### Date : 2024-06-25 14:42 ### Topic : Ménière's Disease #medicine #ENT #neurology ---- ### Ménière's Disease Overview **Ménière's disease** is a disorder of the inner ear that causes episodes of vertigo, tinnitus, hearing loss, and a feeling of fullness or pressure in the ear. It typically affects one ear and can cause significant disruptions in a person's daily life. The exact cause of Ménière's disease is unknown, but it is thought to be related to an abnormality in the volume or composition of fluid (endolymph) in the inner ear. ### Etiology **Possible Contributing Factors:** - **Endolymphatic Hydrops:** An excess buildup of endolymph in the inner ear. - **Genetic Predisposition:** Family history of Ménière's disease. - **Autoimmune Reactions:** Immune system attacking inner ear structures. - **Viral Infections:** Previous viral infections affecting the inner ear. - **Vascular Factors:** Blood flow issues affecting the inner ear. ### Pathophysiology 1. **Endolymphatic Hydrops:** - The inner ear contains a fluid called endolymph, which helps with balance and hearing. - In Ménière's disease, an excess amount of endolymph accumulates in the inner ear, causing swelling and pressure. 2. **Rupture of Membranes:** - Increased pressure can cause the membranes in the inner ear to rupture, leading to a mix of endolymph and perilymph (another inner ear fluid). - This mix disrupts normal inner ear function, leading to the symptoms of Ménière's disease. ### Clinical Features **Symptoms:** - **Vertigo:** - Severe spinning sensation that can last from 20 minutes to several hours. - Often accompanied by nausea and vomiting. - **Hearing Loss:** - Fluctuating hearing loss, initially affecting low frequencies. - Over time, can progress to permanent hearing loss. - **Tinnitus:** - Ringing, buzzing, or roaring noise in the affected ear. - **Aural Fullness:** - A sensation of pressure or fullness in the ear. **Pattern of Episodes:** - Symptoms occur in episodic attacks. - Periods of remission between episodes, where symptoms are minimal or absent. ### Diagnosis **1. Clinical Evaluation:** - Detailed patient history focusing on the characteristics and frequency of episodes. - Physical examination and neurological assessment. **2. Diagnostic Tests:** - **Audiometry:** Hearing tests to evaluate hearing loss patterns and severity. - **Electronystagmography (ENG) or Videonystagmography (VNG):** Tests to assess balance function and detect abnormal eye movements. - **Electrocochleography (ECoG):** Measures electrical potentials in the inner ear to detect endolymphatic hydrops. - **MRI or CT Scan:** Imaging to rule out other causes of symptoms, such as tumors or structural abnormalities. ### Example Case Study **Patient Profile:** - **Name:** Jee Hoon Ju - **Age:** 45 - **Occupation:** Office Worker **Medical History:** - Recurrent episodes of severe vertigo, lasting 1-2 hours. - Fluctuating hearing loss and tinnitus in the right ear. - Sensation of fullness in the right ear. **Clinical Evaluation:** - **Audiometry:** Shows fluctuating low-frequency hearing loss in the right ear. - **ENG:** Reveals abnormal eye movements consistent with vestibular dysfunction. - **MRI:** Normal, ruling out structural abnormalities. **Diagnosis:** Based on the clinical presentation and diagnostic test results, Jee Hoon Ju is diagnosed with Ménière's disease affecting the right ear. ### Management **1. Lifestyle Modifications:** - **Dietary Changes:** Low-sodium diet to reduce fluid retention. - **Hydration:** Regular fluid intake to maintain fluid balance. - **Avoiding Triggers:** Reducing caffeine, alcohol, and stress. **2. Medications:** - **Diuretics:** Such as hydrochlorothiazide to reduce endolymphatic pressure. - **Antihistamines:** Such as meclizine to reduce vertigo symptoms. - **Benzodiazepines:** Such as diazepam to help control acute vertigo episodes. - **Antiemetics:** Such as promethazine to manage nausea and vomiting during vertigo attacks. **3. Rehabilitation:** - **Vestibular Rehabilitation Therapy (VRT):** Exercises to improve balance and reduce dizziness. - **Hearing Aids:** For patients with significant hearing loss. **4. Interventional Treatments:** - **Intratympanic Injections:** Steroids or gentamicin injections into the middle ear to reduce inflammation or ablate vestibular function. - **Endolymphatic Sac Surgery:** To decompress the endolymphatic sac and reduce fluid buildup. - **Labyrinthectomy or Vestibular Nerve Section:** Surgical removal of the inner ear or cutting the vestibular nerve, typically reserved for severe, refractory cases. ### Prognosis - **Variable Course:** The severity and frequency of episodes can vary widely among individuals. - **Chronic Condition:** While Ménière's disease is a chronic condition, many patients find relief with treatment and lifestyle modifications. - **Quality of Life:** Effective management can significantly improve quality of life, although some patients may experience persistent symptoms. ### Conclusion Ménière's disease is a chronic disorder of the inner ear characterized by episodes of vertigo, tinnitus, hearing loss, and aural fullness. Diagnosis involves clinical evaluation and diagnostic tests to assess hearing and balance function. Management includes lifestyle modifications, medications, vestibular rehabilitation, and, in some cases, interventional treatments. Understanding the pathophysiology, clinical features, and management strategies is crucial for effectively treating and improving the quality of life for patients with Ménière's disease.