ACOUSTIC NEUROMA
Saikiran M
Roll No 171
D2 Batch
Contents
◦ Introduction
◦ Pathology and Classification
◦ Clinical Features
◦ Investigations and Diagnosis
◦ Differential Diagnosis
◦ Treatment
◦ Complications and Prognosis
Introduction
◦ Acoustic neuroma is a benign, encapsulated, extrememly slow growing tumor of
VIIIth
cranial nerve.
◦ Acoustic neuroma is also known as vestibular schwannoma, neurilemma or eight
nerve tumour.
◦ It is seen in 80% of all cerebellopontine angle tumours and 10% of all brain
tumours.
◦ It has got high association with Neurofibromatosis Type 2 (NF2), an autosomal
dominant condition.
Pathology
◦ Microscopically, it consists of elongated spindle cells with rod shaped nuclei lying
in rows or palisades.
◦ Neurofibromatosis patients may have bilateral tumours.
◦ It arises from the schwann cells of vestibular part of VIIIth
nerve within the
auditory canal.
◦ As it grows and erodes the canal it appears in the cerebellopontine angke to
grow anterosuperiorly or inferiorly to affect the other nerves (Vth, VIIth, IXth,
Xth, XIth).
◦ In later stages, it can displace the brainstem, put pressure on cerebellum and
raised intracranial tension.
Classification
◦ Depending on its size, the tumour is classified as:
◦ Intracanalicular
◦ Small size (upto 1.5cm)
◦ Medium size (upto 1.5-4cm)
◦ Large size (over 4cm)
Clinical Features
1. Age and Sex : mostly seen in 40-60 years of age and both sexes are equally
affected.
2. Cochleovestibular symptoms: Progressive unilateral sensorineural hearing
loss often with tinnitus is the presenting symptom in majority of cases.
3. Cranial nerve involvement:
◦ Vth nerve: There will be reduced corneal sensitivity, numbness or paresthesia of face.
This shows that the tumour is roughly 2.5 cm in diameter and occupies the
cerebellopontine angle.
◦ VIIth nerve: Sensory fibres affected. Hypoaesthesia of posterior meatal wall, loss of
taste and reduced lacrimation on Schirmer test. Motor fibres are affected late and may
cause delayed blink reflex.
◦ IXth and Xth nerves: There is dysphagia and hoarseness due to palatal,
pharyngeal and laryngeal paralysis.
1. Brainstem involvement: Ataxia, weakness and numbness of the arms and
legs with exaggerated tendon reflexes.
2. Cerebellar involvement: Finger-nose test, knee- heel test,
dysdiadochokinesia, ataxic gait and inability to walk in a straight line with
tendency to fall to affected side.
3. Raised intracranial tension: headache, nausea, vomiting, diplopia due to VIth
nerve involvement and papilledema with blurring of vision.
Investigations and diagnosis
1. Audiological tests :
◦ Pure tone audiometry- sensorineural hearing loss (more marked in higher frequencies).
◦ Speech audiometry- poor speech discrimination. Roll-over phenomenon is observed.
◦ Recruitment phenomenon is absent.
◦ Short Increment Sensitivity Index test will show a score of 0-20% in 70-90% cases.
◦ Threshold tone decay test- retro-cochlear type of lesion.
2. Stapedial Reflex Decay Test:
3. Vestibular tests: Caloric test will show diminished or absent response in 96%.
4. Neurological tests: Complete CNS examination is done + Fundus is examined
for blurring of disc margins or papilledema.
1. Radiological tests:
a) Plain X- rays: Transorbital, Stenver’s, Towne’s and submentovertical views give positive
findings in 80% cases
b) CT scan: Small tumours can be detected, if combined with intrathecal air, an intrameatal
tumour can also be recognised
c) MRI with gadolinium contrast: This is gold standard for diagnosis. Intracanalicular tumours
of even millimetres can be identified.
d) Vertebral angiography: Helps to differentiate acoustic neuroma from other tumours of CPA.
2. Brainstem Evoked Response Audiometry: Used to diagnose retro-cochlear lesions.
3. CSF examination: Protein levels are raised.
Differential Diagnosis
1. Meniere’s disease: Episodic vertigo with fluctuating hearing loss, tinnitus but
imaging is normal.
2. Sudden sensorineural hearing loss (idiopathic): Rapid onset, no vestibular
schwannoma on imaging.
3. Other CPA lesions: Meningioma, cholesteatoma, schwannoma of other cranial
nerves, glomus tumour.
Treatment
1. Observation:
a) Indicated for small, asymptomatic or very slow growing tumours, especially in older
or comorbid patients.
b) Periodic MRI and audiometric evaluations to monitor growth.
2. Surgery: It is the treatment of choice. A surgical approach would depend on
the size of the tumour. The various approaches are:
a) Middle cranial fossa approach
b) Translabyrinthine approach
c) Suboccipital (retrosigmoid) approach
d) Combined Translabyrinthine- suboccipital approach
1. Radiotherapy:
a) X-knife or Gamma knife surgery: A form of stereotactic radiotherapy. The
surrounding tissue is spared. Causes arrest of growth of tumour and also reduces its
size. X-knife surgery is done through linear accelerator and gamma-knife through
a Cobalt 60 source.
b) Cyber knife: It is an improvement over the above and is totally frameless and more
accurate. It uses real-time image guidance technology through computer controlled
robotics.
Complications and Prognosis
1. Complications:
a) Postoperative Facial Nerve Palsy
b) CSF Leak
c) Residual/ Recurrent Tumour
2. Prognosis:
a) Generally good for small tumours, detected early.
b) Even for larger tumours, surgical and radiotherapeutic advances have improved
outcomes.
c) Preservation of facial nerve function is a crucial measure of success.
Acoustic Neuroma- Definition, Clinical features, Investigations, Treatment
Acoustic Neuroma- Definition, Clinical features, Investigations, Treatment

Acoustic Neuroma- Definition, Clinical features, Investigations, Treatment

  • 1.
  • 2.
    Contents ◦ Introduction ◦ Pathologyand Classification ◦ Clinical Features ◦ Investigations and Diagnosis ◦ Differential Diagnosis ◦ Treatment ◦ Complications and Prognosis
  • 3.
    Introduction ◦ Acoustic neuromais a benign, encapsulated, extrememly slow growing tumor of VIIIth cranial nerve. ◦ Acoustic neuroma is also known as vestibular schwannoma, neurilemma or eight nerve tumour. ◦ It is seen in 80% of all cerebellopontine angle tumours and 10% of all brain tumours. ◦ It has got high association with Neurofibromatosis Type 2 (NF2), an autosomal dominant condition.
  • 5.
    Pathology ◦ Microscopically, itconsists of elongated spindle cells with rod shaped nuclei lying in rows or palisades. ◦ Neurofibromatosis patients may have bilateral tumours. ◦ It arises from the schwann cells of vestibular part of VIIIth nerve within the auditory canal. ◦ As it grows and erodes the canal it appears in the cerebellopontine angke to grow anterosuperiorly or inferiorly to affect the other nerves (Vth, VIIth, IXth, Xth, XIth). ◦ In later stages, it can displace the brainstem, put pressure on cerebellum and raised intracranial tension.
  • 7.
    Classification ◦ Depending onits size, the tumour is classified as: ◦ Intracanalicular ◦ Small size (upto 1.5cm) ◦ Medium size (upto 1.5-4cm) ◦ Large size (over 4cm)
  • 8.
    Clinical Features 1. Ageand Sex : mostly seen in 40-60 years of age and both sexes are equally affected. 2. Cochleovestibular symptoms: Progressive unilateral sensorineural hearing loss often with tinnitus is the presenting symptom in majority of cases. 3. Cranial nerve involvement: ◦ Vth nerve: There will be reduced corneal sensitivity, numbness or paresthesia of face. This shows that the tumour is roughly 2.5 cm in diameter and occupies the cerebellopontine angle. ◦ VIIth nerve: Sensory fibres affected. Hypoaesthesia of posterior meatal wall, loss of taste and reduced lacrimation on Schirmer test. Motor fibres are affected late and may cause delayed blink reflex.
  • 9.
    ◦ IXth andXth nerves: There is dysphagia and hoarseness due to palatal, pharyngeal and laryngeal paralysis. 1. Brainstem involvement: Ataxia, weakness and numbness of the arms and legs with exaggerated tendon reflexes. 2. Cerebellar involvement: Finger-nose test, knee- heel test, dysdiadochokinesia, ataxic gait and inability to walk in a straight line with tendency to fall to affected side. 3. Raised intracranial tension: headache, nausea, vomiting, diplopia due to VIth nerve involvement and papilledema with blurring of vision.
  • 10.
    Investigations and diagnosis 1.Audiological tests : ◦ Pure tone audiometry- sensorineural hearing loss (more marked in higher frequencies). ◦ Speech audiometry- poor speech discrimination. Roll-over phenomenon is observed. ◦ Recruitment phenomenon is absent. ◦ Short Increment Sensitivity Index test will show a score of 0-20% in 70-90% cases. ◦ Threshold tone decay test- retro-cochlear type of lesion. 2. Stapedial Reflex Decay Test: 3. Vestibular tests: Caloric test will show diminished or absent response in 96%. 4. Neurological tests: Complete CNS examination is done + Fundus is examined for blurring of disc margins or papilledema.
  • 12.
    1. Radiological tests: a)Plain X- rays: Transorbital, Stenver’s, Towne’s and submentovertical views give positive findings in 80% cases b) CT scan: Small tumours can be detected, if combined with intrathecal air, an intrameatal tumour can also be recognised c) MRI with gadolinium contrast: This is gold standard for diagnosis. Intracanalicular tumours of even millimetres can be identified. d) Vertebral angiography: Helps to differentiate acoustic neuroma from other tumours of CPA. 2. Brainstem Evoked Response Audiometry: Used to diagnose retro-cochlear lesions. 3. CSF examination: Protein levels are raised.
  • 14.
    Differential Diagnosis 1. Meniere’sdisease: Episodic vertigo with fluctuating hearing loss, tinnitus but imaging is normal. 2. Sudden sensorineural hearing loss (idiopathic): Rapid onset, no vestibular schwannoma on imaging. 3. Other CPA lesions: Meningioma, cholesteatoma, schwannoma of other cranial nerves, glomus tumour.
  • 15.
    Treatment 1. Observation: a) Indicatedfor small, asymptomatic or very slow growing tumours, especially in older or comorbid patients. b) Periodic MRI and audiometric evaluations to monitor growth. 2. Surgery: It is the treatment of choice. A surgical approach would depend on the size of the tumour. The various approaches are: a) Middle cranial fossa approach b) Translabyrinthine approach c) Suboccipital (retrosigmoid) approach d) Combined Translabyrinthine- suboccipital approach
  • 17.
    1. Radiotherapy: a) X-knifeor Gamma knife surgery: A form of stereotactic radiotherapy. The surrounding tissue is spared. Causes arrest of growth of tumour and also reduces its size. X-knife surgery is done through linear accelerator and gamma-knife through a Cobalt 60 source. b) Cyber knife: It is an improvement over the above and is totally frameless and more accurate. It uses real-time image guidance technology through computer controlled robotics.
  • 19.
    Complications and Prognosis 1.Complications: a) Postoperative Facial Nerve Palsy b) CSF Leak c) Residual/ Recurrent Tumour 2. Prognosis: a) Generally good for small tumours, detected early. b) Even for larger tumours, surgical and radiotherapeutic advances have improved outcomes. c) Preservation of facial nerve function is a crucial measure of success.