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Middle Ear, Granulomatous Diseases
Updated: Apr 28, 2006
Introduction
Granulomatous diseases comprise a small but important subset of diverse ear problems. By definition, a granuloma is a nodular inflammatory lesion. They are usually small and consist primarily of compact mononuclear phagocytes. Granulomas are different from other inflammatory reactions; they represent an ultimately unsuccessful localized attempt to rid the host of an offending organism or process. Granulomatous diseases in the middle ear may be localized primarily to the ear and surrounding tissues, or they may be a manifestation of a body-wide disseminated problem.
Granulomatous reactions may mimic other far more common middle ear diseases. The common presentation of a draining ear is nearly indistinguishable from that of common otitis media. A high index of suspicion and knowledge about possible causes are required for accurate diagnosis and effective treatment.
Middle Ear Tuberculosis
Description
Rates of infection with Mycobacterium tuberculosis have decreased dramatically over the last century, although pockets of resurgence exist. Tuberculosis usually is due to M tuberculosis, but Mycobacterium avium and Mycobacterium fortuitum may be found in rare cases. Concomitant pulmonary involvement is nearly always present, but isolated middle ear involvement has been reported. Inoculation is thought to occur by means of direct transmission through the eustachian tube, but aerosol contact with the tympanic membrane may be the mode of spread in rare cases. The presence of a perforation or myringotomy increases the risk of inoculation.
Diagnosis
The diagnosis of middle ear tuberculosis may be delayed because of its similarity to other forms of otitis media in the early stages. A thickened tympanic membrane and exudate are the first manifestations. Later, the typical multiple perforations, along with an exuberant polypoid reaction and bony sequestration, may occur. Otitis media with early involvement of vestibular, auditory, or facial nerve function is highly suggestive of middle ear tuberculosis.
A search for pulmonary involvement always should be undertaken if tuberculosis is suspected. Presence of nontender cervical adenopathy should raise the index of suspicion. Accurate diagnosis depends on the results of skin testing, demonstration of acid-fast bacilli in histologic preparations, and culture results.
Treatment
Treatment consists of standard systemic antituberculosis chemotherapy. If bony sequestra are identified, surgical extirpation may be required. In the United States, reporting these cases to the Centers for Disease Control and Prevention (CDC) is mandatory.
Wegener Granulomatosis
Description
Wegener granulomatosis is an autoimmune, granulomatous, necrotizing vasculitis. Like tuberculosis, it rarely is isolated to the middle ear; it usually has systemic manifestations, especially pulmonary and renal manifestations.
Diagnosis
Presentation in the ear is nonspecific. Serous exudates and otalgia are common. The presence of accompanying sinonasal disease is nearly universal. Multiple tympanic membrane perforations may be present, and these may coalesce into a large single perforation.
Wegener granulomatosis is differentiated from tuberculosis by both the presence of necrotizing vasculitis in histologic preparations and characteristic systemic manifestations. Other histologic features include microabscesses and scattered multinucleated giant cells in a highly inflammatory background. Serum antibodies to neutrophil cytoplasmic antigens (ANCAs) may be present, and the erythrocyte sedimentation rate is nearly always elevated.
Treatment
Treatment consists of the administration of systemic corticosteroids and chemotherapeutic agents such as cyclophosphamide. Remission rates are as high as 75%, but relapses are common (Yi, 2001).
Langerhans Cell Histiocytosis (histiocytosis X)
Three related diseases make up histiocytosis X: eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease. Although the diseases share the common finding of histiocytic granulomas, they have markedly different clinical courses. Findings of recent histologic structure studies show that the Langerhans cell line proliferates and predominates; as a result, some advocate a shift in nomenclature to Langerhans cell histiocytosis.
Eosinophilic granuloma
Description
Manifesting as solitary osteolytic granulomas, these lesions may occur in almost any bone in the body, including the temporal bone. Temporal bone lesions may present as a draining, perforated eardrum that fails to respond to normal treatment. As such, it is often misdiagnosed as an infectious process, potentially leading to treatment delays. A slight male preponderance exists, and the disease occurs primarily in children and young adults.
Diagnosis
Radiologic studies, CT scan, or plain films depict the typical punched-out lytic bone lesion and should be considered when recalcitrant, nonresponding otorrhea exists. Biopsy offers a definitive diagnosis.
Treatment
Direct surgical extirpation or curettage is the preferred method of treatment. Low-dose irradiation may also be used as an adjuvant in selected cases. A radiologic search should be made for other skeletal lesions
Hand-Schüller-Christian disease
Description
This more-aggressive subset involves multifocal eosinophilic granulomas that may spread beyond the skeleton to involve the skin and lymphatics. Occurring primarily in pre–school-aged children, it may present as a solitary lesion (see above) but manifests multiple lesions in a few months.
Diagnosis
Initial presenting symptoms are nonspecific and similar to those of viral upper respiratory tract infection (URI). They may include fever, lassitude, adenopathy, otitis media, and splenomegaly. Twenty-five percent of patients exhibit the triad of multiple lytic skull lesions, exophthalmos, and diabetes insipidus due to pituitary involvement. The chest radiograph may show hilar adenopathy.
Treatment
Low-dose chemotherapy is used to control symptoms. A cure is unlikely, but spontaneous regression may occur.
Letterer-Siwe disease
This disease is a disseminated form of Langerhans cell histiocytosis that occurs in very young children. It is fulminating and often fatal. If the lesion is in the temporal bone, draining otitis media is the most common presentation. The actual nature of the disease is indicated by lytic lesions, as seen on radiographs. Treatment is chemotherapy and curettage. Low-dose irradiation is reserved for difficult-to-reach areas.
Sarcoidosis
Description
Sarcoidosis is a systemic and chronic disease with a characteristic histologic finding of noncaseating granuloma. Although it usually affects the lungs, the middle ear may be involved. Sarcoidosis is 10 times more prevalent in blacks than in persons of other races, and it affects females more often than males. Two types of presentations occur: acute and chronic. Acute sarcoidosis develops rapidly and may spontaneously resolve, while the chronic form is the more damaging type, leading to irreparable fibrosis of the pulmonary system.
Diagnosis
Sarcoidosis may affect the pinna and external auditory canal, as well as the middle ear. Granulomas of the middle ear may cause conductive hearing loss due to ossicular erosion and mass effect, but these findings alone are nonspecific. The definitive diagnosis is usually made with chest radiographs; hilar adenopathy is the dominant finding. A chronic cough often leads to chest radiographs being ordered. Other findings include iridocyclitis, keratoconjuctivitis, hepatosplenomegaly myalgias, arthralgias, and lymphadenopathy
Heerfordt syndrome (uveoparotid fever, parotitis, and cranial nerve palsies, especially facial) is seen in patients with sarcoidosis.
Treatment
Corticosteroids are useful in controlling the systemic manifestations.
Syphilis
Description
Syphilis is a sexually transmitted disease caused by the bacteria, Treponema pallidum. The initial lesions are located on or around the genitalia, but if untreated (latent), dissemination may occur (tertiary syphilis). An infected mother may transmit the disease to her child (congenital).
Diagnosis
Infection with T pallidum, congenital or acquired, may manifest as middle ear disease. Early findings include a reddened mucosa and periosteum of the ossicles due to leukocytic infiltration. As larger lesions (gummas) form, infection may present as a perforated tympanic membrane, with drainage and granulomatous changes in the middle ear mucosa.
If gummas invade the inner ear, sensorineural hearing loss and dizziness may occur. Insufflating the ear may cause nystagmus, which mimics a fistula (Hennebert sign).
Syphilitic infection is differentiated from tuberculous infection by means of serologic testing for T pallidum and histologic evaluation for acid-fast bacilli.
Treatment
Intensive antibiotic therapy, coupled with corticosteroids, is effective at eradicating the infection. However, gummatous lesions and their sequelae may persist.
Lyme Disease
Description
Lyme disease is caused by a tick-borne spirochete, Borrelia burgdorferi. After the initial tick bite, a characteristic skin lesion, erythema migrans, occurs in most cases. A viruslike illness occurs as the bacteria spread throughout the body hematogenously. This spread may occur over a few days or a few weeks and may cause headache, fever, migratory arthralgia, adenopathy, and circular red skin lesions, which sometimes are present on the pinna. The last phase involves focal lesions in the joints, heart, or nervous system. The primary otologic manifestation is temporary facial nerve paralysis that is not responsive to steroids. Paralysis is bilateral in as many as 25% of all cases.
Diagnosis
The diagnosis usually is based on a history of a tick bite and the characteristic skin lesion, especially in people from endemic areas. Later diagnosis is based on the recognizable symptom complex and the annular rash. Serologic tests are available.
Treatment
Treatment is the administration of tetracycline. If tetracycline is contraindicated (eg, pregnant patients, patients <12 y), erythromycin, amoxicillin, imipenem, or ceftriaxone may be used.
Fungal Infection
Description
Fungal growths may often be found in otherwise healthy ears as opportunistic colonies growing on accumulated cerumen or keratinous debris. These are harmless and require only local cleaning. However, immunocompromised individuals may have aggressive invasive fungal infections in various regions of the body, including the temporal bone. Chemotherapy, diabetic ketoacidosis, and AIDS compromise the immune defenses of the body, allowing otherwise nonpathogenic fungal organisms to be destructive.
Diagnosis
The presence of hyphae in an immunocompromised individual should alert the examiner to the possibility of a systemic problem. Potassium hydroxide (KOH) preparations may be helpful in identifying the offending organism.
Treatment
Treatment consists of management of the underlying problem and aggressive local debridement. Topical and systemic antifungals are employed as needed.
Keywords
middle ear tuberculosis, Wegener granulomatosis, Wegener's granulomatosis, Langerhans cell histiocytosis, histiocytosis X, sarcoidosis, syphilis, Lyme disease, eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease
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References
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Further Reading
Keywords
middle ear tuberculosis, Wegener granulomatosis, Wegener's granulomatosis, Langerhans cell histiocytosis, histiocytosis X, sarcoidosis, syphilis, Lyme disease, eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease
