eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > External Ear Diseases

External Ear, Malignant External Otitis

Author: Brian Nussenbaum, MD, FACS, Associate Professor, Vice Chair for Clinical Affairs, Co-director of Fellowship in Microvascular Head/Neck Oncology, Department of Otolaryngology-Head and Neck Surgery, Washington University School of Medicine
Coauthor(s): Peter S Roland, MD, Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, University of Texas School of Human Development
Contributor Information and Disclosures

Updated: May 19, 2009

Introduction

Background

Toulmouche was probably the first physician to report a case of malignant external otitis (MEO), in 1838. In 1959, Meltzer reported a case of pseudomonal osteomyelitis of the temporal bone. In 1968, Chandler discussed the clinical characteristics of malignant external otitis (MEO) and defined it as a distinct clinical disease.1 He described this external otitis as malignant because he observed an aggressive clinical behavior, poor treatment outcome, and a high mortality rate for the patients affected by this disease.

The subsequent development of effective antibiotics for treating pseudomonal infections has improved the treatment outcomes for patients with malignant external otitis (MEO). Thus, some physicians have suggested that the term malignant should be abandoned in order to provide a more accurate description of the disease process.

Anatomy of the ear.

Anatomy of the ear.

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Anatomy of the ear.

Anatomy of the ear.

Pathophysiology

Malignant external otitis (MEO) is an infection that affects the external auditory canal and temporal bone. The causative organism is usually Pseudomonas aeruginosa, and the disease commonly manifests in elderly patients with diabetes. The infection begins as an external otitis that progresses into an osteomyelitis of the temporal bone. Spread of the disease outside the external auditory canal occurs through the fissures of Santorini and the osseocartilaginous junction.

Frequency

United States

Malignant external otitis (MEO) is more common in humid and warm climates than in other climates.

Mortality/Morbidity

  • Cranial neuropathy
    • Cranial nerves can be affected by inflammation along the skull base or by a neurotoxin produced by Pseudomonas species. The facial nerve (VII) is affected most commonly, usually at the stylomastoid foramen. As the disease progresses, cranial nerves IX, X, and XI can be affected at the jugular foramen, followed by XII at the hypoglossal canal. Cranial nerves V and VI can be affected if the disease extends to the petrous apex.
    • In 1977, Chandler reported a 32% incidence of facial nerve paralysis.2 The incidence of facial nerve paralysis appears to have decreased with the development of more effective medical therapy as shown by Franco-Vidal et al who reported a 20% incidence of facial nerve paralysis in 46 treated patients.3 The other cranial nerves are affected less frequently than the seventh cranial nerve. The development of cranial neuropathy generally was thought to reflect advanced-stage disease associated with a worse prognosis. More recently, Corey et al, Soudry et al, and Mani et al suggested that the presence of facial nerve paralysis does not worsen the prognosis.4,5 Recovery of facial nerve function is poor and unpredictable, and should not be used as an indicator of successful treatment. Other cranial nerves that are affected have a higher rate of recovery.
  • Intracranial complications: These complications rarely occur in the absence of cranial nerve palsies. Meningitis, brain abscess, and dural sinus thrombosis may ensue. Cranial neuropathies related to the jugular foramen should raise concern for sigmoid sinus thrombosis. Cavernous sinus thrombosis should be considered if cranial nerves V or VI are affected. Intracranial complications reflect severe disease and are commonly fatal.
  • Comorbid conditions: Patients with malignant external otitis (MEO) almost always have diabetes, often with other multiple medical problems. During the course of therapy, Chandler found some deaths related to pneumonia, uremia, myocardial infarction, strokes, and liver failure. Franco-Vidal showed that patients with systemic immunodeficiencies had a worse prognosis.3

Sex

Malignant external otitis (MEO) is more common in males than in females.

Age

Malignant external otitis (MEO) has been reported in all age groups but is most common in patients who are elderly (age, >60 y).

Clinical

History

  • Diabetes (90%) or immunosuppression (illness or treatment related)
  • Severe, unrelenting, deep-seated otalgia
  • Temporal headaches
  • Purulent otorrhea
  • Possibly dysphagia, hoarseness, and/or facial nerve dysfunction

Physical

  • Inflammatory changes are observed in the external auditory canal and the periauricular soft tissue.
  • The pain is out of proportion to the physical examination findings.
    • Marked tenderness is present in the soft tissue between the mandible ramus and mastoid tip.
    • Granulation tissue is present at the floor of the osseocartilaginous junction. This finding is virtually pathognomonic of malignant external otitis (MEO). Otoscopic examination may also reveal exposed bone.
  • The cranial nerves (V-XII) should be examined.
  • Mental status examination should be performed. Deterioration of the mental status may indicate intracranial complication.
  • The tympanic membrane is usually intact.
  • Fever is uncommon.

Causes

  • Diabetes (90% of patients)
    • Diabetes is the most significant risk factor for developing malignant external otitis (MEO).
    • Small-vessel vasculopathy and immune dysfunction associated with diabetes are primarily responsible for this predisposition.
    • The cerumen of patients with diabetes has a higher pH and reduced concentration of lysozyme, which may impair local antibacterial activity.
    • No difference in predisposition is found between diabetes types I and II.
    • The predisposition is not necessarily related to the severity of glucose intolerance or periods of hyperglycemia.
  • Immunodeficiencies, such as lymphoproliferative disorders or medication-related immunosuppression
  • AIDS
    • Malignant external otitis (MEO) associated with AIDS may have a different pathophysiology than classic malignant external otitis (MEO).
    • Patients present with similar symptoms but are generally younger and do not have diabetes.
    • Granulation tissue may be absent in the external auditory canal.
    • Pseudomonas is not necessarily the dominant causative organism.
    • Patients with AIDS generally have a poorer outcome than patients with diabetes.
  • Aural irrigation: As many as 50% of cases of malignant external otitis (MEO) have been reported to be preceded by traumatic aural irrigation in patients with diabetes.

More on External Ear, Malignant External Otitis

Overview: External Ear, Malignant External Otitis
Differential Diagnoses & Workup: External Ear, Malignant External Otitis
Treatment & Medication: External Ear, Malignant External Otitis
Follow-up: External Ear, Malignant External Otitis
Multimedia: External Ear, Malignant External Otitis
References
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References

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Further Reading

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Keywords

malignant external otitis of the external ear, external ear, malignant external otitis, MEO, invasive external otitis, necrotizing external otitis, progressive external otitis, skull base osteomyelitis, ear infection, pseudomonal osteomyelitis of the temporal bone, Pseudomonas aeruginosa, P aeruginosa, diabetes, malignant external otitis

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Contributor Information and Disclosures

Author

Brian Nussenbaum, MD, FACS, Associate Professor, Vice Chair for Clinical Affairs, Co-director of Fellowship in Microvascular Head/Neck Oncology, Department of Otolaryngology-Head and Neck Surgery, Washington University School of Medicine
Brian Nussenbaum, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, Phi Beta Kappa, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

Coauthor(s)

Peter S Roland, MD, Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, University of Texas School of Human Development
Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Alcon labs Honoraria Speaking and teaching; GSK Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear corp Honoraria Board membership; Med El corp travel grants Consulting

Medical Editor

Jack A Shohet, MD, Associate Clinical Professor, Department of Otolaryngology-Head and Neck Surgery, University of California Irvine; Otolaryngologist, Shohet Ear Associates Medical Group, Inc
Jack A Shohet, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, American Neurotology Society, American Tinnitus Association, and California Medical Association
Disclosure: Envoy Medical Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Gerard J Gianoli, MD, Clinical Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Tulane University School of Medicine; Vice President, The Ear and Balance Institute; Chief Executive Officer, Ponchartrain Surgery Center
Gerard J Gianoli, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown

 
 
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