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.
Entire Body System
- Disability
Meniere disease is a disorder characterized by recurring attacks of disabling vertigo (a false sensation of moving or spinning), fluctuating hearing loss (in the lower frequencies), and noise in the ear (tinnitus). [merckmanuals.com]
Topic Resources Meniere disease is a disorder characterized by recurring attacks of disabling vertigo (a false sensation of moving or spinning), fluctuating hearing loss (in the lower frequencies), and noise in the ear (tinnitus). [msdmanuals.com]
A disabling episode of vertigo may involve severe nausea and vomiting. In addition, Meniere's disease can muffle or impair hearing. Individuals may also experience a sensation of increased pressure in the ear. [diversalertnetwork.org]
- Falling
A Matter of Balance Balance disorders fall under several broad categories including peripheral vestibular disorder, central vestibular disorder, systemic disorder and vascular disorder. [tinnitusformula.com]
Vertigo can cause you to lose balance, increasing your risk of falls and accidents. [mayoclinic.org]
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 [winchesterhospital.org]
[…] of fullness in the ears develops shortly before, at the onset, or during episodes. 2 At episode onset, patients may suffer from sudden “drop attacks,” which are periods of strong sensation of directional tilting causing patients to overcompensate and fall [pharmacytimes.com]
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]
- Surgical Procedure
People who still have frequent, severe episodes despite these treatments may need a more invasive surgical procedure. [merckmanuals.com]
Several reliable surgical procedures aimed to control the vertigo associated with Meniere's disease exist. Vestibular preservation and ablative procedures are available with the latter divided between hearing preservation and destructive operations. [ncbi.nlm.nih.gov]
Different surgical procedures have been advocated for patients with persistent, debilitating vertigo from Ménière's disease. An endolymphatic sac decompression and shunt insertion can help improve Meniere’s disease in up to 75% of cases. [earassociates.com]
- 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]
Gastrointestinal
- Nausea
[…] habituation of nystagmus and the vertigo, nausea, and malaise symptoms with repeated maneuvers. [docneuro.com]
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 disabling episode of vertigo may involve severe nausea and vomiting. In addition, Meniere's disease can muffle or impair hearing. Individuals may also experience a sensation of increased pressure in the ear. [diversalertnetwork.org]
Vertigo is often accompanied by nausea and vomiting. Attacks may last for 20 minutes to two hours or longer and fatigue and an off-balance sensation may last for hours to days. [entsurgicalillinois.com]
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. [nlm.nih.gov]
- Vomiting
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 disabling episode of vertigo may involve severe nausea and vomiting. In addition, Meniere's disease can muffle or impair hearing. Individuals may also experience a sensation of increased pressure in the ear. [diversalertnetwork.org]
Symptoms of Meniere disease 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. [msdmanuals.com]
The dizziness can be treated with antivertiginous (stops dizziness) and antiemetic (stops vomiting) drugs. [hear.com]
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. [nlm.nih.gov]
Ears
- Tinnitus
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]
Some people may experience tinnitus or an increase in tinnitus in the affected ear. The fullness in the ear and tinnitus may precede the attacks of vertigo, but they will often occur without warning. [menieres.org.uk]
While not all tinnitus sufferers experience vertigo, nearly everyone with Meniere's disease experiences severe vertigo, tinnitus, hearing loss and a feeling of fullness in the ears. [tinnitusformula.com]
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]
- Aural Fullness
On a daily basis, participants were then asked to rate their vertigo, aural fullness, tinnitus, and hearing on a scale from 0 to 10. [journals.lww.com]
There was no significant difference in the occurrence of postoperative BPPV, CSF leaks, aural fullness, tinnitus, or vertigo spells. [ncbi.nlm.nih.gov]
Aural fullness Grade I outcome measure Salami 1984 found a statistically significant reduction in aural fullness with betahistine (P < 0.02). [doi.org]
- Fluctuating Hearing Loss
Fluctuating, progressive, unilateral (in one ear) or bilateral (in both ears) hearing loss, often initially in the lower frequency ranges. [disabled-world.com]
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]
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]
The hearing generally returns, but over time, the hearing and balance function are lost with each attack of the vertigo. Some patients may just have fluctuating hearing loss without vertigo or episodic vertigo without hearing loss. [ucihealth.org]
- Ear Fullness
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]
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]
This condition is described as ear fullness and tinnitus that is followed by severe spinning attacks or vertigo that last hours to days. Once the dizziness resolves, hearing and balance is back to normal. [youtube.com]
- Benign Paroxysmal Positional Vertigo
Age of onset for Ménière's disease parallels that for benign paroxysmal positional vertigo. [ncbi.nlm.nih.gov]
Neurologic
- Vertigo
This surgery is performed in an outpatient setting, and after a period of recovery, may help 50% of patients improve symptoms of vertigo and dizziness. [masseyeandear.org]
During attacks, patients may be unable to perform their usual activities, needing to lie down until the vertigo resolves. Hearing loss is often intermittent, occurring mainly at the time of the attacks of vertigo. [entsurgicalillinois.com]
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. [symptoma.com]
Usually, the tinnitus is more severe before a spell of vertigo and lessens after the vertigo attack. Attacks are characterized by periods of remission and exacerbation. [en.wikipedia.org]
- Dizziness
This surgery is performed in an outpatient setting, and after a period of recovery, may help 50% of patients improve symptoms of vertigo and dizziness. [masseyeandear.org]
Diuretics with anti-dizziness medication are also used to reduce pressure buildup. Special diets low in sodium and potassium are frequently recommended. [tinnitusformula.com]
Vertigo, often the most debilitating symptom of Ménière's disease, typically involves a whirling dizziness that forces the sufferer to lie down. [earassociates.com]
So called "classic Meniere's" is considered to comprise the following four symptoms: Periodic episodes of rotary vertigo (the abnormal sensation of movement) or dizziness. [disabled-world.com]
- Nystagmus
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]
In Meniere's disease, spontaneous nystagmus beating toward the affected ear (ipsilateral nystagmus) is frequently observed especially during vertiginous periods. [ncbi.nlm.nih.gov]
- Drop Attacks
It’s uncertain what percentage of people with Meniere’s experience drop attacks, but a study published in the Journal of Vestibular Research in 2018 found that 49 percent of the 602 people with Meniere’s surveyed experienced drop attacks that lasted anywhere [american-hearing.org]
usually the most dramatic and disabling part of the disease, often accompanied by nausea and vomiting — this can vary in frequency and duration (may last many hours) Other: fullness in the ear, discomfort with loud noise, drop attacks, constant sense [sunnybrook.ca]
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]
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]
Tumarkin crises (drop-attacks) may ensue in the end-stages of the disease, and are an indication for prompt intervention to prevent serious injury. [umms.org]
- Hyperactivity
Two phenomena in auditory cortex are associated with peripheral deafferentation: (1) hyperactivity in lesion projection zone and (2) increased cortical representation of lesion-edge frequencies (here, C6) in lesion projection zone. [emedicine.medscape.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:
- Low salt diet with a diuretic to reduce fluid content [10].
- Medication for vertigo, nausea and vomiting such as antiemetics.
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.
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.
- Ear infections should be promptly treated
- Low salt diet
- Avoid sudden jerky movements
- Reduce food triggers such as chocolate, caffeine, tea and alcohol
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
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- 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.
- Cawthorne T. Ménière's disease. Annals of Otology.1947; 56: 18–38.
- 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.
- 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.
- Crummer RW, Hassan GA. Diagnostic approach to tinnitus. Am Fam Physician. 2004;69:120-126.
- 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.
- 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.
- Schwaber MK. Transtympanic gentamicin perfusion for the treatment of Meniere's disease. Otolaryngol Clin North Am. 2002;35:287-295.
- Berryhill WE, Graham MD. Chemical and physical labyrinthectomy for Meniere's disease. Otolaryngol Clin North Am. 2002;35:675-682.