Meniere’s disease is an ancient disorder, and nearly a century and a half has passed since it was first described by Prosper Meniere in 1861, leaving behind a great deal of research and repeated exploration that has still not been able to unravel its mysteries. Meniere’s disease is a disabling disease, with episodes of vertigo limiting work and life, sensorineural deafness and speech discrimination disorders affecting work and social life, and especially intractable tinnitus often combined with anxiety and depression, which can be life-threatening. Ménière’s disease has an unknown cause, lacks biological markers, has a wide range of treatments but no efficacy, and is sometimes characterized by contradictory and inexplicable treatments, outcomes, and treatment mechanisms. However, Ménière’s disease is a disease with a certain incidence of about 46 per 100,000 people, so its diagnosis and treatment research has been a clinical hotspot and difficulty.
In 1972, the American Academy of Ophthalmology, Otolaryngology, and Ophthalmology (AAOO) established the diagnostic criteria for Ménière’s disease, and in 1995, the Hearing and Balance Committee of the American Academy of Otolaryngology, Head and Neck Surgery (AAO-HNS) revised the guidelines for the diagnosis and evaluation of the efficacy of Ménière’s disease. In 2015, the International Barany Society (CCBS), the AAO-HNS, the European Academy of Otology and Neuro-Otology (EAONO), the Japanese Society for Balance Research and the Korean Society for Balance jointly developed and promulgated a new version of the 2015 Ménière’s disease diagnostic criteria. The new diagnostic criteria for Ménière’s disease were developed and promulgated. To understand the characteristics of the new version of the criteria, to compare the changes of the old and new versions of the diagnostic criteria, and to discuss the controversial hot issues, it is helpful to carry out basic and clinical research on Ménière’s disease in China, to improve the diagnosis and treatment of vertigo, and to promote the convergence with the international standard.
I. The new diagnostic criteria of Ménière’s disease
In the 1972 version of diagnostic criteria, Meniere’s disease was divided into vestibular Meniere disease and cochlear Meniere disease, the former being defined as patients with episodes of vertigo without hearing loss, and the latter being defined as patients with fluctuating hearing loss but The latter is defined as a patient with fluctuating hearing loss without vertigo onset symptoms.
In the 1995 version of the diagnostic criteria, Meniere’s disease was classified into four categories: certain, definite, probable, and suspicious, and vestibular Meniere’s disease and cochlear Meniere’s disease were no longer used. .
The new 2015 version of the diagnostic criteria simplifies Ménière’s disease into definite and probable categories, as follows.
1, definite Ménière’s disease: (1) vestibular symptoms: more than 2 episodes of spontaneous, episodic vertigo, the duration of each episode 20min~12h; (2) hearing loss characteristics consistent with low-frequency, mid-frequency sensorineural hearing loss, with repeated fluctuations; (3) the affected ear with fluctuating auditory symptoms, including hearing loss, tinnitus and ear stuffiness; (4) exclude other vestibular diseases.
2. Possible Ménière’s disease: (1) vestibular symptoms: more than 2 episodes of spontaneous, episodic vertigo or dizziness, the duration of each episode is 20min~24h; (2) the affected ear is accompanied by fluctuating auditory symptoms, including hearing loss, tinnitus and ear stuffiness; (3) other vestibular diseases are excluded.
In the definition of annotated Ménière’s disease attack time, it refers to the time when the patient is forced to rest and cannot move due to the vertigo attack. 1995 version describes the attack time as 20 min and above, and the new version in 2015 determines it as 20 min~12 h. The clinical significance is to distinguish the attack time of Ménière’s disease from the vestibular migraine criteria of vestibular symptom attack time of 5 min~72 h.
In annotating the subtypes of Ménière’s disease, the new 2015 edition of the criteria no longer has cochlear Ménière’s disease and vestibular Ménière’s disease as in the 1972 edition, and recommends the classification into (1) unilateral type; (2) bilateral type; and (3) co-morbidities [co-morbidities include migraine, benign paroxysmal positional vertigo (BPPV), and systemic autoimmune diseases]. In annotating the degree of hearing loss in Ménière’s disease, the 1995 version of the criteria are: the mean hearing threshold at 0.25, 0.5 and 1 kHz is 15 dB or more higher than the mean hearing threshold at 1, 2 and 3 kHz; in monaural lesions, the mean hearing loss in the affected ear at 0.5, 1, 2 and 3 kHz is 20 dB or more compared to the contralateral ear; in binaural lesions, the mean hearing threshold in the affected ear at 0.5, 1, 2 and 3 kHz The new 2015 version has the following criteria: in the case of monaural lesions, the average hearing loss in the affected ear at 0.5, 1 and 2 kHz is 30 dB or more compared to the contralateral ear; in the case of binaural lesions, the average hearing threshold in the affected ear at 0.5, 1 and 2 kHz is 35 dBHL or more. Compared with the 1995 version, the new 2015 version of the hearing loss criteria increased by 10 dB.
II. Ménière’s disease and inner ear vagal effusion
The only clear-cut pathological change of Ménière’s disease is its endolymphatic hydrops, also known as membranous vagal effusion, but inner ear membranous vagal effusion is not equivalent to Ménière’s disease. First of all, endolymphatic hydrops is the same pathological change after inner ear injury in many diseases. 1983, based on clinical data and pathological findings, Schuknecht and Gulya divided endolymphatic hydrops into two categories, symptomatic and asymptomatic, each of which contains congenital, acquired and idiopathic, of which acquired includes trauma and infection, finally totaling eight types, such as Ménière’s disease is defined as idiopathic, symptomatic membranous vagal effusion. Secondly, the presence of fluid in the membranous vagus of the inner ear does not necessarily present with the clinical manifestations of Ménière’s disease. Temporal bone anatomy studies have found membranous vagal effusion in the contralateral normal ear of some normal people and some patients with monaural Ménière’s disease. Again, some scholars have studied temporal bone specimens from some patients with Ménière’s disease and did not find membranous vagus effusion; others have questioned the necessity of finding membranous vagus effusion through autopsy findings to establish the diagnosis of Ménière’s disease. These ideas and findings that challenge traditional concepts deserve attention and exploration.
III. Ménière’s disease and vestibular migraine
Dizziness or vertigo and headache are common clinical symptoms, and patients with both dizziness or vertigo and headache have been diagnosed with vestibular migraine, migraine-associated vertigo, migraine-associated vestibular disease, and migrainous vertigo, etc. The diagnostic criteria for vestibular migraine were jointly developed by the International Headache Society (HIS) and the International Barany Society in 2012 and were included in the third edition of the 2013 International Classification of Headache It was included in the appendix of the 3rd edition of the International Classification of Headache Disorders (ICHD) beta in 2013, and has become a hot topic of research and reports in the field of vertigo in the past three years. Vestibular migraine is characterized as a headache disorder, with some patients experiencing dizziness or vertigo asynchronously with headache symptoms, and 30% of patients presenting with complaints of dizziness or vertigo, but with poor efficacy of medications targeting dizziness or vertigo, while medications for the treatment and prevention of headache achieve 70% (good) vestibular symptom control. The prevalence of vestibular migraine is significantly higher in patients with Ménière’s disease than in the normal population, and there is symptomatic crossover between the two, with co-morbidity of the two disorders; the choice of different treatment modalities affects the outcome and prognosis, and both vestibular migraine and Ménière’s disease are diseases focused on mutual differentiation in their respective diagnostic criteria. Some scholars consider Ménière’s disease to be atypical vestibular migraine, while others consider vestibular migraine to be vestibular Ménière’s disease. However, it is now accepted that both vestibular migraine and Ménière’s disease are independent disorders, each with clear diagnostic criteria. Since both are diagnosed by symptoms plus exclusion, vestibular migraine can also present with hearing loss, tinnitus, vertigo and stuffy ears, which can be difficult to differentiate from early stage Ménière’s disease, here are some points that may be helpful to distinguish between the two.
Ménière’s disease is an inner ear disease and vestibular migraine is a central vestibular disorder. In vestibular symptoms, patients with Ménière’s disease are vertigo, an illusion of motion, specifically described as spontaneous, rotational vertigo in which there is no objective change in the position of the body relative to the earth’s gravity but subjective perception of body motion. Vestibular migraine is motion intolerance, which is a feeling of body instability, spatial disorientation, or inexplicable uneasiness during active or passive body movements. In terms of cochlear symptoms, vestibular migraine is mostly a subjective sensation of bilateral hearing loss without objective evidence of hearing loss. Although there is bilateral Ménière’s disease, most patients with Ménière’s disease have monaural onset, and fluctuating sensorineural hearing loss can be recorded, with hearing loss worsening with recurrent episodes of vertigo. Vestibular evoked myogenic potentials do not help to differentiate between the two; gadolinium imaging of the inner ear with magnetic resonance imaging of the vestibular membrane vagal effusion in Ménière’s disease helps to differentiate it from vestibular migraine. Patients with vestibular migraine are considered co-morbid with Ménière’s disease if hearing loss and membranous vagal effusion are found on examination.
IV. Refractory Ménière’s disease
Stepwise treatment is the basic treatment strategy for Ménière’s disease, that is, through general treatment, medication and rehabilitation, most patients can have good control of vertigo symptoms, while a small number of patients with poor conservative treatment are called intractable vertigo syndrome, or may receive further destructive treatment or surgery. Refractory vertigo, also known as refractory vertigo syndrome, has no clear and uniform diagnostic criteria in clinical practice, and is expressed in the literature as ineffective for at least 6 months of conservative treatment. For patients with Meniere’s disease, the concept of “refractory” is relative and subjective, but its definition is very important as a basis for choosing destructive or surgical treatment. Some vertigo disorders have atypical early symptoms, and some vertigo disorders are complicated by the presence of a single disorder, and the differential diagnosis of multiple disorders is difficult, so they may be misdiagnosed as “intractable”.
It has been found that some of the possible causes of “intractable Ménière’s disease” include: (1) the cause of vertigo is not removed; (2) the disease itself is misdiagnosed, such as pontocerebellar horn lesions, vestibular migraine, vestibular paroxysms, etc., which are misdiagnosed as Ménière’s disease, making vertigo difficult to treat; (3) vertigo patients are complicated and may be combined with other diseases, such as BPPV, vestibular paroxysms, etc. diseases, such as BPPV, vestibular migraine, cervical spine disease, cardiovascular disease, cerebrovascular disease, congenital malformations and anxiety and depression, etc.; (4) inappropriate drug treatment and inappropriate surgical treatment, etc. Therefore, for patients who are ineffective in conservative treatment and await surgery or potentially damaging treatment, comprehensive evaluation and reasonable intervention before surgery can help reduce the misdiagnosis and mismanagement of Meniere’s disease.
Refractory vertigo is relative, and even if classified as refractory Ménière’s disease, some patients who continue conservative treatment without surgery can have good control of their vertigo symptoms. The rate of complete control of vertigo in patients treated with endolymphatic decompression was 89.6%, and the difference between the two was statistically significant. This result can be used as a reference for doctors and patients when choosing treatment options.
In addition to this, the level and ability of the physicians themselves to diagnose and treat the disease can also influence the conclusion of refractory vertigo. leveque et al. found that the significant decrease in cases of refractory BPPV treated by surgery after 1990 was due to the fact that it was only in the late 1980s that the existence of horizontal and superior semicircular canal otoliths began to be recognized and their differences from posterior semicircular canal otoliths were mastered The clinical features and corresponding, effective manipulation of the otoliths have made the diagnosis of refractory BPPV, which may have been previously misdiagnosed as posterior hemimegalithiasis due to the limitations of the level of awareness, less effective in treatment, less refractory. The above experience can be applied to the judgment of refractory Ménière’s disease. With the increased understanding of vestibular migraine and the advancement of nystagmus detection technology and imaging technology, it will help to reduce the misdiagnosis and mistreatment of patients with Ménière’s disease.
5.Ménière’s disease and endolymphatic sac surgery and chemical vagotomy
In 1923, Portmann first reported endolymphatic sac surgery with the goal of eliminating membranous vagal effusion, and the surgical methods included endolymphatic sac decompression and endolymphatic sac drainage, which has since been recognized as a conservative procedure for the control of intractable vertigo in Ménière’s disease. shunts did not affect the outcome. Thomsen et al. and Bretlau et al. considered endolymphatic sac surgery as a comfort procedure in a controlled study of patients who underwent endolymphatic sac surgery versus mammaplasty alone in a series that lasted for many years; Pullens et al. supported Thomsen et al. through an analysis of evidence-based medical studies. Chung et al. explored direct evidence of whether endolymphatic sac surgery can eliminate membranous vagal effusion in the inner ear by studying temporal bone specimens from patients with Ménière’s disease who underwent endolymphatic sac surgery, and showed that endolymphatic sac surgery does not definitely eliminate membranous vagal effusion in Ménière’s disease; the authors explained that this phenomenon may have some unknown cause for the increase in vertigo attack threshold, in addition to the placebo effect of endolymphatic sac surgery, and not just attributable to the reassuring surgery. To address the question of whether endolymphatic capsule surgery can block the development of binaural Ménière’s disease in patients with monaural Ménière’s disease, Kitahara et al. conducted a study using the glycerol test and cochlear electrogram as reference indicators and found that endolymphatic capsule surgery reduced the appearance of contralateral asymptomatic eustachian vagal effusion but did not prevent the development of binaural Ménière’s disease. In recent years, it was found that the efficacy of semicircular canal obstruction is more exact than that of endolymphatic sac surgery, but the incidence of hearing loss is around 30%, and the procedure is a hot spot for future attention and exploration in vertigo surgery.
Gentamicin tympanic chamber injection for the treatment of recalcitrant peripheral Ménière’s disease has been a clinical hot spot. Pullens and Van Benthem concluded through evidence-based medical analysis that this method can effectively control vertigo symptoms, but the potential, long-term damage to hearing by this treatment cannot be ignored, and there is even the possibility of damaging the function of the balloon and elliptical sacs, thus affecting the balance function. Silverstein et al. found that 57% of patients with Meniere’s disease were free of vertigo episodes within 2 years of onset and 71% of patients were free of vertigo episodes within 8.3 years. These factors should be fully considered when assessing the effect of gentamicin bulbar injection therapy.
Concerned because of the controversy. In the face of many problems in the diagnosis and treatment of Meniere’s disease, only in-depth research and continuous solutions can enable patients to receive the most appropriate treatment and avoid misdiagnosis and mistreatment.