Inflammatory Conditions and Systemic Diseases Affecting the Middle Ear

Eosinophilic Otitis Media

Eosinophilic otitis media (EOM) is a relatively new disease entity. EOM is characterized by a persistent otitis media, with or without suppuration, that is resistant to conventional treatment, in association with adult-onset bronchial asthma, often with nasal polyposis.2

Clinical suspicion should be raised by the discovery on myringotomy of a highly viscous middle ear effusion that is difficult to suction. The underlying middle ear mucosa is thickened and has eosinophil-rich granulation tissue that responds only to high-dose systemic steroids. This should prompt a biopsy. A characteristic of EOM is the high incidence of hearing loss, which is typically a progressive loss but can also be sudden.3

A significant number of patients with EOM have patulous Eustachian tubes, resulting in speculation that the pathological mechanism of EOM is due to the aspiration of antigenic material into the middle ear, causing a localized eosinophilic inflammatory reaction. Whether this is the cause or a sequelae of EOM remains to be established.4

Iino, Tomioka-Matsutani, Matsubara, Nakagawa, and Nonaka has published the largest cohort of EOM patients2 and, with the EOM study group, proposed a diagnostic criteria system for this disease (▶ Table 12.1). In accordance with the diagnostic criteria, investigation mandates the histological examination of the middle ear contents and exclusion of hypereosinophilic syndrome and Churg-Strauss syndrome . Hypereosinophilic syndrome is diagnosed by the presence of a persistent eosinophil count ≥1.5 × 109/L for at least 6 months.2 The diagnosis of Churg-Strauss syndrome will be discussed later. The mainstay of management is early recognition and the commencement of high-dose systemic steroids2 and topical intratympanic steroids.5 Rapid resolution of symptoms and reversal of sensorineural hearing loss has been reported.

Table 12.1 Unvalidated diagnostic criteria system established by the EOM study group

Major (Mandatory)

Otitis media with effusion with an eosinophil-dominant effusion

Minor (2/4 required)

1. Highly viscous middle ear effusion

2. Resistance to conventional treatment for otitis media

3. Association with bronchial asthma

4. Association with nasal polyposis

Exclusion Criteria

1. CSS

2. Hypereosinophilic Syndrome

12.3 Autoimmune Vasculitis

Churg-Strauss syndrome and granulomatosis with polyangitis are closely related autoimmune vasculitic syndromes that are characterized by granulomatous infiltration of small- to medium-sized vessels and are associated with antibodies to neutrophil cytoplasmic antigens (ANCA). These autoimmune vasculitis commonly present with head and neck symptoms; rarely will they present with isolated otologic symptoms. Typical middle ear findings include a thick middle ear effusion on myringotomy, with middle ear granulation that is again resistant to typical treatment. Often, otalgia is significantly worse than would be expected due to middle ear involvement in the granulomatous process.

12.3.1 Granulomatosis with Polyangitis

Granulomatosis with polyangitis (GPA), previously known as Wegener’s granulomatosis, is the most common autoimmune vasculitis to present with otologic symptoms. It can present either as a classic form with all components of the disease or a limited form. Classic GPA typically presents in young adult males and is characterized by a necrotizing, granulomatous vasculitis of the upper and lower respiratory tract, focal glomerulonephritis, and small vessel vasculitis. Because manifestations of GPA in the nose are typically more prominent than manifestations in the ear, the otologist should be alerted when typical GPA findings, such as nasal septal perforation, sinonasal synechiae, saddle nose deformity, friable sinonasal mucosa, and/or polypoid rhinosinusitis, are encountered alongside chronic ear disease. Symptoms of COM are reported in greater than 20% to 40% of patients, but in isolation are quite rare.6,​7 The expected conductive hearing loss may be associated with a sensorineural component and facial nerve palsy that is reversible with steroids.8

Diagnosis is based upon the criteria set forth in 1990 by the American College of Rheumatology (▶ Table 12.2) and laboratory investigations. Greater than 90% of GPA patients have been reported to have ANCA positivity during active disease,9 specifically cytoplasmic-pattern ANCA (c-ANCA) directed against serine proteinase 3 antigen. Establishing a diagnosis of GPA can be difficult early in the disease process given that it is unusual to meet all the criteria shown in ▶ Table 12.2 and, although the c-ANCA is valuable, it is often normal during the early stages of the disease. Therefore, the most critical investigation for early diagnosis of GPA is histological examination of the middle ear contents for evidence of a perivasculitis of small vessels, infiltration of giant cells, and epithelioid cell granulomas.10 However, histopathologic diagnosis is most often made based on a biopsy of an affected sinonasal site.

Table 12.2 1990 criteria for the classification of GPA.

  • Nasal or oral inflammation – Mucosal ulceration with classical appearance of “saddle nose deformity”

  • Abnormal chest radiography with pulmonary lesions

  • Urinary Sediment – Microhematuria or red cast cells

  • Histological – Vasculitis of small vessels, infiltration of giant cells and epithelioid granulomas

Note: A patient is said to have GPA if at least 2 of these 4 criteria are present. These criteria were established before the introduction of c-ANCA.

12.3.2 Churg-Strauss Syndrome

Like GPA, CSS is a granulomatous vasculitis associated with ANCA autoantibodies. Churg Straus Syndrome typically presents with adult-onset asthma, allergic rhinitis often associated with polyposis, a peripheral neuropathy, and systemic eosinophilia.11 There are no reports of presentation with primary otologic symptoms. Excessive granulation tissue is present within the middle ear. Hearing loss generally involves a bilateral mixed loss but can be an isolated sensorineural hearing loss or unilateral in some cases.12

The natural history of the disease is that it mostly occurs in males and has three distinct phases. The first is characterized by a severe, steroid-dependent asthma, which commonly occurs in adulthood and is preceded by chronic recurrent rhinosinusitis. The second phase has a peripheral eosinophilia with eosinophilic organ infiltrates. The third phase is characterized by the appearance of a systemic vasculitis. During these later stages, the asthmatic symptoms generally resolve. It is later in the course of the illness that otologic symptoms present, hence the need for a thorough past medical history.12

Diagnosis is based on the criteria of the American College of Rheumatology, with four of six criteria required (▶ Table 12.3). Unlike GPA, CSS is more commonly associated with perinuclear-pattern ANCA (p-ANCA) directed against myeloperoxidase (MPO) although this is a less reliable test than for GPA.13

Table 12.3 Criteria for diagnosis of Churg-Strauss Syndrome.

  • Asthma

  • Eosinophilia (>10% On white blood cell differential)

  • Migratory Pulmonary infiltrates

  • Neuropathy (Mono/Polyneuritis)

  • Clinical/Radiological Evidence of Sinusitis

  • Biopsy demonstrating extravascular eosinophils

Note: Diagnosis is based on the criteria of the American College of Rheumatology, with 4 of 6 criteria required to achieve sensitivity of 85% and specificity of 99.7%. Data from Masi T, Hunder G, Lie J, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss Syndrome. Arthritis Rheumatology. 1990; 33:1094–1100

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Dec 14, 2016 | Posted by in OTOLARYNGOLOGY | Comments Off on Inflammatory Conditions and Systemic Diseases Affecting the Middle Ear

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