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Necrotizing external otitis, or malignant external otitis, as initially described by Chandler, is a life-threatening Pseudomonas infection of the external auditory canal and skull base, which occurs most commonly in elderly diabetic patients.

Necrotizing (malignant) external otitis is commonly caused by Pseudomonas aeruginosa.  It is rarely caused by Aspergillus and Klebsiella pneumoniae.

The disease spreads rapidly, invading surrounding soft tissues, cartilage and bones causing their necrosis and even spreading to the cranial nerves. The disease can be fatal if treatment is not aggressive and timely, especially if it spreads outside the auditory canal with involvement of the cranial nerves.

Histologically the epithelium is necrotic or ulcerated with pseudoepitheliomatous hyperplasia and marked mixed inflammatory infiltrate in subcutaneous tissue.

                  

Malignant external otitis. Laryngorhinootologie. 2006 Oct;85(10):763-9

Malignant external otitis or skull base osteomyelitis and osteoradionecrosis of the skull base and the skull are potentially life-threatening conditions. The standard methods of treatment involve the use of antibiotics, local treatment and, where necessary, surgical excision of necrotic tissue. These approaches do not provide a complete cure in many cases. Severe functional deficits and even death can occur in advanced stages. We conducted a long-term retrospective follow-up study and report on a multimodal approach that we have been using with great success since 1987. The four cornerstones of this treatment are surgical debridement, combinations of antibiotics, specific immunoglobulins, and adjunctive hyperbaric oxygen therapy. This multimodal treatment approach has proved to be highly effective in improving the survival and quality of life of the patients concerned. These excellent outcomes justify the high costs that this therapy admittedly involves.

Necrotizing external otitis in a patient caused by Klebsiella pneumoniae.Eur Arch Otorhinolaryngol. 2006 Apr;263(4):344-6. 

Necrotizing external otitis is a potentially life-threatening infection involving the temporal and adjacent bones. The most frequent pathogen is attributed to Pseudomonas aeruginosa, but is rarely caused by Klebsiella pneumoniae. Recently, we encountered a 47-year-old diabetic man with a swollen obliterated external ear canal with granulation tissue on the right ear. Image study demonstrated skull base osteomyelitis, epidural abscess and cerebral venous sinus thrombi. It was later proved to be necrotizing external otitis caused by Klebsiella pneumoniae. He then underwent craniotomy for drainage of the epidural abscess, followed by intravenous ciprofloxacin and metronidazole for 2 consecutive weeks until both pus and blood cultures depicted no growth of pathogens. Based on this case, synergistic antibiotic therapy using a third-generation cephalosporin or quinolone (ciprofloxacin), accompanied by metronidazole, and even a short-term aminoglycoside is recommended for the treatment of severe Klebsiella-induced necrotizing external otitis. Surgical intervention should be limited without shedding of the pathogens.

The changing face of malignant (necrotising) external otitis: clinical, radiological, and anatomic correlations.Lancet Infect Dis. 2004;4(1):34-9.

Malignant (necrotising) external otitis is an invasive infection of the external auditory canal. Although elderly patients with diabetes remain the population most commonly affected, immunosuppressed individuals (eg, from HIV infection, chemotherapy, etc) are also susceptible to malignant external otitis. Pseudomonas aeruginosa is isolated from the aural drainage in more than 90% of cases. The pathophysiology is incompletely understood although aural water exposure (eg, irrigation for cerumen impaction) has been reported as a potential iatrogenic factor. The typical patient presents with exquisitely painful otorrhoea. If untreated, cranial neuropathies (most commonly of the facial nerve) can develop due to subtemporal extension of the infection. The diagnosis of malignant external otitis is based on a combination of clinical findings, an increased erythrocyte sedimentation rate, and radiographic evidence of soft tissue with or without bone erosion in the external canal and infratemporal fossa. Treatment consists of prolonged administration (6-8 weeks) of an antipseudomonal agent (typically an orally administered quinolone). With the introduction and widespread use of both oral and topical quinolones, there are reports of less severe presentation of malignant external otitis and even the emergence of ciprofloxacin resistance. Reservation of systemic quinolones for the treatment of invasive ear infections is recommended.

Necrotizing (malignant) external otitis. Am Fam Physician. 2003 Jul 15;68(2):309-12.

Necrotizing (malignant) external otitis, an infection involving the temporal and adjacent bones, is a relatively rare complication of external otitis. It occurs primarily in immunocompromised persons, especially older persons with diabetes mellitus, and is often initiated by self-inflicted or iatrogenic trauma to the external auditory canal. The most frequent pathogen is Pseudomonas aeruginosa. Patients with necrotizing external otitis complain of severe otalgia that worsens at night, and otorrhea. Clinical findings include granulation tissue in the external auditory canal, especially at the bone-cartilage junction. Facial and other cranial nerve palsies indicate a poor prognosis; intracranial complications are the most frequent cause of death. Diagnosis requires culture of ear secretions and pathologic examination of granulation tissue from the infection site. Imaging studies may include computed tomographic scanning, technetium Tc 99m medronate bone scanning, and gallium citrate Ga 67 scintigraphy. Treatment includes correction of immunosuppression (when possible), local treatment of the auditory canal, long-term systemic antibiotic therapy and, in selected patients, surgery. Family physicians and others who provide medical care for immunocompromised patients should be alert to the possibility of necrotizing external otitis in patients who complain of otalgia, particularly if they have diabetes mellitus and external otitis that has been refractory to standard therapy. Susceptible patients should be educated to avoid manipulation of the ear canal (i.e., they should not use cotton swabs to clean their ears) and to minimize exposure of the ear canal to water with a high chloride concentration. Appropriate patients should be referred to an otolaryngologist.

Malignant otitis externa: a review.Pac Health Dialog. 2002 Mar;9(1):64-7.

Malignant otitis externa is a rare but potentially fatal disease of the external auditory canal seen mostly among elderly, diabetic or immunocompramised patients. The causative organism is mainly Pseudomonas aeruginosa. The disease spreads rapidly, invading surrounding soft tissues, cartilage and bones causing their necrosis and even spreading to the cranial nerves. The disease can be fatal if treatment is not aggressive and timely, especially if it spreads outside the auditory canal with involvement of the cranial nerves. Treatment is mainly medical with antipseudomonal drugs like the third generation cephalosporin and the fluoroquinolones and local debridement. With aggressive treatment the mortality rate from this disease, which used to be 50% in the past has now been reduced to 10-20%. The pathophysiology of the disease, clinical presentation, diagnosis, treatment and the outcome has been discussed and reviewed.

Malignant or necrotizing otitis externa: experience in 22 cases. Ann Otolaryngol Chir Cervicofac. 2000 Nov;117(5):291.

Malignant or progressive necrotizing otitis extrema is an uncommon but severe infectious condition of the external auditory canal. Over a period of four years, we treated 22 patients: 60% had diabetes (1/4 insulin dependent) and 13% were immunodepressed. The causal germ was Pseudomonas aeruginosa in 87% of cases. The pretherapeutic work-up included a computed tomography scan and a technetium scintigraphy to confirm diagnosis and assess extension. Repeated scintigraphies with gallium were used to follow the course under treatment. Medical treatment was used in most cases (16/22) with parenteral antibiotic therapy using a third-generation cephalosporin (ceftazidime or ceftriaxone) and a fluoroquinolone (ciprofloxacin or ofloxacin) and, if there was no contraindication, hyperbaric oxygen. Surgery is not indicated in malignant otitis externa. We had a 95% cure rate with only 10% recurrence. We reviewed the data in the literature on malignant otitis externa and present the important diagnostic, imaging and therapeutic aspects.

Necrotizing external otitis caused by Aspergillus fumigatus: computed tomography and high resolution magnetic resonance imaging in an AIDS patient.J Laryngol Otol. 1998 Jan;112(1):98-102.

Most necrotizing (malignant) external otitis (NEO) occurs in diabetic patients and is commonly caused by Pseudomonas aeruginosa. We report an acquired immunodeficiency syndrome (AIDS) patient with NEO caused by Aspergillus fumigatus in which computed tomography (CT) showed destructive petrous bone involvement and magnetic resonance imaging (MRI) of the ear discovered extensive soft tissue and facial nerve involvement. Dedicated MRI studies of the ear in this type of pathology provide new insights relating to nerve dysfunction, that cannot be obtained with CT.

Necrotizing external otitis in patients with AIDS.Laryngoscope. 1997 Apr;107(4):456-60.

In a retrospective review of seven patients with AIDS who were diagnosed with necrotizing external otitis between 1990 and 1995, it was found that the presentation of necrotizing external otitis in patients with AIDS differed from the classic description of malignant external otitis in several respects. The patient population was significantly younger and nondiabetic. Granulation tissue was usually absent from the external auditory canal and Pseudomonas aeruginosa was not the predominant pathologic organism. Also, outcome was found to be significantly worse. Thus a high index of suspicion must be entertained and vigorous local and systemic treatment initiated early in the course of disease to achieve a satisfactory outcome.

Malignant external otitis: a case report and review. Am Fam Physician. 1994 Feb 1;49(2):427-31.

Malignant external otitis is an unusual but serious and potentially fatal condition that has only recently been described. It is an invasive pseudomonal infection of the external auditory canal and deep periauricular tissues that characteristically involves the bone and adjacent cartilaginous structures, and it may lead to osteomyelitis of the base of the skull. It typically occurs in elderly diabetic patients. Malignant external otitis can cause severe pain, necrosis of the external auditory canal and progressive palsies of the facial and cranial nerves. Treatment consists of debridement of external auditory canal granulation tissue and long-term therapy with an antipseudomonal cephalosporin or an antipseudomonal penicillin plus an aminoglycoside.

Malignant otitis externa in AIDS patients: case report and review of the literature.Ear Nose Throat J. 1994 Oct;73(10):772-4, 777-8.

Malignant otitis externa is a necrotizing infection of the external ear canal and surrounding soft tissue and bone, usually caused by Pseudomonas aeruginosa. The infection classically occurs in diabetic patients, however recently, several patients with the acquired immunodeficiency syndrome (AIDS) have been reported to have malignant otitis externa. A patient with AIDS who had malignant otitis externa with skull base osteomyelitis is presented and reported cases in patients with AIDS are reviewed. Predisposing factors include immunologic abnormalities (notably neutropenia), dermatitis, medications, neoplasm, and iatrogenic procedures, e.g., ear lavage. Treatment of malignant otitis externa has traditionally included anti-pseudomonal cephalosporins/penicillins and aminoglycosides for prolonged durations. Recently, ciprofloxacin has been shown to be effective as an oral regimen. With the increasing number of patients with AIDS being seen in the outpatient clinics, the diagnosis of malignant otitis externa should be considered in any patient with persistent ear pain or otorrhea who does not respond to conventional treatment for external otitis.

Adjuvant hyperbaric oxygen in malignant external otitis.Arch Otolaryngol Head Neck Surg. 1992 Jan;118(1):89-93.

Necrotizing invasive pseudomonal infection of the external auditory canal (malignant external otitis) is an uncommon but important disorder in the elderly. The high morbidity, and even mortality, of this disorder has been reduced by the early and intensive use of combination antipseudomonal antibiotics. However, in severely immunocompromised patients or in infection involving the base of the skull, multiple cranial nerves, or the meninges, conventional therapy has been prolonged, intensive, and relatively ineffective. We treated 16 patients with malignant external otitis with adjuvant hyperbaric oxygen therapy. In six patients, infection was in advanced stages, infections were recurrences after previous treatment, and repeated treatment with antipseudomonal antibiotics had failed. All 16 cases responded promptly when a 30-day course of hyperbaric oxygen was added to the antibiotic regimen, and all patients remained free of infection or neurologic deficit during 1 to 4 years of follow-up. No complications of this treatment modality were noted. Hyperbaric oxygen therapy reverses tissue hypoxia, which enhances phagocytic killing of aerobic microorganisms, and stimulates neomicroangiogenesis. In addition, hyperbaric oxygen augments the action of aminoglycoside antibiotics. Adjuvant hyperbaric oxygen therapy should be considered in advanced or recurrent cases of malignant external otitis.

                   

 
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Necrotizing 'malignant' external otitis caused by Staphylococcus epidermidis.Arch Otolaryngol Head Neck Surg. 1992 Jan;118(1):94-6.

Necrotizing "malignant" external otitis is a life-threatening skull base infection that originates in the external auditory canal and is characterized by otalgia and purulent aural discharge with external auditory canal cellulitis and granulation. Necrotizing external otitis, seen almost exclusively in elderly diabetics, is almost always caused by Pseudomonas aeruginosa. To our knowledge, there have been only six nonpseudomonal cases reported to date. We describe a 70-year-old diabetic man with necrotizing external otitis caused by Staphylococcus epidermidis, confirmed by serial cultures. This case was characterized by otalgia, purulent otorrhea, preauricular swelling, bony external auditory canal erosion, and a conductive hearing loss. Despite prolonged intravenous antistaphylococcal antibiotic therapy and frequent local débridement, the patient's symptoms never completely resolved. As demonstrated by the treatment failure, S epidermidis necrotizing external otitis, may represent a more refractory form of this already virulent disease process. We believe this to be the first reported case of necrotizing external malignant otitis caused by S epidermidis.

Malignant otitis externa (2 cases).Tunis Med. 1993 Nov;71(11):541-5.

Malignant external otitis is a progressive necrotizing otitis. It's a rare severe and evolutive clinical entities, old diabetics are the most victim. Pseudomonas aeruginosa is the bacteria responsible in the most cases. Prognostic vital can be affected, treatment must be energic, rapid and well adapted. The authors report two old diabetic women presenting a malignant external otitis and discuss etiopathogenic, physiopathologic, diagnosis and treatment of this illness.


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