Summary about Disease
Otitic hydrocephalus, also known as pseudotumor cerebri secondary to otitis media, is a rare condition characterized by increased intracranial pressure (pressure inside the skull) associated with ear infections (otitis media), particularly in children. Unlike true hydrocephalus, there's no obstruction of cerebrospinal fluid (CSF) flow, but the pressure is elevated, leading to symptoms mimicking a brain tumor.
Symptoms
Common symptoms include:
Headache
Papilledema (swelling of the optic disc, visible during an eye exam)
Vision changes (blurred vision, double vision, temporary vision loss)
Nausea and vomiting
Tinnitus (ringing in the ears)
Dizziness
Neck stiffness
Sixth nerve palsy (eye muscle weakness, leading to double vision)
Causes
The exact cause isn't fully understood, but it's believed to be related to:
Ear Infections: Otitis media (middle ear infection) or mastoiditis (infection of the mastoid bone) are almost always present.
Venous Sinus Thrombosis: Inflammation from the ear infection may spread to the dural venous sinuses (major veins in the brain), potentially causing a partial blockage or thrombosis (blood clot). This can impair CSF absorption.
Inflammation and Increased CSF Production: The infection may trigger an inflammatory response leading to increased CSF production.
Possible Genetic Predisposition: Some people may be more susceptible due to individual variations.
Medicine Used
Treatment typically involves:
Antibiotics: To treat the underlying ear infection.
Acetazolamide (Diamox): A diuretic medication that reduces CSF production.
Topiramate (Topamax): Can be used alone or in combination with Acetazolamide to reduce CSF production.
Corticosteroids: May be used to reduce inflammation, but their long-term use is generally avoided.
Pain relievers: For headaches. In severe cases, procedures may be necessary:
Lumbar punctures: To temporarily reduce CSF pressure.
Shunting: A surgical procedure to divert CSF to another part of the body (e.g., the abdomen).
Optic nerve sheath fenestration: A surgical procedure to relieve pressure on the optic nerve, to preserve vision.
Venous Sinus Stenting: If venous sinus stenosis is significant.
Is Communicable
No, otitic hydrocephalus is not communicable. It is a complication of an ear infection, not an infection itself. The ear infection might be contagious, but the hydrocephalus is not.
Precautions
Prevention focuses on managing ear infections effectively:
Prompt treatment of ear infections with antibiotics.
Regular follow-up appointments with a doctor, especially if headaches or vision changes develop during or after an ear infection.
Maintaining good hygiene to prevent ear infections.
Avoidance of smoking and exposure to secondhand smoke, as they can increase the risk of ear infections.
How long does an outbreak last?
The duration of symptoms varies depending on the severity of the condition and the effectiveness of treatment. With prompt and appropriate treatment, including antibiotics and medications to lower CSF pressure, symptoms can often resolve within weeks to months. However, some individuals may experience chronic symptoms or require long-term management to prevent recurrence.
How is it diagnosed?
Diagnosis typically involves:
Physical Exam: Including a neurological exam and fundoscopic exam (examining the back of the eye for papilledema).
Detailed Medical History: Including information about ear infections and other relevant medical conditions.
Eye Exam: To assess visual acuity, visual fields, and optic nerve swelling.
Neuroimaging:
MRI of the brain with and without contrast: To rule out other causes of increased intracranial pressure (e.g., brain tumor, hydrocephalus, venous sinus thrombosis) and to assess for venous sinus stenosis or thrombosis.
CT scan of the head: May be used if MRI is not possible.
Lumbar Puncture: To measure CSF pressure and rule out infection. CSF analysis to rule out meningitis or other inflammatory conditions.
Audiometry: To assess hearing function.
Timeline of Symptoms
The onset of symptoms can be gradual or relatively rapid.
Initial Stage: Often starts with symptoms of an ear infection (ear pain, drainage). Headaches may begin subtly.
Progressive Stage: Headaches become more frequent and severe. Vision changes (blurred vision, double vision, temporary vision loss) may develop.
Advanced Stage: Papilledema becomes pronounced. Nausea, vomiting, and other neurological symptoms (e.g., sixth nerve palsy) may occur. The specific timeline varies significantly from person to person.
Important Considerations
Early diagnosis and treatment are crucial to prevent permanent vision loss.
Otitic hydrocephalus is a diagnosis of exclusion. Other causes of increased intracranial pressure must be ruled out.
Long-term follow-up is often necessary to monitor for recurrence and manage any residual symptoms.
Children are more frequently affected than adults.
Cooperation between multiple specialists (e.g., otolaryngologist, neurologist, ophthalmologist) is essential for optimal care.