Diseases of the Temporal BoneINCLUDING GRANULOMAS (SYSTEMIC BONE DISEASE, INFECTIVE AND GRANULOMATOUS DISEASE, AUTOIMMUNE INNER EAR DISEASE)

1. Diseases of the Temporal Bone
INCLUDING GRANULOMAS (SYSTEMIC BONE DISEASE, INFECTIVE
AND GRANULOMATOUS DISEASE, AUTOIMMUNE INNER EAR DISEASE)
DR. RAJIV MAHASETH
MS ORL- HNS, 1ST
Year Resident
GMSM Academy of ENT – Head & Neck Studies
MMC-TUTH, IOM

2. Road map
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
•Systemic bone diseases
•Infective and granulomatous disease
•Autoimmune inner ear disease

3. Systemic bone diseaseSystemic bone disease
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
 Paget’s disease
 Osteogenesis imperfecta
 Fibrous dysplasia
 Osteopetrosis
Neurofibromatosis
Craniofacial dysostosis
Osteopathia striata

4. Paget’s Disease
1876 - Sir James Paget - Osteitis deformans
1888 - Sir Jonathan Hutchinson referred osteitis
deformans as Paget's disease of bone
Progressive,focal disorder of bone remodelling
▪ Osteoclastic - Bone resorption
▪ Osteoblastic - Bone deposition
Clinical Presentation
▪ Bone pain, stiffness and fatiguability
▪ Enlargement of skull, pelvis, tibia and femur
www. Google.com/image
Disease of temporal bone - Dr Rajeev Mahaseth 2017
(Scott & Brown 7th
edition)

5. 2nd
most common bone disease after
osteoporosis
Affects -3 % of population > 40 yrs
- 11 % > 80 yrs
Male : Female = 3:2
Common : Britain, Australia, New
Zealand,
North America
Uncommon: Asia (Non-white race)
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Paget’s disease contd.
(www.google.com/image)

6. Etiology :
 Unknown
 Inherited as autosomal dominant
 Slow viral infection
 Polyclonal antibodies reveal paramyxovirus antigens
in osteoclasts
 Monoclonal antibodies - Measles, Human
Parainfluenza Virus
 Electron microscopic study
- Fingerprint pattern of osteoclast
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Paget’s disease contd.
(Cummings 6th
edition)

7. Paget’s disease contd.
Multinucleated osteoclast
Courtesy of Pierre Delmas, MD.
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Bone affected
Pelvis > Femur >Skull > Tibia >Vertebrae
> Clavicle > Humerus
Phases:
Osteolytic - Mixed - Osteoblastic
- Lamellar remodeling (Temporal
Bone)
Bossing of temporal bone
Histology
Periosteum - Enchondral - Endosteal
layer

8. Clinical Feature
 Bilateral progressive hearing loss: 13-40%
 Sensorineural , mixed or conductive
 Bone pain - constant deep boring
 Joint stiffness and fatiguability
 Vestibular symptoms - 20- 30%
- May present with vertigo
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Paget’s disease contd.
(Cummings 6th
edition)

9. Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Courtesy of Pierre Delmas, MD
Skull
enlargement
Dilated
scalp
veins
Paget’s disease contd.

10. Diagnosis :
Incidental finding in x - ray
Early stage: Osteolytic lesion
Advanced stage:
Bone density
Abnormal architecture
Overgrowth
 Microfractures
 CT Scan:
Temporal bone - washed out appearance of
petrous apex
Obliteration of otic capsule , narrowing of IAC
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott & Brown 7th
edition)
Paget’s disease contd.

11. Courtesy of Pierre Delmas, MD.
Disease of temporal Bone - Dr Rajeev Mahaseth
2017
Early Stage
(Osteoporosis
Circumscripta)
Lytic
border
Diffuse
sclerotic
changes
Advanced Stage
(Cotton wool skull)
Paget’s disease contd.

12. Laboratory tests
 Marker of new bone formation
▪ Alkaline phosphatase
 Elevated markers of bone resorption:
▪ Urinary hydroxyproline/creatinine
▪ Urinary and serum deoxypyridinoline
▪ C - telopeptide
▪ N - telopeptide )
 Bone scan to assess extent of
disease
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott & Brown 7th
edition)
Paget’s disease contd.
www. google.com/image

13.  Pain from site of known Paget`s disease
 Early, potentially deforming disease
 Osteolytic lesions in weight bearing bones
 Skull disease
 Complications:
a) Progressive neurological syndrome
b) Fissure fractures
c) Immobilization hypercalcaemia
d) High-out put cardiac failure
(Scott & Brown 7th
edition)
Indications forTreatment
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

14. Indications
Age <55yrs
Serum alkaline phosphate/urine hydroxyproline > 2
times
 6 months prior to joint replacement surgery
Treatment
 Physiotherapy
 Antiresorptive therapy (Bisphosphonates )
- Tab Alendronate 40mg/day PO OD for 6 mths
 Calcitonin was previously used, alternative
therapy
 Analgesics
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott & Brown 7th
edition)
Paget’s disease contd.

15. Surgery :
Fractures
Bone deformities
Osteoarthritis
Complication
Development of osteosarcoma : < 0.1 %
Cranial nerve involvement
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott & Brown 7th
edition)
Paget’s disease contd.

16. Hereditary disorder of collagen synthesis
Incidence : 2 - 15/100000 births
Type 1
 AD,Mild
 Blue sclera, non deforming fracture
 Normal stature, hearing loss
Type 2
 Severe form, multiple fractures in utero
 Still birth
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/ima
ge)
(Cummings 6th
edition)
OSTEOGENESIS IMPERFECTA

17. Type 3
 Multiple fracture, progressive bone deformity
 Hearing loss
Type 4
Similar to type 1 but there is white sclera
 95% spontaneous fracture
 50-60% hearing loss
 Hypermobility/laxity of joints
 Thin skin/subcutaneous bruising
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Cummings 6th
edition)
Osteogenesis imperfecta contd.

18. Clinical Feature
▪ 95% spontaneous fractures
▪ 85% blue sclera
▪ 50-60% hearing loss
▪ Hypermobility/laxity of joints
▪ Thin skin/subcutaneous bruising
CHL accompanies blue sclera appear in
- 20-25 yrs of age
SNHL seen in 40 % of patients - grey/white
sclera
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Cummings 6th
edition)
(www.google.com/ima
ge)
Osteogenesis imperfecta contd.

19. Investigations
 Level of FGF 23 is raised
 PCR - mutations of the gnas gene
 Low serum phosphate
Radiology:
 Enlarged temporal bone
 Sclerosis(23%)
 Ground glass appearance (56%)
 Cystic lesion(21%)
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/ima
ge)
Osteogenesis imperfecta contd.

20.  History of fracture after minor trauma
 Positive family history
 Blue sclerae
 Early onset of hearing loss
Stapes footplate - Thick, soft, chalk-like/granular,
fixed
Stapedectomy - Similar result otosclerosis
Treatment
- Biphosphonate
Disease of temporal Bone - Dr Rajeev Mahaseth
2017
(Cummings 6th
edition)
D/Dwith
otosclerosis

21. FIBROUS DYSPLASIA
▪ 1900 - Von Recklingshausen - Ostitis Fibrosa
▪ Genetic noninherited disease
▪ Somatic activating missense mutations
of GNAS1
▪ Age – 2 – 5th
decade
▪ Tumour like lesion - Replacement of normal
bone with cellular connective tissue
Types:
 Monostotic : Skull base in 15 % ,maxilla .
mandible
 Polyostotic : More than one bone lower
limbs,skull Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott Brown 6th
edition)
(www.google.com/image)

22. McCune-Albright syndrome
Polyostotic fibrous dysplasia
Skin hyperpigmentation
Endocrine dysfunction
Presentation
Skull base involved in 15 % cases
1/3rd cases are located in maxilla or
mandible
Palpable visible swelling in 75 % cases
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Fibrous dysplasia contd.

23. Fibrous dysplasia contd.
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/image)

24. Presentation
 Pain
 Progressive hearing impairment - 57%
 Localized temporal bone swelling - 51%
 Bony occlusion of EAC - 42%
 SNHL (erosion of otic capsule) ,pain,
vertigo
 VII Nerve paralysis
 Decreased vision – compression of optic
nerve
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Fibrous dysplasia contd.

25. Differential Diagnosis
Ossifying fibroma
Histiocytosis X
Paget’s disease
Aneurysmal bone cyst
Giant cell tumour
Brown tumour of hyperthyroidism
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Fibrous dysplasia contd.

26. Treatment
No known conservative treatment
Surgery
 Curettage
 External auditory meatus stenosis
 Decompression of optic nerve
Radiotherapy :
 Predispose to malignant degeneration
(Scott Brown 6th
edition)
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Fibrous dysplasia
contd.

27. OSTEOPETROSIS
 Rare genetic disorder
 Defective osteoclast
- Failure of normal bone resorption
 Normal bone formation by osteoblast
 Thick, dense, brittle bones
Malignant
 AR, rapidly progressive, Infancy
 Encroachment of bone marrow - Anemia,
thrombocytopenia, susceptibility to infection,
hepatospleenomegaly
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/imag
e)
(Cummings 6th
edition)

28. Osteopetrosis contd.
 Optic atrophy, facial paralysis, SNHL,
hydrocephalus, mental retardation
AD type1
 Rare disorder , asymptomatic
 Some have pain and hearing loss
 No fracture
AD type 2 (Marble bone disease)
 Frequent type with normal life expectancy
 May be asymptomatic
 Increased fracture of bones
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

29. Otological manifestation
 Dense calcified cartilage
 Non-pneumatized mastoid
 Fetal form stapes
 Normal inner ear
 Dehiscence of tympanic segment of VII Nerve
 Facial nerve paralysis : -
Unilateral/Bilateral
 Recurrent AOM, CHL, SNHL
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Osteopetrosis contd.
(Cummings 6th
edition)

30. Radiological:
 Increase density all bones
 Increase serum acid phosphatase
Treatment
 Not definitive
 Steroid
 Interferon
 BMT
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Osteopetrosis contd.
(Cummings 6th
edition)

31. Infective and granulomatous Disease
 Tuberculosis
 Syphilis
 Sarcoidosis
 Histiocytosis X
 Wegener’s
granulomatosis
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

32. TUBERCULOSIS
 Chronic granulomatous infection
 Age: 1 - 5 yrs
 Incidence : 0.05 – 0.9 % of COM cases
 Etiology: M. tuberculosis, M.Bovis, M.avium,
M.fortuitum
 Risk factors: Active pulmonary tuberculosis, HIV
infection
 Mode of transmission:
 Hematogenous/Lymphatic
 Eustachian tube
 Intracranial, extracranial infection
 Maternal systemic tuberculosisDisease of temporal Bone - Dr Rajeev Mahaseth 2017
(Cummings 6th
edition)

33. Diagnosis
Chronic otorrhoea resistant to usual antibiotic
treatment, surgery
Non tender high jugular chain lymphadenopathy, cold
abscess
Otoscopic examination
 TM thickened ,landmark obliterated
 Multiple perforation in TM with seropurulent
discharge
 Middle ear mucosa - Pale
Conductive hearing loss
 Effusion in middle ear & ossicles destructionDisease of temporal Bone - Dr Rajeev Mahaseth 2017
(Cummings 6th
edition)
Tuberculosis contd.

34.  Otic capsule – Loss of auditory and vestibular
function
 Histopathology
 Granuloma with Langerhans cells – 1/3rd
case
 Acid fast organism
 Culture – Gold standard
 Skin test , biopsy & PCR - TB
 CT Scan – Soft tissue in middle ear,
intracranial/extracranial foci
 ANCA (Antineutrophil cytoplasmic antibody) - WG
Tuberculosis contd.
(Cummings 6th
edition)
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

35. Management
 Antituberculous therapy - Dry ear in 1-3 months
 Ear surgery minimal role
Complications
 Profound SNHL
 Facial paralysis
 Petrous apicitis
 Labyrinthitis, meningitis
 Post auricular abscesses
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott Brown 7th
edition)
Tuberculosis contd.

36. SYPHILIS
 Congenital and acquired syphilis affect middle ear
 Less frequently seen in clinical practice
 Male : Female – 4:1
 Acquired form 25 times more frequent than congenital
 SNHL (sudden/fluctuating ) tinnitus & vertigo
 Systemic feature
- Cardiac and aortic involvement
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott Brown 6th
edition)

37. Syphilis contd.
Early syphilis:
 Congenital bilateral hearing loss – 50 %
 Secondary syphilis
- Transient vestibular symptoms (Positional
with tinnitus)
- Ocular palsy ,facial paralysis
 SNHL – High frequency involved
 Speech descrimination worse
 Caloric response reduced
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

38. Late syphilis: 10 - 50 yrs of primary infection
Otological features :
 SNHL – B/L in congenital , U/L in acquired
 Onset of aural symptoms :
- Sudden, fluctuant , episodic vertigo
 Severe vestibular damage
- Imbalance, ataxia
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott Brown 6th
edition)
Syphilis contd.

39. Hennebert’s sign
Ocular deviation with positive or negative pressure in
external auditory canal
 Fistula between middle and inner ear due to rarefying
osteitis
Tullio’s sign
 Transient vertigo and nystagmus due to exposue to loud
sound
Diagnosis:
 VDRL, TPHA, TPI, FTA-ABS
 CSF examination - Raised globulin, IgG level &
lymphocytosisDisease of temporal Bone - Dr Rajeev Mahaseth 2017
Syphilis contd.

40. Treatment:
 Penicillin - 600000units I/M OD x 21 days
Probenecid - 500mg x 6hrly
 Ampicillin
 - 1.5 gm x 6hrly x 4wks.
 Prednisolone
- 30mg x 8hrly x 4wks (Jarish –Herxeimer rxn)
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Syphilis contd.

41. Sarcoidosis
 Rare systemic granulomatous disease of unknown
etiology
 Non-caseating granuloma
 Female predominance
 Onset - 3rd
- 4th
decade
 Bilateral hilar lymphadenopathy, cough and
granulomatous skin rash
 Organs: Parotid gland, facial nerve, nasal cavity and
larynx
 Uveitis 80 %, lymphadenopathy 55 %, parotid swelling
20 %,
facial nerve palsy 43%
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott Brown 6th
edition)

42. Otological Feature
 SNHL – sudden fluctuating or progressive
 vestibular dysfunction
 Granulomatous disease external , middle ear &
mastoid
 PTA - Low or high frequency hearing loss
Heerfordt syndrome
 Parotitis, uveitis, facial nerve paralysis and
pyrexia
Histopathology
 Perivascular lymphocytic infiltration and
granulomatous
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott Brown 6th
edition)
Sarcoidosis contd.

43. Laboratory Tests
 Serum ACE ( Angiotensin converting enzyme)
elevated
 Hypercalcemia & hypercalciuria
 Raised ESR
Treatment:
 Steroids
 Methotrexate, Cyclophosphamide
 Hydroxychloroquine
 Infliximab
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Sarcoidosis contd.

44. Histiocytosis X
 Proliferation of cytologically benign histiocytes,
lymphocytes, eosinophlis in the affected tissue
 Immunologic dysfunction
 Organs: Reticuloendothelial system
3 types:
 Eosinophilic granuloma
 Hand-Schuller-Christian disease
 Letterer-Siwe disease
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

45. Eosinophilic granuloma
 Children , young adults
 Male : Female - 2 : 1
 Osteolytic lesion - Femur , pelvis , scapula, vertebrae
,ribs, mandible
 Maxilla of skull
Clinical features:
 Asymptomatic
 Local Pain
 Local swelling
 Pathologic fracture
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Histiocytosis x contd.

46. Histopathology :
• Sheets of histiocytes
• Scattered eosinophils
• Plasma cells
• Areas of haemorrhage
• Necrosis with giant cell
Radiological appearance
• Punched out / lytic appearance
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
www.google.com/image
Histiocytosis x contd.

47. Hand-Schuller Christian disease
Multiple focal lesions
Early onset (<5yrs)
Common site – Skull bone
Destruction of temporal bone
- Secondary infection & otorrhoea
Systemic manifestation
 Fever / Recurrent URTI
 Otitis media
 Cervical Lymphadenopathy,
hepatosplenomegaly
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
www.google.com/image
Histiocytosis x contd.

48. Letterer- Siwe Disease
 Disseminated disease
 Early onset (< 3 yrs)
 Multiple organ involvement (Skin,
Lymph node, viscera)
Systemic manifestation:
 Fever
 Eczema like rash ,oral lesions
 Lymphadenopathy
 Multiple bony lesion
 Respiratory failure- pulmonary
infiltration
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Cummings 6th
Edition)
Histiocytosis x contd.
(www.google.com/image)

49. Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Letterer- Siwe Disease contd.
Otological manifestation (15-61%)
 Otorrhoea
 Post auricular swelling, hearing loss, vertigo
 Granulation /Polyp in EAC
Diagnosis
 Inflammatory disorder not responding to antibiotic
 Bilateral destructive ear disease
 High ESR
 Immunohistochemistry
Treatment
 Aural polypectomy +/- steroid
 Low dose chemotherapy
Histiocytosis x contd.

50. Autoimmune inner ear
disease
1. Vasculitis
▪ Wegener
granulomatosis
▪ Polyarteritis nodosa
▪ Temporal arteritis
▪ Cogan syndrome
▪ Behcet’s disease
2. Relapsing
polychondritis
3. Systemic lupus
erythmatosis
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
4. Immunodeficiency
disease
 T cell deficiency
 B cell deficiency
 Disorder of phagocytosis
 Complement system
disorder

51. Wegener’s Granulomatosis
Friedrich Wegener – German pathologist
Also called as Granulomatosis with polyangiitis
Age : 15 - 73 yrs
Necrotizing vasculitis small to medium sized vessels
Triad : Airway, lung & renal disease
Clinical features:
 Epistaxis, nasal obstruction, crusting
 Saddle nose deformity (perforated septum)
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott- Brown 7th
edition)
(www.google.com/ima
ge)

52. Wegener’s Granulomatosis
contd.
Otological features:
 Ear involved in 15 - 35%
 Seromucinous otitis media, frank otorrhoea
 Necrotizing granulation tissue - Tympanic
cavity
 TM - Multiple perforations (granulation)
 Rapidly progressive SNHL
 Loss of vestibular function
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott- Brown 7th
edition)

53. Wegener’s Granulomatosis contd.
Diagnosis:
 C-ANCA - Positive in 95% patient
 Diagnosis & activity of disease
 Anaemia, thrombocytosis, hyperglobulinemia [IgA]
 Raised ESR & CRP level
Treatments:
 Steroids - Prednisolone 60-80 mg/day
 Cyclophosphamide 2mg/kg, azathioprine
200mg/day
 Complete remission - 90%
 Duration : 3 – 6 months
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott-Brown 6th
edition)

54. Polyarteritis nodosa
 Systemic necrotizing vasculitis of small and medium
sized arteries
 Male : Female - 3:1
 Renal, coronary, hepatic and visceral circulation
involved
 Otological feature:
 SNHL - Sudden, bilateral symmetrical
 CHL - Middle ear granulation
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Scott-Brown 6th
edition)

55. Polyarteritis nodosa contd.
Lab tests :
 Anaemia, raised ESR
 Leukocytosis
Treatment
 Systemic steroid
 Immunosuppressive drug - Chlorambucil
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

56. Temporal arteritis
Older age group
Vasculitis of extracranial arteries
Aneurysm
formation Stenosis Occlusion
Features:
 Pyrexia
 Bitemporal headache
 Tender palpable thickening of
temporal arteries
 Blindness: 30% (Ophthalmic artery)Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/image)

57. Temporal arteritis contd.
Otological features:
 Hearing loss (rapid, progressive,
vertigo)
Lab.tests:
 ESR
 Serum globulin raised
 Biopsy
Ultrasound/ Doppler help to identify
involved area
Treatments:
 Systemic steroidsDisease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/image)

58. Cogan Syndrome
 Dr. David Cogan in 1945
 Rare, autoimmune, young adults
 Non syphilitic interstitial keratitis
 Fluctuant, aggressive cochleovestibular
damage
Acute episode
- Nausea, vomiting, tinnitus followed by
SNHL
Otological features:
- B/L SNHL (Progressive , fluctuating
,episodic )
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/image)
(Ballenger’s 17th
edition)

59. Cogan`s Syndrome contd.
Ocular symptoms:
▪ Excessive lacrimation,
▪ Photophobia
▪ Blurred vision
Treatment:
▪ Steroid
▪ Immunosuppresive drugs
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/image)

60. Behcet’s disease
Prof. Hulusi Behcet named in 1973
Uncommmon
Chronic relapsing inflammatory
disorder
 Recurrent mouth ulcers (89%)
 Genital ulceration
 Inflammed eyes
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/image)

61. Behcet’s disease contd.
Otological:
 Slow progressive, bilateral SNHL
 Vestibular symtoms
Treatment:
 Immunosuppresive
 Steroids
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

62. Relapsing polychondritis
Rare disease of episodic inflammation
Degeneration of multiple cartilagenous structure
Pearson coined the term in 1960
Pathology
 Cartilage and proteoglycan rich structures
(eyes and cardiovascular system)
 Neutrophil infilteration
 Loss of basophilia of cartilage
Etiology:
 Autoimmune
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(www.google.com/image)

63. Disease of temporal Bone - Dr Rajeev Mahaseth 2017
Relapsing polychondritis contd.

64. Relapsing polychondritis contd.
Disease of temporal Bone - Dr Rajeev Mahaseth 2017
(Wallace et al.)

65. Clinical presentation
Otological
 Pinna involved in 89 %
 Eustachian tube - Otitis media with effusion
 Inner ear - Hearing loss and dysequilibrium
Nasal chondritis:
 Pain, feeling of fullness , sudden painless saddle
deformity
Larynx and trachea
 Hoarseness , tenderness, airway obstruction
Occular inflammationDisease of temporal Bone - Dr Rajeev Mahaseth 2017
Relapsing polychondritis contd.

66. Outcomes
Follows a course of acute exacerbation and
remission
Treatments
I. Medical
 Steroids – Prednisolone 30-60mg/daily
 Immunosupressive
( Azathioprine,Methotrexate)
 Anti-CD4monoclonal antibody,
 Minocycline.
II. Surgical
 TracheostomyDisease of temporal Bone - Dr Rajeev Mahaseth 2017
Relapsing polychondritis contd.

67. Systemic lupus erythematosus
Multisystem connective tissue disorder
Etiology:
 Autoimmune
 Immune complex deposition
Otological features
Bilateral SSNHL - 8 %
 Loss of spiral ganglion cell and hair cell and
atrophy of the
stria vascularis
 Circulating immune complex sludge in stria
vascularis
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

68. Systemic lupus erythematosus
contd.
Lab Tests:
 Antinuclear antibody
 Anti dsDNA
 Anaemia
 Raised ESR
Treatment:
Steroids - Prednisolone 40 mg daily for 2 wks
 Plasma exchange at 6 months interval
Disease of temporal Bone - Dr Rajeev Mahaseth 2017

69. References
1. Scott-Brown 6th
& 7th
edition
2. Cumming’s otorhinolaryngology, 6th
edition
3. Ballenger’s otorhinolaryngology 17th
edition
4. https://scholar.google.com
5. Current Diagnosis and treatment 3rd
edition
6. Glasscock and sambough surgery of ear 6th
edition
Disease of temporal Bone - Dr Rajeev Mahaseth 2017