Otologic Manifestation of

Systemic Disease

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classification

1- Granulomatous & infectious

disEASE
2- Neoplastic disEASE
3- Disease of bone
4- Storage disEASE
5- Collagen vascular disEASE
6- Immunodeficiency disorder
Systemic disease that can affect the ear
 1- Granulomatous and • 4- Storage and metabolic
infectious disease disease
• a. Mucopolysaccharidoses
 a. Langerhans cell histiocytosis • b. Gout
(LCH) • c. Ochronosis
 b. Tuberculosis • 5- Collagen vascular and
 c. Wegners granulomatosis autoimmune disease
 • 6- Immunodeficiency disorders
d. Sarcoidosis •
 a. Primary or congenital
e. Syphilis • i. Humoral immunodeficiency
 f. Lyme disease disorders
 g. Mycotic disease • ii. Cellular immunodeficiency
 2- Neoplastic disease disorders
 • iii. Disorders of phagocyte
a. Multiple myeloma
 function
b. Leukemia • iv. Complement system defects
 c. Metastatic neoplasm • b. Acquired
 d. Paraganglioma • i. Acquired immunodeficiency
 3- Disease of bone syndrome
 a. Pagets disease
 b. Osteogenesis imperfecta
 c. Fibrous dysplesia
 d. Osteopetroses
 e. Osteitis fibrosa cystica 3
Introduction
 A variety of systemic disorders can manifest as
otologic complaints

 Primary systemic but have manifestation in otologic

 Infectious processes, autoimune disease, disorder of

bone, metabolic and granulomatous disease

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Infectious Diseases

• Herpes Simplex • Lyme Disease

• Varicella Zoster • Leprosy

• Mumps • Tuberculosis

• Measles • Human
Immunodeficiency
• Syphilis Virus
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Herpes Simplex

• Occurs in patients without prior immunity to

the virus

• Manifest as ginggivostomatitis,
keratokonjunctivitis or meningoenchepalitis

• The vesicular eruption can involve the skin of

the auricle and external ears

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Herpes Simplex
• Self limiting
• Treatment : Symptomatic
- Analgesic and antibiotic for secondary
infection
- Primary infection :
Acyclovir 400 mg 3 times day, 7-10 days
- Prophylactic : Acyclovir 400 mg twice a day

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Varicella Zoster

• Two distinct clinical disease

1. Varicella (chikenpox)
2. Herpes zoster (shingles)

Chikenpox
• an extremely contangious disease
• Transmission is to be respiratory
• Self limiting

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 Varicella (chikenpox)

 Hearing loss after chikenpox is not uncommon but

its ussualy due to a secondary bacterial infection
causing a conductive hearing loss
 Maculopapular exanthem often involve the skin of
the auricle

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Herpes Zoster Oticus

 Viral infection caused by varicella zoster

 Infection along one or more cranial nerve

dermatomes (shingles)

 Herpes zoster oticus or Ramsay-Hunt syndrome is

believed to result from reactivation of latent herpes
zoster virus within the geniculate ganglion
Symptoms

 Ramsey Hunt syndrome:

herpes zoster of the
pinna with otalgia and
facial paralysis
 Early: burning pain in
one ear, headache,
malaise and fever
 Late (3 to 7 days):
vesicles, facial paralysis

Herpes Zoster Oticus

 Treatment

- Acyclovir 800 mg orally 5 times a day for 7-10 days

- Valcyclovir 1000 mg 3 times a day for 7 days.

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Mumps
• RNA Paramyxovirus
• Transmitted by salivary secretion, usually via
the respiratory tract
• Manifestation include salivary adenitis,
epididimo-orchitis, pancreatitis, thyroiditis,
polyartritis and miocarditis.
• Frequency equal in both sex

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 Mumps
 Hearing loss in 5 of 10,000 patient.
 Hearing loss is unilateral 80%, permanent
 Tinnitus and aural fullness, vestibular symptom in
several week
• Treatment : no specific treatment
- corticosteroid reported to help diminish fever
and pain
- vaccination provide protection to 75% to 95%

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Measles
• RNA virus, Rubeola
• Hearing loss occur in fewer than 0,1% of all
measles infection.
• Hearing loss usually bilateral and begins
abruptly as the same time as the macular
rash
• 45% develop profound hearing loss

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Measles

• Hearing loss may be asymmetric and tends to

be maximal at higher frequencies
• Tinnitus and vertigo
• Hearing loss is permanent and 70% have
absent vestibular responses

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Syphilis
• Causative organism is the spirochete Treponema
pallidum
Stage :
• Latent syphilis infection, middle ear and mastoid
osteitis is seen with a leukocytic infiltration of the osicles
and temporal bone
• Secondary stage, is usually abrupt, bilateral and
progesive, vestibular symptom, rash and headache
• Tertier syphilis is characterized by gumma, a
granulomatous lession with central nekrosis and
obliterative arteritis.
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Syphilis

• Gumma may effect the middle ear with

tympanic membrane perforation and
granular appearance of the middle ear
mucosa

• Tertiary stage hearing loss asymmetric and

fluctuating, progressive tinnitus and vertigo,
positive fistula test (Hennebert’s sign)

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 Syphilis
• Diagnosis :
Presence of a positive serum fluorescent treponemal antibody
absorption test (FTA-ABS), or microhemaglutinastion assay-
Treponema pallidum (MHA-TP).

• Treatment :
- benzathine penicilin 2.4 million unit im weekly for 6 to 12 week
- aqueous penicillin 600.000 unit im daily for 6 to 12 weeks
- prednisone 30-60 mg per day for 3-6 month and tapered slowly

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Lyme Disease

 Infection with the spirochete Borrelia burgdorferi,

 Transmitted by certain species of ticks

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Lyme Disease

 Late symptom : multiple neurophaty,

artheritis, meningitis, myocarditis
 To ENT  facial
paralysis,temporomandibular joint pain,
tinnitus, SNHL, sudden HL, otalgia, facial
pain, cervical adenopathy
 50% acute facial paralysis in children, it
resolve slowly in 6-12 month
 Treatment : doxycyclin ,amoxicilin in 14 days
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Tuberculosis

• Caused by Mycobacterium tuberculosis

• Transmission due to aerosolized respiratory

secretion expelled by the cough of an infected
person

• Can effect multiple sites in the head and neck

including the larynx, pharynx,oral cavity, nasal
cavity, salivary gland, cervical spine, middle ear
and mastoid
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Tuberculosis

 Lymphadenopathy is seen in 5-10%

 May present as
- Facial paralyzed
- Persistent aural discharge
- Profuse granulation tissue
- Pericondritis
- Refractory chronic otitis media :
hematogenous, infection through the Eustachian
tube
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 Tuberculosis
• The infection is generally painless, with
multiple small perforation that may quickly
coalesce to total tympanic membrane
perforation, conductive hearing loss due to
osicular destruction, jugular
lymphadenopathy

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Tuberculosis
 Definitive diagnosis  isolation and identification of
M tuberculosis in culture from a diagnostic
specimen

 Treatment : standard multy drug antituberculous

 Surgery may sometimes be needed to remove

intractably pathologic tissue and sequestrated bone

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HIV

 RNA retrovirus
 Immunocompromised patient are more to
opportunistic infection
 Otologic manifestation :
 Otitis externa : pseudomonas, proteus, aspergillus
 Polyp of the external canal  biopsy
 Recurrent otitis media
 Serous otitis media ; 80 % in pediatric HIV infected
 SNHL ; prevalence 21- 41 %
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Imunologic Disease

 Wagener’s Granulomatosis
 Relapsing Polychondritis
 Chronic Discoid Lupus Erythematosus
 Polyarteritis Nodosa
 Rheumatoid Arthritis

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Wagener Granulomatosis
• Mean age of onset is approximately 40 years

• Common symptoms involve the upper and

lower respiratory tract and include sinus pain,
bloody rhinorhoe, septal ulceration, cough,
dypsnea, pleritic chest pain and nodular
infiltrate on chest radiograph

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 Wegener’s Granulomatosis
 General
 necrotizing granulomas of upper airway, lower airway,
kidney
 bilateral pneumonitis 95%
 chronic sinusitis 90%
 mucosal ulceration of nasopharynx 75%
 renal disease 80%
 The nasal cavity and paranasal sinuses are involved in 85-
100% of the case
 Otologic involvement is seen in approximately 35%
Wegener’s Granulomatosis

• Otologic manifestation
▪ serous otitis media (20-25%)
▪ suppurative otitis media
▪ pinna changes similar to polychondritis
▪ facial nerve palsy
▪ CHL and SNHL

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Wegener’s Granulomatosis

 Positive laboratory findings are an elevated

erythrocyte sedimentaation rate, anemia,
hematuria, elevated serum creatinine and
presense of serum antineutrophil cytoplasmic
antiboby (ANCA)

 Treatment :
- corticosteroid
- cyclophosphamide
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Relapsing Polychondritis
 More common in women, average in 40
 Episodic inflamation of cartilaginos structur
 Other sign episkleritis,arthtropathies, anemia,
weight loss, myalgia fever
 Recurent inflamation  atrophy, scaring, distortion
of the involved cartigale.

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 Relapsing Polychondritis

Otologic Manifestation
 Red, tender, edematous
pinna
 Fever, pain
 Swelling, erythema

 Treatment : corticosteroid
Chronic Discoid Lupus Erythematosus
• Condition that raised erythematous lesions
on the skin of head, neck and chest
• The cause is unknown, but demonstration of
immune complexes in dermal-epidermal
junction suggest an autoimmune
phenomenon

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Chronic Discoid Lupus Erythematosus

Treatment:
 Rheumatologist involvement
 Avoidance of sun
 Use of sunscreens
 NSAID, topical and low dose steroids
 Low dose methotrexate instead of steroids
 Azothioprine, cyclophosphamide, high dose
steroids for serious visceral involvement
Polyarteritis Nodosa
 Necrotizing vasculitis of small and medium
diameter arteries
 Diagnosis  biopsy showing polymorphonuclear
neutrophils infiltrating all layers
 Sistemic  ecchymosis, myocardial ischemia, renal
failure and hypertensi
 Otologic HL, facial nerve paralisis
 Treatment : corticosteroid combine with
cyclophosphamide

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Rheumatoid Arthritis

 Inflammation of the synovial tissue

(lymphocytic) with synovial proliferation
 Symmetric involvement of peripheral joints,
hands, feet and wrists
 Women : man 3:1 , 4th and 5th decade

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Rheumatoid Arthritis
 Characterized :
Persistent inflamatory synovitis
causing progressive cartilage
and bone destruction within joint

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Rheumatoid Arthritis

 Rheumatoid nodule are both cutaneous and

subcutaneous and may involve the external
ear,painful, raised lesion with necrotic center
 SNHL, CHL
 Treatment :
Aspirin
Nonsteroidal antiinflamatory drugs
Glucocorticoid

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Metabolic Disorder

 Gout
 Ochronosis
 Mucopolysaccharidoses

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 Gout
• Disorder of purine metabolism that causing
arthritis and tophaceous nodule

• Categorized as
- overproducer  with abnormally high production
of uric acid
- underexcreters  with abnormally low renal
excretion of uric acid

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Gout

 Common among men in the 4th or 5th

decade of life

 Treatment
- overproducer alloprinol
- underexcreters  uricosuric (probenecid)

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Ochronosis

• A form alkaptonuria, is an autosomal recessive

disorder of homogentisic acid metabolisme
• 3rd decade
• Accumulate homogentisic acid, which is
oxidized to a black compound that adheres to
cartilage and thus discolors the ear, finger,
buccal mucosa and nose
• Urine turns black on exposure to sunlight
• Treatment : low protein diet
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Mucopolysaccharidoses
• Group of storage disease characterized by inherited
deficiencies of enzymes needed for degradation of
mucopolisacharides

• Hunter syndrome
 lack of alpha-iduronidase, x linked

• Hurler syndrome
 same enzym, autosomal resesive disease

• Morquio syndrome
 deficiency of N acetylgalactosamine-6-sulfatase
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Mucopolysaccharidoses

• sensorineural maybe due to abnormal lipid

metabolism within nerve cell
• HL usually mixed with the conductive
component due to Eustachian tube
dysfunction

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Disease of Bone

 Osteogenesis imperfecta
 Osteoporosis
 Paget’s Disease
 Fibrosis Dysplasia

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Osteogenesis imperfecta
• Hoeve-deKleyen syndrome, autosomal
dominant, disorder of connective tissue 
high susceptibility to fracture

• Cause by mutation in either of the two chains

that form type I procolagen, the major
structural protein of bone, skin and tendon.

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Osteogenesis imperfecta

• Type I : mildest form, autosomal dominant, HL,

multiple fracture and blue sclera

• Type II : lethal in utero

• Type III : more severe with age, sclera slightly

bluish in infancy and white in adult

• Type IV : sclera slightly bluish in infancy and

• white in adult
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Osteopetrosis

• An inherited disorder causing faulty

remodeling of bone
• Dominant or a recessive pattern

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Osteopetrosis

Characterized dominant form

 Progressive enlargement of the head of the
mandible and long bone clubbing.
 Progressive cranial neuropathies are cause by
nerve decompression at the foramina, resulting
in :
 Optic atrophy
 Trigeminal hypoesthesia
 Recurent facial paralysis
 SNHL
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Osteopetrosis

Characterized recessive form

 Recessive form is more severe
 Hepatospenomegaly
 Mental retardation.
 Facial nerve paralysis is caused by compression of
the nerve along its intratemporal course

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Paget’s Disease
 Osteitis deformans
 Etiology : unknown
 Chronic, progressive bone metabolism
 Bony hypertrophy and remodeling due to increased
activity of osteoclast and osteoblast
 Men : woman = 4:1, sixth decade

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Paget’s Disease

 Paget in temporal bone can changes in bone

density, mass and geometry causes SNHL
and CHL
 Treatment :
 calcitonin
 etidronate disodium to decrease osteoclast
activity

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Fibrous Dysplasia
 Idiopathic disorder
 Characterized by replacement of normal cancellous
bone by spicules of woven bone in a fibrous stroma
 Involvement of temporal bone
 painless progresive enlargment of the squamosa mastoid
and external canal
 CHL,progressive occlusion of external auditory canal,
canal colesteatoma
 Treatment : surgical

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Idiopathic Disease

• Histiocytosis X
• Include eosinophilic granuloma, Hand-Schuller-
Christian disease and Letterer-Siwe disease.
• Usually children
• Caracterized :
• Acumulation of histiocytes, lymphochites and
eosinofil in the skin, bone marrow, lymph node, lung,
liver,thymus, spleen and central nervus system.
• Deficiency supresor lymphocyte and elevated helper
lymphocyte
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Idiopathic Disease

Head and neck involvement in 73%

Otologic manifestation :
 otorhea, aural granulation, mastoid swelling,
deafness, collaps of EAC, swelling of mandible
or maxilla  complication include SNHL,
facial nerve paralysis and secondary infection
• Treatment :
• systemic steroid  vincristine, vinblastine.
• surgical debulking
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Thank You

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