Meniere's Disease: Clinical Guidelines

List of Abbreviations

MD – Meniere's disease

VNG – videonystagmography

BPPV – benign paroxysmal positional vertigo

ABR – auditory brainstem responses

A-B gap – air-bone gap

MRI – magnetic resonance imaging

US – ultrasound

SISI / RRec – recruitment phenomenon

EcoG – electrocochleography

ES – endolymphatic sac

AAO-HNS – American Academy of Otolaryngology–Head and Neck Surgery

Terms and Definitions

Meniere's disease (MD) – an inner ear disorder characterized by periodic attacks of systemic vertigo, tinnitus, and sensorineural hearing loss.

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

Meniere's disease (MD) – an inner ear disorder characterized by periodic attacks of systemic vertigo, tinnitus, and sensorineural hearing loss.

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

To date, the etiology and pathogenesis of MD remain insufficiently studied. According to the classical theory of pathogenesis, the development of MD is associated with idiopathic endolymphatic hydrops, which represents an increase in the volume of endolymph filling the membranous labyrinth of the inner ear and causing distension of Reissner's membrane.

Possible causes for the development of endolymphatic hydrops include:

• increased production of endolymph by the vascular stria of the spiral organ and the cells of the saccule and utricle;

• impaired resorption of endolymph.

Recurrent vertigo attacks in MD are associated with the periodic rupture of Reissner's membrane, which is overstretched due to increased pressure, the mixing of endolymph and perilymph, and the entry of potassium-rich endolymph into the perilymph, followed by depolarization of the vestibular nerve and its hyperexcitation. Hearing loss and tinnitus are apparently caused by the gradual degeneration of spiral ganglion neurons.

Currently, the relationship between labyrinthine hydrops and MD attacks is a subject of debate. It is known that endolymphatic hydrops can coexist with other middle and inner ear disorders, such as otosclerosis. According to literature data, labyrinthine hydrops is found at autopsy in individuals who did not manifest MD symptoms during their lifetime; therefore, it is logical to assume that labyrinthine hydrops is not the sole pathogenetic factor in the development of the disease's symptoms, suggesting the presence of additional factors.

1.3 Epidemiology of the Disease or Condition

The incidence of MD varies across different countries from 3.5 to 513 per 100,000 population. The first MD attack most frequently occurs between the ages of 40 and 60 years. Women are affected more often than men. According to statistics, 0.5% of the European population is diagnosed with MD, totaling approximately 1 million people. MD ranks 2nd in frequency among peripheral causes of vertigo, second only to benign paroxysmal positional vertigo. Patients with MD account to 9.5% of all inpatients undergoing treatment for cochleovestibular disorders.

1.4 Features of Coding the Disease or Condition

H81.0 – Ménière's disease

1.5 Classification of the disease or condition

According to the classification of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS)[11], 4 forms of MD are distinguished:

• Definite MD;

• Certain MD;

• Probable MD;

• Possible MD.

There are also three main variants of the disease course. In the first variant (cochlear form), auditory disorders appear first, followed by vestibular ones. In the second (classic) variant, auditory and vestibular disturbances appear simultaneously; the first vertigo attack is accompanied by hearing loss and tinnitus. In the third (rarer) variant of the course (vestibular form of MD), the disease begins with attacks of vestibular vertigo, which are subsequently joined by auditory disorders. According to several authors, hearing fluctuation with vertigo is observed in 82.7% of cases, and without vertigo in 17.3%. Others observed cochlear monosymptomatology at the onset of MD in 54.4% and the classic form in 45.6% [2,4,5].

1.6 Clinical presentation of the disease or condition

MD is characterized by a clinical triad of symptoms, brilliantly described back in 1861 by the renowned French physician Prosper Ménière.

Vertigo attacks. Attacks of systemic vertigo in Ménière's disease are highly characteristic. They occur suddenly, at any time of the day and in any season, against the background of "complete health", are unprovoked, and sometimes have an aura in the form of increased or newly onset aural fullness in the affected ear, tinnitus, etc. These attacks are always accompanied by various autonomic manifestations, the main one being nausea and vomiting; they last for several hours (most commonly 4–6 hours) and resolve spontaneously without the use of any medications. Tinnitus is unilateral, typically low- or mid-frequency, intensifying before and during the systemic vertigo attack.

Hearing loss. Hearing loss in MD also has its characteristic features. It is primarily unilateral, fluctuating, and audiologic testing reveals so-called scalar or false sensorineural hearing loss—a horizontal or ascending audiometric curve with an air-bone gap (ABG of 5–10 dB) in the low or middle frequency range. Normal ultrasound thresholds (UST) and its lateralization toward the worse-hearing ear.

Three stages are distinguished in the development of MD.

First stage – initial stage. Systemic vertigo attacks occur infrequently, 1–2 times a year or even every 2–3 years. These attacks appear at any time of the day, last on average from 1 to 3 hours, and are accompanied by nausea and vomiting. Tinnitus, aural fullness, or a feeling of pressure in the ear occur before or during the attack but are not constant symptoms. Unilateral hearing loss occurs or intensifies at the time of the attack; that is, the first stage of the disease is characterized by hearing fluctuation—its periodic deterioration, usually before the onset of the vertigo attack, followed by improvement. Some patients report significant hearing improvement immediately after the attack, followed by its deterioration back to the usual level over the next 24 hours.

Second stage – height of the disease. Attacks acquire the typical character of MD with intense systemic vertigo and pronounced autonomic manifestations, occurring several times a week (daily) or several times a month. Tinnitus bothers the patient constantly, often intensifying at the time of the attack. A daily sensation of aural fullness and discomfort in the affected ear is characteristic. Hearing loss progresses from attack to attack.

Third stage – fading stage. Typical systemic vertigo attacks become less frequent or disappear completely, but the patient is constantly troubled by a sensation of swaying and unsteadiness. There is pronounced hearing loss in the affected ear; often, the second ear becomes involved in the process at this stage. Tumarkin's otolithic crises may occur—conditions characterized by sudden drop attacks resulting from a sharp mechanical displacement of otolithic receptors, leading to the sudden activation of vestibular reflexes. Such severe symptoms can lead to serious injuries. Taking into account the frequency and duration of vertigo attacks and the preservation of working capacity, three degrees of MD severity are distinguished: severe, moderate, and mild.

In severe MD, vertigo attacks are frequent (daily or weekly), lasting several hours, with the full complex of statokinetic and autonomic disorders; working capacity is lost. In moderate MD, vertigo attacks are also quite frequent (weekly or monthly), lasting several hours. Statokinetic disorders are moderate, and autonomic ones are pronounced. Working capacity is lost during the vertigo attack and for several hours afterward.

In mild MD, vertigo attacks are short-lived, with prolonged remissions (lasting several months or years).

2. Diagnostics, medical indications and contraindications for diagnostic methods

The American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) developed diagnostic criteria for definite, certain, probable, and possible MD (1972, 1985, 1995) [11]. Data from Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS), and the Korean Balance Society, 2015 [12].

Definite MD:

• histologically confirmed endolymphatic hydrops;

• two or more vertigo attacks lasting from 20 minutes to 12 hours each;

• audiometrically confirmed sensorineural hearing loss in low and middle frequencies during or after a vertigo attack;

• fluctuating auditory symptoms: hearing, tinnitus, aural fullness.

Certain MD:

• two or more spontaneous vertigo attacks lasting from 20 minutes to 12 hours;

• audiometrically confirmed sensorineural hearing loss in low and middle frequencies during or after a vertigo attack;

• fluctuating auditory symptoms: hearing, tinnitus, aural fullness;

• exclusion of other causes.

Probable MD:

• at least one vertigo attack;

• sensorineural hearing loss confirmed by at least one audiometry session;

• tinnitus or aural fullness in the affected ear;

• exclusion of other causes explaining the listed symptoms.

Possible MD:

• vertigo attacks without confirmed hearing loss;

• sensorineural hearing loss, stable or fluctuating, with balance disturbance but without definitive vertigo attacks;

• exclusion of other causes explaining the listed symptoms.

2.1 Complaints and Anamnesis

Ménière's disease manifests as attacks of progressive deafness, tinnitus, periodic loss of balance, vertigo, and nausea progressing to vomiting.

2.2 Physical Examination

2.3 Laboratory Diagnostics

2.4 Instrumental Diagnostics

1. pure tone audiometry [1, 2, 4, 5, 20-22] for all patients to verify the degree of hearing loss;

2. impedance audiometry (tympanometry and acoustic reflexometry) [2, 5, 20, 22] for all patients to rule out middle ear pathology;

3. determination of ultrasound sensitivity thresholds and the lateralization phenomenon and/or electrocochleography (ECochG), and/or a dehydration test [2, 4-7, 20, 22, 23] to verify endolymphatic hydrops of the labyrinth when there are clinical signs of probable Meniere's disease, as well as in cases of conservative therapy failure;

4. assessment of evoked otoacoustic emissions and recording of short-latency auditory evoked potentials in case of contraindications to brain MRI (e.g., presence of metal implants in the body, etc.), as well as in case of questionable pure tone audiometry results (e.g., exaggeration, malingering, etc.) [20];

5. vestibulometry [6, 7, 20] for differential diagnosis when it is impossible to establish a diagnosis based on clinical data, as well as when planning surgical treatment.

GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)

Comments: there is no single universal method for diagnosing labyrinthine hydrops. Given the specific course of the disease and the combination of various symptoms of inner ear damage, a wide range of clinical studies must be performed and evaluated dynamically to confirm the diagnosis. The results of these studies largely depend on the stage of the disease. During the interictal period, spontaneous vestibular symptoms are absent upon vestibular function testing, and static and dynamic balance (including posturographic (stabilographic) examination) are within normal limits. During an attack, peripheral vestibular syndrome develops, accompanied by systemic vertigo, nausea, vomiting, balance impairment, and horizontal vestibular nystagmus. Vestibulometry in the interictal period reveals depression of vestibular function, which is more pronounced on the affected side. Audiological testing reveals so-called scalar or false sensorineural hearing loss—a horizontal or ascending type of audiometric curve with an air-bone gap (ABG of 5–10 dB) in the low or middle frequency range. Ultrasound (US) thresholds are normal and lateralize toward the worse-hearing ear. In Meniere's disease, a decrease in the amplitude of spontaneous otoacoustic emission peaks may be observed. At the retrocochlear level of lesion, an increase in acoustic reflex thresholds relative to normal hearing thresholds is noted. Criteria for retrocochlear dysfunction of the auditory system include: absence of all ABR components from wave I to wave V, which is difficult to explain by hearing loss; absence of all components following wave I or wave III; a pathological increase in the I–V interpeak latency interval greater than 4.4 ms; a pathological decrease in the V/I amplitude ratio below 0.5, especially when accompanied by other ABR alterations. A pathological increase in the parameter difference between right and left ear stimulation (inter-aural difference), particularly regarding interpeak intervals—which is difficult to explain by unilateral or asymmetric peripheral hearing loss in the middle or inner ear—also has diagnostic significance.

Comments: neoplasms (such as acoustic neuroma in the presence of concurrent unilateral sensorineural hearing loss), demyelinating processes, neurovascular conflict, and acquired or congenital structural changes of the temporal bones must be ruled out.

2.5 Other Procedures

No.

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

All applied treatment methods for patients with Meniere's disease can be divided into three groups:

1. relief of vertigo attacks (pharmacotherapy);

2. prophylactic measures during the interictal period (pharmacotherapy);

3. surgical treatment.

3.1 Conservative Treatment

Comments: Centrally acting vestibular blockers, namely systemic H1-antihistamines that cross the blood-brain barrier, are used during the first 24 hours (maximum up to 3 days) to abort an acute vestibular vertigo attack [2-8].

Comments: During an acute attack of Meniere's disease, due to autonomic symptoms (nausea and vomiting), parenteral administration of medications is preferred; as symptoms resolve, a transition to oral formulations is possible [2-8].

Comments: Betahistine** is an H1 histamine receptor agonist in the inner ear blood vessels and an H3 histamine receptor antagonist in the vestibular nuclei of the central nervous system. By relaxing the precapillary sphincters of the inner ear vessels, it improves blood circulation in the stria vascularis of the inner ear. It dose-dependently reduces action potential generation in the neurons of the lateral and medial vestibular nuclei. It accelerates the recovery of vestibular function, including by activating central vestibular compensation (due to antagonism with H3 histamine receptors) [6-8, 10, 12].

Comment: Acetazolamide** is used to treat cerebrospinal fluid dynamics disorders and intracranial hypertension by reducing excessive cerebrospinal fluid production at the level of the choroid plexuses of the brain. This effect helps reduce the manifestations of labyrinthine hydrops in Meniere's disease.

Loop diuretics should not be used due to potential ototoxic effects. With prolonged use of acetazolamide**, blood electrolyte levels must be monitored, and replenishment of lost electrolytes may be necessary [2, 4].

Comments: In Meniere's disease, in addition to medical treatment, proper nutrition plays an important role, aimed at reducing the fluid volume in the patient's body. This diet recommends consuming as little salt and processed foods as possible, eliminating alcohol, caffeine, nicotine, and consuming moderate amounts of fluids [1-8, 19, 20, 22].

3.2 Surgical Treatment

Surgical treatment is recommended when conservative therapy is ineffective for 6-12 months [11, 22].

Performing hearing-preserving surgeries (e.g., chordoplexectomy, intratympanic glucocorticoid administration, endolymphatic sac decompression/drainage, or selective laser destruction of the horizontal semicircular canal ampullary receptor) is recommended as the first-line approach in stages I and II of the disease.

Destructive surgeries (e.g., intratympanic gentamicin sulfate administration, labyrinthine laser destruction, vestibular neurectomy) are recommended as a second-line approach when hearing-preserving surgery is ineffective or in cases of socially insignificant hearing [2, 4, 5, 11, 18-20, 22].

4. Medical Rehabilitation and Sanatorium-Resort Treatment

5. Prevention and Dispensary Observation

Prevention of Ménière's disease: promoting a healthy lifestyle among the population, early detection of inflammatory diseases of the ENT organs (especially in pediatric practice), and secondary prevention of recurrences of chronic inflammatory ENT diseases through clinical follow-up (dispensary observation).

6. Organization of Medical Care

Primary specialized health care for patients with Ménière's disease is provided by an outpatient otorhinolaryngologist.

In case of a severe condition, the patient is hospitalized in an otorhinolaryngological department to receive specialized medical care. In severe cases, the patient is admitted via emergency medical services directly to a specialized otorhinolaryngological department, bypassing the outpatient stage. Criteria for hospital discharge are: cessation/reduction in the frequency of vertigo attacks and/or improvement/stabilization of hearing thresholds.

7. Additional Information

The course and outcome of Ménière's disease are influenced by factors such as disease duration and severity, comorbid pathology, patient age, and the presence and nature of complications.

Criteria for Evaluating the Quality of Medical Care

Table 1 – Quality assessment criteria for outpatient medical care

Table 2 – Quality assessment criteria for inpatient medical care

References

Appendix A1. Working Group Composition

1. Kunelskaya NL, D.Sc. in Medicine, Professor, is a member of the National Medical Association of Otorhinolaryngologists;

2. Diab HM, D.Sc. in Medicine, is a member of the National Medical Association of Otorhinolaryngologists;

3. Popadyuk VI, D.Sc. in Medicine, Professor, is a member of the National Medical Association of Otorhinolaryngologists;

4. Garov EV, D.Sc. in Medicine, is a member of the National Medical Association of Otorhinolaryngologists;

5. Kuzovkov VE, D.Sc. in Medicine, is a member of the National Medical Association of Otorhinolaryngologists;

6. Zaitseva OV, Ph.D. in Medicine, is a member of the National Medical Association of Otorhinolaryngologists;

7. Machalov AS, D.Sc. in Medicine, Associate Professor, is a member of the National Medical Association of Otorhinolaryngologists;

8. Lilenko SV, D.Sc. in Medicine, Professor, is a member of the National Medical Association of Otorhinolaryngologists;

9. Levina YV, Ph.D. in Medicine, is a member of the National Medical Association of Otorhinolaryngologists;

10. Savelyeva EE, D.Sc. in Medicine, Associate Professor, is a member of the National Medical Association of Otorhinolaryngologists;

There is no conflict of interest.

Appendix A2. Methodology for Developing Clinical Guidelines

These guidelines are based on the methodological recommendations for "Ménière's disease", reviewed and approved at the meeting of the National Medical Association of Otorhinolaryngologists on December 1–2, 2014.

Target audience of these clinical guidelines (in accordance with the Order of the Russian Ministry of Health No. 1183n dated December 20, 2012 (as amended on September 4, 2020) "On Approval of the Nomenclature of Positions for Medical and Pharmaceutical Workers"):

1. Otorhinolaryngologist;

2. Audiologist-otorhinolaryngologist;

3. Neurologist;

4. General practitioner (internist);

5. Psychotherapist

6. Psychiatrist;

7. Neurosurgeon;

8. General practitioner (family physician).

As diagnostic, treatment, and rehabilitation methods for patients with Ménière's disease evolve and improve, and as new regulatory acts and health insurance practices develop, these clinical guidelines will be updated and supplemented. Given the interdisciplinary nature of Ménière's disease, it is advisable to update the guidelines following scientific and practical conferences and symposia every 3 years.

Table 1. Scale for grading levels of evidence (LE) for diagnostic methods (diagnostic interventions)

Table 2. Level of Evidence (LoE) grading scale for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitative interventions)

Table 3. Scale for grading the grades of recommendation (GR) for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, rehabilitation interventions)

Procedure for updating clinical guidelines

Clinical guidelines will be updated every 3 years

Appendix A3. Reference Materials

1. Order of the Ministry of Health of the Russian Federation No. 178n dated April 9, 2015, "On Approval of the Procedure for Providing Medical Care to the Population Profiled in 'Audiology-Otorhinolaryngology'."

2. Order of the Ministry of Health of the Russian Federation No. 905n dated November 12, 2012, Moscow, "On Approval of the Procedure for Providing Medical Care to the Population Profiled in 'Otorhinolaryngology'."

3. Government Decree No. 2406-r dated October 12, 2019 (as amended on June 9, 2023) "On Approval of the List of Vital and Essential Medicines, as well as Drug Lists Required for Medical Care Provision."

4. State Register of Medicines. Internet resource http://grls.rosminzdrav.ru/Default.aspx

Differential diagnosis of Ménière's disease

Appendix B. Physician Action Algorithms

Meniere's disease

Appendix C. Patient Information

• impermissible sharp changes in middle ear pressure during scuba diving, forceful nose blowing, or air travel;

• diet with restriction of carbohydrates, salty, spicy, and fatty meat products;

• the daily diet must include foods rich in potassium, in particular, baked potatoes, cottage cheese, and dried apricots;

• exclude alcohol consumption, smoking, caffeine intake, and foods containing preservatives, especially monosodium glutamate;

• avoid overwork, working at heights, and working with moving objects.

Tinnitus remains a complex problem to this day, with significant medical and socio-medical aspects.