This syndrome is caused most commonly by a cerebellopontine angle tumor, the most common of which being the Acoustic Neuroma or Vestibular Schwannoma.
This is the most common Schwannoma of Head and neck region. It is benign and extremely slow growing most of the times being unilateral. It is seen bilaterally in cases of Von Recklinghausen's disease or Neurofibromatosis type 2.
This is a benign tumor of the Schwann Cells covering the vestibular part of the VIII cranial nerve. It involves the Inferior vestibular branch or the singular nerve most commonly.
*Signs and Symptoms-
(due to the mass effect of the tumor)
# The most common presentation of a CP angle tumor is loss of Corneal reflex due to involvement of V CN.
# There is associated ipsilateral progressive deafness, tinnitus, vertigo, nystagmus and nausea due to involvement of VIII CN.
# Ipsilateral ataxia due to involvement of middle and inferior cerebellar peduncles.
# Ipsilateral facial paralysis, positive Bell's phenomenon, lag ophthalmos due to involvement of VII CN.
# Hitzelberger's sign - the VII CN supplies sensation to a small part of skin in the posterior wall of the external auditory canal....which is lost in this condition.
# Loss of gag reflex and hoarseness of voice due to involvement of IX & X CN.
# Increased ICP and herniation of brain stem in severe cases.
*Investigation of choice - Gadolinium MRI
*Treatment- Surgical excision or Radiotherapy.
Mithil Jagannath.
Medicowesome 2017.
8....turn it horizontally....●●....two eyes....associated with NF2
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