- Ménière’s disease (MD) is a clinical syndrome affecting approximately 50–200/100,000 adults and is most common between the ages of 40–60.
- The diagnosis of MD is made clinically since the disease typically presents with unilateral ear symptoms that can last for several decades. MD attacks are typically random and episodic (approximately 6–11/year), with periods of remission that may last months to years.
- As such, the diagnosis of MD is typically not made at one point in time; rather it may take months or even years to fully appreciate the clinical manifestations leading to definitive diagnosis.
- The natural course of MD is typically progressive and fluctuates unpredictably.
- It is important to clinically distinguish MD from other independent causes of vertigo such as otosyphilis, vestibular neuritis and acute labyrinthitis that may mimic MD since they also present with hearing loss, tinnitus, and aural fullness.
- The goals of MD treatment are to prevent or at least reduce the severity and frequency of vertigo attacks. In addition, treatment approaches aim to relieve or prevent hearing loss, tinnitus, and aural fullness and to improve overall quality of life.
Table 1. Diagnostic Criteria
|Two or more spontaneous attacks of vertigo, each lasting 20 min to 12 hours|
|Audiometrically documented fluctuating low- to mid-frequency sensorineural hearing loss in the affected ear on at least one occasion before, during, or after one of the episodes of vertigo|
|Fluctuating aural symptoms (hearing loss, tinnitus, or fullness) in the affected ear|
|Other causes excluded by other tests|
|At least two episodes of vertigo or dizziness lasting 20 min to 24 hours|
|Fluctuating aural symptoms (hearing loss, tinnitus, or fullness) in the affected ear|
|Other causes excluded by other tests|
Table 2. Key Definitions for Ménière’s Disease (MD) Guideline
|Vertigo||Sensation of self-motion (rotary spinning) or movement of the environment when neither is occurring or the sensation of distorted self-motion (rotation or spinning) during an otherwise normal head movement|
|Imbalance||Sense of unsteadiness, or instability; discrete from vertigo; may be ongoing and not episodic|
|Acute MD attack||Vertigo episode that lasts for 20 min to 12 hours and aural symptoms (timing impacted by treatment onset)|
|Active MD||Describes periods during which episodic acute attacks of MD occur with some regularity|
|Definitive MD||See Table 1|
|Drop attacks (Tumarkin’s Otolithic Crisis)||Sudden fall associated with discrete MD attacks with no warning; the patient does not lose consciousness. Drop attacks may be experienced during later stages of MD, and they are not present in every patient.|
Levels of adequate hearing perception often defined by the patient; may be audiometrically defined based on level of hearing loss (HL), pure tone average (PTA) and word recognition/discrimination scores (WRS) from vestibular schwannoma literature:
|Probable MD||See Table 1|
|PTA||Pure Tone Average measured by audiometry|
|Hearing loss in MD||Often fluctuates from low- to mid-frequency but over time may involve all frequencies|
Table 3. Summary of Key Action Statements (KAS)
|1. Diagnosis of Ménière’s Disease||Clinicians should diagnose definite or probable Ménière’s disease in patients presenting with two or more episodes of vertigo lasting 20 minutes to 12 hours (definite) or up to 24 hours (probable) and fluctuating or non-fluctuating sensorineural hearing loss, tinnitus or pressure in the affected ear, when these symptoms are not better accounted for by another disorder.||Recommendation|
|2. Assessing for Vestibular Migraine||Clinicians should determine if patients meet diagnostic criteria for vestibular migraine when assessing for Ménière’s disease.||Recommendation|
|3. Audiometric Testing||Clinicians should obtain an audiogram when assessing a patient for the diagnosis of Ménière’s disease.||Strong Recommendation|
|4. Utility of Imaging||Clinicians may offer magnetic resonance imaging (MRI) of the internal auditory canal (IAC) and posterior fossa in patients with possible Ménière’s disease and audiometrically verified asymmetric sensorineural hearing loss.||Option|
|5. Vestibular or Electrophysiologic Testing||Clinicians should not routinely order vestibular function testing or electrocochleography to establish the diagnosis of Ménière’s disease.||Recommendation Against|
|6. Patient Education||Clinicians should educate patients with Ménière’s disease about the natural history, measures for symptom control, treatment options, and outcomes.||Recommendation|
|7. Symptomatic Management of Vertigo||Clinicians should offer a limited course of vestibular suppressants to patients with Ménière’s disease for management of vertigo only during Ménière’s disease attacks.||Recommendation|
|8. Symptom Reduction and Prevention||Clinicians should educate patients with Ménière’s disease on dietary and lifestyle modifications that may reduce or prevent symptoms.||Recommendation|
|9. Oral Pharmacotherapy for Maintenance||Clinicians may offer diuretics and/or betahistine for maintenance therapy to reduce symptoms or prevent Ménière’s disease attacks.||Option|
|10. Positive Pressure Therapy||Clinicians should not prescribe positive pressure therapy for patients with Ménière’s disease.||Recommendation Against|
|11. Intratympanic Steroid Therapy||Clinicians may offer, or refer to a clinician who can offer, intratympanic steroids to patients with active Ménière’s disease not responsive to non-invasive treatment.||Option|
|12. Intratympanic Gentamicin Therapy||Clinicians should offer, or refer to a clinician who can offer, intratympanic gentamicin to patients with active Ménière’s disease not responsive to non-ablative therapy.||Recommendation|
|13. Surgical Ablative Therapy||Clinicians may offer, or refer to a clinician who may offer, labyrinthectomy in patients with active Ménière’s disease who have failed less definitive therapy and have non-usable hearing.||Recommendation|
|14a. Role of Vestibular Therapy for Chronic Imbalance||Clinicians should offer vestibular rehabilitation/physical therapy for Ménière’s disease patients with chronic imbalance.||Recommendation|
|14b. Role of Vestibular Therapy for Acute Vertigo||Clinicians should not recommend vestibular rehabilitation/physical therapy for managing acute vertigo attacks in patients with Ménière’s disease.||Recommendation Against|
|15. Counseling for Amplification and Hearing Assistive Technology||Clinicians should counsel patients, or refer to a clinician who can counsel patients, with Ménière’s disease and hearing loss on the use of amplification and hearing assistive technology.||Recommendation|
|16. Patient Outcomes||Clinicians should document resolution, improvement, or worsening of vertigo, tinnitus, and hearing loss and any change in quality of life in patients with Ménière’s disease after treatment.||Recommendation|
Table 4. Common Causes of Vertigo/Dizziness and Their Differentiating Features from MD
|Condition||Clinical Presentation||Differentiation from MD|
|Autoimmune (e.g., multiple sclerosis)||Often progressive fluctuating bilateral hearing loss that is steroid responsive.||May present with vision, skin, and joint problems|
|Benign Paroxysmal Positional Vertigo (BPPV)||Positional vertigo lasting less than a minute (i.e., seconds).||Not associated with hearing loss, tinnitus, or aural fullness; short duration of vertigo spells.|
|Infectious (e.g., Lyme disease)||Viral (i.e. adenovirus) or bacterial (e.g., staph/strep); can lead to complete hearing loss and vestibular crisis event with prolonged vertigo and/or hearing loss.||Losses are often permanent and do not fluctuate. Can present with severe otalgia and fever.|
|Otosyphilis||Sudden, unilateral or bilateral, sensorineural, fluctuating hearing loss, tinnitus and/or vertigo.||Vertigo attacks not typically associated with aural symptoms immediately before or after attacks.|
|Stroke/Ischemia||Vertigo may last for minutes with nausea, vomiting, severe imbalance. May also include visual blurring and drop attacks.||Insults are often permanent and do not fluctuate. May be comorbid with dysphagia, dysphonia, or other neurologic symptoms and signs. Usually no associated hearing loss, tinnitus.|
|Vestibular Migraine||Presents with attacks lasting hours but can also present with attacks lasting minutes or >24 hours.||Timing of attacks may be shorter or longer than MD. Hearing loss less likely. Patients often have a migraine history; more photophobia than visual aura.|
|Vestibular Schwannoma||May present with vertigo. Majority present with chronic imbalance, asymmetric hearing loss, and tinnitus.||Chronic imbalance more likely than profound episodic vertigo. Hearing loss does not typically fluctuate.|
|Labyrinthitis||Sudden severe vertigo with profound hearing loss and prolonged vertigo (i.e., >24 hours).||Vertigo, nausea with hearing loss, not episodic, not fluctuating.|
|Vestibular Neuritis||Viral infection of vestibular system: Leads to acute, prolonged vertigo with prolonged nausea, vomiting without hearing loss, tinnitus, or aural fullness. Severe rotational vertigo lasts 12–36 hours with decreasing disequilibrium for the next four to five days.||Vertigo, nausea without hearing loss.|
Table 5. International Classification of Headache Disorders (ICHD) Diagnostic Criteria for Migraine
|1.1 Migraine without aura|
|Previously used terms:|
Common migraine; hemicrania simplex.Description:
Recurrent headache disorder manifesting in attacks lasting 4–72 hours. Typical characteristics of the headache are unilateral location, pulsating quality, moderate or severe intensity, aggravation by routine physical activity and association with nausea and/or photophobia and phonophobia.Diagnostic criteria:
1. One or a few migraine attacks may be difficult to distinguish from symptomatic migraine-like attacks. Furthermore, the nature of a single or a few attacks may be difficult to understand. Therefore, at least five attacks are required. Individuals who otherwise meet criteria for 1.1 Migraine without aura but have had fewer than five attacks should be coded 1.5.1 Probable migraine without aura.
2. When the patient falls asleep during a migraine attack and wakes up without it, duration of the attack is reckoned until the time of awakening.
3. In children and adolescents (aged under 18 years), attacks may last 2–72 hours (the evidence for untreated durations of less than two hours in children has not been substantiated).
|1.2 Migraine with aura|
|Previously used terms:|
Classic or classical migraine ophthalmic, hemiparaesthetic, hemiplegic or aphasic migraine, migraine accompagnée, complicated migraine.Description:
Recurrent attacks, lasting minutes, of unilateral fully reversible visual, sensory, or other central nervous system symptoms that usually develop gradually and are usually followed by headache and associated migraine symptoms.Diagnostic criteria:
1. When, for example, three symptoms occur during an aura, the acceptable maximal duration is 3×60 minutes. Motor symptoms may last up to 72 hours.
2. Aphasia is always regarded as a unilateral symptom; dysarthria may or may not be.
3. Scintillations and pins and needles are positive symptoms of aura.
Table 6. Barany Diagnostic Criteria for Vestibular Migraine
|1. Vestibular migraine|
|2. Probable vestibular migraine|
• Spontaneous vertigo including:
◦ Internal vertigo, a false sensation of self-motion, and
◦ External vertigo, a false sensation that the visual surrounding is spinning or flowing
• Position vertigo, occurring after a change in head position
• Visually-induced vertigo, triggered by a complex or large moving visual stimulus
• Head motion-induced vertigo, occurring during head motion
• Head motion-induced dizziness with nausea. Dizziness is characterized by a sensation of disturbed spatial orientation. Other forms of dizziness are currently not included in the classification of vestibular migraine. b Vestibular symptoms are rated “moderate” when they interfere with but do not prohibit daily activities and “severe” if daily activities cannot be continued.
c Duration of episodes is highly variable: About 30% of patients have episodes lasting minutes, 30% have attacks for hours and another 30% have attacks over several days. The remaining 10% have attacks lasting seconds only and tend to occur repeatedly during head motion, visual stimulation, or after changes of head position. In these patients, episode duration is defined as the total period during which short attacks recur. At the other end of the spectrum, there are patients who may take four weeks to fully recover from an episode. However, the core episode rarely exceeds 72 hours.
d Migraine categories 1.1 and 1.2 of the ICDH.
e One symptom is sufficient during a single episode. Different symptoms may occur during differentepisodes. Associated symptoms may occur before, during or after the vestibular symptoms.
f Phonophobia is defined as sound-induced discomfort. It is a transient and bilateral phenomenon that must be differentiated from recruitment, which is often unilateral and persistent. Recruitment leads to an enhanced perception and often distortion of loud sounds in an ear with decreased hearing.
g Visual auras are characterized by bright scintillating lights or zigzag lines, often with a scotoma that interferes with reading. Visual auras typically expand over 5–20 minutes and last for less than 60 minutes. They are often, but not always, restricted to one hemifield. Other types of migraine aura (e.g., somatosensory or dysphasic aura) are not included as diagnostic criteria because their phenomenology is less specific. and most patients also have visual auras.
h History and physical examinations do not suggest another vestibular disorder, or such a disorder is considered but ruled out by appropriate investigations, or such disorder is present as a comorbid or independent condition, but episodes can be clearly differentiated. Migraine attacks may be induced by vestibular stimulation. Therefore, the differential diagnosis should include other vestibular disorders complicated by superimposed migraine attacks.
* Reprinted from Journal of Vestibular Research, Vol 22, Author(s), Vestibular migraine: diagnostic criteria, 167-172, Copyright 2012, with permission from IOS Press.
Table 7. IT Steroid Therapy Dosing and Frequency
|Dexamethasone sodium phosphate||Stock: 4 mg/mL or 10 mg/mL|
Compounded: 16 mg/mL or 24 mg/mL
|Inject 0.4–0.8 mL into middle ear space, from once only or up to 3–4 sessions every 3–7 days depending on clinical response.|
|Methylprednisolone sodium succinate||Stock: 30 mg/mL or 40 mg/mL|
Compounded: 62.5 mg/mL
Table 8. The American Academy of Otolaryngology-Head and Neck Surgery Hearing Classification Criteria
|Hearing Category||Average PTA||Speech Discrimination|
|A||≤30 dB HL||>70%|
|B||>30– ≤50 dB HL||≥50%|
|C||>50 dB HL||≥50%|