The document discusses neoplasms of the middle ear, detailing primary tumors, including benign and malignant types, and their classification along with symptoms, investigations, and treatment options. It also covers acoustic neuroma, highlighting its characteristics, manifestations, and approaches for management. Key imaging techniques and surgical methods are described for both types of tumors, emphasizing the importance of diagnostics and staging in determining effective interventions.

1. NEOPLASMS OF
MIDDLE EAR
Dr Ashish khadgi

2. Classification
A. Primary tumours
1. Benign
I. Neoplastic: Paragangliomas, Facial Nerve schwannoma,
Haemangioma, adenoma
II. Non-neoplastic: Congeneital cholesteatoma, choristoma,
abberant internal carotid artery, high jugular bulb
2. Malignant: Carcinoma of middle ear, sarcoma
B. Secondary tumours
I. From adjacent areas: External ear canal, parotid gland,
Nasopharynx
II. Metastatic: From carcinoma of breast, bronchus, kidney, thyroid,
prostrate

3. GLOMUS
TUMOURS

4. Introduction
Synonym:  Chemodectoma
 Non-chromaffin paraganglioma
Commonest benign tumour of middle ear derived from
glomus bodies distributed along parasympathetic
nerves of head & neck
Consists of paraganglionic cells derived from
embryonic neuroepithelium

5. Introduction
Histologically benign but locally invasive, highly
vascular, non-encapsulated, slow growing tumors
10 % tumors: familial
10 % tumors: multicentric
3 % tumors: functional (secrete catecholamines)
4 % tumors: metastatic

6. Histopathology
Typical cellular groups ("Zellballen") surrounded by a capillary network

7. Types
Glomus jugulare
Arises along jugular bulb & superior vagal
Ganglion, near floor of middle ear
Glomus tympanicum
Arises along tympanic plexus on promontory
formed by tympanic branch of Glossopharyngeal nerve,
near medial wall of middle ear

8. Spread

9. Common Symptoms
■ Seen in 40-60 yrs
■ Female : male = 5:1
■ U/L deafness: progressive, conductive
■ Pulsatile tinnitus: synchronous with pulse
decreases on carotid occlusion
■ Blood stained otorrhoea
■ Ear ache & vertigo: rare

10. Signs
■ Rising sun sign: red reflex on otoscopy
■ Browne’s pulsation sign on siegalization: Positive
pressure  tumor engorges  tumor blanches 
pressure released  tumor engorges
■ Aural mass: bleeds on touch
■ Systolic bruit: over mastoid on auscultation
■ Neurological: 9th
, 10th
11th
cranial nerve palsy

11. Rising sun sign

12. Blood-stained otorrhoea

13. Bleeding polyp

14. Investigations
1. Pure Tone Audiometry: Conductive deafness
2. High resolution C.T. scan with contrast:
erosion of carotico-jugular spine (Phelp’s sign)
3. Magnetic Resonance Imaging with Gadolinium
contrast: for soft tissue & intra-cranial extension
4. M. R. Angiography: non-invasive. For invasion of
Internal jugular vein & internal carotid compression

15. Investigations
5. Digital Subtraction Angiography
6. Four Vessel Angiography
 Tumour blush
 Feeding arteries
 Contra lateral circulation
 Embolization (within 48 hours of surgery)
 Other carotid body tumors

16. Investigations
7. 24 hour urine Vanillyl Mandelic Acid level:
> 7 mg  Catecholamine secreting tumor
 Initial hypertension during surgery followed by
hypotension
8. Careful biopsy of mass in ext. auditory canal: rule
out malignancy. Ear packing done for profuse
bleeding.

17. C.T. scan plain
Glomus Jugulare

18. Plain & contrast C.T. scan

19. M.R.I. with contrast

20. Fisch Staging
■ Stage A: tumor limited to middle ear cleft
■ Stage B: tympano-mastoid tumor sparing
infra-labyrinthine bone
■ Stage C: tympano-mastoid tumor eroding
infra-labyrinthine bone
■ Stage D1: Intra-cranial extension < 2 cm
■ Stage D2: Intra-cranial extension > 2 cm

21. Surgical Treatment
■ Anterior Tympanotomy: small stage A
■ Extended facial recess approach: large stage A
■ Modified Radical Mastoidectomy: small Stage B
■ Combined Modified Radical Mastoidectomy +
Fisch’s Infratemporal fossa approach:
large stage B, Stage C
■ Subtotal temporal bone resection: Stage D1

22. Other Treatments
Radiotherapy (4000 – 5000 rads) or Stereotactic
Radiotherapy:
■ Inoperable, residual or recurrent tumors;
■ Pt unfit for surgery or refuses surgery
Observation: Pt > 70 yr with minimal symptoms
Embolization:
■ Before surgery: reduces vascularity
■ After RT: for residual or recurrent tumor

23. ACOUSTIC
NEUROMA

24. Introduction
A.K.A.: vestibular schwannoma / neurilemmoma
Benign, encapsulated, slow growing tumour
arising from Schwann cells of superior vestibular
division of 8th
nerve within internal auditory canal
Rarely from inferior vestibular or cochlear division

25. Tumour growth
Tumor expansion within internal auditory canal
 causes widening & erosion of I.A.C.
 appears in cerebello-pontine angle (> 2.5 cm)
 involves 5th
, 7th
, 9th
, 10th
, 11th
cranial nerves
 displacement of brainstem & cerebellum
 raised intracranial pressure
 Involvement of 6th
& 3rd
cranial nerves

26. Classification as per size
1. Intra-canalicular: confined to I.A.C.
2. Small: up to 1.5 cm
3. Medium: 1.5 to 4 cm
4. Large: over 4 cm

27. Tumor size

28. Epidemiology
■ 10% of all brain tumors
■ 80% of all Cerebello-pontine angle tumors
■ Age: 40-60 yrs
■ Male : Female = 3:2
■ Unilateral (90%); Bilateral (10%)
■ Bilateral = von Recklinghausen’s
neurofibromatosis

29. Clinical Staging
1. Otological stage: due to pressure on 8th
nerve
2. Other Cranial nerve involvement
3. Brainstem + Cerebellar involvement
4. Raised intra-cranial tension
5. Terminal stage: failure of vital centers of
brainstem & cerebellar tonsil herniation

30. Otological symptoms & signs
1. Progressive, unilateral sensorineural deafness
2. Poor speech discrimination (disproportionate)
3. Tinnitus
4. Mild vertigo
5. Nystagmus
Vestibular symptoms appear late due to slow tumor
growth & vestibular compensation

31. Other Cranial nerve palsy
Trigeminal: first nerve to be involved
■ Loss of corneal reflex
■ Pain, numbness and paresthesia of the face
Facial:
■ Hypoaesthesia of posterior external auditory canal
wall (Hitselberger’s sign)
■ Facial weakness, Loss of taste, ed lacrimation

32. Other Cranial nerve palsy
Glossopharyngeal, Vagus & Accessory Spinal:
– Dysphagia
– Hoarseness
– Nasal regurgitation
– Decreased gag reflex
Abducent & Oculomotor:
– Diplopia

33. Brainstem involvement
 Ataxia  Weakness of arms & legs  Tendon
reflexes exaggerated
Cerebellar involvement
 Ataxic gait (fall on affected side)  Intention
tremors  Past-pointing  Dysdiadochokinesia
Increased Intra-cranial tension
 Headache  Projectile vomiting  Blurred vision
 Papillodema  Abducent nerve palsy

34. First Symptoms
Hearing loss: 80-100 %
Vertigo: 10-50 %
Tinnitus: 5-10 %
Ear ache: 5 %
Sudden hearing loss: 5%
Facial paralysis: 1-2 %

35. Investigations
■ Pure Tone Audiometry: high frequency SNHL
■ Speech audiometry: SD scores < 30%
■ Tone decay test: positive
■ Stapedial Reflex: Decay > 50 % in 10 sec
■ B.E.R.A.: wave V >4.2 ms; inter-wave V >0.2 ms
■ Caloric test: I/L canal paresis or no response
■ C.T. scan with contrast: for tumor > 0.5 cm
■ M.R.I. with gadolinium contrast: best

36. Pure Tone Audiogram

37. Speech Audiometry
Roll over
phenomenon

38. Contrast C.T. Scan

39. Contrast M.R.I.

40. Treatment
1. Observation
2. Microsurgical removal: (partial or total)
■ Trans-labyrinthine approach
■ Retro-sigmoid or Sub-occipital approach
■ Middle Cranial Fossa approach
■ Combined approach
3. Proton Stereotactic Radiotherapy
4. Brainstem Implant: after B/L tumor excision

41. Observation
Indications:
1. Age > 60 years with small tumor & no symptoms
2. Tumour in only hearing / better hearing ear
Serial MRI used to follow growth pattern.
Treatment recommended if hearing is lost or tumor
size becomes life threatening.

42. Surgical Approach Protocol
1. Intra-canalicular: Middle cranial fossa approach
2. Small (<1.5 cm): Retrosigmoid approach
3. Medium (1.5 - 4 cm)
a. Hearing fine**: Retrosigmoid approach
b. Hearing bad: Trans-labyrinthine approach
4. Large (>4 cm): Trans-labyrinthine / Combined
** Pure Tone Average < 30 dB, S.D. Score >70%

43. Intra-operative photograph

44. Proton stereotactic radiotherapy
Single high dose of radiation delivered on a small
area to arrest or kill tumor cells. Minimal injury to
surrounding nerves & brain tissue
Gamma Knife: radioactive cobalt
LINAC X-knife: linear accelerator
Cyber-Knife: robotic radio-surgery system
Indication: 1. Surgery refused / contraindicated 2.
Post-operative residual tumour

45. Thank you for your
attention