Meniere disease is an inner ear disorder characterized by hearing loss, tinnitus, and vertigo. It often develops gradually and is associated with considerable social impairment among patients. However, all three symptoms are relatively common and may have numerous causes, whereas Meniere disease is diagnosed mainly by eliminating other possible causes. Current diagnostic criteria proposed by Lopez-Escamez et al. on behalf of the Barany Society have been formulated to distinguish between probable and definite Meniere’s disease. 

According to the Barany Society, patients with definite Meniere disease have: 

Two or more unexpected episodes of vertigo, each lasting between twenty minutes and twelve hours. Low to medium frequency sensorineural hearing loss in one ear audiometrically confirmed prior to, during or following a vertigo episode. 

Subjective hearing loss and/or tinnitus in the affected ear that can vary depending on the position of the head. 

Meniere illness affects 3.5 to 513 individuals per 100,000, with a greater incidence in elderly, white, female patients. Several comorbidities are more frequently observed in patients with Meniere disease: 

Migraine: Migraines occur in most patients with Meniere disease. There will be considerable cases of incorrect identification and overlapping diagnosis of basilar migraine with the Meniere disease. Some theories propose that there is vascular cause for Meniere disease but this is not proven. 

Autoimmune Diseases: Several autoimmune disorders are also associated with Meniere disease as they include rheumatoid arthritis, systemic lupus erythematosus, and ankylosing spondylitis. A few studies suggest IgE as a possible factor due to middle ear samples from patients with Meniere disease. 

Genetic Component: Meniere disease is an inherited condition that has a genetic component. Familial cases account for 10% of the cases recorded in patients of European descent. The condition can manifest autosomal dominant or recessive mode of inheritance but is mostly acquired sporadically. Further research continues to be focused on the exact genetics of Meniere disease. 

Due to this fact the exact pathophysiology of Meniere disease is not known. One of the most common histological findings is endolymphatic hydrops but hydrops does not necessarily lead to MD in all cases of hydrops. It is the inflammation of endolymphatic chamber as well as the dilation of Reissner’s membrane. The exact mechanisms that cause endolymphatic hydrops are also not clearly understood with the current state of knowledge suggesting that it may be due to a disruption of the secretion and resorption of the endolymph in the cochlea. 

It is idiopathic which implies that if a cause for the disease is established, such a case cannot be categorized as Ménière disease. But since the cause of Ménière disease is endolymphatic hydrops – an increase in pressure within the endolymphatic system – it is crucial to discuss other conditions that may lead to similar pressure increase and differentiate Ménière disease from them. 

Conditions that may cause increased endolymphatic pressure include metabolic abnormalities, endocrine diseases, trauma, and numerous infections. Some autoimmune diseases like lupus and rheumatoid arthritis can also elicit inflammation in the labyrinth. Autoimmune etiology has been hypothesized based on the linkage of thyroid autoantibodies to Ménière disease. 

Perez-Garrigues et al. made a survey on Meniere disease and stated that the frequency of vertigo attack is maximum in the first year of the disease and is decreasing over the next years and this dependence is not affected by any kind of treatment. But most patients eventually enter the “steady-state phase” and there is no vertigo, though there is profound hearing loss. 

Hearing loss evolves similarly to vertigo – it is maximal during the first years of illness and progression slows down later with minimal to no recovery. People diagnosed with Meniere disease in one ear have an increased risk of developing it in the other ear; a systematic review finds that the organ of balance becomes affected in the second ear in up to 47% of patients over 20 years. 

Meniere disease mostly occurs in adults between the age of 20 and 60 years but is most predominant between 40 and 50 years. It is frequently connected with headaches, autoimmune diseases, allergies, metabolic disorders, endocrine disorders, infectious diseases, and inheritance, especially in patients of European origin. Tinnitus and vertigo are the most common and incapacitating in the first years of the disease ranging from several minutes to several hours and occasionally associated with nausea and vomiting. Hearing loss develops in the following way: it begins as low and then maintains a steady level. It is often associated with tinnitus and aural fullness that varies in intensity with vertigo and hearing loss. Eventually, vertigo episodes become less frequent and most patients enter a compensated stage where the vertigo is no longer present although hearing loss and other symptoms may remain. 

Neurological Examination: 

Cranial Nerves: Assessment of cranial nerves especially Vestibulocochlear nerve to check for any signs of vestibular- cochlear dysfunction. 

Gait and Balance: Performing gait and balance tests to identify if there is any irregularity that can suggest vestibular system issues. 

Otoscopic Examination: Assessment of the external auditory canal and tympanic membrane to look for any visible abnormalities or signs of infection. 

Audiometric Testing: Quantitative estimation of the presence, nature and severity of hearing loss typical for Meniere disease. 

Lifestyle Modifications: 

Dietary Changes: Restriction of salt consumption to prevent/ alleviate fluid accumulation in inner ear and thus make symptoms worse. 

Hydration: Fluid balance in the body – proper hydration to ensure that the body acquires the required fluids. 

Avoidance of Triggers: Avoiding the consumption of potentially aggravating substances, including caffeine, alcohol, and nicotine. 

Medications: 

Diuretics: To decrease fluid accumulation and assist in regulating pressure within the inner ear. 

Vestibular Suppressants: Medications including meclizine or benzodiazepines may be administered to relieve the person from vertigo during the acute attack. 

Anti-nausea Medications: To prevent nausea and vomiting during vertigo attacks. 

Corticosteroids: Pain relievers to decrease inflammation and relieve symptoms during acute attacks or on short-term use. 

Invasive Interventions: 

Intratympanic Gentamicin Injection: Gentamicin injected into the middle ear to exact damage on the vestibular hair cells to reduce the function of the vestibular system/ labyrinth and relieve the vertigo. This procedure comes with a risk of hearing loss and is usually done after other treatments have proven ineffective. 

Endolymphatic Sac Decompression: Procedure in which an opening is made in the mastoid bone behind the ear to create a channel for draining the inner ear to relieve pressure. 

Vestibular Nerve Section: A surgical technique that involves the disconnection of the vestibular nerve to decrease the amount of input that the vestibular system receives, and which is therefore useful primarily in cases of extreme vertigo that is resistant to other treatments. 

Hearing Aids or Cochlear Implants: In case of sensorineural or SNHL, hearing aids or in some cases cochlear implant may be prescribed to improve the auditory function. 

Cognitive Behavioral Therapy (CBT): CBT can also be important in managing the mind of the patient to overcome fear, anxiety and depression that can be associated with Meniere disease. 

Neurology

Dietary Modifications: 

Low-Salt Diet: Limiting salt consumption improves the function of membranous labyrinth and reduces the frequency and intensity of vertigo episodes. 

Hydration: Fluid balance may be helpful to the inner ear and staying hydrated probably helps. 

Stress Management: 

Stress Reduction Techniques: Meditation techniques like mindfulness meditation, yoga, and other types of relaxation have been found to reduce stress levels, which could be a contributing factor to symptom aggravation. 

Vestibular Rehabilitation Therapy (VRT): 

Balance Exercises: Specific training to re-accommodate balance, stability, and adaptation to changes in the vestibular system can be beneficial to alleviate the symptom of vertigo and possibly improve the overall functioning of the vestibular system. 

Gaze Stabilization Exercises: Training to maintain gaze stability during head movements and reduce dizziness after loss of function. 

Tinnitus Management: 

Sound Therapy: Creating and using white noise or other calming sounds to try and cover up or quieten the perception of tinnitus for patients. 

Avoidance of Triggers: 

Identifying and Avoiding Triggers: Informing the patients on possible triggers like caffeine, alcohol, nicotine, and urging them to stay off these substances to help in the mitigation of symptom flare-ups. 

Assistive Devices: 

Hearing Aids: Hearing aids are devices used in the management of the impaired hearing and they may be used to enhance the hearing function for patients with severe impairment in communication. 

Balance Support Devices: Canes or walkers may be advised to ensure that stability is acquired in situation the patient experiences vertigo attacks. 

Cognitive Behavioral Therapy (CBT): The use of the CBT techniques will assist in the management of the anxiety, depression, and stress that is associated with the living with the Meniere disease condition for better patient’s wellbeing. 

Neurology

Thiazide diuretics primarily act by blocking sodium reabsorption in the kidneys and thereby cause sodium and water loss. This reduction in fluid volume would help to decrease the pressure within the endolymphatic system in the inner ear and may lead to decreased frequency and severity of vertigo attacks associated with Meniere disease. 

Hydrochlorothiazide (HCTZ): It is commonly prescribed among thiazide diuretics for Meniere disease in men. It is commonly administered as a pill that aids in relieving fluid retention by increasing urination. 

Chlorthalidone: A thiazide-like diuretic that can be considered for some patients. It has a more prolonged action than hydrochlorothiazide and is also useful in edema. 

Neurology

Betahistine is a drug used in the treatment of Meniere disease, which is designed to reduce the frequency and severity of vertigo attacks and symptoms of endolymphatic hydrops. It is believed to work as a histamine H1 receptor agonist and H3 receptor antagonist. It enhances blood circulation to the internal ear where this may enhance the balance of pressure stages among the endolymph fluid compartments therefore lowering incidents of vertigo. 

Betahistine Hydrochloride: The hydrochloride salt is the most used form of betahistine. It comes in different strengths and delivery forms (e. g. tablets of 8 mg, 16 mg, and 24 mg). 

Neurology

Intratympanic steroid injections are one of the therapeutic options that are applied in the management of Meniere disease and especially where the conventional treatments are not successful or where the vertigo attacks are too frequent or severe. 

Systemic steroids like dexamethasone or methylprednisolone administrated intratympanically act on the inner ear fluid by suppressing inflammation within the inner ear. 

Intratympanic steroids may be beneficial in Meniere disease by decreasing inflammation and fluid accumulation in the endolymphatic sac and cochlea, which can cause vertigo attacks and fluctuating hearing loss. 

Dexamethasone: It is administered as an injection in the middle ear through the eardrum. It is advantageous in that it has higher concentrations of the medication reaching the inner ear structures thus potentiating the medication while at the same time minimizing the systemic adverse effects of the medication. 

Neurology

Vestibulosuppressants have one of the most important uses in the treatment of Meniere’s disease and they work to reduce the frequency of vertigo attacks and increase the quality of life of patients with the disease. The main mechanism of action of vestibulosuppressants is the reduction in the activity of the vestibular system which is concerned with the inner ear and its role in balance and spatial orientation. 

Meclizine: It is an antihistamine and has anticholinergic effects and therefore helpful by reducing the excitability of the inner ear. Usually taken for acute vertigo attacks and PRN for chronic vertigo. 

Diazepam: A benzodiazepine which possesses central nervous system depressant properties both on the central vestibular system. Helps with acute vertigo attacks and may also mitigate anxious feelings brought about by Meniere’s disease. 

Scopolamine: An antimuscarinic drug that acts by masking vestibular receptors for acetylcholine. Being handed down via transdermal shell out to treat vertigo accompanied by nausea due to the fact of Meniere’s disorder. 

Neurology

Role of Endolymphatic Sac Decompression 

Endolymphatic sac decompression is a surgical procedure in the management of Meniere’s disease which involves relieving of the more severe and frequent symptoms in the condition. 

Meniere’s disease is an inner ear disorder characterized by abnormalities in inner ear fluid mechanisms that cause endolymphatic system pressure to rise. The principle of ESS surgery is to reduce fluid pressure by opening up the endolymphatic sac located in the posterior cranial fossa to control symptoms including vertigo attacks, fluctuating hearing loss, tinnitus, and ear fullness. 

The procedure can be done through various methods like mastoidectomy with open cavity or endoscopic method with minimal cavity. In the surgical treatment, the surgeon normally reduces the space present in the endolymphatic sac or inserts a shunt to facilitate the drainage of the fluid and thereby bring down the fluid pressure. 

The main aim of labyrinthectomy is to prevent the occurrence of vertigo episodes completely. In occasional cases vertigo in Meniere’s disease can be severe and medically refractory. Labyrinthectomy is a surgery in which the balance part of the inner ear (labyrinth) is completely or partially removed or disabled with the intention of providing permanent relief from vertigo episodes. Labyrinthectomy causes total loss of balance function and total deafness on the side of the lesion. Thus it is regarded for patients with poor hearing already in the affected ear or those that seek relief from severe vertigo more than preservation of the residual hearing. 

Labyrinthectomy is a destructive surgical approach in which the labyrinth (the semicircular canals and vestibule) are either excised or rendered non-functional by the surgeon during the operation. This procedure may be performed via open approach or laparoscopic or robotic assisted techniques depending on patients condition and on the surgical preference. 

Neurology

The diagnosis of Meniere’s disease should also be based on a thorough medical history and physical examination as well as hearing tests. These diagnostic tests may include audiometry and tests of vestibular function and imaging procedures. The first step in treatment is symptom control which deals with symptom such as vertigo, tinnitus, and ear fullness. This may include some medications such as vestibulosuppressants, diuretics or corticosteroids which are used to reduce inflammation. Patients may also be advised to follow a low sodium diet to reduce fluid in the inner ear.  

Maintenance therapy is the process of long-term management of the condition that can include administering medication, changing the diet, or making other adjustments to a patient’s life. Vestibular rehabilitation is a form of physical therapy that can help reduce a person’s dizziness episodes and improve their balance.  

Hearing assessment is important to measure any changes in their hearing ability in future. Surgical treatments may be recommended in extreme cases wherein the symptoms do not respond to non-operative management. Rehabilitation after surgery is required to improve balance and adjust to changes in hearing or vestibular function.