Meniere's Disease Part 2 - Clinical Features, Variants, Diagnostic Criteria, Investigations & Staging

👂 Meniere's Disease - Clinical Features, Diagnosis & Investigations

🗣️ Symptoms of Meniere's Disease

⭐ Triad of Symptoms

1- Vertigo

2- Sensorineural hearing loss

3- Tinnitus

👉 A fourth symptom is aural fullness.

1- Vertigo

Definition of a Definitive Spell

A spontaneous rotational vertigo lasting at least 20 minutes, and which may last up to 24 hours.

What is Spontaneous rotational vertigo?

What are the features of vertigo seen in Meniere's disease?

Associated Symptoms

• Nausea
• Vomiting
• Ataxia
• Nystagmus

⚠️ There is NO loss of consciousness during or after the vertigo. ⭐ Key point.

⭐ An attack may be heralded by aural fullness, a change in the character of tinnitus, or a decrease in hearing.

In Severe Attacks - Vagal Disturbances are seen.

What are the vagal disturbances seen in severe attacks of Meniere's disease?

• Tullio Phenomenon is also seen

What is Tullio Phenomenon? Why does it occur in Meniere's disease?

How can you classify the Severity of Vertigo in Meniere's disease?

2- Hearing Loss

Type of hearing loss:

👉 Sensorineural hearing loss, most commonly unilateral, fluctuating and progressive.

Diplacusis and Recruitment are also seen.

What are the characteristics of the Hearing Loss seen in Meniere's Disease?

Name some causes of fluctuating sensorineural hearing loss.

Which frequencies are involved in hearing loss seen in Meniere's disease?

What is the percentage of Bilateral hearing loss in Meniere's disease?

What is Diplacusis?

What is Recruitment? What are the tests of Recruitment?

3- Tinnitus

• Non-pulsatile
• Low pitched
• Whistling, roaring or hissing in character
• May be continuous or intermittent
• Becomes louder or changes pitch as an attack approaches
• Improves after the attack passes
• May disappear or persist during periods of remission

4- Aural Fullness

• Fluctuating in nature
• Completely disappears during periods of remission
• May accompany or precede an attack of vertigo

🩺 Signs of Meniere's Disease

Otoscopy

👉 No abnormality detected

Nystagmus

⭐ Seen only during the acute attack. The direction changes with the phase of the attack.

What is the direction of nystagmus in each phase of attack - Irritative phase, Paretic phase and Recovery phase?

Tuning Fork Tests

Show features of sensorineural hearing loss:

🔍 Variants of Meniere's Disease

1️⃣ Cochlear hydrops

Only cochlear signs and symptoms are present.

What is the pathogenesis behind Cochlear hydrops?

2️⃣ Vestibular hydrops

Only vestibular symptoms are present.

• Patient gets attacks of episodic vertigo
• Cochlear functions remain normal, so there is no hearing loss
• The typical picture of Meniere's disease develops only after a long period

⭐ Many of these cases are referred to as recurrent vestibulopathy.

3️⃣ Tumarkin's otolithic crisis (Drop attacks)

⭐ Seen in only about 2% of cases of Meniere's disease.

What are the features of Drop attacks in Meniere's disease?

What is the mechanism behind development of Tumarkin's otolithic crisis?

4️⃣ Lermoyez syndrome

The same as Meniere's disease, but with the symptoms occurring in reverse order.

📋 Diagnostic Criteria of Meniere's Disease (2015 Guidelines)

The current criteria are a joint consensus given by five committees:

1- Classification Committee of the Barany Society

2- Japan Society for Equilibrium Research

3- European Academy of Otology and Neurotology

4- Equilibrium Committee of the American Academy of Otolaryngology, Head and Neck Surgery

5- Korean Balance Society

⭐ The 1995 guidelines had four classes. The 2015 guidelines have only two: Definite Meniere's disease and Probable Meniere's disease.

⭐ Diagnosis is based on the clinical symptoms together with exclusion of other identifiable etiologies, rendering the disease idiopathic.

What are the latest diagnostic criteria for Definite Meniere's Disease?

What are the latest diagnostic criteria for Probable Meniere's Disease?

What are the Etiologies to be Excluded to make the diagnosis of Meniere's Disease?

🧪 Investigations of Meniere's Disease

1- Pure Tone Audiometry

2- Speech Audiometry

• Discrimination score usually 55 to 85% between attacks
• Discrimination ability is impaired during and immediately following an attack

3- Special Audiometry Tests

Done to differentiate cochlear from retrocochlear disease.

What are the Audiometry Tests to differentiate between cochlear and retrocochlear disease?

⭐ Meniere's disease is a cochlear disease; acoustic neuroma is a retrocochlear disease.

4- Electrocochleography

5- Caloric Test

• Reduced response on the affected side in about 75% of cases
• Reveals canal paresis of the affected side (most common finding)
• May also show directional preponderance to the healthy side, or a combination of canal paresis and directional preponderance

6- VEMP (Vestibular Evoked Myogenic Potential)

What findings do you see in VEMP in Meniere's Disease?

7- CHAMP (Cochlear Hydrops Analysis Masking Procedure)

What is the Hypothesis behind CHAMP?

⚠️ Shows conflicting results across different groups, so it is not a reliable investigation.

8- Glycerol Test

Principle

Glycerol is a dehydrating agent. It reduces the endolymphatic pressure, thereby producing an improvement in hearing.

What is the procedure of Glycerol test?

What are the findings of Glycerol test in Meniere's disease?

⭐ The glycerol test has both diagnostic and prognostic value.

9- Imaging

⭐ Imaging is not required for diagnosis. It is done to rule out secondary causes.

What will you check in CT and MRI of temporal bone in a case of Meniere's disease?

📊 Staging of Meniere's Disease

Staging is based on the pure tone average of 0.5, 1, 2 and 3 kHz of the worst audiogram in the preceding 6 months.

What is the Differential Diagnosis of Meniere's Disease?

🧠 Viva Pearls

• The triad of Meniere's disease is vertigo, sensorineural hearing loss and tinnitus, with aural fullness as a fourth symptom.
• A definitive spell of vertigo lasts at least 20 minutes and may last up to 24 hours.
• There is no loss of consciousness during or after an attack of vertigo.
• Meniere's disease is one of the most common causes of fluctuating sensorineural hearing loss.
• Tinnitus is non-pulsatile and low pitched, described as whistling, roaring or hissing.
• Otoscopy shows no abnormality.
• Tumarkin's otolithic crisis is a drop attack without loss of consciousness, seen in about 2% of cases.
• Lermoyez syndrome shows the symptoms of Meniere's disease in reverse order.
• The 2015 guidelines classify the disease as definite or probable.
• Electrocochleography shows an SP/AP ratio greater than 30%.
• Caloric test shows canal paresis on the affected side in about 75% of cases.

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