Analysis of Clinical Characteristics of Middle Ear Congenital Cholesteatoma

Background and Objectives: Many studies have been put forward to explain the origin of congenital middle ear cholesteatoma (CMEC) ; however, none of the existing theories so far have convincingly shown the exact pathogenesis of CMEC. The aim of this study was to describe the clinical characteristics of CMEC based on clinical symptoms, radiological evaluation and surgical findings at the site of the lesion. Subjects and Method: The study was conducted retrospectively by examining the medical records of 12 patients diagnosed as having CMEC from August 1996 to October 2004. CMEC was classified into two categories according to the site of lesion : the anterior and the posterior lesion. We compared clinical symptoms of the different sites of lesion and described intraoperative findings with respect to the extent of the disease and ossicular chain involvement. Results: We found 2 cases of anterior lesions and 10 cases of posterior lesions. Of the 2 cases with anterior lesion, one complained of earfullness whereas the lesion in the other one was detected incidentally without any otologic symptoms. Of 10 cases with posterior lesion, 7 cases complained of conductive hearing loss as the most common symptom. With respect to the intraoperative findings, the disease in one of 2 cases with the anterior lesion was confined to the anterosuperior lesion while it extended to the epitympanum in another. Of 10 cases with the posterior lesion, the disease was confined to the posterosuperior lesion in 2 cases while it extended to the epitympanum in 1 case; the remaining 7 had the disease progressed to the mastoid antrum. The ossicular chain involvement was not observed in any of the anterior lesions but found in 9 of the 10 cases with the posterior lesion. Conclusion: The two lesions that differed in the sites of formation showed different clinical symptoms and the extent of disease. Therefore, the present study suggests that they may be explained in terms of disparate theories of pathogenesis rather than explaining them by a single theory. (J Clinical Otolaryngol 2006;17:73–78)