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VESTIBULAR SCHWANNOMA

  (ACOUSTIC NEUROMA)
DEFINITION
• Benign tumor of vestibular nerve schwan
  cells
VESTIBULAR SCHWANNOMA

•   6 % of all Intracranial tumors
•   80 - 90% of CPA tumors
•   Majority adulthood
•   95% Unilateral
•   5% Neurofibromatosis type 2 (bilateral)
•   No known race, gender predilection
ANATOMY OF CP ANGLE
•   Triangular area
•   Lateral       Posterior surface of temporal bone
•   Medial        Edge of pons
•   Posterior     Anterior surface of cerebellum
•   Superior      Trigeminal nerve
•   Inferior      IX, X, XI cranial nerves
Petrous Bone
Po
ns


     •Cerebrum
SITE OF ORIGIN
• Schwann cells which envelop distal potion
  of VIIIth nerve
• Within the IAC
GROWTH PATTERN

• Grows medially
• Invested by double
  arachnoid layer
EFFECTS ON INNER EAR
• Compressive effect on cochlear nerve
• Vascular occlusion of internal auditory
  artery
• Atrophy of organ of Corti
• Vacuolization of stria vascularis
APPEARANCE
•   Firm
•   Well encapsulated
•   Interior soft
•   Sometimes filled with serous or
    hemorrhagic fluid
STAGES
1   Otological stage                      (< 2 cm)
2   Trigeminal nerve involvement          (2-2.5 cm)
3   Brain stem and cerebellar compression (2.5-4 cm)
4   Increasing intracranial pressure
5   Terminal stage
STAGE 1
       OTOLOGICAL STAGE
• Deafness
  – Unilateral, gradual
• Tinnitus
  – Non-pulsatile
• Imbalance
• Trigeminal nerve involvement
• Facial nerve involvement
STAGE 2
    TRIGEMINAL NERVE INVOLVEMENT
•   Tumor size 2-2.5 cm
•   Irritation in eye
•   Pain, tingling, numbness on face
•   Feeling of cold on face
STAGE 3
      BRAIN STEM AND CEREBELLAR
                 COMPRESSION
•   Ataxia
•   Gait disturbance
•   Tremors
•   Nystagmus
STAGE 4
  INCREASING INTRACRANIAL PRESSURE

• Headache occipital
• Nausea Vomiting
• Papilloedema
STAGE 5
          TERMINAL STAGE
• Failure of vital centres in brain stem
EXAMINATION
• Cutaneous lesions (neurofibromas)
• Otoscopy:     Normal
• Tuning fork tests:
  – Unilateral senorineural deafness
• Trigeminal Nerve
• Facial nerve
• Eye examination
EXAMINATION
• Finger nose test
• Romberg’s test
• Unterberg’s test
INVESTIGATIONS
•   Speech test
•   Speech audiometry
•   PTA
•   Stapedial reflex
•   Electrocochleography
•   ABR
•   Caloric test
RADIOLOGICAL INVESTIGATIONS

• Temporal bone radiology (PLAIN)
CT-SCAN WITH CONTRAST
MRI BRAIN
DIFFERENTIAL DIAGNOSIS
•   Meningioma
•   Primary cholesteatoma
•   Arachnoid cysts
•   Facial neuromas
•   Lipoma
•   Choroid plexus papilloma
•   Glomus jugulare
TREATMENT
• Observation
• Surgery
• Radiotherapy
  – Conventional
  – Stereotactic
STEREOTACTIC RADIOSURGERY
         Indications
• Patient who is poor surgical risk (age,
  medical condition, etc.)
• Tumor < 3 cm
• Younger patients with < 3 cm tumor who
  refuse surgery
SURGERY
• Middle fossa approach
• Translabyrinthine approach
• Retrosigmoid approach
CONSERVATIVE MANAGEMENT
•   Advanced age (> 65 )
•   Short life expectancy (< 10 years)
•   Slow growth rate
•   Poor surgical candidate / poor general health
•   Minimal symptoms
•   Only hearing ear
•   Patience preference
THANK YOU
GLOMUS TUMOURS
Glomus Tumors
• They are the most common benign
  neoplasm of middle ear
• Arise from glomus bodies, which are
  similar to carotid bodies in structure.
• They consist of paraganglionic cells derived
  from the neural crest.
• 1. Glomus jugulare
• 2. Glomus tympanicum
GLOMUS JUGULARE
• Collection of ganglionic tissue within the
  temporal bone in close relation with the
  jugular bulb
TYPES
• Glomus jugulare
  – Dome of jugular bulb
  – IX, XII nerve involvement
• Glomus tympanicum
  – Promontory
  – Aural smptoms
  – VII nerve involvement
PATHOLOGY
• Not very active
• Well-defined thin fibrous capsule
• Locally invasive
• They are sheets of epithelial cells with
  number of thin walled blood sinusoids so
  they are highly vascular.
• Destructive to bone and facial nerve
• Invasion to mastoid air cells and Skull base
EPIDEMIOLOGY
• SEX
  – Female: Male 6:1
• AGE
  – Middle age groups
• INCIDENCE
  – Familial
ENDOCRINE ACTIVITY
• Non-chromaffin paragangliomas
• 10% secretory
• Urine for VMA
MULTICENTRICITY
• Both ears
• With other paragangliomas
SPREAD
•   Initially middle ear
•   TM perforation
•   Labyrinth, petrous pyramid, mastoid
•   Jugular foramen
•   Eustachian tube
•   Intracranially
•   Metastasis to lungs, bones
CLASSIFICATION (FISCH)
• Type A localized to middle ear cleft
• Type B Tympanomastoid tumor no bone
         destruction in infralabyrinthine
         compartment
• Type C Invading infralabyrinthine region and its
         destruction
• Type D Intracranial extension
CLINICAL FEATURES
•   Slow growing
•   Pulsatile tinnitus
•   Conductive deafness
•   Otalgia
•   Aural bleeding
•   Cranial nerve involvement (Facial)
EXAMINATION
• Red mass behind intact tympanic membrane
  – (Rising sun sign)
INVESTIGATIONS
•   Baseline
•   CT-scan head with contrast
•   MRI
•   MR Angiography
RADIOLOGICAL ASSESSMENT
          Radiological assessment of ‘rising sun’
                     Axial CT-scan

   Jugular fossa enlarged          Normal Jugular fossa

 Cortex eroded   Cortex normal            Coronal CT-scan

                            Normal carotid    Laterally placed

                                  canal             carotid canal

Glomus jugulare High jugular bulb Glomus tympanicus
                         Aberrant
MANAGEMENT
•   No active treatment observation
•   Primary radiotherapy
•   Surgical resection
•   Surgery + Radiotherapy
RADIOTHERAPY
• Slow growing tumors
• Elderly
SURGERY
•   Pre-op embolization
•   Transmeatal approach
•   Extended facial recess approach
•   Infratemporal fossa approach
OTALGIA
Innervation of ear
• 5th, 7th, 9th, 10th
• C2, C3
Referred pain
•   Pinna
•   Meatus
•   Middle ear
•   Mastoid
•   Inner ear
Other causes
•   Tonsillitis
•   Parotitis
•   Thyroid
•   Mumps
•   Tuberculosis larynx
•   Styloid process
•   Teeth
Other causes
•   Oral ulceration
•   TM joint
•   MI
•   Malignancy
CENTRAL CAUSES
•   Herpes zoster oticus
•   Glossopharyngeal zoster
•   Post-herpetic neuralgia
•   Trigeminal Neuralgia
•   Glossopharyngeal neuralgia
•   Geniculate neuralgia
•   Migraine
•   Acoustic neuroma
THANK YOU
TINNITUS
CAUSES
• Otologic
  –   Wax
  –   Fluid in middle ear
  –   ASOM CSOM
  –   Meniere’s disease
  –   Otosclerosis
  –   Noise trauma
  –   Ototoxic drugs
  –   Vestibular schwanomma
OTHERS
•   CNS
•   Anaemia
•   Hypertension
•   Hypotension
•   Hypoglycaemia
•   Epilepsy
•   Migraine
•   Psychogenic
TREATMENT
•   Reassurance
•   Treatment of underlying cause
•   Sedation
•   Masking
VERTIGO
VERTIGO
• Rotation
  – Episodic (seconds to hours)
  – Hours (weeks)
• Unsteadiness
  – Episodic (second to hours)
  – Prolonged (weeks to months)
ROTATORY
• Short lived (seconds)
  –   Benign Paroxysmal positional vertigo
  –   Labyrinthine fistula
  –   Post-concussional
  –   Vertebrobasilar insufficiency
  –   Cervical
  –   Caloric effect
ROTATORY
• Hours
  –   Meniere’s disease
  –   Syphilitic labyrinthitis
  –   Delayed endolymphatic hydrops
  –   Middle ear surgery
ROTATORY
• Prolonged (days)
  –   Vestibular neuronitis
  –   Trauma
  –   Ear surgery
  –   Labyrinthectomy
  –   Labyrinthitis
  –   Vascular lesions
UNSTEADINESS
• Seconds
  –   Rapid movement
  –   Abnormal visual input
  –   Visual inadequacy
  –   Vestibular inadequacy
UNSTEADINESS
• Hours
  –   Drugs
  –   Travel sickness
  –   Perilymph fistula
  –   ASOM
  –   Functional
UNSTEADINESS
• Weeks to months
  –   Elderly
  –   Drugs
  –   CNS lesions
  –   CSOM
OTHER CAUSES
• Head trauma
  –   Post concussional
  –   BPPV
  –   Labyrinthine destruction
  –   Perilymph fistula
  –   Functional
SUDDEN SENSORINEURAL
    HEARING LOSS
COCHLEAR
•   Inflammatory (bacterial, viral, fungal)
•   Traumatic
•   Vascular (hypertension)
•   Hematological
•   Autoimmune
•   Endolymphatic hydrops
•   Metabolic
•   Skeletal
•   Ototoxicity
RETROCOCHLEAR
              CNS
•   Meningitis
•   Multiple sclerosis
•   Lateral sclerosis
•   Tumours
•   Friedreich’s ataxia
•   Idiopathic
AUDIOMETRY
• Pure tone audiometry
  – Differentiate conductive and sensorineural
    hearing loss
Speech audiometry
• Speech reception threshold (SRT)
  – Minimum intensity at which 50% of words are
    repeated by patient correctly
• Speech discrimination score
  – 30-40 dB above SRT ---> percentage of words
    heard by the patient
TYMPANOMETRY
• To measure pressure in middle ear
• Calculate EAC volume
• Types
  – Type A (normal)
  – Type B (Conductive)
  – Type C
STAPEDIAL REFLEX
• A loud sound of 70dB above normal
  threshold causes bilateral contraction of
  stapedial    muscles      detected     by
  tympanometry
CALORIC TEST
• Supine position
• Head at 30 degree up
• Irrigate warm (44C) and cold water (30C)
  in the ear  40 seconds
• Observe nystagmus
  – Cold water other side
  – Warm water same side
THANK YOU

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Accustic neuroma 1

  • 1. VESTIBULAR SCHWANNOMA (ACOUSTIC NEUROMA)
  • 2. DEFINITION • Benign tumor of vestibular nerve schwan cells
  • 3. VESTIBULAR SCHWANNOMA • 6 % of all Intracranial tumors • 80 - 90% of CPA tumors • Majority adulthood • 95% Unilateral • 5% Neurofibromatosis type 2 (bilateral) • No known race, gender predilection
  • 4. ANATOMY OF CP ANGLE • Triangular area • Lateral Posterior surface of temporal bone • Medial Edge of pons • Posterior Anterior surface of cerebellum • Superior Trigeminal nerve • Inferior IX, X, XI cranial nerves
  • 5. Petrous Bone Po ns •Cerebrum
  • 6.
  • 7. SITE OF ORIGIN • Schwann cells which envelop distal potion of VIIIth nerve • Within the IAC
  • 8. GROWTH PATTERN • Grows medially • Invested by double arachnoid layer
  • 9. EFFECTS ON INNER EAR • Compressive effect on cochlear nerve • Vascular occlusion of internal auditory artery • Atrophy of organ of Corti • Vacuolization of stria vascularis
  • 10. APPEARANCE • Firm • Well encapsulated • Interior soft • Sometimes filled with serous or hemorrhagic fluid
  • 11. STAGES 1 Otological stage (< 2 cm) 2 Trigeminal nerve involvement (2-2.5 cm) 3 Brain stem and cerebellar compression (2.5-4 cm) 4 Increasing intracranial pressure 5 Terminal stage
  • 12. STAGE 1 OTOLOGICAL STAGE • Deafness – Unilateral, gradual • Tinnitus – Non-pulsatile • Imbalance • Trigeminal nerve involvement • Facial nerve involvement
  • 13. STAGE 2 TRIGEMINAL NERVE INVOLVEMENT • Tumor size 2-2.5 cm • Irritation in eye • Pain, tingling, numbness on face • Feeling of cold on face
  • 14. STAGE 3 BRAIN STEM AND CEREBELLAR COMPRESSION • Ataxia • Gait disturbance • Tremors • Nystagmus
  • 15. STAGE 4 INCREASING INTRACRANIAL PRESSURE • Headache occipital • Nausea Vomiting • Papilloedema
  • 16. STAGE 5 TERMINAL STAGE • Failure of vital centres in brain stem
  • 17. EXAMINATION • Cutaneous lesions (neurofibromas) • Otoscopy: Normal • Tuning fork tests: – Unilateral senorineural deafness • Trigeminal Nerve • Facial nerve • Eye examination
  • 18. EXAMINATION • Finger nose test • Romberg’s test • Unterberg’s test
  • 19. INVESTIGATIONS • Speech test • Speech audiometry • PTA • Stapedial reflex • Electrocochleography • ABR • Caloric test
  • 23.
  • 24. DIFFERENTIAL DIAGNOSIS • Meningioma • Primary cholesteatoma • Arachnoid cysts • Facial neuromas • Lipoma • Choroid plexus papilloma • Glomus jugulare
  • 25. TREATMENT • Observation • Surgery • Radiotherapy – Conventional – Stereotactic
  • 26. STEREOTACTIC RADIOSURGERY Indications • Patient who is poor surgical risk (age, medical condition, etc.) • Tumor < 3 cm • Younger patients with < 3 cm tumor who refuse surgery
  • 27.
  • 28. SURGERY • Middle fossa approach • Translabyrinthine approach • Retrosigmoid approach
  • 29. CONSERVATIVE MANAGEMENT • Advanced age (> 65 ) • Short life expectancy (< 10 years) • Slow growth rate • Poor surgical candidate / poor general health • Minimal symptoms • Only hearing ear • Patience preference
  • 32. Glomus Tumors • They are the most common benign neoplasm of middle ear • Arise from glomus bodies, which are similar to carotid bodies in structure. • They consist of paraganglionic cells derived from the neural crest. • 1. Glomus jugulare • 2. Glomus tympanicum
  • 33. GLOMUS JUGULARE • Collection of ganglionic tissue within the temporal bone in close relation with the jugular bulb
  • 34. TYPES • Glomus jugulare – Dome of jugular bulb – IX, XII nerve involvement • Glomus tympanicum – Promontory – Aural smptoms – VII nerve involvement
  • 35. PATHOLOGY • Not very active • Well-defined thin fibrous capsule • Locally invasive • They are sheets of epithelial cells with number of thin walled blood sinusoids so they are highly vascular. • Destructive to bone and facial nerve • Invasion to mastoid air cells and Skull base
  • 36. EPIDEMIOLOGY • SEX – Female: Male 6:1 • AGE – Middle age groups • INCIDENCE – Familial
  • 37. ENDOCRINE ACTIVITY • Non-chromaffin paragangliomas • 10% secretory • Urine for VMA
  • 38. MULTICENTRICITY • Both ears • With other paragangliomas
  • 39. SPREAD • Initially middle ear • TM perforation • Labyrinth, petrous pyramid, mastoid • Jugular foramen • Eustachian tube • Intracranially • Metastasis to lungs, bones
  • 40. CLASSIFICATION (FISCH) • Type A localized to middle ear cleft • Type B Tympanomastoid tumor no bone destruction in infralabyrinthine compartment • Type C Invading infralabyrinthine region and its destruction • Type D Intracranial extension
  • 41. CLINICAL FEATURES • Slow growing • Pulsatile tinnitus • Conductive deafness • Otalgia • Aural bleeding • Cranial nerve involvement (Facial)
  • 42. EXAMINATION • Red mass behind intact tympanic membrane – (Rising sun sign)
  • 43. INVESTIGATIONS • Baseline • CT-scan head with contrast • MRI • MR Angiography
  • 44.
  • 45. RADIOLOGICAL ASSESSMENT Radiological assessment of ‘rising sun’ Axial CT-scan Jugular fossa enlarged Normal Jugular fossa Cortex eroded Cortex normal Coronal CT-scan Normal carotid Laterally placed canal carotid canal Glomus jugulare High jugular bulb Glomus tympanicus Aberrant
  • 46. MANAGEMENT • No active treatment observation • Primary radiotherapy • Surgical resection • Surgery + Radiotherapy
  • 47. RADIOTHERAPY • Slow growing tumors • Elderly
  • 48. SURGERY • Pre-op embolization • Transmeatal approach • Extended facial recess approach • Infratemporal fossa approach
  • 50. Innervation of ear • 5th, 7th, 9th, 10th • C2, C3
  • 51. Referred pain • Pinna • Meatus • Middle ear • Mastoid • Inner ear
  • 52. Other causes • Tonsillitis • Parotitis • Thyroid • Mumps • Tuberculosis larynx • Styloid process • Teeth
  • 53. Other causes • Oral ulceration • TM joint • MI • Malignancy
  • 54. CENTRAL CAUSES • Herpes zoster oticus • Glossopharyngeal zoster • Post-herpetic neuralgia • Trigeminal Neuralgia • Glossopharyngeal neuralgia • Geniculate neuralgia • Migraine • Acoustic neuroma
  • 57. CAUSES • Otologic – Wax – Fluid in middle ear – ASOM CSOM – Meniere’s disease – Otosclerosis – Noise trauma – Ototoxic drugs – Vestibular schwanomma
  • 58. OTHERS • CNS • Anaemia • Hypertension • Hypotension • Hypoglycaemia • Epilepsy • Migraine • Psychogenic
  • 59. TREATMENT • Reassurance • Treatment of underlying cause • Sedation • Masking
  • 61. VERTIGO • Rotation – Episodic (seconds to hours) – Hours (weeks) • Unsteadiness – Episodic (second to hours) – Prolonged (weeks to months)
  • 62. ROTATORY • Short lived (seconds) – Benign Paroxysmal positional vertigo – Labyrinthine fistula – Post-concussional – Vertebrobasilar insufficiency – Cervical – Caloric effect
  • 63. ROTATORY • Hours – Meniere’s disease – Syphilitic labyrinthitis – Delayed endolymphatic hydrops – Middle ear surgery
  • 64. ROTATORY • Prolonged (days) – Vestibular neuronitis – Trauma – Ear surgery – Labyrinthectomy – Labyrinthitis – Vascular lesions
  • 65. UNSTEADINESS • Seconds – Rapid movement – Abnormal visual input – Visual inadequacy – Vestibular inadequacy
  • 66. UNSTEADINESS • Hours – Drugs – Travel sickness – Perilymph fistula – ASOM – Functional
  • 67. UNSTEADINESS • Weeks to months – Elderly – Drugs – CNS lesions – CSOM
  • 68. OTHER CAUSES • Head trauma – Post concussional – BPPV – Labyrinthine destruction – Perilymph fistula – Functional
  • 69. SUDDEN SENSORINEURAL HEARING LOSS
  • 70. COCHLEAR • Inflammatory (bacterial, viral, fungal) • Traumatic • Vascular (hypertension) • Hematological • Autoimmune • Endolymphatic hydrops • Metabolic • Skeletal • Ototoxicity
  • 71. RETROCOCHLEAR CNS • Meningitis • Multiple sclerosis • Lateral sclerosis • Tumours • Friedreich’s ataxia • Idiopathic
  • 72. AUDIOMETRY • Pure tone audiometry – Differentiate conductive and sensorineural hearing loss
  • 73. Speech audiometry • Speech reception threshold (SRT) – Minimum intensity at which 50% of words are repeated by patient correctly • Speech discrimination score – 30-40 dB above SRT ---> percentage of words heard by the patient
  • 74. TYMPANOMETRY • To measure pressure in middle ear • Calculate EAC volume • Types – Type A (normal) – Type B (Conductive) – Type C
  • 75. STAPEDIAL REFLEX • A loud sound of 70dB above normal threshold causes bilateral contraction of stapedial muscles detected by tympanometry
  • 76. CALORIC TEST • Supine position • Head at 30 degree up • Irrigate warm (44C) and cold water (30C) in the ear  40 seconds • Observe nystagmus – Cold water other side – Warm water same side