II. Risk Factors

  1. Diabetes Mellitus
  2. Elderly
  3. Immunocompromised state
  4. Human Immunodeficiency Virus (HIV)

III. Pathophysiology

  1. Necrotizing infection of the soft tissue of the external auditory canal
    1. Pseudomonas aeruginosa is most common causative organism
  2. Complication of Otitis Externa
    1. Infection extends into ear canal cartilage
    2. Passes to Temporal Bone via Santorini's Fissures
  3. Severe extension of external Otitis Media
    1. Mastoiditis
    2. Osteitis of Temporal Bone

IV. Symptoms: Severe Otitis Externa

  1. Severe, unrelenting Ear Pain and Headache
  2. Persistent discharge
  3. Hearing Loss
  4. Does not respond to topical medications
  5. Commonly associated with Diabetes Mellitus

V. Signs

  1. Purulent Otorrhea
  2. Tender and swollen external auditory canal
  3. Tympanic Membrane spared
  4. Granulation tissue in posterior and inferior canal and possible exposed bone
    1. Pathognomonic for necrotizing otitis
    2. Occurs at bone-cartilage junction
  5. Extra-auricular findings
    1. Cervical Lymphadenopathy
    2. Trismus (TMJ involvement)
    3. Facial Nerve Palsy or paralysis (Bell's Palsy)
      1. Associated with poor prognosis

VI. Labs

  1. Complete Blood Count
  2. Culture ear discharge
  3. Erythrocyte Sedimentation Rate (ESR) markedly elevated
  4. Serum Glucose
  5. Serum Creatinine
  6. Histology of granulation tissue excised from canal

VII. Imaging

  1. CT Scan or MRI of ear
    1. CT findings lag behind clinical findings (but best for bony involvement evaluation)
  2. Nuclear imaging
    1. Technetium Tc 99m medronate methylene bone scanning
    2. Gallium citrate Ga 67 scintography
      1. High sensitivity for current infection
      2. Useful for follow-up for resolution

VIII. Management

  1. Admit to hospital
  2. Anti-pseudomonal antibiotics
    1. Intravenous Antibiotic options
      1. Ciprofloxacin 400 mg IV q12 hours
      2. Imipenem 0.5 mg IV q6 hours
      3. Meropenem 1.0 grams IV q8 hours
      4. Ceftazidime 2.0 grams IV q8 hours
      5. Cefepime 2.0 grams IV q12 hours
      6. Gentamicin 1 to 1.66 mg/kg IV or IM/IV with
        1. Ticarcillin or
        2. Piperacillin
      7. Timentin 3.0 grams IV q4 hours
    2. Oral antibiotic options (after initial IV course)
      1. Ciprofloxacin 750 mg PO q12 hours
    3. Course
      1. Start with IV antibiotics
      2. Continue antibiotics for 4-8 weeks
    4. Alternative course in a well appearing reliable patient
      1. Ceftazidime can be given IM and could be used with follow-up within 8-12 hours
      2. Hospital admission with IV antibiotics is safest course
  3. Consult Otolaryngology (ENT)
    1. Surgical debridement may be required
  4. Clean ear canals meticulously on a daily basis
    1. Clean and debride canal
    2. Apply topical antibiotic agents
  5. Other modalities to consider
    1. Hyperbaric oxygen chamber
    2. Davis (1992) Arch Otolaryngol Head Neck Surg 118:89 [PubMed]

IX. Complications

  1. Skull Osteomyelitis
  2. Cranial Nerve palsy
  3. Septic venous sinus thrombosis
  4. Meningitis
  5. Cerebral abscess

X. Prognosis

  1. Mortality reportedly as high as 20 to 53%

XI. Prevention

  1. Avoid use of cotton swabs in ear and other canal Trauma
  2. Use caution when irrigating ear of high risk patients
  3. Treat Eczema of ear canal and other pruritic dermatitis

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Ontology: Malignant otitis externa (C0155395)

Concepts Disease or Syndrome (T047)
ICD9 380.14
ICD10 H60.2 , H60.20
SnomedCT 194206006, 94146005
Dutch maligne otitis externa, otitis; externa, Pseudomonas aeruginosa, otitis; externa, maligne, Maligne otitis externa
French Otite externe maligne
German boesartige Otitis externa, Otitis externa maligna
Italian Otite esterna maligna
Portuguese Otite externa maligna
Spanish Otitis externa maligna, otitis externa maligna (trastorno), otitis externa maligna, otitis externa por Pseudomonas aeruginosa
Japanese 悪性外耳炎, アクセイガイジエン
Czech Maligní infekce zevního ucha
Korean 악성 바깥귀길염
English Malignant otitis externa, unspecified ear, externa malignant otitis, malignant otitis externa, otitis externa malignant, malignant otitis externa (diagnosis), Malignant otitis externa, Otitis externa due to Pseudomonas aeruginosa, Malignant otitis externa (disorder), otitis; externa, Pseudomonas aeruginosa, otitis; externa, malignant
Hungarian Rosszindulatú otitis externa