Mastoiditis and facial paralysis as initial manifestations of temporal bone systemic diseases - the significance of the histopathological examination.

UNLABELLED: Several systemic diseases, including granulomatous and infectious processes, tumors, bone disorders, collagen-vascular and other autoimmune diseases may involve the middle ear and temporal bone. These diseases are difficult to diagnose when symptoms mimic acute otomastoiditis. CASE REPORTS: The present report describes our experience with three such cases initially misdiagnosed. Their predominating symptoms were otological with mastoiditis, hearing loss, and subsequently facial nerve palsy. The cases were considered an emergency and the patients underwent tympanomastoidectomy, under the suspicion of otitis media with cholesteatoma, in order to remove a possible abscess and to decompress the facial nerve. The common features were the presence of severe granulation tissue filling the mastoid cavity and middle ear during surgery, without cholesteatoma. The definitive diagnoses was made by means of biopsy of the granulation tissue from the middle ear, revealing granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) in one case, middle ear tuberculosis and diffuse large B-cell lymphoma respectively. After specific associated therapy facial nerve functions improved, and atypical inflammatory states of the ear resolved. CONCLUSIONS: As a group, systemic diseases of the middle ear and temporal bone are uncommon, but aggressive lesions. After analyzing these cases and reviewing the literature, we would like to stress upon the importance of microscopic examination of the affected tissue, required for an accurate diagnosis and effective treatment.

Silent sinus syndrome--report of a case.

INTRODUCTION: The "silent sinus syndrome" is a rare entity that was first described in 1964 and given this name 30 years later. Although it is well described both from clinically and radiologically point of view we consider that its rarity still makes it a subject for report. CASE PRESENTATION: A 46-year-old patient was admitted for facial asymmetry, diplopia, unilateral left enophthalmos, and inferior displacement of the eye globe, and decreased occlusal pressure in left dentate region. CT scan revealed interior bulging of all left maxillary sinus walls with osteolysis and intense opacification, enlargement of the left middle meatus especially in the posterior part and lateralization of the uncinate process. Nasal endoscopy with 00 rigid scope visualized mild deviation to the right of the nasal septum, enlargement of the left middle meatus by the lateral deviation of the left intersinusal septum and uncinate process. Surgery was scheduled and performed a left maxillary sinus antrostomy. Histopathological examination on the biopsies revealed inflammation. A complete study was performed to assess the elements of inflammation. Postoperative course was simple. Follow-up visit at three and six months, showed significant regression of diplopia and improved facial aspect. CONCLUSIONS: Silent sinus syndrome is a well-defined clinical entity with characteristic imagistic findings. Surgical intervention that restores sinus drainage will interrupt the pathogenesis of the disease and lead to its progressive regression. Topographic associations and density of inflammatory elements analyzed in relation with neoforming vessels suggest their implication in reparatory angiogenesis characteristic to chronic inflammation. Modulating activity in the frame of inflammatory process, of the T-lymphocytes and especially of T-lymphocytes may represent a target for the therapeutic management. Surgery can and should be performed by an endoscopic approach.

Molecular biology of cholesteatoma.

Cholesteatoma is a non-neoplastic, keratinizing lesion, characterized by the proliferation of epithelium with aberrant micro-architecture into the middle ear and mastoid cavity. The exact pathogenic molecular mechanisms behind the formation and propagation of cholesteatoma remain unclear. Immunohistochemical examinations of the matrix and perimatrix have considerably improved the knowledge of cholesteatoma pathogenesis. In this review, the current concepts of cholesteatoma pathogenesis are discussed. Currently, the most widely acknowledged pathogenesis of acquired cholesteatoma is the theory that negative pressure, dysfunction of the Eustachian tube, causes a deepening retraction pocket that, when obstructed, desquamated keratin cannot be cleared from the recess, and a cholesteatoma results. Local infection leads to a disturbance of self-cleaning mechanisms, with cell debris and keratinocytes accumulate inside the retraction pocket, and this is followed by an immigration of immune cells, i.e., Langerhans' cells, T-cells, macrophages. There is an imbalance and a vicious circle of epithelial proliferation, keratinocyte differentiation and maturation, prolonged apoptosis, and disturbance of self-cleaning mechanisms. The inflammatory stimulus will induce an epithelial proliferation along with expression of lytic enzymes and cytokines. Bacteria inside the retraction pocket produce some antigens, which will activate different cytokines and lytic enzymes. These cytokines lead to activation and maturing of osteoclasts with the consequence of degradation of extracellular bone matrix and hyperproliferation, bone erosion and finally progression of the disease. Further research is necessary for a better understanding of the pathogenetic mechanisms and to expand the spectrum of therapeutic options.