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Meniere's disease (MD) is an idiopathic chronic inner ear disorder characterized by recurrent episodes of spontaneous vertigo and fluctuating sensorineural hearing loss, tinnitus, and ear fullness
.
At present, the academic community believes that Meniere's disease is a dynamic and progressive inner ear disease, and it will show different clinical features at different stages of disease development and different pathological changes
.
Today, let's systematically learn about the diagnosis and treatment of MD
.
Prevalence and Pathophysiological Mechanisms The prevalence of MD is 5 to 500 per 100,000 inhabitants, and familial MD accounts for about 9% of cases
.
Studies have shown that MD symptoms are associated with the accumulation of endolymph in the cochlear duct and inner ear balloon
.
It is thought that this endolymphatic edema begins with a disturbance in the ionic composition of the cochlear mediator
.
However, current data support the hypothesis that endolymphedema is an epiphenomenon associated with multiple inner ear diseases, with familial clustering suggesting that genetic and environmental factors contribute to its development
.
Diagnostic criteria The International Committee on the Classification of Vestibular Nerve Disorders of the Barany Society has developed diagnostic criteria in collaboration with the American Academy of Otolaryngology-Head and Neck Surgery, the European Society of Otology and Neurootology, the Japanese Society for the Study of Balance, and the Korean Society of Balance
.
These criteria define two categories: definite MD and probable MD (Table 1)
.
Table 1 Diagnostic criteria for MD A national survey in the United States showed that only 26.
9% - 46.
7% of otolaryngologists rely solely on history, physical examination and audiometry to diagnose MD
.
Therefore, auxiliary examinations help to support the clinical diagnosis, especially given the high rate of misdiagnosis (mainly overdiagnosis) of MD
.
➤ Eye movement examination should assess the presence of spontaneous and gaze-evoked nystagmus, vestibulo-ocular reflex (head impulse test), tracking, and integrity of saccadic eye movements
.
Postural manipulation should be performed in any patient with paroxysmal vertigo to rule out benign paroxysmal vertigo
.
➤ Bedside audiometric assessment may not be sufficient to determine hearing loss, and any patient with suspected MD should obtain an audiogram, including tympanometric measurements
.
Hearing loss is usually in the low frequency range, and MD can lead to a marked intolerance to loud sounds
.
In the early stages of the disease, hearing loss and tinnitus are fluctuating, but in later stages, they may become permanent
.
➤ Brain imaging, preferably MRI, helps exclude secondary etiologies and posterior fossa lesions that may present with progressive hearing loss and vertigo
.
In addition, endolymphedema in MD was visualized on high-resolution MRI following transtympanic gadolinium injection
.
➤ In electrocochleography (EChG), evoked summed potentials (SP) and action potentials (AP) elicited by click or burst stimulation recorded by intratympanic or extratympanic electrodes were recorded
.
In a large case series, an increased SP/AP ratio (>0.
4) was found in 72% of patients with a clinical diagnosis of MD, and the results of the test increased with disease severity and duration
.
Although EChG is not yet widely available in routine clinical practice, it is valuable, especially in the presence of diagnostic uncertainty
.
➤The cervical vestibular evoked myogenic potential (cVEMP) is a short-latency ipsilateral sternocleidomastoid muscle inhibitory potential evoked by a brief, loud (>85 dB) monaural click or short sound stimulation
.
cVEMP is thought to be of cystic origin, mediated by the inferior vestibular nerve
.
Compared with controls, MD patients had elevated cVEMP thresholds or absent VEMP
.
Consistent with the otolithic basis, abnormal CVEMP was more common in patients with Tumarkin crisis and was also seen in the ears of 27% of patients with contralateral asymptomatic MD
.
➤ Nystagmus has a limited role in the diagnosis of MD, but may help differentiate MD from central causes of vertigo
.
Although 42% to 73% of MD patients have pathological semicircular canal paresis, complete loss of function is rare
.
A temperature test is useful to assess contralateral function prior to ablation procedures, to assess postoperative residual function, and to determine whether the patient has preserved ipsilateral neural canal function, and non-destructive treatment options may be preferred
.
The natural history of treating MD is unpredictable, although the onset is usually unilateral
.
Bilateral involvement was more common with longer disease course: 15% in the first two years, 35% after 10 years, and as high as 47% after 20 years
.
➤ In acute attacks of MD, treatment is symptomatic
.
Nausea and vomiting can be treated with standard antiemetics, such as phenothiazines, antihistamines, and anticholinergics
.
It is reasonable to use benzodiazepines to relieve anxiety during an acute attack
.
➤ One intervention is dietary salt restriction (daily sodium intake <2 g/day), which is thought to reduce osmotic pressure build-up in the endolymphatic compartment
.
Using similar logic, diuretics have also been used in MD
.
➤High-dose betahistine may have a preventive effect on MD attack frequency at least in the first year, although its effect on vestibular and hearing function is unclear
.
➤Although the evidence is still lacking, systemic steroids should be considered in patients with co-morbid autoimmune disease, especially bilateral sensorineural hearing loss
.
There is also weak evidence that intratympanic dexamethasone reduces the onset of MD without significant systemic side effects
.
➤Intratympanic gentamicin therapy may be effective in reducing seizures in patients with significant loss of hearing vestibular function in the affected ear but with persistent severe seizures
.
➤ Finally, given the volatility of vestibular symptoms in these patients, the role of vestibular rehabilitation in the acute phase of the disease is controversial
.
However, there is evidence that vestibular neurorehabilitation can improve both subjective and objective balance function in MD patients
.
➤ Expert referral refers to patients with clinically suspected MD
.
Such patients may also require referral for medical and surgical intervention if symptoms persist or are severe, and ensuring the participation of a multidisciplinary team including an otolaryngologist, neurootologist, physical therapist, and audiologist
.
Given the unpredictable and disabling nature of the disease, psychological support plays an important role in long-term management to improve quality of life
.
Compiled from: Seemungal B, Kaski D, Lopez-Escamez JA.
Early Diagnosis and Management of Acute Vertigo from Vestibular Migraine and Ménière's Disease.
Neurol Clin.
2015 Aug;33(3):619-28, ix.
doi: 10.
1016/j.
ncl.
2015.
04.
008.
Epub 2015 Jun 12.
PMID: 26231275.
.
At present, the academic community believes that Meniere's disease is a dynamic and progressive inner ear disease, and it will show different clinical features at different stages of disease development and different pathological changes
.
Today, let's systematically learn about the diagnosis and treatment of MD
.
Prevalence and Pathophysiological Mechanisms The prevalence of MD is 5 to 500 per 100,000 inhabitants, and familial MD accounts for about 9% of cases
.
Studies have shown that MD symptoms are associated with the accumulation of endolymph in the cochlear duct and inner ear balloon
.
It is thought that this endolymphatic edema begins with a disturbance in the ionic composition of the cochlear mediator
.
However, current data support the hypothesis that endolymphedema is an epiphenomenon associated with multiple inner ear diseases, with familial clustering suggesting that genetic and environmental factors contribute to its development
.
Diagnostic criteria The International Committee on the Classification of Vestibular Nerve Disorders of the Barany Society has developed diagnostic criteria in collaboration with the American Academy of Otolaryngology-Head and Neck Surgery, the European Society of Otology and Neurootology, the Japanese Society for the Study of Balance, and the Korean Society of Balance
.
These criteria define two categories: definite MD and probable MD (Table 1)
.
Table 1 Diagnostic criteria for MD A national survey in the United States showed that only 26.
9% - 46.
7% of otolaryngologists rely solely on history, physical examination and audiometry to diagnose MD
.
Therefore, auxiliary examinations help to support the clinical diagnosis, especially given the high rate of misdiagnosis (mainly overdiagnosis) of MD
.
➤ Eye movement examination should assess the presence of spontaneous and gaze-evoked nystagmus, vestibulo-ocular reflex (head impulse test), tracking, and integrity of saccadic eye movements
.
Postural manipulation should be performed in any patient with paroxysmal vertigo to rule out benign paroxysmal vertigo
.
➤ Bedside audiometric assessment may not be sufficient to determine hearing loss, and any patient with suspected MD should obtain an audiogram, including tympanometric measurements
.
Hearing loss is usually in the low frequency range, and MD can lead to a marked intolerance to loud sounds
.
In the early stages of the disease, hearing loss and tinnitus are fluctuating, but in later stages, they may become permanent
.
➤ Brain imaging, preferably MRI, helps exclude secondary etiologies and posterior fossa lesions that may present with progressive hearing loss and vertigo
.
In addition, endolymphedema in MD was visualized on high-resolution MRI following transtympanic gadolinium injection
.
➤ In electrocochleography (EChG), evoked summed potentials (SP) and action potentials (AP) elicited by click or burst stimulation recorded by intratympanic or extratympanic electrodes were recorded
.
In a large case series, an increased SP/AP ratio (>0.
4) was found in 72% of patients with a clinical diagnosis of MD, and the results of the test increased with disease severity and duration
.
Although EChG is not yet widely available in routine clinical practice, it is valuable, especially in the presence of diagnostic uncertainty
.
➤The cervical vestibular evoked myogenic potential (cVEMP) is a short-latency ipsilateral sternocleidomastoid muscle inhibitory potential evoked by a brief, loud (>85 dB) monaural click or short sound stimulation
.
cVEMP is thought to be of cystic origin, mediated by the inferior vestibular nerve
.
Compared with controls, MD patients had elevated cVEMP thresholds or absent VEMP
.
Consistent with the otolithic basis, abnormal CVEMP was more common in patients with Tumarkin crisis and was also seen in the ears of 27% of patients with contralateral asymptomatic MD
.
➤ Nystagmus has a limited role in the diagnosis of MD, but may help differentiate MD from central causes of vertigo
.
Although 42% to 73% of MD patients have pathological semicircular canal paresis, complete loss of function is rare
.
A temperature test is useful to assess contralateral function prior to ablation procedures, to assess postoperative residual function, and to determine whether the patient has preserved ipsilateral neural canal function, and non-destructive treatment options may be preferred
.
The natural history of treating MD is unpredictable, although the onset is usually unilateral
.
Bilateral involvement was more common with longer disease course: 15% in the first two years, 35% after 10 years, and as high as 47% after 20 years
.
➤ In acute attacks of MD, treatment is symptomatic
.
Nausea and vomiting can be treated with standard antiemetics, such as phenothiazines, antihistamines, and anticholinergics
.
It is reasonable to use benzodiazepines to relieve anxiety during an acute attack
.
➤ One intervention is dietary salt restriction (daily sodium intake <2 g/day), which is thought to reduce osmotic pressure build-up in the endolymphatic compartment
.
Using similar logic, diuretics have also been used in MD
.
➤High-dose betahistine may have a preventive effect on MD attack frequency at least in the first year, although its effect on vestibular and hearing function is unclear
.
➤Although the evidence is still lacking, systemic steroids should be considered in patients with co-morbid autoimmune disease, especially bilateral sensorineural hearing loss
.
There is also weak evidence that intratympanic dexamethasone reduces the onset of MD without significant systemic side effects
.
➤Intratympanic gentamicin therapy may be effective in reducing seizures in patients with significant loss of hearing vestibular function in the affected ear but with persistent severe seizures
.
➤ Finally, given the volatility of vestibular symptoms in these patients, the role of vestibular rehabilitation in the acute phase of the disease is controversial
.
However, there is evidence that vestibular neurorehabilitation can improve both subjective and objective balance function in MD patients
.
➤ Expert referral refers to patients with clinically suspected MD
.
Such patients may also require referral for medical and surgical intervention if symptoms persist or are severe, and ensuring the participation of a multidisciplinary team including an otolaryngologist, neurootologist, physical therapist, and audiologist
.
Given the unpredictable and disabling nature of the disease, psychological support plays an important role in long-term management to improve quality of life
.
Compiled from: Seemungal B, Kaski D, Lopez-Escamez JA.
Early Diagnosis and Management of Acute Vertigo from Vestibular Migraine and Ménière's Disease.
Neurol Clin.
2015 Aug;33(3):619-28, ix.
doi: 10.
1016/j.
ncl.
2015.
04.
008.
Epub 2015 Jun 12.
PMID: 26231275.
