The labyrinth (inner ear) contains the organs of hearing and balance, located in the petrosal part of the temporal bone. The labyrinth consists of the following: ^1,2,6

1. The bony labyrinth consists of: semicircular canals, vestibule, and cochlea.

2. The membranous labyrinth, which is located within the bony labyrinth, consists of: semicircular canals, utricle, saccule, sac, and endolymphatic duct and cochlea.

Between the bony and membranous labyrinths there is a space containing perilymph fluid which originates from cerebrospinal fluid. and filtration of blood. In the membranous labyrinth there is endolymph fluid, produced by the stria vascularis and the endolymphatic sac.

The apex or tip of the cochlea is called the helicotherma which connects the perilymph of the scala tympani and scala vestibuli. A transverse section of the cochlea shows the vestibular scale at the top, the tympani scale at the bottom and the media scale in between. ^1,2,6

Scale vestibuli and scale timpani containing perilymph whereas The media scale contains endolymph. The base of the vestibuli scale is called the Reissner membrane while the base of the media scale is called the basilar membrane which contains the organ of Corti. On the media scale there is a tongue-shaped part called the tectorial membrane and on the basilar membrane are attached the inner hair cells, outer hair cells, and the canal of Corti. The basilar membrane is narrow at its base (tone tall) And widen at the apex (low tone). ^1,2,6

Located on membrane basilaris from base to apex is organ of corti which contains organelles important for the peripheral nervous mechanism of hearing. The organ of corti consists of one line cell hair inner (3,000) and three rows of outer hair cells (12,000). The afferent and efferent nerve endings are attached to the lower ends of the hair cells. The vestibule of the inner ear is formed by the utricle, saccule, and semicircular canals. The utricle and saccule contain maculae covered by hair cells. Covering the hair cells is a gelatinous layer penetrated by cilia and in this layer there are also otoliths containing calcium and will stimulate the receptors. The saccule is connected to the utricle through a narrow ductus which is a channel to the endolymphatic sac. The macula of the utricle lies in a plane perpendicular to the macula of the saccule. Third canal semicircular culminate on utricle. Each canal has one widened end which forms an ampulla and contains the crista hair cells and is covered by a gelatinous layer called the cupula. Movement of endolymph in the canal semicircular will to move cupula which will then bend the cilia of the cristae hair cells and stimulate the receptor cells. ^1,2,6
Physiology of Balance

In body man, There is system Which arrange balance and the orientation of a person's body towards the surrounding environment which depends on sensory input from vestibular receptors in the labyrinth, the organ of vision and the proprioceptive organ. The combination of information from the three sensory receptors will be processed in the central nervous system so that it will cause description about condition position body on at some point and how to position the body as desired at that time. ^3.4

System organ vision accept stimulation through receptor in the retina, namely in the macula lutea. The stimulus is transmitted through the optic nerve (N.II) to the visual cortex in the occipital lobe. The function of vision provides information about position And motion body and the surrounding environment. Proprioceptive organs receive movement stimuli through musculoskeletal receptors, especially in the neck area, which are distributed through the spinal nerves, then the spinal cord, medulla oblongata, thalamus and end in the sensory cortex (post centralis). ⁴

Based on  guidelines  of  the  American  Academy  Of

Otolaryngology-Head and Neck Surgery (AAO-HNS), Meniere's disease is characterized by 4 symptoms, namely: vertigo (spinning dizziness), fluctuating hearing loss, tinnitus and a feeling of fullness in the ear. ^2.5 This disease is one of the diseases that causes humans to be unable to maintain an upright body position. This is caused by hydrops (swelling) of the endolymph cavity in the cochlea and vestibule. This disease was discovered by Meniere in 1861 and he believed that the disease That was in the ear. However, experts at that time suspected that the disease was in the brain. Meniere's opinion was later proven by Hallpike and Cairn in 1938, with the discovery of endolymphatic hydrops after examining the temporal bone of a patient suspected of suffering from Meniere's disease. ^6,7

Symptoms of Meniere's Disease

The definition of vertigo is a sensation of movement or a feeling of movement from the body or the surrounding environment which can be accompanied by other symptoms, especially from the tissue. autonomous consequence disturbance tool balance body. Say Vertigo comes from the Greek word meaning spinning motion. Vertigo may not consist of just one symptom of dizziness, but rather a collection of symptoms or a syndrome consisting of symptoms. somatic (nystagmus, unstable ), autonomic symptoms such as paleness, cold sweat, nausea, vomiting and dizziness. ^6,7

Tinnitus is defined as hearing loss with constant complaints. hear sound However without There is stimulation sound from outside. The source of the sound comes from the sufferer's body itself (self-impulse). However, tinnitus is only a symptom, not a disease, so the cause must be sought tinnitus. ^7,8 Hearing loss is usually fluctuating and progressive with hearing getting worse over days. Severe Meniere's disease hearing loss can result in permanent hearing loss. ^8,9

Epidemiology

Most cases of Meniere's disease are unilateral and about 10-20% of cases are bilateral. The incidence of this disease reaches 0.5-7.5: 1000 in the UK and Sweden. Meniere's disease is one of one of the most common causes of vertigo in the inner ear. ³ This case is rarely found in children. Most cases occur in adult men or women. Most often found at the age of 20-50 years. There is a possible genetic component that plays a role in Meniere's disease, with 21% of patients with Meniere's disease having a family history of the disease. Also experience complaint Which The same. People who have history allergies, smoke, stress, fatigue alcoholism and patients who regularly consume aspirin have a high risk of developing Meniere's disease. ^3.9

Etiology

The exact cause of Meniere's disease is not yet known. However, there are various theories that could possibly cause it. the occurrence disease Meniere's. A number of reason Which It is thought that this can be a trigger for Meniere's disease, including abnormal neurochemical and hormonal influences on blood flow to the labyrinth, electrolyte disturbances in the labyrinth fluid, allergic reactions, and autoimmune. ^6,7,

Meniere's disease is thought to occur due to an abnormal imbalance of inner ear fluid which is thought to be caused by malabsorption in the endolymphatic sac. In addition, experts also say that there is a tear in the membrane in the cochlear labyrinth causing the endolymph and perilymph to mix. ⁶ According to experts, this can cause symptoms of the disease Meniere's. Researchers are also conducting investigations and research into other possible causes of Meniere's disease and each has its own beliefs about the cause of this disease, including environmental factors such as loud noises, HSV virus infection, blood vessel pressure on nerves ( microvascular compression syndrome ). In addition, Meniere's disease symptoms can be caused by trauma. head, infection channel breathing on, aspirin, smoke, alcohol or excessive salt consumption. Basically, no one knows for sure what causes Meniere's disease. ^7,9,10

Meniere's disease has clinical symptoms caused by endolymph hydrops (increased endolymph causing the membranous labyrinth to dilate) in the cochlea and vestibule. Hydrops that occurs and recurs is thought to be caused by increased hydrostatic pressure at the end of the artery, decreased osmotic pressure in the capillaries, increased osmotic pressure of the extracapillary space, the outlet of the endolymphatic saccule is blocked (due to scar tissue or a birth defect). ^11,12 This endolymph hydrops over time causes pressure which, when it reaches maximum dilation, will rupture the membranous labyrinth and the endolymph will mix with the perilymph. This mixing causes potential action in ear deep, causing symptoms of vertigo, tinnitus and disturbances hearing as well as flavor full in ear. When pressure Already The same, then the membrane will heal itself and the perilymph fluid and endolymph do not mix again, but this healing does not always occur perfectly. ^10,11

Meniere's disease can cause :

1. Death cell hair on organ corti in inner ear

In the case of repeated attacks of Meniere's disease causes the death of hair cells of the organ of Corti. Within a year it can cause unilateral sensorineural deafness. Vestibular hair cells can still function, but caloric tests show a decline in function. ^13,14

2. Changes in ear mechanism

The period of enlargement and then chronic shrinkage of the utricle and saccule causes changes in the ear mechanism. Histopathological examination of the temporal bone found morphological changes in the Reissner membrane. There is a protrusion into the vestibuli scale, especially in area apex cochlea (Helicotrema). Sacculus Also experiencing widening that can press on the utricle. Initially, the widening of the media scale starts from the apex of the cochlea and can then spread to the middle and basal parts of the cochlea. This can explain the occurrence of low tone nerve deafness in Meniere's disease. ^14,15

Clinical Symptoms

Meniere's disease usually begins with one symptom and then progressively other symptoms increase. Clinical symptoms of the disease Meniere's typical often called Trias Meniere that is: vertigo, tinnitus and fluctuating sensorineural deafness especially in low tones. The first attack is felt very heavy, that is vertigo accompanied by nauseous And vomit. Every When trying to stand up, the patient will feel dizzy, nauseous and vomit again. This lasts for several days to several weeks, then the condition will gradually improve. ⁸ This disease can be cured without medication and the symptoms of the disease can disappear completely. In the second and subsequent attacks, it is felt to be lighter, unlike the first attack. In Meniere's disease, vertigo is periodic and subsides in subsequent attacks. ^9,14

One attack of Meniere's disease is usually accompanied by hearing loss, and if there is no attack, your hearing will feel good again. Other accompanying symptoms attack is tinnitus Which sometimes settle down although in outside the attack. Another symptom that is a special sign is a feeling of fullness in the ear. ^6,7

Periodic vertigo is usually felt in 20 minute until 2 hours or more during the attack period a week or a month Which interspersed with periods of remission. Vertigo causes nystagmus, nausea, vomiting. Each attack is usually accompanied by hearing and balance disorders so that one cannot be active and is in a state of no hearing loss will recover. From the vertigo complaint we can already differentiate it from disease Which others who also have symptoms of vertigo such as N.VIII tumors, multiple sclerosis, vestibular neuritis or benign paroxysmal positional vertigo (BPPV). ^5,6,7

In N.VIII tumors, vertigo attacks are periodic, initially weak and gradually stronger. In multiple sclerosis, vertigo is periodic with the same intensity in each attack. In vestibular neuritis, vertigo attacks are not periodic. And more and more long disappear. On VPPJ complaints of vertigo came consequence change position head And complaint which is felt very heavy, sometimes accompanied by nausea and vomiting but does not last long. ^5,6

Tinnitus is sometimes persistent (seconds to minutes), even outside of attacks. Tinnitus often worsens before an attack of vertigo. Tinnitus is often described by patients as a motor sound, engine, rumbling, ringing, buzzing, and ringing in the ears. ⁷

Hearing loss may seem to be only slightly reduced at the beginning of the attack, but over time permanent hearing loss may occur. Meniere's disease may involve damage to all nerves at all frequencies. voice hearing but most general happen on frequency Which low. Voice Which loud may be uncomfortable ^and very disturbing to the affected ear.9,10

A feeling of fullness in the ear is felt as when we experience a change pressure air (climbing) And down hill, aircraft fly, and so on) but the difference is that this feeling of fullness is not is lost with Valsalva and Toynbee maneuvers. ^8,11

Diagnosis

Other disease conditions can produce similar symptoms to Meniere's disease, so other possible diseases must be ruled out in order to establish an accurate diagnosis. Evaluation beginning based on on anamnesis Which very be careful. ^8,10

Diagnosis of this disease can be made easier by criteria diagnosis as follows: ^8,10

1. Vertigo that comes and goes with tinnitus and a feeling of fullness in the ears.

2. Fluctuating hearing loss in the form of sensorineural deafness.

Eliminate the possibility of central causes, for example a tumor of the VIII nerve. Some differential diagnoses for Meniere's disease are: tumor N.VIII, sclerosis multiple, neuritis vestibular or Benign paroxysmal positional vertigo (BPPV). In N.VIII tumors, vertigo attacks are periodic, initially weak and gradually stronger. In Multiple sclerosis periodic vertigo with the same intensity in each attack. In vestibular neuritis vertigo attacks are not periodic and gradually disappear. In VPPJ vertigo complaints come due to changes position head And complaint Which felt very heavy sometimes accompanied by nausea and vomiting but does not last long.

A careful physical examination is required to confirm the diagnosis. If results inspection physique ear possibility external ear disorder And middle can removed And confirmed abnormality originating from the inner ear, for example, if anamnesis reveals a complaint of fluctuating nerve deafness and this is confirmed by the results of the examination, then we can diagnose Meniere's disease, because there is no nerve deafness that improves except in Meniere's disease.

Inspection   support  Which  can  done  For

1. Electronystagmography (ENG) and balance tests, to objectively determine the quantity of balance disorders in patients. Most patients with Meniere's disease experience a decrease in nystagmus response to stimulation with hot and cold water used in this test. ^18,19

2. Electrocochleography (ECOG), measures fluid accumulation in the inner ear by recording the action potentials of auditory neurons through electrodes that placed near with cochlea. On patients with Meniere's disease, this test also shows increased pressure Which due to by fluid Which excessive on ear in which is indicated by existence widening form wave with multiple peaks. ¹⁹

3. Brainstem Evoked Response Audiometry (BERA ), usually normal in patients with Meniere's disease, although sometimes there is mild hearing loss in patients with disorders of the central nervous system. ^18,19

4. Magnetic Resonance Imaging (MRI) with contrast Which called gadolinium specific visualize N.VIII. If There is part

Nerve fibers that are not filled with contrast indicate the presence of an acoustic neuroma. In addition, MRI examination can also visualize the cochlea and semicircular canals. ^18,19

Management

Patients who come with typical complaints of Meniere's disease are initially only given symptomatic treatment, such as sedatives and if necessary, anti-emetics. The best treatment is according to the cause. ^11,12

1. Diet and lifestyle changes

A low-salt diet has little effect on plasma sodium concentrations, because the body has a regulatory system in the kidneys to maintain plasma sodium levels . To maintain the balance sodium concentration, the kidneys adjust the capacity for ion transport capability based on sodium intake . This adjustment is played by the hormone aldosterone which functions to control the amount of ion transport in the kidneys so that it will affect sodium regulation in the endolymph, thereby reducing Meniere's disease attacks. ¹⁵

Many patients can control their symptoms simply by following a low-salt diet (2000 mg/day). The amount of sodium is one of the factors that regulates fluid balance in the body. Retention of sodium and fluid in the body can disrupt the balance between endolymph and perilymph in the ear. Sodium salts added to food are usually in the form of sodium chloride or table salt, monosodium glutamate ( MSG ), sodium bicarbonate (baking soda), sodium benzoate (corned beef). ¹⁵

Consumption cigarette, alcohol, chocolate must stopped. Caffeine and nicotine is also a vasoactive stimulant and can cause vasoconstriction and decreased blood flow. artery small Which give nutrition nerve from ear middle.

Avoiding both substances can reduce symptoms. Regular exercise can stimulate blood circulation so it needs to be recommended to patients. Patients should also avoid the use of ototoxic drugs such as aspirin because it can worsen tinnitus. ¹⁵

During an acute attack it is recommended to lie down on a hard surface, try not to move, fix your eyes on one immovable object, do not try to drink even if you feel like vomiting, after the vertigo disappears the patient is asked to get up slowly because usually after an attack there will be fatigue and the patient should find a comfortable place to sleep for a few hours to restore balance. ^15,16

2. Pharmacology

Symptoms of Meniere's disease are usually treated with pharmacotherapy in the form of peripheral vasodilators, antihistamines, anticholinergics, steroids. And diuretic For reduce pressure on endolymph. Anti-ischemic drugs can also be given as alternative drugs and neurotonics to strengthen the nerves. In addition, if there is a viral infection, antivirals such as acyclovir can be given. ¹⁸Tranquilizers such as diazepam (Valium) can be used in acute cases to help control vertigo, but because of their addictive nature are not used as long-term treatment. Antiemetics such as promethazine not only reduce nausea and vomiting but also vertigo. Diuretics such as thiazides can help reduce the symptoms of Meniere's disease by reducing pressure in the endolymphatic system. Patients should be reminded to eat foods containing potassium such as bananas, tomatoes and oranges. when use diuretic which causes potassium loss. ^17,18

Caption :

On Figure 1, first sitting position, head turned to the left (on

Caption :

First The movement is done in a sitting position, direct the head to the left, drop the body to the right position, then turn the sitting position, turn the head to the right then drop the body to the left side, each movement is waited for about 1 minute, can be done repeatedly, first enough 1-2 times left and right, the next day it will increase. It is also better to have it checked first formerly For ensure reason vertigo disturbance its balance. ¹³

This surgical procedure decompresses excess fluid in the inner ear and causes pressure on the vestibulocochlear nerve endings to return to normal. The incision is made behind ear Which infected And water cell mastoid lifted in order to see the inner ear. A small incision is made in the endolymphatic sac to drain fluid into the mastoid cavity. ^18,19 Overall, about 60% of patients with vertigo attacks are controlled, 20% do not get a decrease in symptoms, 20% experience worse attacks. Hearing function remains stable but rarely improves and tinnitus persists, 2% experience total deafness and vertigo persists. ^17,18,19

• Labyrinthectomy

This labyrinthectomy operation is performed by removing the semicircular canals and vestibulocochlear nerves. It is performed with an incision in the back of the ear and the mastoid air cells are removed, when the inner ear is visible, the entire bony labyrinth is removed. After One or two day post operation, No seldom happen vertigo heavy. Matter This can overcome with administration of medication. After a week, the patient experienced a period of moderate imbalance without vertigo, after which the ear Which normal take transfer all over function balance. This operation removes the hearing function of the ear. ^17,18

If patient Still can hear, neurectomy vestibular is an option for treating vertigo and hearing Which remaining. Done incision in behind ear and the mastoid air cells are removed, an opening is made in the fossa dura mater and N.VIII and the balance nerve is cut. The choice of this operation is similar to a labyrinthectomy. However, because this operation involves the intracranial area, so strict post-operative supervision must be carried out. This surgery is indicated in healthy patients under 60 years of age. Approximately 5% experience total deafness in the infected ear, paralysis face temporary can happen during A few days to months, about 85% of vertigo can be controlled. ^16,18,19

• Labyrinthectomy with chemicals

Surgical procedures performed using antibiotics (strepomycin) or gentamicin dose small) Which entered to inner ear. This operation aims to reduce the process of destruction of the balance nerve and maintain the remaining hearing. In cases of Meniere's disease, intramuscular streptomycin or gentamicin can cure vertigo attacks and hearing can be maintained. ^16,19

• Endolymph shunt

This surgical procedure still attracts some controversy because many researchers Which consider operation This is a placebo. ¹⁷

There are two types of this operation, namely :

1. Endolymph subarachnoid shunt : with put

tube shunt between the endolymph and the cranium.

2. Endolymph mastoid shunt : by placing a shunt tube between the endolymphatic sac and the mastoid cavity. ^15,18,19

Prognosis

Until with moment This disease Meniere Not yet can cured and in some cases is progressive, but not fatal, but interferes with quality of life. There are many treatment options to treat the symptoms and various measures can be taken to prevent attacks and disease progression. Meniere's disease tends to be different for each patient. Some patients experience spontaneous remission over a period of days to years. Other patients experience a rapid worsening of symptoms. However, there are Also patients whose disease progresses slowly. Patients with severe vertigo should be advised not to drive, climb stairs and swim. ^1,6.18

DISCUSSION

Meniere's is a disorder of endolymphatic cavity swelling. A disease with a classic triad of clinical symptoms, namely vertigo for several hours, tinnitus, and fluctuating hearing loss . According to Anderson et al., Meniere's is only diagnosed if there is a Meniere's triad. 4

Until now the exact cause of Meniere's disease is unknown. According to Paparella et al., the increase in endolymph volume is thought to be due to biochemical disorders of endolymph fluid and clinical disorders of the labyrinthine membrane. Syphilis infection, hypothyroidism and head trauma have also been reported to cause Meniere's disease. Recent literature according to Rutka et al., suggests that in some cases bilateral Meniere's can be caused by an immune phenomenon. ^7,9

The occurrence of swelling disorders in the endolymphatic cavity is called hydrops. Hydrops endolymphatic This can happen on cochlea and vestibule. According to Rutka et al., hydrops occurs suddenly and is lost It is thought that the cause of the occurrence is: increased hydrostatic pressure on end arteries, decrease pressure osmotic in the capillaries, increasing pressure osmotic room extracapillary, road go out endolymphatic sac clogged, so that happen hoarding fluid endolymph. ⁹ Based on Paparella et al, on inspection histopathology temporal bone, found widening And change Morphology of Reissner's membrane. There is a protrusion into the scala vestibuli, esp in the apex of the cochlea helicotrema. The saccule also experiences widening which can press on utricle. On at first widening scale media started from apex cochlea, possibility can expand about part middle and basal cochlea. Matter This can explain the occurrence deaf nerve tone low

in Meniere's disease . ⁹

Typical symptoms of Meniere's disease are a triad of vertigo, tinnitus, and sensorineural deafness, especially low tones. The first attack very heavy, that is vertigo accompanied by vomit. Every time stand he feeling dizzy, nauseous and then vomiting. This lasted a number of days to weeks, although the condition gradually improves. This disease can be cured without medication and the symptoms of the disease can disappear completely. In the second attack And furthermore symptom Which felt lighter, unlike the first attack. According to Adams et al., in Meniere's disease, vertigo is periodic and subsides in subsequent attacks. According to Becker et al., diagnosis is made easier by standardizing diagnostic criteria, namely: vertigo that comes and goes, fluctuations in hearing loss in the form of nerve deafness, eliminating the possibility of central causes, such as N.VIII tumors if these typical symptoms can be found, then a diagnosis of Meniere's disease can be made. Physical examination is only needed to confirm the diagnosis of this disease. If in the anamnesis there is a history of hearing fluctuations, while in the hearing examination there is sensorineural deafness, then we can diagnose Meniere's disease, because there is no other disease that can cause improvement in sensorineural deafness, except for Meniere's disease. ^6,12

Based on Harold et al, in case Which doubtful, We can prove the presence of hydrops with the glycerin test. Besides That test glycerin This is useful for determining the prognosis of operative action in the creation of a " shunt " if there is hydrops, then the operation is thought to be successful. According to Stapleton et al., when arriving, symptomatic drugs are usually given, such as sedatives and if necessary, anti-vomiting drugs, Prochlorperazine (Compazine) suppositories can be given. Dietary regulation by restricting sodium consumption such as salt (≤1500 mg/day), as well as caffeine, nicotine, cigarettes, alcohol, and foods with high theophylline content ( chocolate ), can reduce symptoms. In acute exacerbations, it can respond to oral steroids. Intratympanic steroids can also be used to treat ongoing infections and prevent complications. If a diagnosis has been found, the best treatment is according to the cause. According to Horold et al., specifically For disease Meniere's, given medicines vasodilator peripheral

For reduce pressure hydrops lymph. Can also pressure endolymph This is channeled to another place through surgery, or a " shunt " is created. Anti-ischemic drugs can also be given as alternative drugs and also given as a neurotonic drug to strengthen the nerves. ¹⁶ According to Stapleton et al., other therapies can be with intratympanic gentamicin, labyrinthectomy, and vestibular nerve section. ^15,19

CONCLUSION

Meniere's disease is also called idiopathic endolymphatic hydrops . This disease is a disorder of the inner ear which is generally There are symptoms called Meniere's triad, namely vertigo, tinnitus and low-pitched nerve deafness that is fluctuating. The exact cause of Meniere's disease is not yet known, but it is suspected that the clinical symptoms of Meniere's disease are caused by endolymph hydrops (increased pressure). endolymph Which cause labyrinth membranous dilated) on cochlea and vestibule. The diagnosis of Meniere's disease is facilitated by the standardization of diagnostic criteria, namely the Meniere triad and eliminating the possibility of central causes. A careful anamnesis and physical examination are needed to confirm the diagnosis. Supporting examinations that can be performed to assist in the diagnosis are audiometry, Electronystagmography, Electrocochleography Brainstem Evoked Response Audiometry, Magnetic Resonance Imaging . Patients with typical symptoms of Meniere's disease at the beginning of symptoms are usually given symptomatic drugs, peripheral vasodilator drugs to reduce the pressure of lymph hydrops. Dietary regulation by restricting consumption sodium like salt (≤1500 mg/day), as well as caffeine, nicotine, cigarette, alcohol, And food with content tall theophylline ( chocolate ), can reduce symptoms. Rehabilitation and training of the vestibular system. Drug treatment by giving antiemetic drugs, tranquilizers and diuretics. Surgical management is performed if vertigo is severe and uncontrolled.

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20. E Stapleton, R mailing list. Clinical diagnosis of Me´nie` re's disease: how useful are the American Academy of Otolaryngology Head and Neck Surgery Committee on Hearing and Equilibrium guidelines. The Journal of Laryngology & Otology. 2018: 122; 7