A Deaf Young Man with Poor balance

Monday, March 8, 2010
A 30-year-old young man came to the ENT clinic complaining of  increasing tinnitus in his left ear with a tendency to fall to the left. He has no significant previous medical history and on examination he has left sided ataxia, a gaze paresis to the left and audiogram shows sensorineural deafness on that side.


 What is the most propable Diagnosis?




ANSWER

ANSWER - LEFT SIDED ACOUSTIC NEUROMA
An acoustic neuroma(or vestibular schwannoma), is a slow growing benign primary intracranial tumor of the myelin-forming cells of the vestibular portion of the 8th cranial nerve.
It comprises about 6% of all intracranial tumors
lesions are usually located in the internal auditory canal or the cerebellopontine angle causing compression of the vestibular nerve and resulting eventually in deafness
Bilateral acoustic neuromas are the hallmark in patient with type 2 neurofibromatosis

CLINICAL FEATURES

1. Occipital pain on the side of the tumour
2. VIII nerve damage - unilateral sensorineural deafness developing gradually over a period of months or years. vertigo with associated nausea and vomiting,tinnitus.Caloric paresis may be demonstrable.
3. V nerve damage - depression of corneal reflex occurs early. Facial pain, paraesthesia and numbness develop.
4. VII nerve damage - facial weakness is unusual.If involved it may lead to ipsilateral facial weakness, sensory impairment, impairment of glandular secretions and loss of taste sensation in the anterior two-thirds of the tongue
5. IX, X, XI nerve damage - rare; presents as dysphagia, change in voice, palatal weakness.
6. Compression effects of large tumours:

* cerebellum - ataxia, loss of co-ordination on ipsilateral side, nystagmus
* pons - contralateral hemiparesis,gaze palsies
* aqueduct and 4th ventricle - raised intracranial pressure, e.g. headache

INVESTIGATIONS

Neuro-otological
Pure tone audiometry - demonstrates unilateral sensorineural hearing loss
Brainstem audiometry - to distinguish cochlear deafness from retrocochlear disease

Caloric test - expect depressed or absent response

Contrast CT scan as acoustic neuromas are often isodense

MRI with gadolinium enhancement - especially for small intracanalicular tumours

TREATMENT

Conservative management - due to the risk of interventions, small asymptomatic tumors may be managed by a "watch, wait and rescan" approach. This is more suitable in elderly patients with co morbid diseases

Stereotactic radiotherapy

Microsurgery - is useful in treating large acoustic neuromas. three surgical methods are used in approaching the CP angle

1. retrosigmoid approach
2. translabyrinthine approach
3. middle fossa approach




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11 comments

  1. Anonymous Says:
  2. Vestibular Schawnnoma

     
  3. Anonymous Says:
  4. I agree with Meningioma of VIII pair of nerve...atassia, tinnitus and sensorineural deafness...

     
  5. Anonymous Says:
  6. acoustic neuroma

     
  7. Anonymous Says:
  8. arash from iran


    acoustic neuroma with enhancement in involved side

     
  9. Anonymous Says:
  10. left sided acoustic neuroma

     
  11. Anonymous Says:
  12. acoustic neuroma
    Azhar

     
  13. Anonymous Says:
  14. thanx for taking my suggestion into consideration
    dr.Winzo

     
  15. acoustic neuroma

     
  16. Anonymous Says:
  17. why symptoms in left side and enhancement in right?

     
  18. i guess it's acoustic neuroma

     
  19. Anonymous Says:
  20. Unilateral tinnitus with SNHL,acoustic neuroma is possible diagnosis

     

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