A 42-year-old woman from Mumbai presents with a 6-month history of episodic severe vertigo lasting 2–3 hours, accompanied by tinnitus and a sensation of fullness in the left ear. Between episodes, she experiences progressive hearing loss in the low frequencies. Audiometry shows sensorineural hearing loss at 250–1000 Hz. Caloric testing reveals reduced vestibular response on the left. MRI of the internal acoustic meatus is normal. What is the most likely diagnosis?

## Diagnosis: Meniere Disease ### Clinical Presentation **Key Point:** Meniere disease is characterized by the classic tetrad of episodic vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness — all present in this patient. ### Diagnostic Features | Feature | Finding in This Case | Significance | |---------|----------------------|---------------| | **Vertigo** | Episodic, 2–3 hours | Typical duration (20 min–several hours) | | **Hearing Loss** | Low-frequency SNHL (250–1000 Hz) | Pathognomonic for Meniere; high frequencies spared early | | **Tinnitus** | Present | Fluctuates with disease activity | | **Aural Fullness** | Yes | Due to endolymphatic hydrops | | **Caloric Response** | Reduced on left | Indicates vestibular involvement | | **MRI** | Normal | Rules out retrocochlear pathology (e.g., acoustic neuroma) | ### Pathophysiology **High-Yield:** Meniere disease results from **endolymphatic hydrops** — abnormal accumulation of endolymph in the cochlea and vestibule. This causes: 1. Rupture of Reissner's membrane → mixing of endolymph and perilymph 2. Acute potassium influx into vestibular and cochlear hair cells 3. Depolarization → vertigo, hearing loss, tinnitus ### Diagnostic Criteria (Definite Meniere Disease) **Mnemonic: AAAA** - **A**ural fullness (aural symptoms) - **A**udiometric evidence (low-frequency SNHL) - **A**ttacks of vertigo (2 or more, 20 min–12 hours) - **A**ll other causes excluded ### Why MRI is Important **Clinical Pearl:** Normal MRI excludes retrocochlear lesions (acoustic neuroma, MS). Gadolinium-enhanced MRI may show endolymphatic sac enlargement in Meniere disease, but diagnosis is primarily clinical. ### Management Overview ```mermaid flowchart TD A[Meniere Disease Diagnosed]:::outcome --> B{Frequency & Severity?}:::decision B -->|Mild/Infrequent| C[Conservative: Salt restriction, diuretics]:::action B -->|Moderate| D[Intratympanic corticosteroids or gentamicin]:::action B -->|Severe/Refractory| E[Surgical: Endolymphatic sac decompression or labyrinthectomy]:::action C --> F[Reassess in 3 months]:::action D --> F E --> G[Symptom control achieved]:::outcome ``` ### Acute Attack Management - Bed rest, antiemetics (prochlorperazine, dimenhydrinate) - Vestibular suppressants (betahistine, meclizine) - Avoid sudden head movements [cite:Hazarika ENT Ch 8]