Ear

Inflammatory / infectious / autoimmune / systemic disorders

Infectious / inflammatory



Last author update: 1 October 2013
Last staff update: 17 January 2025

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PubMed Search: Meniere disease, Necrotizing malignant external otitis, Otitis media, Otomycosis, Relapsing polychondritis ear pathology, Cystic chondromalacia of auricular cartilage


Nat Pernick, M.D.
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Cite this page: Pernick N. Infectious / inflammatory. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earinfectiousinflam.html. Accessed April 2nd, 2025.
Idiopathic cystic chondromalacia of auricular cartilage
Definition / general
  • Also called auricular or endochondral pseudocyst
  • Benign cystic degeneration of auricular cartilage of unknown cause
  • Usually men age 20 - 40 years
  • Unilateral swelling of cartilage over weeks to years, most commonly on scaphoid fossa of auricle
  • May be due to minor trauma

Case reports

Treatment
  • Excision

Microscopic (histologic) description
  • Fluid filled distended mass composed of cyst like wall with fibrous and granulation tissue lining but no epithelium
  • Cyst contains 1 - 2 mm rim of cartilage
  • Cyst fluid resembles olive oil
  • No / mild atypia

Microscopic (histologic) images

Images hosted on other servers:
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Central cystic degeneration of auricular cartilage

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Factor VIII+ lining of pseudocyst



Differential diagnosis

Additional references
Meniere disease
Definition / general
  • Also called endolymphatic hydrops
  • Idiopathic disorder of inner ear associated with episodic attacks of vertigo, fluctuating sensorineural hearing loss, tinnitus and sensation of aural fullness
  • Incidence varies from 7.5 per 100,000 in France to 157 per 100,000 in England
  • 60% women, peaks in 40s to 60s but wide age range
  • Rarely occurs in children (J Laryngol Otol 2006;120:343)
  • Associated with HLA B8 / DR3
  • May be due to accumulation of endolymph in membranous labyrinth, perhaps due to inadequate absorption by endolymphatic sac

Treatment
  • Dietary modification, intermittent dehydration, diuretics, vasodilators in increase microcirculation of ear
  • 60 - 80% improve
  • Surgery includes shunting and decompression of endolymphatic sac, labyrinthectomy, sectioning of vestibular nerve (Laryngoscope 2005;115:1454)

Microscopic (histologic) description
  • Initially involves cochlear duct and saccule
  • Later entire endolymphatic system with dilation, rupture and collapse of membranous labyrinth with possible fistula
  • May have severe atrophic changes with loss of cochlear neurons

Microscopic (histologic) images

Images hosted on other servers:
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Section through cochlea



Additional references
Necrotizing malignant external otitis
Definition / general
  • Potentially fatal external otitis due to Pseudomonas aeruginosa (Ann Otolaryngol Chir Cervicofac 2000;117:291), Aspergillus or other fungal infection
  • Usually older patients, often with diabetes, chronic debilitation or immunodeficiency; also undernourished African infants (Rev Laryngol Otol Rhinol 2002;123:225)
  • Initially affects external auditory canal with symptoms of acute otitis externa; later pain, purulent otorrhea and swelling; may progress to cellulitis, chondritis, osteomyelitis, involve middle ear space or base of skull and cause cranial nerve palsies, meningitis, venous thrombosis or brain abscess (Rev Stomatol Chir Maxillofac 2006;107:167)
  • Up to 75% mortality if treatment is delayed
  • Due to tissue ischemia (from above primary pathologic state) plus neutrophilic migratory defect plus virulence of Pseudomonas

Treatment

Case reports

Gross description
  • Ulcerated skin near osseous portion of external auditory canal, often with abundant necrotic and granulation tissue

Microscopic (histologic) description
  • Epithelium is necrotic or ulcerated with pseudoepitheliomatous hyperplasia, marked mixed inflammatory infiltrate in subcutaneous tissue, necrotizing vasculitis
  • Necrotic bone and cartilage with heavy inflammatory infiltrate in viable bone
  • Variable sequestra of nonviable bone or cartilage

Positive stains
  • Gram stain (Gram negative rods)

Differential diagnosis

Additional references
Otitis media
Definition / general
  • Acute or chronic infectious disease of middle ear
  • Usually childhood disease caused by Streptococcus pneumoniae or Haemophilus influenzae; also coinfection by viruses (Pediatr Infect Dis J 2004;23:1142, Clin Infect Dis 2006;43:1417)
  • Rarely caused by fungi or pneumocystis in HIV+ patients
  • Hyperemic, opaque and bulging tympanic membrane with limited mobility; may have purulent otorrhea
  • Infection probably occurs post pharyngitis via eustachian tube
  • Severe cases are associated with destruction of ossicles
  • Tympanosclerosis: dystrophic calcification of tympanic membrane or middle ear associated with recurrent cases of otitis media, occurs in 3 - 33% of cases; may be reversible in children, usually irreversible in adults and associated with conductive hearing loss

Treatment

Gross description
  • Not a common specimen but may have small fragments of soft / rubbery granulation tissue

Microscopic (histologic) description
  • Acute and chronic inflammatory cells, haphazard glandular metaplasia with cilia, fibrosis, hemorrhage, foci of calcification (tympanosclerosis), cholesterol granulomas and reactive bone formation (Laryngoscope 1982;92:273)
  • Cholesterol granulomas: foreign body granulomas in response to cholesterol crystals from rupture of red blood cells and breakdown of lipid bilayer in cell membrane, prominent cholesterol clefts; associated with interference to drainage or ventilation of middle ear space; not related to cholesteatomas

Differential diagnosis

Additional references
Otomycosis

Microscopic (histologic) images

Contributed by Veena Maheshwar, M.D., Kiran Alam, M.D., Anshu Jain, M.D.
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Various images

Relapsing polychondritis
Definition / general
  • Also called polychondropathia
  • Uncommon systemic episodic or relapsing disease with progressive degeneration of cartilage throughout the body
  • Probable autoimmune process (antibodies to type II collagen) associated with other autoimmune disorders
  • Whites, no gender preference, usually symptomatic in 40s to 60s although affects all ages
  • 90% have involvement of auricular cartilage, usually bilateral, with swelling, erythema and tenderness
  • Earlobes are typically spared
  • Variable relapsing of disease
  • May cause cauliflower ear and saddle node deformities
  • Clinical diagnosis requires 3 of the following
    • Recurrent chondritis of both auricles
    • Nonerosive inflammatory arthritis
    • Chondritis of nasal cartilage
    • Ocular inflammation including conjunctivitis, keratitis, scleritis, episcleritis or uveitis
    • Chondritis of upper respiratory tract including larynx or tracheal cartilage
    • Cochlear or vestibular damage with sensorineural hearing loss, tinnitus or vertigo

Laboratory
  • Nonspecific elevated sedimentation rate, mild leukocytosis, normochromic normocytic anemia; variable elevated ANCA
  • Prognosis varies from prolonged course to aggressive and fulminant disease leading to death from respiratory tract or cardiovascular involvement (aortic insufficiency)

Treatment
  • Responds to steroids or dapsone (this also confirms diagnosis)
  • Advanced cases require immunosuppressive agents

Microscopic (histologic) description
  • Mixed inflammatory infiltrate (lymphocytes, plasma cells, neutrophils, occasional eosinophils) extending into cartilage with blurring of interface between cartilage and adjacent soft tissue
  • Cartilage shows loss of normal basophilia, loss of chondrocytes and destruction of lacunar architecture at advancing edge of inflammation with cartilage replaced by fibrous tissue

Positive stains

Additional references
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