1. Pathogenesis Classification Complications
CSOM
Pathogenesis
Classification
Complications
Vishav
Yadav

2. Introduction
Keratin-producing squamous epithelium in the middle ear, mastoid or petrous apex
Johannes Müller (1838):German Physiologist coined cholesteatoma i.e. layered paerly tumpour of fat
Schuknecht : keratoma

3. DEFINITION
Abramson
3D epithelial and connective tissue structure usually in form of sac , mostly confined to middle ear spaces , with tendency of independent growth on cost of underlying bone , with tendency of recurrence despite complete removal .

4. Histologically made up of :
Cystic content – anucleate keratin squames
Matrix – keratinizing squamous epithelium
Perimatrix – granulation tissue in contact with bone (produces proteolytic enzymes)

5. Classification Congenital Acquired
Primary acquired (retraction pocket)
Secondary acquired
Tertiary acquired

6. Pathogenesis

7. Congenital cholesteatoma
The incidence of CC - 1% to 5% of all cholesteatomas
Congenital cholesteatoma of the middle ear was first described by Howard House
Location (AS,PS , petrous pyramid, mastoid and middle ear cleft , CP Angle)

8. Congenital cholesteatoma
Levenson criteria (Modified Derlacki & Clemis)
White mass medial to normal TM
Normal pars flaccida and tensa
No history of otorrhea or perforations
No prior otologic procedures
Prior bouts of otitis media not grounds for exclusion

9. Congenital cholesteatoma :theories
The accepted cause of CC remains controversial
Teed’s epithelial cell rest theory
Friedberg’s implantation theory
Ruedi’s invagination theory
Aimi’s epithelial migration theory
Michael’s epidermoid formation theory

10. Epithelial rest Most commonly accepted and quoted theory Teed
An epidermal structure : these rests as ectodermal implants in the fusion plates between the first and second branchial arches that appear around 10 weeks
It aided in middle ear and tympanic membrane development. Initially dormant, it undergoes rapid proliferation before resorption around 33 weeks’ gestation
CC is thought to form if resorption is incomplete

11. Implantation theory
Friedberg observed viable squamous cells in the amniotic fluid present in the middle ear of neonates.
Could they be a cause for congenital cholesteatoma?

12. INVASION Ruedi Inflammatory injury to an intact tympanic membrane
Microperforations in the basal layer that lead to invasion of the squamous epithelium by proliferating epithelial cones through a macroscopically intact but microscopically injured tympanic membrane.
Epithelial cones fuse and expand forming a middle ear cholesteatoma

13. Epithelial migration theory
First proposed by Aimi
He suggested that ectoderm of external canal managed to grow / migrate in to the middle ear cavity somehow overcoming the restraining influence of the annulus.

14. Epidermoid formation theory
First proposed by Michael
He observed nests of squamous epithelium in the lateral wall of tympanic cavity below the level of pars flaccida
These nests normally involute
Failure of this involution process can possibly cause cholesteatoma

15. Acquired inclusion theory

16. Staging (1) aid the clinician in preoperative planning of treatment
(2) Indicate prognosis
(3) facilitate exchange of information between different clinicians
(4) evaluate the results of treatment.

17. Derlacki and Clemis STAGING
Petrous Pyramid
Mastoid
Tympanic

18. Potsic STAGING
Stage 1: Single quadrant with no ossicular or mastoid involvement
Stage 2: Multiple quadrants with no ossicular or mastoid involvement
Stage 3: Ossicular involvement but no mastoid involvement
Stage 4: Mastoid extension

19. Nelson STAGING Type 1: Mesotympanum with no incus or stapes erosion
Type 2: Mesotympanum or attic with ossicular erosion but no mastoid extension
Type 3: Mesotympanum with mastoid extension
Clinical Stage
Recurrence Rates
TYPE 1 15
TYPE 2 59 34
TYPE 3 26 55

20. Acquired cholestaetoma

21. Primary acquired Pathogenesis
Invagination theory of Wittmaack
Eustachian tube dysfunction
Poor aeration of the epitympanic space
Retraction of the pars flaccida
Normal migratory pattern altered
Accumulation of keratin, enlargement of sac

22. Primary acquired Pathogenesis
Basal cell hyperplasia (Ruedi theory)
Inflammatory injury to an intact tympanic membrane
Microperforations in the basal layer that lead to invasion of the squamous epithelium by proliferating epithelial cones through a macroscopically intact but microscopically injured tympanic membrane.
Epithelial cones fuse and expand forming a middle ear cholesteatoma

23. Primary acquired Pathogenesis
Squmaous metaplasia (Sade theory)
transformation of cuboidal epithelium to squamous epithelium from chronic infection

24. Secondary acquired Pathogenesis
Presence of preexisting perforation in pars tensa
Often associated with posterosuperior marginal perforation or large central perforation

25. Secondary acquired Pathogenesis
Immigration & Invasion:
Habermann
medial migration along permanent perforation of TM
Implantation & invasion –foreign body, blast injury
Metaplasia – transformation of cuboidal epithelium to squamous epithelium from chronic infection
Papillary ingrowth – intact pars flaccida, inflammation in Prussack’s space, break in the basal membrane, cords of epithelium migrate inward

26. Tertiary acquired Pathogenesis
iatrogenic – surgery, foreign body, blast injury
Tympanoplasty
Ossiculoplasty
Stapedotomy
Typmpanostomy tubes

27. spread

28. Anatomic Considerations
Epitympanic cholesteatoma patterns of spread from Prussack’s space
Posterior epitympanum
Posterior mesotympanum
Anterior epitympanum

29. Prussak’s space Medially :neck of malleus
laterally :Shrapnell’s membrane
Sup : lat malleal fold
Anteriorly anterior malleolar fold & anterior ligament

30. Cholesteatoma spread

31. Cholesteatoma spread
Posterior epitympanum – through superior incudal space to mastoid antrum

32. Cholesteatoma spread
Posterior mesotympanum – inferiorly through posterior pouch of Von Troeltsch to stapes, round window, sinus tympani and facial recess

33. Cholesteatoma spread
Anterior epitympanum – anterior to head of malleus, may gain access to supratubal recess
To ant mesotympanum via anterior pouch of von Troeltsch

34. Sinus Tympani (Infrapyramidal Reccess)
*Medially – Medial Wall Of Middle Ear with Oval & Round Window Niches *Laterally – Pyramid & Facial Nv *Superiorly– Ponticulus *Inferiorly – Subiculum -can extend as far as 9mm into the mastoid -probably the most inaccessible site in middle ear & mastoid

35. Pathology Molecular models Preneoplastic transformation events
Defective wound-healing process
Collision between host inflammatory response, normal middle ear epithelium, and bacterial infection

36. Preneoplastic transformation
Hyperproliferative keratinocytes
Increased proliferation
Decreased terminal differentiation
Expression of epithelial markers in the basal and suprabasal layers (cytokeratins –10,13,16, filaggrin, involucrin); confirm they arise from pars flaccida and overlying EAC skin
High expression of epidermal growth factor receptor, transforming growth factor
Upregulation of p53

37. Defective wound healing
Chronic inflammatory response around matrix (granulation/perimatrix)
Infiltration of activated T-cells and macrophages
Production of cytokines (TGF,TNF,IL-1,IL- 2,FGF,PDGF)
Causes increased migration and invasion of cholesteatoma epithelium and fibroblasts

38. Host response vs. bacteria
Bacterial related antigens producing host inflammatory response may stimulate the migrating epithelium’s uncoordinated proliferation
Granulation induces invasion of keratinocytes
Granulation – contains proteases, acid phosphatases, bone resorption proteins, osteoclast-activating factors, prostaglandins

39. complications

40. Intracranial
Extradural abscess
Subdural abscess (empyema)
Sigmoid sinus thrombophlebitis
Meningitis
Brain abscess
Otitic hydrocephalus

41. Extratemporal complications:
Subperiosteal abscess
Migrating thrombophlebitis of IJV
Intratemporal
Facial paralysis
Labrynthine infections
Labyrinthine fistula
Petrositis
Mastoiditis
Other rare complications:
Abscess:
Bezold’s abscess
Luc’s abscess
Citeli’s abscess
Distant pyaemic abscess

42. Possible routes of spread:
Extension through bone
Infected clot within small veins
Normal anatomical pathways
Non anatomical bony defects
Surgical defects
Into brain tissue along
periarteriolar spaces of Robin Virchow

43. General principles:
The complications are multiple in about 1/3rd of the cases
The symptoms of intracranial spread of infection are those of infection and those of brain tissue compression
Otalgia is never a symptom of uncomplicated cholesteatoma
Investigation and treatment must run concurrently
The principles of treatment :
Systemic antibiotic therapy
Local neurosurgical attention to the complication(s)
Treatment of the ear lesion

44. Organisms involved: multiple organisms
Intracranial complication:
G-ve organisms
Staphylococci- beta lactamase producing
Obligate anaerobes Bacteroides fragilis
Extratemporal and temporal complications:
Pseudomonas
Proteus

45. When to suspect complications
High grade fever
Headache with vomiting
Copious ear discharge
Gait changes
Visual alterations
Seizure activity
Neck stiffness
Unusual persistent pain

46. Extradural abscess
Etiology :Bone erosion
Sigmoid perisinus abscess

47. Pott’s puffy tumour

48. Clinical features: Diagnosis : Site and size Duration and rate
Incidental finding
Headache with malaise
Intermittent relief from pain during episodes of aural discharge
Diagnosis :
Operative findings
CT scan

50. Management : Surgical exploration
????Don’t remove the granulation tissue attached to the dura
Appropriate antibiotics

51. Subdural Abscess(empyema)
Spread of infection
Loculated infection
Thrombophlebitis
Obliteration of subdural space
Commonly associated organisms:
Strp. milleri
H. influenzae

53. Clinical features: Severe headache Fever Drowsiness
Focal neurological deficit
Seizure
Paralysis
The course is much more rapid than brain abscess
Papilledema is uncommon, as are the cranial nerve palsy
Seizures and FND differentiates from meningitis

54. Diagnosis CT scan MRI LP : helpful but risky ↑pressure
Normal sugar content
Cultures are sterile
Marked pleocytosis

55. Management : Removal of subdural fluid Massive antibiotics
Treatment of ear disease
Antiepileptics

56. Lateral sinus thrombophlebitis
Sigmoid sinus
Transverse sinus
Usually preceded by the development of extradural perisinus abscess
Spread of infection
Torcula herophili (confluence of sinuses)
Superior sagittal sinus
Superior and inferior petrosal sinus
Cavernous sinus
Brain abscess
Internal jugular vein
Subclavian vein

59. Commonly associated organisms:
Beta hemolytic streptococci
Staphylococus aureus
Now a days : mixed flora :
Venezio et al in 1982 grew Proteus mirabilis, staphylococci, streptococcus pneumoniae and Bacteroides oralis

60. Clinical features: Classical picture before antibiotics:
Severe pyrexial wasting illness
Develop over several weeks
Picket fence fever
Headache and neck pain
Emaciation with anaemia
Spread along the IJV:
Perivenous inflamation
Perivenous edema
Suppuration of the lymph node
Paralysis of the lower cranial nerves
↑ICP
Hydrocephalus
Superior extension
Superior sagittal sinus
Cavernous sinus

61. Septic embolisation (sinus thrombophlebitis)
Lung fields
Large joints
Subcutaneous tissue
Other viscera
Pleuroperitoneal cavity
Mastoid emissary vein: suboccipital abscess
Griesinger’s sign:
oedema of the postauricular soft tissues overlying the mastoid process as a result of thrombosis of the mastoid emissary vein

62. Investigations : Hemogram Blood cultures Lumbar puncture :
Low WBC count
Normal CSF pressure
Queckstedt or Tobey-Ayer test
CT scan:
An empty triangle at the level of the sigmoid sinus, clot surrounded by a high-intensity rim of contrast-enhanced dura: delta sign
Angiography
Venography
MRI

63. Tobey-Ayer test (for sinus thrombosis)
The jugular vein on the side of the suspected thrombosis is compressed
A rise in spinal fluid pressure should occur
Its absence indicates presence of thrombosis.

66. Treatment : ???? Anticoagulants : Administration of antibiotics
Exposure of the sinus: incision and removal of contents
Perisinus cells need to be cleared: chances of delayed sinus thrombosis if not cleared
???? Anticoagulants :
If in spite of the treatment the infection is progressively involving the cortical veins
Role of IJV ligation:
if the thrombus is getting dislodged then the need to ligate IJV

67. Meningitis( leptomeningitis )
The commonest intracranial complication
Childhood otogenic meningitis is seen most often as a complication of acute middle ear infection
In adults, it is now more commonly a complication of chronic disease

68. Organisms responsible for acute infection:
Pathways of spread:
Bone erosion
Suppurative labrynthitis
Rupture of established brain abscess
Organisms responsible for acute infection:
Hemophilus influenzae type B
Streptococcus pneumoniae type III
Organisms responsible in chronic infection:
G-ve enteric organisms
Proteus
Pseudomonas
Anaerobes such as Bacteroides species

69. Clinical features: Headache and neck stiffness Malaise
Pyrexia mental hyperactivity
Exaggerated Tendon reflexes
Photophobia
Vomiting

70. Diagnisis : Lumbar puncture
Rise in fluid pressure above the normal mm Hg
Gross appearance of fluid: cloudy and then turbid
On histological inspection PMN cells ranging from x109 /l
Biochemical tests:
Protein content may rise from normal of mg/l to a raised level of 2-3g/l
Chloride content may fall from the normal 120mmol/l to 80mmol/l
Fall in glucose level from the normal value of mmol/l to zero
Bacteriological examination : gram staining and then culture
D/d :
Rupture of brain abscess
Rupture of subdural abscess
CT scan
MRI
PCR

71. Treatment : Medical : Surgical :
Repeated LP to lower the raised pressure
Large doses of systemic antibiotics
Systemic antibiotics must continue for 10 days after apparent clinical recovery
Dexamethasone??
Surgical :
Certainly deterioration or failure of response over 48hrs implies loculated infection in the mastoid, needing surgical drainage

73. Failure of an adequate response to antimicrobial therapy may be a result of :
Organism resistant to chosen antibiotics
Persistent leakage of infected material into the CSF
Persistence of a previously unidentified other complication/Leakage into the CSF from an unrecognized brain abscess.

74. Brain abscess
It is a focal suppurative process within the brain parenchyma serrounded by a region of encephalitis.
Bimodal age of distribution with peaks in the paediatric age group and in the 4th decade of life.
Almost always develop in the temporal lobe or the cerebellum
Temporal lobe abscess is twice as common as cerebellum

75. Various routes of spread:
Through an osteitic tegmen tympani , with middle fossa extradural abscess
Local pachymeningitis followed by thrombophlebitis
Extension of infection along periarteriolar Virchow-Robin spaces
Cerebellar abscess are frequently preceded by lateral sinus thrombophlebitis

77. Bacteriology :polymicrobial (also extradural)
Anaerobes
Streptococcus & staphylococcus
G- organism:
Escherichia coli
Proteus
Klebsiella
Pseudomonas

78. Clinical presentation:
Very toxic and drowsy
Brain abscess is associated with the triad of:
Headache
High grade fever
Focal neurological deficits
Cerebellar abscess:
Dizziness
Ataxia
Nystagmus
Vomiting
Temporal lobe lesion:
Seizure
Visual field defects
Nominal aphasia

80. Diagnosis : Imaging : Computed tomography
Hypodense area by an area of edema, ring sign
MRI: superior to CT scan except for delineation of temporal bone is poor.

83. Other d/d: Meningitis Subdural abscess Lateral sinus thrombophlebitis
Otitic hydrocephalus
Other masses such as brain tumour.

84. Management : High-dose antimicrobial medication
Patient should be first stabilized neurologically:
? Aspiration with high dose of antibiotics
?Total excision
?parenteral antibiotics

85. Otitic hydrocephalus
Syndrome (pseudotumour cerebrii) associated with otitis media with
Increased intracranial pressure
Normal CSF findings
Spontaneous recovery
No abscess
No associated ventricular dilation: benign raised intracranial tension.

86. Pathophysiology:
Symonds: decreased absorption of CSF secondary to blockage of arachnoid villi
Sahs and Joynt: secondary to brain edema
Weed and Flexner: disruption in venous circulation as a cause .

88. Clinical presentation
Symptoms :
Headache
Drowsiness
Vomiting
Blurring of vision
Diplopia
Signs:
Papilledema
6th cranial nerve palsy
Optic disc atrophy

89. Diagnosis : Management: ↑CSF pressure Normal CSF biochemistry
MRI is the modality of choice
Management:
Lowering of the elevated intracranial pressure
Medical therapy:
Corticosteroids
Mannitol
Diuretics
Acetazolamide
Eradication of ear disease

90. Facial nerve paralysis
In CSOM ostietis and subsequent bone erosion likely expose the nerve to infection
This results in inflammation and ultimately facial nerve compression
The tympanic segment is the most common area of involvement
HRCT of the temporal bone
Management :
Intravenous antibiotics
Prompt surgical intervention:
Any attached granulation to the nerve should not be removed
Healthy bone should be removed from the nerve on either side of the diseased segment.
Concomitant corticosteroids

91. Labyrithitis It has two clinically distinguishable subtypes:
Localised, circumscribed, or serous labyrinthitis without total or permanent loss of function
Diffuse, purulent or suppurative labyrinthitis with permanent total destruction of the sensory elements within the labyrinth

92. Serous labrynthitis
As the result of osteitis of the labyrinth capsule or of extension along preformed pathways
Potential pathway include:
oval window
Round window membrane
Cholesteatomatous fistula

93. Clinical features: Symptoms Signs:
Vestibular symptoms precede cochlear symptoms by hours to days when the site of invasion is semicircular canal
Symptoms
Profound vertigo
Nausea
Vomiting
Signs:
Spontaneous nystagmus towards the unaffected ear.
Some degree of ataxia with pastpointing
High frequency sensorineural hearing loss
Distortion of hearing.

94. Treatment :
Parenteral antibiotics

95. Suppurative labrynthitis
Usually following the serous labyrinthitis
Involvement of labyrinth secondary to generalized meningitis
Symptoms: same as serous labyrinthitis including the lack of fever
Key differences:
Symptoms are more rapid and intense
The onset of severe vestibular symptoms is accompanied by a complete loss of cochleovestibular response
A caloric response is conspicuously absent from the disease ear.
d/d: cerebellar abscess

96. Treatment:
Close and continuous monitoring for symptoms of intracranial extension
Bed rest with minimal head movements
Antiemetics
Antibiotics
If meningeal signs are noted: LP
Any signs of intracranial spread: labyrinthectomy
Premature surgical trauma can promote dissemination of infection: mastoid exploration to be deferred until acute symptoms subside.

97. Labyrinthine fistula Bone erosion by cholesteatoma
Most common site: dome of the lateral semicircular canal.
Symptoms :
Episodic vertigo
Signs:
Fistula test
Negative test does not rule out fistula
Tullio’s phenomenon
Site of fistula:
The dome of the LSCC: deviation of eyes towards normal ear.
LSCC fistula anterior to the ampulla: deviation towards the affected ear.
Erosion of the vestibule: rotatory horizontal deviation towards the diseased ear.
SSCC: rotatory movements towards the normal ear
PSCC: vertical deviation of the eyes.

98. Investigations: Treatment: HRCT temporal bone Surgery :
Managed according to the site, size and status of hearing
The rate of total hearing loss is 8% to 56%

99. Acute petrositis
It is the infected petrosal cells with inadequate drainage causing bone changes of coalescence in the cell walls and resulting in symptoms referable to the petrosa.
Symptoms:
Gradneigo’s syndrome:
1.Deep-boring pain: depending upon the site of involvement:
Posterior group of cells:
occipital, parietal, or temporal
Discharge is from the mastoid
Anterior petrositis:
Frontal or behind the eye
Drainage is from the tympanum
2.Aural drainage
3,Diplopia : 6th cranial nerve palsy occurs when the apex is involved

100. Investigations:
HRCT temporal bone
Gallium 67 scan
Technetium 99m scan
Management :
Parenteral antibiotics
Surgical drainage

101. Chronic Mastoiditis Can occur with:
Long standing perforation
Cholesteatoma
Once any type of mastoiditis causes continuous purulent drainage for 8 or more weeks, the likelihood of complete resolution with antibiotics significantly decreases.
Hallmark triad:
Otalgia
Postauricular pain
Fever
Signs:
Postauricular tenderness
Protrusion of pinna
Postauricular erythema
Induration over the mastoid: impending subperiosteal abscess.

102. Investigaton : Treatment : Parenteral antibiotics Mastoidectomy:
HRCT temporal bone
Treatment :
Parenteral antibiotics
Mastoidectomy:
Significant bony destruction
Poor response to up to 2 weeks of conservative management.

103. Subperiosteal abscess
Direct destruction of cortical bone / hematogenous spread through small vascular channels.
Well pneumatized mastoids are more susceptible
The most common site of cortex breakdown is through the thin trabecular bone Macewen’s triangle
Signs and symptoms:
Auricle is displaced forward and outward
Fluctuant mass can be palpated behind the ear
Bezold’s abscess
Luc’s abscess

104. Investigations: HRCT temporal bone Management:
Simple mastoidectomy
Antibiotics

105. Benzold abscess:
Breach in the bony plate forming the inner surface of mastoid tip.
Erosion of inner or outer cortex of the mastoid if periphlebitis or phlebitis propagate the infection.
Pus track below the sternocleidomastoid or even the layer of cervical fascia.
d/d : inflamed lymph nodes
Investigation : CT scan
Treatment :
Complete excision of mastoid pathology
Drainage of the abscess
Removal of associated granulation tissue.

106. thanks