--- title: "Meniere's Disease Part 1 - Definition, Etiology, Pathophysiology & Histopathology" description: "ENT notes on Meniere's Disease - Definition, Meniere's syndrome, etiology, pathophysiology and histopathology." source: "The ENT Resident" author: "Dr. Mausumi Das" canonical_url: "https://www.theentresident.com/ent-notes-lectures/menieres-disease-causes-pathophysiology-pathology" categories: ["Otology"] video: "https://www.youtube.com/watch?v=NvTA2bZ1NQ8" image: "https://media.theentresident.com/og-images/menieres-disease-causes-pathophysiology-pathology.png" --- # Meniere's Disease Part 1 - Definition, Etiology, Pathophysiology & Histopathology ENT notes on Meniere's Disease - Definition, Meniere's syndrome, etiology, pathophysiology and histopathology. Watch the full video lecture on YouTube: https://www.youtube.com/watch?v=NvTA2bZ1NQ8 ## 👂 Meniere's Disease - Definition, Etiology & Pathophysiology ### 📝 Definition of Meniere's Disease Meniere's disease is an **idiopathic inner ear disorder** characterized by **recurrent spontaneous vertigo**, accompanied by **fluctuating or progressive sensorineural hearing loss, tinnitus and aural fullness** in the affected ear, occurring due to **distension of the endolymphatic system**. > What does the term Idiopathic mean? > [What is the Triad of Symptoms seen in Meniere's Disease?](https://www.theentresident.com/ent-notes-lectures/menieres-disease-symptoms-signs-diagnosis.md) #### 👨‍⚕️ Eponym Named after **Prosper Meniere**, who first reported the disease. --- ### 📊 Epidemiology of Meniere's Disease | Parameter | Detail | | --- | --- | | **Incidence** | 7.5 to 157 per 1 lakh persons | | **Race** | More common in Caucasians | | **Age** | 2nd to 6th decade of life | | **Sex** | No sex preponderance; equal incidence in males and females | | **Laterality** | Mostly unilateral | > What is the prevalence of Meniere's disease in the pediatric age group? > What is the percentage of Bilateral involvement of Meniere's disease? > What is the percentage of sporadic and familial Meniere's disease? > What are the Features of Familial Meniere's Disease? > What are the Genes Associated with Meniere's Disease? --- ### ⚖️ Meniere's Disease vs Meniere's Syndrome Distension of the endolymphatic system is termed **endolymphatic hydrops**. | Feature | Meniere's Disease | Meniere's Syndrome | | --- | --- | --- | | **Type of hydrops** | Primary (idiopathic) endolymphatic hydrops | Secondary endolymphatic hydrops | | **Underlying cause** | No identifiable cause | Identifiable underlying cause present | | **Clinical features** | Same | Same | > What are the causes of Meniere's Syndrome? --- ### 🔍 Etiology of Meniere's Disease **1- Endolymphatic Hydrops** ⭐ The main etiological factor. Distension of the endolymphatic system occurs due to an **increased volume of [endolymph](https://www.theentresident.com/ent-notes-lectures/perilymph-endolymph.md)**, which may result from: a. **Increased production** of endolymph b. **Reduced or faulty absorption** of endolymph c. Both occurring together > What is the Sequence of Events leading to the development of vertigo in Meniere's disease? ⚠️ Endolymphatic hydrops is **not pathognomonic** of Meniere's disease, as it is seen in other conditions too. > What other conditions do you see Endolymphatic hydrops in? **2- Autoimmune Factor** > What are the Theories of Autoimmunity in Meniere's disease? > Why is an Autoimmune etiology considered in Meniere's disease? **3- Viral Factor** > What is the most common virus associated with Meniere's disease? **4- Allergy** **Potential Mechanisms** * The **endolymphatic sac** is targeted by the allergic reaction * Deposition of circulating immune complexes in the fenestrated blood vessels of the **endolymphatic sac** and the **stria vascularis** * Viral antigen and allergic interaction, with **T cell homing** to the endolymphatic sac **5- Vasomotor Disturbance** > How does vasomotor disturbance cause Meniere's disease? **6- Hormonal Factor** ⭐ About **3%** of cases are associated with **hypothyroidism**, and these patients often benefit from **thyroid replacement therapy**. --- ### 🧠 Pathophysiology of Meniere's Disease Increased production or faulty absorption of endolymph → **Increased volume of endolymph** → **Distension of the endolymphatic system** → **Thinning and atrophy of the Reissner's membrane**, which bulges into the scala vestibuli → **Rupture of the Reissner's membrane**, with sudden release of a large volume of endolymph into the perilymphatic space → Sensory and neural structures exposed to **potassium rich endolymph** → **Sudden hearing loss and vertigo** → Excess pressure is released and the perilymphatic compartment is restored to normal → **Symptoms subside and the patient goes into remission** → The rupture **heals**, pressure builds up again and the **whole cycle repeats** > What are the Histopathological changes seen in Meniere's Disease? --- ### 🧠 Viva Pearls * Meniere's disease is an idiopathic inner ear disorder due to distension of the endolymphatic system. * Named after Prosper Meniere. * Most common in the 2nd to 6th decade of life, with no sex preponderance. * Mostly unilateral. * Meniere's disease is primary (idiopathic) endolymphatic hydrops. * Meniere's syndrome is secondary endolymphatic hydrops with an identifiable cause. * Endolymphatic hydrops is not pathognomonic of Meniere's disease. * Increased endolymph volume results from increased production or faulty absorption. * Reissner's membrane bulges into the scala vestibuli and may rupture. * Rupture causes mixing of endolymph and perilymph, producing an attack of vertigo. * Bulging may herniate through the helicotrema into the apical part of the scala tympani. --- ## About This Note This free ENT note on Meniere's Disease Part 1 - Definition, Etiology, Pathophysiology & Histopathology was written by Dr. Mausumi Das and is paired with a corresponding video lecture. 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