3 Systemic disease that can affect the ear 1- Granulomatous and infectious diseasea. Langerhans cell histiocytosis (LCH)b. Tuberculosisc. Wegners granulomatosisd. Sarcoidosise. Syphilisf. Lyme diseaseg. Mycotic disease2- Neoplastic diseasea. Multiple myelomab. Leukemiac. Metastatic neoplasmd. Paraganglioma3- Disease of bonea. Pagets diseaseb. Osteogenesis imperfectac. Fibrous dysplesiad. Osteopetrosese. Osteitis fibrosa cystica4- Storage and metabolic diseasea. Mucopolysaccharidosesb. Goutc. Ochronosis5- Collagen vascular and autoimmune disease6- Immunodeficiency disordersa. Primary or congenitali. Humoral immunodeficiency disordersii. Cellular immunodeficiency disordersiii. Disorders of phagocyte functioniv. Complement system defectsb. Acquiredi. Acquired immunodeficiency syndrome
4 Langerhans cell histiocytosis (LCH) Histiositosis X - Proliferation of cytologically benign histiocytes- Etiology and pathogenesis remain unknowna- unifocal eosinophilic granuloma- M>F, No systemic manifestation, prognosis is excellent- Dx: Local curettage + low dose irradiation, Follow-up with radiographic skeletal surveyb- Hand-Schuller-Christian disease- <5 y, Multifocal osteolytic lesions, With limitedInvolvement of skin, lymph nodes & viscera- Systemic manifestations include fever, anorexia, recurrent URTI, anterior cervical lymphadenopathy, otitis media, and hepatosplenomegaly- Dx: Low dose chemotherapyC- Letterer-Siwe disease- <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
5 Otologic manifestation - 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
6 two lytic lesions of the skull showing beveled edges (arrows) and nonsclerotic margins, which are typical of histiocytosis X.
8 TuberculosisTuberculosis otitis media 0.05% to 0.9% of all cases of COMHematogenous or lymphatic or by extension through eustachian tubeTM becomes thickened, CHL due SOMENo pain or tenderness, lymphanopathy in high jugular chainMultiple small perforation of the TMThe middle ear mucosa appears to be hyperemic with polypoid granulationDestruction of mastoid tip may result in Bezolds abscessDefinitive diagnosis is made by histopathologic examinationOf tissue from the ME or mastoid showing a granulomatousProcess with multinucleated giant cells (langerhans cells) and histologic demonstration of acid-fast organismsD.D with wegener granulomatosis and distinguished on the basis of skin test, cultures of the ME, ANCADx: Systemic use of standard anti-TB chemotherapy however mastoid surgery may be required to remove sequestrated bone
9 The TM is intact, but greatly thickened by tuberculous granulation tissue containing the typical epithelioid cells, round cells, and multinucleated giant cells
11 Wegener’s granulomatosis A granulomatouse inflammatory process with necrotizing vasculitisPrimarily affects the upper and lower respiratory tract and kidneys but can involve any organM=F, mean age 40 yCommon presenting symptom: headache, sinusitis, rhinorrhea, otitis media, fever, arthralgiaUpper 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 ESRPositive ANCA test, especially proteinase 3, specificity>95%, sensitivity>90% in active and systemic, in limited or inactive 65% to70%
12 ContinueThe diagnose of WG is made histologically by the presence of necrosis granulomatous inflammation with multinucleated giant cells, vasculitis and microabscess formationEtiology 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-sulfamethoxazoleOtologic 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
13 Segmental vasculitis in Wegener granulomatosis with inflammation involving a portion of the arterial wall
14 SARCOIDOSISChronic multisystem disease of unknown etiology that’s characterized by noncaseating granulomasIt most frequently affects lungs, F>M, and is 10 times more common in blacks, third to fourth decadeCommon presenting symptoms: Bilateral hilar adenopathy, cough, granulomatous skin rashOthers: iridocyclitis, keratocojectivitis, peripheral lymphadenopathy, hepatosplenomegaly, cardiac failure, myalgia, and arthralgiaThe facial & optic nerves are the most commonly affected cranial nervesLaboratory findings: Hilar adenopathy in CXR, hypercalcemia, and elevated serum ACEIt has been suggested that the etiology is linked to genetically determined enhancement of the T-helper immune response to a limited number of microbial pathogens
15 Spontaneous resolution occurs in many patients ContinueSpontaneous resolution occurs in many patientsCorticosteroids are beneficial for those with progressive symptoms or with ocular, cardiac or CNS involvementInfliximab (inhibits release of TNF) has been reported as being effective for some cases that are refractory to other treatmentsOtologic manifestation: SNHL, vestibular dysfunction and facial nerve paralysis, or occasionally granulomatous disease of the external or middle ear and mastoidThe 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
16 sarcoidosisBilateral hilar adenopathy and linear parenchymal densities in pulmonary sarcoidosis.
17 SYPHILISBoth congenital and acquired syphilis may affect the middle ear in the late latent and tertiary formsIn late latent form the ME & mastoid affected by rarefying osteitis with leukocytic infiltrationIn 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 mucosaDefinitive diagnosis of syphilis requires a positive serologic test and a histologic demonstration of Treponema pallidumSyphilis may mimic TBHeneberts 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 labyrinthDx: Combined antibiotic and corticosteroid
20 LYME DISEASEMultisystem inflammatory disorder that affects skin, nervous system, heart, jointsSpirochete Borrelia burgdorferiTransmitted by Ixodes TicksPrimary reservoirs are white-footed mice and white-tailed deerThree clinical stages are recognized :a- The first stage (early, localized infection) begins 3 to 33 days after a tick bite (erythema migrans), this lesion occursin 60%-80% of patients and may accompanied by minor constitutional symptomsb- 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 lesionc-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
23 Inflammatory innate immune responses are critical in the pathogenesis ContinueInflammatory innate immune responses are critical in the pathogenesisDiagnosis is based on the recognition of the characteristic clinical features, a history of exposure and detection of a specific antibody to B. burgdorferiDx: The spirochete is highly sensitive to doxycycline, other effective antibiotics include amoxicillin, erythromycin, cefuroxime, ceftriaxone, imipenem. Steroids for carditis and arthritis.Vaccine is now availableOtologic 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 outcomeLymphocytoma a red and violet nodules occur on the earlobe during the second stageSNHL, sudden hearing loss, vertigo, meniere-like symptom have been described
25 MYCOTIC DISEASESystemic invasive clinical disease reflects some defect in host defense, such as DKA, chemotherapy, AIDSDiagnose is made by biopsy and cultureTreatment consists of control of the underlying predisposing condition, surgical debridement of necrotic tissue and Amphotercin- BOtologic manifestation: destruction of the middle ear cleft ensues, often with extention to the surrounding structures , including thrombosis or rupture of the internal carotid arteryOther routes: hematogenous embolic dissemination
26 NEOPLASTIC DISEASEMultiple myeloma: malignancy of plasmacells derived from B lymphocytes, M>F, 60 ySevere bone pain, pathologic fractures, renal failure, failure of the bone marrow, hypercalcemia and recurrent infections.Laboratory findings: M component on serum or urine electrophoresisnormochromic, 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 diseaseTx: Autologous stemcell transplantation, thalidomide, bisphosphonate and erythropoietinExtramedullary plasmacytoma (soft tissue) and solitary bone plasmacytoma (bone): in younger individual, M component in 30%, indolent course, survival rates of 10y or moreDx: local radiotherapy (4000 cGY),Periodic evaluation should be performed to detect conversion to multiple myeloma
27 large lytic destructive lesion (arrows) of the clivus (CL), petrous temporal bone, middle ear, and jugular foramen area caused by multiple myeloma
28 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 (PF) and upper neck (UN).
29 LEUKEMIACommon in the submucosa of the pneumatized areas of middle ear and mastoid and bone marrow of the petrous apexSecondary bacterial infection due to immunocompromised state or chemotherapy, hemorrage in ME, mastoid or inner earClinical 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 canalGranolocytic sarcoma or chloroma: exteramedullary tumore in AML or CMLManagement is by local irradiation and chemotherapy
30 PARAGANGLIOMA Is the most common neoplasm after the acoustic neuroma Divided into two groups: the glomus tympanicum and glomus jugulareSymptom:The glomus tympanicum appears with pulsatile tinnitus and a CHLThe 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 labyrrinthBoth may erode the TM and presenting by bleeding mass10% of nonfamilial and 50% of familial have at least one additionalLesionA few PG, both benign and malignant may secrete catecholaminesA history of headache, hypertension and flushingDx: surgery, radiotherapy is useful for management of recurrences and unresectable lesions
31 METASTATIC NEOPLASM Hematogenous dissemination The most common sites: Breast, lung, prostate, skinPetrous apex and internal auditory canalThe otic capsule relatively resistantCHL, pain, SNHL, vertigo, facial paralysisIn meningial carcinomatosis unilateral or bilateral SNHL is a common presenting symptom, diagnosis is made by cytology of the CSF
32 metastatic breast adenocarcinoma showing a large lytic lesion (arrows) destroying the mastoid.
34 PAGETS DISEASE (osteitis deformans) Osteolytic and osteoblastic changes affect the axial skeletonAD, 3% of the population, 40y old and older, M>FEnlarging skull, progressive kyphosis, deformities of the pelvis, femur, tibiaEtiology is uncertain, slow virus infection have suggestedTx: bisphosphonate, calcitonin, mithramycin, ipriflavone, gallium nitrateOtologic manifestation: HL, tinnitus, mild vestibular dysfunction,facial nerve is sparedHL 5% to 44%, SN, mixed or rarely conductiveD.D: otosclerosis, paget is late in onset, old age, greater SNHL, enlarged calvaria, enlargement of the superficial temporal artery, elevated serum ALP
35 Paget's disease. There is diffuse expansion of the skull table and involvement of both temporal bones, with patchy demineralization.
36 Lateral skull radiograph in a patient with Paget's disease Lateral skull radiograph in a patient with Paget's disease. Findings include thickening of the skull table, multiple patchy densities, and platybasia.
37 The pagetic bone encroaches on the posterior margin (arrow) of the internal auditory canal (IAC). The mastoid is largely replaced by pagetic bone
38 OSTEOGENESIS IMPERFECTA Type I through IVType 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 sporadicType 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, bisphosphonateOtologic 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 stapesRehabilitation by amplification or surgeryStapedectomy can give similar results to otosclerosis
40 FIBROUS DYSPLASIABenign, chronic, slowly progressive, unknown etiologyReplacement of normal bone with fibrous tissue and woven bone7% 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 puberty23% polystotic form: skull lesions in more than 50%, can continue to progressClinical manifestation: bony deformity, pathologic fracture, cranial nerve palsyNormal serum calcitonin and phosphorus levels, elevated serum ALP in polystotic formRadiographic finding: radiolucent area, ground-glass appearanceOtologic manifestation: progressive narrowing of the external auditory canal with CHL is the most common (80%), facial nerve paralysis, SNHL, vertigoManagement is symptomatic, radiotherapy is contraindicated
41 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 the ground-glass appearance of the fibrous phase.
42 Coronal tomographic radiograph of a patient with fibrous dysplasia Coronal tomographic radiograph of a patient with fibrous dysplasia. New bone formation causes a dense appearance of the involved left temporal bone.
43 OSTEOPETROSESRare 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 deathOtologic 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 paralysisTx: symptomatic, decompression of the facial nerve
44 OSTEITIS FIBROSA CYSTICA Von Recklinghausens diseaseExcess parathormoneOsteoclastic bone resorption, marrow fibrosis, bone cysts, bone pain, and fracturesIn most cases is caused by hyperparathyroidism due to an adenomaInvolvement of temporal bone is very rare, the otic capsule is replaced by abnormal bone, SNHL has been attributed to osteitis fibrosa
46 MUCOPOLYSACCHARIDOSES MPS an inherited deficiency of one of several lysosomal enzymes that degrade MPSClassified into seven types, all are AR except for Hunters syn (MPS II) which is X-linked recessiveManagement is supportive and symptomaticMPS III: Hurlers syn, accumulation of heparan sulfate, corneal clouding, abnormal facies, hepatosplenomegaly, MR, joint stiffness, and herniasMPS II: Hunters syn, accumulation of heparan sulfate and dermatan sulfate, similar to hurler, but corneal clouding is not seenMPS IV: Moquios syn, spondyloepiphyseal dysplasiaOtologic manifestations: CHL( Eustachian tube dysfunction & thickening of the mucosa ), SNHL ( may be a result of abnormal metabolism of the inner ear).
48 GOUTDeposition of crystals of monosodium urate within joint space & cutaneous structuresSerum urate level>7mg/dl, risk factors include: alcohol use, exposure to loop diuretics, hypertension & renal insufficiencyClinical manifestation: acute gouty arthritis, tophi, urate urolithiasis, and gouty nephropathyOtologic manifestation: Tophaceous deposits in the helical rim of the pinna, asymptomaticTreatment: bed rest, analgesics, colchicine, probencid, allopurinol
50 OchronosisOchronosis 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 andcutaneous 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.
53 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 ).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.
56 Immunodeficiency disorders Primary:Humoral: recurrent & chronic RTI with extracellular bacteria.Cellular: dysfunction of T lymp, recurrent infection with intracellular opportunistic ( viruses, fungi, protozoa & some bacteria ).Dis of phagocyte: pyogenic bacteria & fungi.Complement sys:C5, C6, C7, C9( Neisseria ), C3b inactivator ↓ ( staphylococcus) , C1, C2, C4( lupus like syn ), C1 esterase inhibitor ↓( angioedema ).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.
57 Cont.AcquiredOtologic 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.Pneumocystis carinii is common cause of middle & external ear, may be the initial presenting sym of AIDS, Tx is cotri.SNHL( otosyphilis, cryptococcal, tuberculous, toxoplasmosis meningitis ), vertigo, tinnitus, facial paralysis by Herpes zoster.
58 AIDS The virus is lymphotropic & attacks T helper lymphocytes Otologic manifestation: infrequent except in children, SOM is common, microbiology is similar to non AIDSAOM, acute mastoiditis, SOM, and bullous myringitisOtitis externa, Kaposi sarcomaPneumocystis carinii is common cause of middle ear & external ear disease, subcutaneous masses, aural polyps, CHL, otorrhea, otalgia,Dx: co-trimoxazolSNHL, vertigo, tinnitus, facial nerve palsy