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Hagen Frickmann,Andreas Erich Zautner*
The article provides an overview on the current state-of-science of middle ear cholesteatoma, a non-neoplastic, keratinizing lesion that is characterized by the proliferation of epithelium with aberrant micro-architecture. Pathogenetic mechanisms including morphological, immunological, epidemiological and microbiological aspects of the disease are summarized. The importance of penicillinase-expressing anaerobic bacteria and biofilm formation for maintaining the chronic middle ear inflammation is stressed. Nevertheless, the role of the isolated pathogens in the primarily non-sterile compartiment of the middle ear cavity is so far not completely understood and data on the isolated species are contradictious. Heredity was demonstated for some variants of the disease. Therefore, further studies on the etiological role of microbial agents and potential benefits of resistance-adapted antimicrobial therapy seem advisable. Local and systemic complications of the potentially life-threatening disease like conductive and sensorineural hearing loss and cranial abscesses are reported. The prognosis is limited due to frequent recurrence in spite of surgical therapy. Further research is necessary for a better understanding of the pathogenetic mechanisms and to expand the sectrum of therapeutic options.