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A 60 year old male presented with sensorineural hearing loss and ataxia.

1. What is the Diagnosis? A. Looks normal to me B. Superficial Siderosis C. Cavernoma(s) D. Leptomeningeal Spread of CA (or infection) 2. Wh...

1. What is the Diagnosis?
A. Looks normal to me
B. Superficial Siderosis
C. Cavernoma(s)
D. Leptomeningeal Spread of CA (or infection)

2. What is the classic presentation?
A. Seizure
B. Thunderclap Headache
C. Gelastic Seizures
D. Sensorineural Hearing Loss

3. What is the classic history?
A. AVM or Aneurysm
B. Down Syndrome
C. NF -2
D. Pituitary Teratoma

4. What is the “next step”?
A. PET CT
B. CT with Perfusion
C. Conventional Angiogram
D. No further imaging needed.

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ANSWERS

1. What is the Diagnosis? Superficial Siderosis  -Deposition of hemosiderin along the leptomeninges. 

2. What is the classic presentation? Sensorineural Hearing Loss

3. What is the classic history?
AVM or Aneurysm

4. What is the “next step”?
Conventional Angiogram 

 

KEY FEATURES

  • The etiology is typically recurrent or extensive subarachnoid hemorrhage. This can be from any cause; trauma, bleeding brain tumor, AVM, or aneurysm.
  • If you are forced into a “next step” situation, you would want to look for the cause of bleeding. Angiogram (conventional or CTA) would be the answer I would choose.
  • Sensorineural hearing low is found in 95% of patients, and is the most common clinical presentation. It is typically bilateral and gradual in onset.
  • The second most common symptom (found in 88% of cases) is ataxia. 
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