Isfahan university of medical sciences
M.Rogha M.D.
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classification
Systemic disease that can affect the ear
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1- Granulomatous and
infectious disease
a. Langerhans cell
histiocytosis (LCH)
b. Tuberculosis
c. Wegners granulomatosis
d. Sarcoidosis
e. Syphilis
f. Lyme disease
g. Mycotic disease
2- Neoplastic disease
a. Multiple myeloma
b. Leukemia
c. Metastatic neoplasm
d. Paraganglioma
3- Disease of bone
a. Pagets disease
b. Osteogenesis imperfecta
c. Fibrous dysplesia
d. Osteopetroses
e. Osteitis fibrosa cystica
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4- Storage and metabolic
disease
a. Mucopolysaccharidoses
b. Gout
c. Ochronosis
5- Collagen vascular and
autoimmune disease
6- Immunodeficiency disorders
a. Primary or congenital
i. Humoral immunodeficiency
disorders
ii. Cellular immunodeficiency
disorders
iii. Disorders of phagocyte
function
iv. Complement system
defects
b. Acquired
i. Acquired
immunodeficiency syndrome
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- Proliferation of cytologically benign histiocytes
 - Etiology and pathogenesis remain unknown
 a- unifocal eosinophilic granuloma
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- M>F, No systemic manifestation, prognosis is excellent
 - Dx: Local curettage + low dose irradiation, Follow-up with radiographic
skeletal survey
 b- Hand-Schuller-Christian disease
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- <5 y, Multifocal osteolytic lesions, With limited
Involvement of skin, lymph nodes & viscera
 - Systemic manifestations include fever, anorexia, recurrent URTI, anterior
cervical lymphadenopathy, otitis media, and hepatosplenomegaly
 - Dx: Low dose chemotherapy
 C- Letterer-Siwe disease
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- <3y, diffuse involvement of multiple organs, manifestations include fever,
Seborrheic or eczema-like rash. Oral lesions, lymphadenopathy,
hepatosplenomegaly,
Multiple bony lesions, diffuse replacement of marrow, and pulmonary
infiltration
 - Virulent, poor prognosis and high mortality ate
 - Dx: Corticosteroids and cytotoxic drugs
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OTOLOGIC MANIFESTATION
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- Mastoid is a common site of involvement
- Otic capsule & facial nerve are relatively resistant
- Otorrhea is the most common symotom followed by
postauricular swelling, HL, & vertigo
- The most common sign is granulation tissue or aural polyps
Perforation of the TM, otitis media, otitis external, a fistula
between the mastoid and the external canal
- Diagnosis of LCH is suggested by an inflammatory disorder of
middle ear and mastoid that does not respond to routine antibiotic
therapy, bilateral destructive ear disease, an elevated ESR in the
absence of acute infection, exuberant granulation tissue after
mastoid surgery, and associated skin and systemic lesions
- Radiographs show destructive lesions in the mastoid
- The diagnosis is established by biopsy
- A definitive diagnosis of LCH is made by immunostaining and
electron microscopic studies
two lytic lesions of the skull showing beveled edges (arrows) and
nonsclerotic margins, which are typical of histiocytosis X.
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TUBERCULOSIS
Tuberculosis otitis media 0.05% to 0.9% of all cases of COM
 Hematogenous or lymphatic or by extension through eustachian
tube
 TM becomes thickened, CHL due SOME
 No pain or tenderness, lymphanopathy in high jugular chain
 Multiple small perforation of the TM
 The middle ear mucosa appears to be hyperemic with polypoid
granulation
 Destruction of mastoid tip may result in Bezolds abscess
 Definitive diagnosis is made by histopathologic examination
Of tissue from the ME or mastoid showing a granulomatous
Process with multinucleated giant cells (langerhans cells) and
histologic demonstration of acid-fast organisms
D.D with wegener granulomatosis and distinguished on the basis of
skin test, cultures of the ME, ANCA
Dx: Systemic use of standard anti-TB chemotherapy however
mastoid surgery may be required to remove sequestrated bone 8
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The TM is intact, but greatly thickened by tuberculous granulation tissue containing
the typical epithelioid cells, round cells, and multinucleated giant cells
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WEGENER’S GRANULOMATOSIS
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A granulomatouse inflammatory process with necrotizing
vasculitis
Primarily affects the upper and lower respiratory tract and
kidneys but can involve any organ
M=F, mean age 40 y
Common presenting symptom: headache, sinusitis, rhinorrhea,
otitis media, fever, arthralgia
Upper airway and sinus involvement in 75% to 90%, pulmonary
manifestations(couph, pleuritic chest pain, hemoptysis and
nodular or cavitary infiltrates) in 65% to 85%
Glomerulonephritis in 60%-75%, eye involvement
(conjunctivitis, iritis, scleritis, proptosis) in 15%-50%,
dermatologic findings (necrotic ulceration, vesicles or petechiae)
Laboratory findings: normochromic, normocytic anemia,
thrombosytosis, positive RF, hyperglobulinemia particularly
IgA, elevated ESR
Positive ANCA test, especially proteinase 3, specificity>95%,
sensitivity>90% in active and systemic, in limited or inactive 11
65% to70%
CONTINUE
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The diagnose of WG is made histologically by the presence of
necrosis granulomatous inflammation with multinucleated giant
cells, vasculitis and microabscess formation
Etiology and pathogenesis unknown, but currently considered to
be an autoimmune disease that is perhaps the result of
stimulation by an infectious agent (or agents)
Prognosis of WG has dramatically improved from mortality rate
of 80% to the current remission rate of >75%
Dx. High doses of corticosteroids, cyclophosphamide or
methotrexate for 3 to 6 m followed by maintenance of remission
using lower doses of corticosteroids and less toxic
immunosuppresants such as azathioprime, methotrexate,
trimethoprime-sulfamethoxazole
Otologic manifestation: Middle ear and mastoid are the most
common sites within the temporal bone, SOM due to obstruction
of the eustachian tube, purulant OM, granulomatous involvement
of the ME & mastoid, facial nerve involvement and inner ear can
be involved
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Segmental vasculitis in Wegener granulomatosis with inflammation involving
a portion of the arterial wall
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SARCOIDOSIS
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Chronic multisystem disease of unknown etiology that’s
characterized by noncaseating granulomas
It most frequently affects lungs, F>M, and is 10 times more
common in blacks, third to fourth decade
Common presenting symptoms: Bilateral hilar adenopathy,
cough, granulomatous skin rash
Others: iridocyclitis, keratocojectivitis, peripheral
lymphadenopathy, hepatosplenomegaly, cardiac failure, myalgia,
and arthralgia
The facial & optic nerves are the most commonly affected cranial
nerves
Laboratory findings: Hilar adenopathy in CXR, hypercalcemia,
and elevated serum ACE
It has been suggested that the etiology is linked to genetically
determined enhancement of the T-helper immune response to a 14
limited number of microbial pathogens
CONTINUE
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Spontaneous resolution occurs in many patients
Corticosteroids are beneficial for those with progressive
symptoms or with ocular, cardiac or CNS involvement
Infliximab (inhibits release of TNF) has been reported as
being effective for some cases that are refractory to other
treatments
Otologic manifestation: SNHL, vestibular dysfunction and
facial nerve paralysis, or occasionally granulomatous
disease of the external or middle ear and mastoid
The facial nerve is the most commonly affected cranial
nerve, it is often bilateral, it may resolve spontaneously and
is usually involved as part of the triad of uveoparotid fever
(Heerfordts syndrome) parotitis, uveitis, facial nerve
paralysis, and mild pyrexia
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sarcoidosis
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Bilateral hilar adenopathy and
linear parenchymal densities in
pulmonary sarcoidosis.
SYPHILIS
• Both congenital and acquired syphilis may affect the middle ear in the late
latent and tertiary forms
• In late latent form the ME & mastoid affected by rarefying osteitis with
leukocytic infiltration
• In tertiary the gumma demonstrates obliterative arteritis and central
necrosis, A gumma of the ear canal or ME may result in perforation of TM
and a granulomatous appearance of mucosa
• Definitive diagnosis of syphilis requires a positive serologic test and a
histologic demonstration of Treponema pallidum
• Syphilis may mimic TB
• Heneberts sign (induction of ocular deviation with positive or negative
pressure in the external canal) probably due to fibrous adhesion between the
stapes footplate and the membranous labyrinth
• Dx: Combined antibiotic and corticosteroid
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active round cell
osteitis (O)
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Syphilitic gumma
LYME DISEASE
 Multisystem inflammatory disorder that affects skin, nervous
system, heart, joints
 Spirochete Borrelia burgdorferi
 Transmitted by Ixodes Ticks
 Primary reservoirs are white-footed mice and white-tailed deer
 Three clinical stages are recognized :
a- The first stage (early, localized infection) begins 3 to 33 days
after a tick bite (erythema migrans), this lesion occurs
in 60%-80% of patients and may accompanied by minor
constitutional symptoms
b- The second stage (early, disseminated infection) occurs within
days or weeks after inoculation, symptoms include fever,
migratory arthralgia, myalgia, headache, meningismus,
generalized lymphadenopathy, malaise, fatigue, and secondary
annular skin lesion
c-The third stage occurs more than a year after onset and can
result in chronic, prolonged arthritis, chronic encephalomyelitis,
chronic axonal peripheral polyradiculopathy, keratitis,
acrodermatitis chronic`atrophicans, localized scleroderma-like
lesions
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Erythema migrans
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acrodermatitis chronica atrophicans
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Continue
 Inflammatory innate immune responses are critical in the
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pathogenesis
Diagnosis is based on the recognition of the characteristic clinical
features, a history of exposure and detection of a specific
antibody to B. burgdorferi
Dx: The spirochete is highly sensitive to doxycycline, other
effective antibiotics include amoxicillin, erythromycin,
cefuroxime, ceftriaxone, imipenem. Steroids for carditis and
arthritis.
Vaccine is now available
Otologic manifestation: Facial nerve paralysis is the most
common Otologic manifestation (3% to 11%), bilateral in
(25%), in second stage, in all ages and both sexes, acute in onset,
return is spontaneous and complete, antibiotics or steroids do
not appear to influence the duration or outcome
Lymphocytoma a red and violet nodules occur on the earlobe
during the second stage
SNHL, sudden hearing loss, vertigo, meniere-like symptom have
been described
Skin lesion of lyme disease, ” lymphocytoma”
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MYCOTIC DISEASE
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Systemic invasive clinical disease reflects some defect in
host defense, such as DKA, chemotherapy, AIDS
Diagnose is made by biopsy and culture
Treatment consists of control of the underlying
predisposing condition, surgical debridement of necrotic
tissue and Amphotercin- B
Otologic manifestation: destruction of the middle ear cleft
ensues, often with extention to the surrounding
structures , including thrombosis or rupture of the
internal carotid artery
Other routes: hematogenous embolic dissemination
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Multiple myeloma: malignancy of plasmacells derived from B
lymphocytes, M>F, 60 y
Severe bone pain, pathologic fractures, renal failure, failure of the
bone marrow, hypercalcemia and recurrent infections.
Laboratory findings: M component on serum or urine electrophoresis
normochromic, normocytic anemia, hypercalcemia and elevated
BUN.
Otologic manifestation: Lytic lesions of the temporal bone and otic
capsule,
Symptoms are usually overshadowed by manifestation of diffuse
disease
Tx: Autologous stemcell transplantation, thalidomide,
bisphosphonate and erythropoietin
Extramedullary plasmacytoma (soft tissue) and solitary bone
plasmacytoma (bone): in younger individual, M component in 30%,
indolent course, survival rates of 10y or more
Dx: local radiotherapy (4000 cGY),
Periodic evaluation should be performed to detect conversion to
multiple myeloma
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large lytic destructive lesion (arrows) of the clivus (CL), petrous temporal bone,
middle ear, and jugular foramen area caused by multiple myeloma
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Coronal CT scan with contrast enhancement and a soft tissue technique shows a slightly
enhancing mass that has destroyed the mastoid bone and extends to the posterior fossa
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(PF) and upper neck (UN).
LEUKEMIA
• Common in the submucosa of the pneumatized areas of middle ear and
mastoid and bone marrow of the petrous apex
• Secondary bacterial infection due to immunocompromised state or
chemotherapy, hemorrage in ME, mastoid or inner ear
• Clinical manifestation: ME effusion, acute and chronic suppuration in
the ME and mastoid, thickening of the TM, CHL, SNHL, vertigo,
facial paralysis, skin lesions in the external auditory canal
• Granolocytic sarcoma or chloroma: exteramedullary tumore in AML or
CML
• Management is by local irradiation and chemotherapy
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PARAGANGLIOMA
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Is the most common neoplasm after the acoustic neuroma
Divided into two groups: the glomus tympanicum and glomus jugulare
Symptom:
The glomus tympanicum appears with pulsatile tinnitus and a CHL
The glomus jugulare appears late, after considerable growth and bony
destruction, may cause a neurologic defect in CNs IX to XII, facial nerve
paresis caused by tumor extension into the mastoid, or SNHL caused by
bony erosion of the labyrrinth
Both may erode the TM and presenting by bleeding mass
10% of nonfamilial and 50% of familial have at least one additional
Lesion
A few PG, both benign and malignant may secrete catecholamines
A history of headache, hypertension and flushing
Dx: surgery, radiotherapy is useful for management of recurrences and
unresectable lesions
 Hematogenous dissemination
 The most common sites:
 Breast, lung, prostate, skin
 Petrous apex and internal auditory canal
 The otic capsule relatively resistant
 CHL, pain, SNHL, vertigo, facial paralysis
 In meningial carcinomatosis unilateral or bilateral SNHL is a
common presenting symptom, diagnosis is made by cytology
of the CSF
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metastatic breast adenocarcinoma showing a large lytic lesion
(arrows) destroying the mastoid.
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Diseases of the bone
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PAGETS DISEASE (osteitis deformans)
 Osteolytic and osteoblastic changes affect the axial skeleton
 AD, 3% of the population, 40y old and older, M>F
 Enlarging skull, progressive kyphosis, deformities of the pelvis, femur, tibia
 Etiology is uncertain, slow virus infection have suggested
 Tx: bisphosphonate, calcitonin, mithramycin, ipriflavone, gallium nitrate
 Otologic manifestation: HL, tinnitus, mild vestibular dysfunction,
facial nerve is spared
 HL 5% to 44%, SN, mixed or rarely conductive
 D.D: otosclerosis, paget is late in onset, old age, greater SNHL, enlarged
calvaria, enlargement of the superficial temporal artery, elevated serum ALP
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Paget's disease. There is diffuse expansion of the skull table and
involvement of both temporal bones, with patchy demineralization.
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Lateral skull radiograph in a patient with Paget's disease. Findings include thickening
of the skull table, multiple patchy densities, and platybasia.
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The pagetic bone encroaches on the posterior margin (arrow) of the internal
auditory canal (IAC). The mastoid is largely replaced by pagetic bone
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OSTEOGENESIS IMPERFECTA
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Type I through IV
Type I: AD, mildest form, blue sclera, nondeforming fructures, normal
stature, HL in 30%-50%
Type II: most severe, multiple fracture in uterus, stillbirth, AR or sporadic
Type III: multiple fracture, bone deformity, HL in 50%
Type IV: AD, similar to type I except that the sclera are white, HL in 10%30%
Tx: management of fractures, orthopedic surgery, bisphosphonate
Otologic manifestation: SNHL in 40%, high correlation with gray or white
sclera, CHL accompanies blue sclera, CHL reflects structural change in the
ossicles, microfractures of the manuberium, fragility of the long process of
the incus, fracture or resorption of the crura of the stapes
Rehabilitation by amplification or surgery
Stapedectomy can give similar results to otosclerosis
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Osteogenesis imperfecta
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FIBROUS DYSPLASIA
• Benign, chronic, slowly progressive, unknown etiology
• Replacement of normal bone with fibrous tissue and woven bone
• 7% as part of Albrights syndrome (bony lesions, abnormal
pigmentation, endocrine dysfunction, precocious puberty in
women)
• 70% monostotic form: most common, skull, ribs, femur, tibia, may
become quiescent at puberty
• 23% polystotic form: skull lesions in more than 50%, can continue
to progress
• Clinical manifestation: bony deformity, pathologic fracture, cranial
nerve palsy
• Normal serum calcitonin and phosphorus levels, elevated serum
ALP in polystotic form
• Radiographic finding: radiolucent area, ground-glass appearance
• Otologic manifestation: progressive narrowing of the external
auditory canal with CHL is the most common (80%), facial nerve
paralysis, SNHL, vertigo
• Management is symptomatic, radiotherapy is contraindicated
Lateral radiograph of the skull of a patient with fibrous dysplasia showing lytic (L)
and fibrous (F) phases of disease. Spicules of new bone are responsible for the41
ground-glass appearance of the fibrous phase.
Coronal tomographic radiograph of a patient with fibrous dysplasia. New bone
formation causes a dense appearance of the involved left temporal bone.
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OSTEOPETROSES
 Rare genetic disorder, greatly increased bone density,
 Defective function of osteoclasts,
 Malignant osteopetrosis ; AR, high mortality rate, anemia,
thrombocytopenia, hepatosplenomegaly, susceptibility to infection,
encroachment of the neural foramina, optic atrophy, facial paralysis,
SNHL, hydrocephalus, MR, and death
 Otologic manifestation: mastoid is non pneumatized, inner ear
normal, herniation of facial nerve a consistent finding, recurrent
episodes of AOM, SOM, CHL, SNHL, unilateral or bilateral facial
nerve paralysis
 Tx: symptomatic, decompression of the facial nerve
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Von Recklinghausens disease
Excess parathormone
Osteoclastic bone resorption, marrow fibrosis, bone
cysts, bone pain, and fractures
In most cases is caused by hyperparathyroidism
due to an adenoma
Involvement of temporal bone is very rare, the otic
capsule is replaced by abnormal bone, SNHL has
been attributed to osteitis fibrosa
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MUCOPOLYSACCHARIDOSES
 MPS an inherited deficiency of one of several lysosomal enzymes that
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degrade MPS
Classified into seven types, all are AR except for Hunters syn (MPS II)
which is X-linked recessive
Management is supportive and symptomatic
MPS III: Hurlers syn, accumulation of heparan sulfate, corneal clouding,
abnormal facies, hepatosplenomegaly, MR, joint stiffness, and hernias
MPS II: Hunters syn, accumulation of heparan sulfate and dermatan
sulfate, similar to hurler, but corneal clouding is not seen
MPS IV: Moquios syn, spondyloepiphyseal dysplasia
Otologic manifestations: CHL( Eustachian tube dysfunction & thickening
of the mucosa ), SNHL ( may be a result of abnormal metabolism of the
inner ear).
MUCOPOLYSACCHARIDOSIS
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GOUT
 Deposition of crystals of monosodium urate within joint
space & cutaneous structures
 Serum urate level>7mg/dl, risk factors include: alcohol use,
exposure to loop diuretics, hypertension & renal
insufficiency
 Clinical manifestation: acute gouty arthritis, tophi, urate
urolithiasis, and gouty nephropathy
 Otologic manifestation: Tophaceous deposits in the helical
rim of the pinna, asymptomatic
 Treatment: bed rest, analgesics, colchicine, probencid,
allopurinol
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Tophus
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Ochronosis
 Ochronosis is a rare disease that is caused by an inherited lack of
the enzyme homogentisic acid oxidase.
 The presence of homogentisic acid in urine is called
alkaptonuria. The result of this inborn error of metabolism is the
deposition of a dark pigment in tissues that are rich in collagen.
 Patients often present with symptoms and signs during the third
decade of life.
 Manifestations include ochronotic arthropathy, ocular and
cutaneous pigmentation, obstruction of the genitourinary tract by
ochronotic calculi, and cardiovascular manifestations as a result of
ochronosis affecting the aortic valve.
 Ochronosis has manifestations in the external ear; cartilage is a
site of predilection for the deposition of the pigment of
ochronosis. Blue or mottled-brown macules can appear on the
pinna and in other areas of the head and neck, including the
nose, buccal mucosa, tonsils, pharynx, larynx, and esophagus.
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Ochronosis
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Collagenvascular
&
Auto immune diseases
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Relapsing polychondritis
 Episodic & progressive autoimmune inflammatory dis, F>M, auricles &
nasal septom are most common & first sites. nonerosive & nondeforming
arthritis of hands & knees. Other cartilaginous sites…, eyes, aorta, heart &
skin.
 Sudden, painful, tender uniform reddish swallowing of auricle, lobule
remains NL in color. CHL( as a result of Eustachian tube involvement ),
SNHL & vestibular symptom( because of endorgan vascular etiology ).
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 DDx: erysipelas( irregular margin of erithema extend to priauricular skin ),
chondritis & prichondritis( don’t uniformly involve the entire auricle, often
fluctuance, not spare the lobule ).
 Dx is clinical, non specific lab findings ( ↑ ESR, no ↑ WBC), Bx is
unnecessary.
 Corton & immunosupresive for sever ,progresive & lethal cases. In less
sever cases Dapsone , NSAID, Colchicine, Salisylates.
Relapsing poly chondritis
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Immunodeficiency disorders
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Immunodeficiency disorders
Primary:
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Humoral: recurrent & chronic RTI with extracellular bacteria.
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Cellular: dysfunction of T lymp, recurrent infection with
intracellular opportunistic ( viruses, fungi, protozoa & some
bacteria ).
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Dis of phagocyte: pyogenic bacteria & fungi.
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Complement sys:C5, C6, C7, C9( Neisseria ), C3b inactivator ↓
( staphylococcus) , C1, C2, C4( lupus like syn ), C1 esterase
inhibitor ↓( angioedema ).
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Otologic manifestations: recurrent AOM, SOM, refractory COM.
anomalies of external, middle & inner ear with CHL, SNHL or mixed
HL and high incidence of Mondini’s dysplasia, in DiGeorge’s syn.
Cont.
Acquired
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Otologic manifestations are rare, except in children( SOM ),
microbiology is similar to non AIDS population with addition of
unusual opportunistic organism. Severity depends on immune
status, Bx or tympanocentesis is indicated before Tx.
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Pneumocystis carinii is common cause of middle & external ear,
may be the initial presenting sym of AIDS, Tx is cotri.
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SNHL( otosyphilis, cryptococcal, tuberculous, toxoplasmosis
meningitis ), vertigo, tinnitus, facial paralysis by Herpes zoster.
AIDS
• The virus is lymphotropic & attacks T helper lymphocytes
• Otologic manifestation: infrequent except in children, SOM is
common, microbiology is similar to non AIDS
• AOM, acute mastoiditis, SOM, and bullous myringitis
• Otitis externa, Kaposi sarcoma
• Pneumocystis carinii is common cause of middle ear & external ear
disease, subcutaneous masses, aural polyps, CHL, otorrhea,
otalgia,
• Dx: co-trimoxazol
• SNHL, vertigo, tinnitus, facial nerve palsy
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