--- title: "Meniere's Disease Part 2 - Clinical Features, Variants, Diagnostic Criteria, Investigations & Staging" description: "ENT notes on Meniere's Disease - Clinical Features, Variants, Diagnostic Criteria, Investigations & Staging." source: "The ENT Resident" author: "Dr. Mausumi Das" canonical_url: "https://www.theentresident.com/ent-notes-lectures/menieres-disease-symptoms-signs-diagnosis" categories: ["Otology"] video: "https://www.youtube.com/watch?v=wT8G2vXc6cg" image: "https://media.theentresident.com/og-images/menieres-disease-symptoms-signs-diagnosis.png" --- # Meniere's Disease Part 2 - Clinical Features, Variants, Diagnostic Criteria, Investigations & Staging ENT notes on Meniere's Disease - Clinical Features, Variants, Diagnostic Criteria, Investigations & Staging. Watch the full video lecture on YouTube: https://www.youtube.com/watch?v=wT8G2vXc6cg ## 👂 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](https://www.theentresident.com/ent-notes-lectures/tullio-phenomenon-hennebert-sign.md)? 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](https://www.theentresident.com/ent-notes-lectures/tuning-fork-tests-rinne-weber-absolute-bone-conduction.md) Show features of **sensorineural hearing loss**: | Test | Finding | | --- | --- | | Rinne's test | Positive | | Weber's test | Lateralized to the better ear | | Absolute bone conduction test | Reduced in the affected ear | --- ### 🔍 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**. | Disease | Sequence of Symptoms | | --- | --- | | **Meniere's disease** | Vertigo, then sensorineural hearing loss and tinnitus | | **Lermoyez syndrome** | Hearing loss and tinnitus first, then vertigo | --- ### 📋 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** | Stage of Disease | Frequencies Affected | Type of Curve | | --- | --- | --- | | Early | Lower frequencies only | **Rising curve** | | Progressing | Lower and middle frequencies | **Flat curve** | | Advanced | All frequencies | **Falling curve** | **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** | Curve | SP/AP Ratio | | --- | --- | | Normal | 30% | | Meniere's disease | **Greater than 30%**, due to an increased summating potential | **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**. | Stage | Pure Tone Average | | --- | --- | | **Stage 1** | 25 dB or less | | **Stage 2** | 26 to 40 dB | | **Stage 3** | 41 to 70 dB | | **Stage 4** | More than 70 dB | > 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. --- ## About This Note This free ENT note on Meniere's Disease Part 2 - Clinical Features, Variants, Diagnostic Criteria, Investigations & Staging was written by Dr. Mausumi Das and is paired with a corresponding video lecture. 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