occur. Symptoms get worse with sudden movement. Often, you will need to lie down and close your eyes. You may feel dizzy and off-balance for anywhere from 20 minutes to 24 hours.[medlineplus.gov]
  • Symptoms include sudden (acute), unprovoked attacks of severe, disabling vertigo, nausea, and vomiting. Vertigo is a false sensation that people, their surroundings, or both are moving or spinning.[merckmanuals.com]
  • A 51-year-old woman, with complete remission after optimal maximal debulking and chemotherapy for an International Federation of Gynecology and Obstetrics IIIc primary ovarian carcinoma, presented with nausea, vomiting, vertigo and headache 18 months[ncbi.nlm.nih.gov]
  • The two medicines usually recommended by GPs are: prochlorperazine – helps relieve severe nausea and vomiting antihistamines – help relieve mild nausea, vomiting and vertigo The aim is to get the medicine into the body as soon as possible at the first[nhs.uk]
Tinnitus
  • Tinnitus (ringing in the ears, from mild to severe) Often the tinnitus is accompanied by ear pain and a feeling of fullness in the affected ear. Usually, the tinnitus is more severe before a spell of vertigo and lessens after the vertigo attack.[en.wikipedia.org]
  • (mean pre/postoperative tinnitus handicap inventory test: 77.2/6), and finally increasing the overall quality-of-life parameters.[ncbi.nlm.nih.gov]
  • Thus, a typical attack consists of vertigo, tinnitus and loss of hearing. Towards the later stages vertigo reduces while tinnitus and deafness remain permanent. The presentation may vary from individual to individual.[symptoma.com]
  • Tinnitus Grade I outcome measure Ricci 1987 : All 10 patients had tinnitus and none reported improvement on betahistine or placebo. Schmidt 1992 found no difference in tinnitus between the betahistine and placebo groups over four months. 4.[doi.org]
Fluctuating Hearing Loss
  • The pathogenesis is endolymphatic hydrops and osteitis of the otic capsule which produces the characteristic fluctuating hearing loss, tinnitus and ear pressure associated with episodic spells of vertigo.[ncbi.nlm.nih.gov]
  • Clinical Information Fluctuating hearing loss, tinnitus, and vertigo resulting from nonsuppurative disease of the labyrinth; swelling of the endolymph-containing structures is the main pathologic finding Applies To Endolymphatic hydrops Lermoyez's syndrome[icd9data.com]
  • Some patients may just have fluctuating hearing loss without vertigo or episodic vertigo without hearing loss. These are termed cochlear hydrops and vestibular hydrops respectively.[ucirvinehealth.org]
Benign Paroxysmal Positional Vertigo
  • Age of onset for Ménière's disease parallels that for benign paroxysmal positional vertigo.[ncbi.nlm.nih.gov]
Ear Fullness
  • Ménière's disease is an inner ear disorder characterized by vertigo attacks, fluctuating low-frequency hearing loss, ear fullness, and tinnitus. Endolymphatic hydrops has long been thought to be the pathological basis for Ménière's disease.[ncbi.nlm.nih.gov]
  • Triggers Meniere's Disease Spinning Hours to Days Hearing Fluctuation, Ear Fullness, Tinnitus Salt, Alcohol BPPV Spinning Seconds to Minutes None Head Position Changes TMJ Variable Seconds to Days Hearing Fluctuation, Ear Fullness, Tinnitus, Ear Pain[fauquierent.net]
  • Tinnitus or ear fullness (aural fullness) need to be present to make the diagnosis An audiogram is helpful to show a hearing loss, and to rule-out other abnormalities.[medicinenet.com]
  • David Geffen School of Medicine at UCLA The molecular and ultrastructural biology of the human blood-labyrinthine barrier in Ménière’s disease Ménière’s disease is characterized by hearing loss, vertigo, tinnitus, and the sensation of ear fullness.[hearinghealthfoundation.org]
Hearing Impairment
  • Two of the three patients initially complained only of recurrent bouts of vertigo, without any tinnitus, ear fullness or hearing impairment.[ncbi.nlm.nih.gov]
  • Definition Meniere's disease or syndrome is a condition characterized by hearing impairment, ringing in the ears (tinnitus), and periodic vertigo (disturbance of balance and equilibrium).[healthcentral.com]
Vertigo
  • Usually, the tinnitus is more severe before a spell of vertigo and lessens after the vertigo attack.[en.wikipedia.org]
  • […] for vertigo complaints.[doi.org]
  • In Case 1 and 3, vertigo, hearing loss and tinnitus recovered soon after medical therapy. In Case 2, however, vertigo recurred and the hearing level on the right side markedly deteriorated.[ncbi.nlm.nih.gov]
Dizziness
  • Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people will have single attacks of dizziness separated by long periods of time.[web.archive.org]
  • These include hearing loss, ringing in the ears, or dizziness. You can't prevent Ménière disease. Treating early symptoms right away may help prevent the condition from getting worse.[medlineplus.gov]
  • This prospective study showed that MIVN improved dizziness and anxiety in these patients.[ncbi.nlm.nih.gov]
Nystagmus
  • Periodic alternating nystagmus (PAN) is a spontaneous horizontal nystagmus that reverses direction periodically. PAN has been reported in acquired and congenital forms.[ncbi.nlm.nih.gov]
  • The patient’s response relative to symptoms and nystagmus should be noted. When observing nystagmus, whether it is horizontal or rotatory, its fatigability, and its extinction with repetition are all important factors.[docneuro.com]
Drop Attacks
  • Vertigo can cause nausea, vomiting and occasionally “drop attacks” which is a sudden onset of severe vertigo so severe that it causes the patient to fall.[californiaearinstitute.com]
  • Some people with Meniere's disease have "drop attacks" during which the dizziness is so bad they lose their balance and fall. Scientists don't yet know the cause.[medlineplus.gov]
  • An uncommon symptom is to have sudden unexplained falls (drop attacks). These are falls without losing consciousness. Drop attacks last just a short time with little associated vertigo. They occur in about 1 in 25 people with Ménière's disease.[patient.info]
  • Tumarkin crises (drop-attacks) may ensue in the end-stages of the disease, and are an indication for prompt intervention to prevent serious injury.[umm.edu]
  • Failure to warn patients of the possibility of drop attacks, which could result in injury, is a pitfall.[emedicine.com]
Ataxia
  • Headache and ataxia may persist for days following an acute episode. Some of the attacks may be severe enough to cause patients to collapse—an event known as a Tumarik crisis.[docneuro.com]
  • Hearing should be good in the opposite ear and there should be no ataxia. There must be objective evidence of unilateral inner ear disease, including a sensorineural hearing loss, usually worse in the low frequencies.[audiologyonline.com]

Workup

The diagnosis is variable as there is no specific diagnostic test. It is based on history and clinical examination [8]. An important feature to note in Meniere's disease which helps differentiate it from other clinical conditions is the intermittent nature of its clinical course.

A complete history as regards to the frequency, intensity, duration and type of attacks should be noted. Degree of hearing loss and tinnitus should also be assessed. An audiometry test should be done to conclude the type of hearing loss. Speech discrimination should be tested for especially in the affected ear. An electronystagmogram is conducted to check for balance and function. Eye movements in a dark room should also be checked as they work in coordination [9]. CT or MRI scans of the skull can be done to rule out any other lesions.

Treatment

Meniere's disease is not curable but various treatments are available to alleviate symptoms and reduce frequency of attacks. After understanding the cause and pattern of the individual case, the physician can very well judge the best treatment option for the patient. Initial line of treatment consists of:

These two therapies alone can help a majority of cases. If symptoms are not controlled with the above methods, there are two non destructive methods which are invasive but do not hamper the anatomy of the ear.

  • Intratympanic injections of either gentamicin or dexamethsone. A temporary opening is made in the ear drum and the drug is injected into the middle ear space once or several times [11].
  • Air pressure pulse generator, in this pressure is transmitted through the ear drum to alter the pressure in the inner ear.

Surgery is the last resort when attacks of vertigo cannot be controlled by above methods.

  • Endolymphatic sac shunt or decompression which actually helps to improve the function of the ear actively by reducing fluid production and improving fluid drainage.
  • Destructive surgeries such as vestibular nerve section help reducing vertigo.
  • Labyrinthecotomy is a surgery where the entire inner ear is removed thus eliminating the function of hearing and balance in the ear [12].

Vestibular rehabilitation can improve the attacks. These exercises also help to maintain the sense of balance. Hearing aids can be used for hearing loss.

Prognosis

There is no permanent cure for this condition though various treatment strategies can improve the outlook. The intensity and severity can vary from individual to individual, thus it is very unpredictable. Some cases have long periods of remission which can last for months to years.

With progress of time, permanent sensorineural deafness and tinnitus sets in. Initially, it begins in one ear but over a period of time, both ears are involved. The approximate number of patients who end up with bilateral Meniere's disease is about 17 to 75%.

Sometimes the condition might be so severe that it can disable a person completely but this is rare. Most of the cases do well with or without any major medical help. The outlook is as unpredictable as the disease [6]. In the later stages when hearing loss is permanent, hearing aids and cochlear implants are used.

Etiology

Although histologically Meniere's disease has been well characterized by endolymphatic hydrops, the exact etiology still remains unclear. Recent research shows a possible role of autoimmune processes, endocrine function, infection, allergy and genetic factors.

The inner ear has a membranous labyrinth which contains endolymph, if there is some problem with the drainage of this fluid it leads to the membranes becoming distended like a balloon due to pressure. This is the hallmark of hydrops. The reason for drainage being affected can be anything from a swelling of endolymphatic sac or other parts of the vestibular system. In some cases, an excess of fluid is secreted or there is presence of an obstruction in the outlet of the endolymphatic duct. This was the traditional thinking regarding the etiology.

Nowadays, it is hypothesized that an immunologic component seems to play in role in the causation. Individuals with autoimmune disorders seem to have Meniere's disease. Immunosuppressive therapy seems to improve hearing loss. Viral infections also maybe involved in the etiopathogenesis, especially the herpes simplex virus.
Strong genetic predisposition exists with a strong familial tendency. Other factors such as middle ear infection, head injury, or acoustic trauma have been postulated as etiological factors.

Epidemiology

As there is no specific diagnostic test for Meniere's disease, the incidence and prevalence of this condition vary. The typical onset is seen between 20 to 50 years of age. Recent studies document that it affects women slightly more than men. Hardly 3% belong to pediatric group [3].

In the United States of America, it affects about 0.2% of the population. In Great Britain, it affects about 157 individuals out of 100,000. It tends to occur with increasing age and is common as a balance related disorder in the elderly [4]. Racial predisposition is still not clearly known. There is no correlation between age of the individual and bilateral involvement, both ears are affected equally.

Sex distribution
Age distribution

Pathophysiology

Endolymphatic hydrops is the single most distinctive feature histologically and there is no general accepted theory regarding the pathophysiology. In Meniere's disease, how the hydrops forms is still unknown with no clear understanding of the etiopathogenesis.

The most widely accepted theory is the reabsorption of endolymph which leads to collection of fluid and distension of the membranes like a balloon. Electron microscopy shows perisacular degeneration and fibrosis while epithelial cells are replaced by mesenchymal cells [5]. With increase in fluid, a rupture of the membranous labyrinth mainly the Reissner's membrane takes place, as it is the thinnest. This leads to mixing of perilymph and endolymph which changes the electrochemical properties of the two fluids. This produces vertigo and nystagmus due to potassium rich endolymph.

Due to rupture of the membrane, open fistula spaces form between perilymph and endolymphatic spaces. Neural damage especially sensorineural deafness for low frequencies, tinnitus and aural fullness occur. Healing of ruptured membranes accompanied by release of pressure follows. Repeated episodes lead to deafness for all frequencies and ultimately, irreversible pathological changes.

Prevention

There is no known preventive measure for Meniere's disease, but certain steps can be followed to reduce attacks.

Summary

Recurrent spontaneous attacks of vertigo, hearing loss, tinnitus and a sense of aural fullness are the symptoms commonly experienced by an individual suffering from a condition called Meniere's disease. This disorder is also known as endolymphatic hydrops. It is a condition related to the inner ear wherein there is irregularity in the flow of the fluid in the inner ear. Initially it affects only one ear, but with time it tends to become bilateral [1].

The exact etiopathogenesis is not known and most of the cases remain idiopathic. Symptoms fluctuate and typically each attack lasts for 20 minutes to about 4 hours or more. Vertigo is rotational. Hearing loss which ultimately leads to deafness also occurs. Diagnosis is mainly based on history and examination. Acute attacks require bed rest and assurance. Surgery or pharmacological ablation of vestibular organ is considered in very severe disease [2].

Patient Information

Meniere's disease is a condition of the inner ear, which leads to episodes of vertigo, hearing loss and noises in the ear (tinnitus). Each episode may vary and there can be long periods of no symptoms at all. The reason for this condition is not known. A combination of genetic and autoimmune factors seem to play a role. This condition is more common in advanced age groups. As the disease progresses hearing loss becomes permanently impaired and by that time, both ears are affected.

Diagnosis is made by a medical physician based on clinical history and certain tests such hearing tests and scans. There is no cure and the goal of treatment is to reduce frequency of attacks and relieve symptoms. During an attack patient is advised to lie down and take complete rest without focusing on moving objects. Sleep will improve the condition of the patient. Driving is not advised unless symptoms are controlled. Medications and low salt diet can help to obtain a good control over this disease but if still no improvement then surgery can be resorted to as a last option.

References

Article

  1. Ervin SE. Meniere's disease: identifying classic symptoms and current treatments. AAOHN J. 2004;52:156-158.
  2. Beasley NJ, Jones NS. Menière's disease: evolution of a definition. J Laryngol Otol.1998; 110 (12): 1107–13.
  3. Lempert T, Neuhauser H. Epidemiology of vertigo, migraine and vestibular migraine. Journal of Neurology.2008; 256 (3): 333–338.
  4. Eaton DA, Roland PS. Dizziness in the older adult, part 2: treatments for causes of the four most common symptoms. Geriatrics. 2003;58:46, 49-52.
  5. Cawthorne T. Ménière's disease. Annals of Otology.1947; 56: 18–38.
  6. Kinney SE, Sandridge SA, Newman CW. Long-term effects of Meniere's disease on hearing and quality of life. Am J Otol 1997 Jan;18(1):67-73.
  7. Mancini F, Catalani M, Carru M, Monti B. History of Meniere's disease and its clinical presentation. Otolaryngol Clin North Am. 2002;35:565-580.
  8. Crummer RW, Hassan GA. Diagnostic approach to tinnitus. Am Fam Physician. 2004;69:120-126.
  9. Black FO, Effron M Z and Burns DS. Diagnosis and management of drop attacks of vestibular origin: Tumarkin's otolithic crisis. Otolaryngol Head Neck Surg.90.1982;(2): 256-62.
  10. Santos PM, Hall RA, et al. Diuretic and diet effect on Meniere's disease evaluated by the 1985 Committee on Hearing and Equilibrium guidelines. Otolaryngol Head Neck Surg.1993; 109(4): 680-9.
  11. Schwaber MK. Transtympanic gentamicin perfusion for the treatment of Meniere's disease. Otolaryngol Clin North Am. 2002;35:287-295.
  12. Berryhill WE, Graham MD. Chemical and physical labyrinthectomy for Meniere's disease. Otolaryngol Clin North Am. 2002;35:675-682.

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Last updated: 2019-07-11 21:46