Updated: 8/4/2019

Vestibular Schwannoma (Acoustic Neuroma)

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Snapshot
  • A 44-year-old man presents to his primary care physician for worsening hearing loss. His hearing loss is most severe in his right ear. He has noticed decreased facial sensation on the right side of the face. Physical examination is notable for a sensorineural hearing loss affecting the right ear and decreased sensation to pin-prick in the right face. An MRI brain demonstrates an enlarged mass in the internal acoustic meatus, extending to the cerebellopontine angle.
Introduction
  • Overview
    • a Schwann cell-derived tumor arising from the 8th cranial nerve
      • typically the vestibular portion
      • usually unilateral (~90% of cases)
  • Epidemiology
    • demographics
      • median age is ~50 years
  • Associated conditions
    • neurofibromatosis type 2 
      • characteristically presents as bilateral acoustina neuromas
        • secondary to a mutation in merlin in chromosome 22  
Presentation
  • Symptoms/physical exam 
    • cochlear nerve involvement
      • sensorineural hearing loss
      • tinnitus
    • vestibular nerve involvement
      • unsteady gait
    • can have other cranial nerve involvement
      • facial and trigeminal nerve involvement
        • facial nerve
          • facial paresis
          • taste disturbaces
        • trigeminal nerve
          • facial paresthesia and pain
Imaging
  • MRI brain with gadolinium
    • indication
      • imaging study of choice
    • fundings
      • enhancing lesion in the internal auditory canal that may extend into the cerebellopontine angle  
Studies
  • Audiometry
    • indication
      • best initial screening laboratory test
Differential
  • Neurofibromatosis type 2
    • differentiating factors
      • associated with bilateral acoustic neuromas, cataracts, meningiomas, and ependymomas
  • Meningioma
    • differenting factors
      • tumor involving the meninges only
Treatment
  • Medical
    • radiation therapy
      • indication
        • small tumors or patients who are not surgical candidates
      • options
        • sterotactic radiosurgery
        • sterotactic radiotherapy
  • Surgical
    • microsurgical tumor removal
      • indication
        • large tumors or rapidly growing tumors
Complications
  • Hearing loss
  • Brainstem compression

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(M2.ON.16.4694) A 45 year old male presents to clinic complaining of a ringing in his ears. The patient's vitals are: BP 120/65, RR 16, 100% on room air, HR 78 bpm and he appears to be in no acute distress. Upon obtaining a history you learn that this patient also complains that at times the "room feels like it's spinning." You also notice throughout the interview that you have to speak loudly as the patient has difficulty hearing. The patient is then lost to follow up for his current condition. The patient returns several years later with worsening symptoms and as part of this patient's workup a MRI is obtained (Image A). Which of the following chromosomes is most likely mutated in association with this patient's condition?

QID: 107884
FIGURES:
1

Chromosome 13

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(0/1)

2

Chromosome 7

100%

(1/1)

3

Chromosome 17

0%

(0/1)

4

Chromosome 22

0%

(0/1)

5

The X chromosome

0%

(0/1)

M 6 E

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