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Acoustic neuroma
Dr Ranjana D Telkar
• Acoustic neuroma: definition, histopathology
• Etiopathogenesis
• Classification
• Clinical features
• Management
Acoustic neuroma
• Definition:
Tumor of eight cranial nerve
• Eponyms
Vestibular schwannoma
Neurilemmoma
• Incidence
80% of cerebellopontine angle tumors
10% of all brain tumors
Pathology
• Gross:
Benign, Encapsulated, slow growing
• Microscopy:
Elongated spindle cells
Rod shaped nuclei in rows or palisades
Origin & growth
• Origin:
– Schwann cells of vestibular nerve, rarely from
cochlear nerve
• Growth:
– Causes widening and erosion of the canal and appears
in the CP angle
– Anterosuperior growth: 5th
– Inferior: 9th 10th & 11th
– Later stages: displacement of brainstem, pressure on
cerebellum and raised intracranial tension
Origin and growth
classification
Acoustic
neuroma
intracellular
Small size
<1.5cm
Medium size
1.5 – 4 cm
Large size
>4cm
Clinical features
• Age: 40 – 60 years
• Sex: M = F
• Symptoms:
– Progressive unilateral SNHL
– Tinnitus
– Marked difficulty in understanding speech
– Imbalance/Unsteadiness
– Vertigo
– Sudden hearing loss
– Fullness in the ear
Cranial nerve involvement
• 5th nerve: EARLIEST
Reduced cornea sensitivity, paresthesia of face
Involvement indicates: tumor size = 2.5cm &
occupies CP angle
• 9th & 10th: dysphagia & hoarseness due to palatal,
pharyngeal, laryngeal paralysis
• Other cranial nerves: affected only when tumor
size is very large
Cranial nerve involvement
• Facial nerve:
– Sensory fibres are affected early
– Hitzelberger’s sign: hypoaesthesia of posterior
meatal wall
– Loss of taste (Electrogustometry)
– Schirmer test: Reduced lacrimation
– Motor fibres: affected late
– Delayed blink reflex
Brainstem involvement
• Ataxia
• Weakness & numbness of arms & legs
• Exaggerated tendon reflexes
Raised intracranial tension
Headache, nausea, vomiting, diplopia (6th ) &
papillo-edema with blurring of vision
Cerebellar involvement
• Pressure symptoms on cerebellum are seen in
large tumors
• Revealed by
• Finger-nose test
• Knee-heel test
• Dysdiadochokinesia
• Ataxic gait
• Inability to walk along a straightline (tendency to
fall on the affected side
Investigations
• Audiological tests
– PTA
– Speech audiometry
– Recruitment phenomena: absent
– Short increment sensitivity index: 0-20%
– Threshold tone decay test: Retrocochlear type of
lesion
Vestibular tests
• Caloric test
– Diminished or absent
– Respone in 96% of patients
– Maybe normal when tumor is small
Neurological tests
• Complete examination of:
– Cranial nerves
– Cerebellar functions
– Brainstem signs of pyramidal and sensory tracts
– Fundus
Radiological tests
• Plain X-ray
– Positive in 80% of patients
– Different views
• Transornital
• Stenver’s
• Towne’s
• Submentovertical
• Vertebral angiography
– Helps in differentiating AN from other tumors
Radiological tests
• CT scan
– More sensitive than X-ray
– Can detect even intrameatal and posterior fossa
tumors
• MRI with Gadolinium contrast
– GOLD standard
– Can detect even intracanalicular tumors of few
mm
CT
MRI
Other tests
• BERA:
– A decay of >0.2ms in wave V between 2 ears in
case of 8th nerve tumor
• Stapedial reflex :delayed
• CSF examination:
– Protein levels raised, lumbar punture should be
avoided
Investigations
• Important tests for AN workup
– PTA
– Speech discrimantion score
– Roll-over curve
– Stapedial reflex decay
– BERA
– MRI with Gadolinium contrast
Differential diagnosis
• Meniere’s disease
• Tumors of CP angle:
– Meningioma
– Epidermoid
– Arachnoid cyst
– Schwannoma of other cranial nerves
– Aneurysm
– Glomus tumor
– Metastasis
Treatment
Treatment
Surgical Radiotherapy
Surgical Mx
• Approaches
• Middle cranial fossa approach
• Translabyrinthine
• Suboccipital
• Combined translabyrinthine suboccipital
Radiotherapy
• Conventional
• X knife / gamma knife surgery
• Cyber knife
Thank you

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Acoustic neuroma.pptx

  • 2. • Acoustic neuroma: definition, histopathology • Etiopathogenesis • Classification • Clinical features • Management
  • 3. Acoustic neuroma • Definition: Tumor of eight cranial nerve • Eponyms Vestibular schwannoma Neurilemmoma • Incidence 80% of cerebellopontine angle tumors 10% of all brain tumors
  • 4.
  • 5.
  • 6. Pathology • Gross: Benign, Encapsulated, slow growing • Microscopy: Elongated spindle cells Rod shaped nuclei in rows or palisades
  • 7.
  • 8.
  • 9. Origin & growth • Origin: – Schwann cells of vestibular nerve, rarely from cochlear nerve • Growth: – Causes widening and erosion of the canal and appears in the CP angle – Anterosuperior growth: 5th – Inferior: 9th 10th & 11th – Later stages: displacement of brainstem, pressure on cerebellum and raised intracranial tension
  • 12. Clinical features • Age: 40 – 60 years • Sex: M = F • Symptoms: – Progressive unilateral SNHL – Tinnitus – Marked difficulty in understanding speech – Imbalance/Unsteadiness – Vertigo – Sudden hearing loss – Fullness in the ear
  • 13. Cranial nerve involvement • 5th nerve: EARLIEST Reduced cornea sensitivity, paresthesia of face Involvement indicates: tumor size = 2.5cm & occupies CP angle • 9th & 10th: dysphagia & hoarseness due to palatal, pharyngeal, laryngeal paralysis • Other cranial nerves: affected only when tumor size is very large
  • 14. Cranial nerve involvement • Facial nerve: – Sensory fibres are affected early – Hitzelberger’s sign: hypoaesthesia of posterior meatal wall – Loss of taste (Electrogustometry) – Schirmer test: Reduced lacrimation – Motor fibres: affected late – Delayed blink reflex
  • 15. Brainstem involvement • Ataxia • Weakness & numbness of arms & legs • Exaggerated tendon reflexes Raised intracranial tension Headache, nausea, vomiting, diplopia (6th ) & papillo-edema with blurring of vision
  • 16. Cerebellar involvement • Pressure symptoms on cerebellum are seen in large tumors • Revealed by • Finger-nose test • Knee-heel test • Dysdiadochokinesia • Ataxic gait • Inability to walk along a straightline (tendency to fall on the affected side
  • 17. Investigations • Audiological tests – PTA – Speech audiometry – Recruitment phenomena: absent – Short increment sensitivity index: 0-20% – Threshold tone decay test: Retrocochlear type of lesion
  • 18. Vestibular tests • Caloric test – Diminished or absent – Respone in 96% of patients – Maybe normal when tumor is small
  • 19. Neurological tests • Complete examination of: – Cranial nerves – Cerebellar functions – Brainstem signs of pyramidal and sensory tracts – Fundus
  • 20. Radiological tests • Plain X-ray – Positive in 80% of patients – Different views • Transornital • Stenver’s • Towne’s • Submentovertical • Vertebral angiography – Helps in differentiating AN from other tumors
  • 21. Radiological tests • CT scan – More sensitive than X-ray – Can detect even intrameatal and posterior fossa tumors • MRI with Gadolinium contrast – GOLD standard – Can detect even intracanalicular tumors of few mm
  • 22. CT
  • 23. MRI
  • 24. Other tests • BERA: – A decay of >0.2ms in wave V between 2 ears in case of 8th nerve tumor • Stapedial reflex :delayed • CSF examination: – Protein levels raised, lumbar punture should be avoided
  • 25. Investigations • Important tests for AN workup – PTA – Speech discrimantion score – Roll-over curve – Stapedial reflex decay – BERA – MRI with Gadolinium contrast
  • 26. Differential diagnosis • Meniere’s disease • Tumors of CP angle: – Meningioma – Epidermoid – Arachnoid cyst – Schwannoma of other cranial nerves – Aneurysm – Glomus tumor – Metastasis
  • 28. Surgical Mx • Approaches • Middle cranial fossa approach • Translabyrinthine • Suboccipital • Combined translabyrinthine suboccipital
  • 29. Radiotherapy • Conventional • X knife / gamma knife surgery • Cyber knife