Congenital Middle Ear Cholesteatoma

Kim, Sung Won; Jung, Min Kyo; Kwun, Yong Sig; Kang, Jun Myung; Chang, Ki Hong; Yeo, Sang Won; Suh, Byung Do

Abstract

BACKGROUND AND OBJECTIVES: Congenital middle ear cholesteatoma (CMEC) is a keratinous mass behind an intact tympanic membrane. CMEC does not have a history of instrumentation and is less common than acquired one. Many theories have been put forward to explain the pathophysiology of CMEC, however, none of these so far have been convincingly proven. This clinical study was performed to investigate the characteristic features of CMEC and to evaluate the correlation between pathophysiology and CMEC by retrospectivly reviewing the cases. MATERIALS AND METHOD: The medical records of patients who underwent otologic procedures at the hospitals of the Catholic university from January 1993 to September 1998 have been reviewed. They were ten males and four females, ranging in age from 4 to 59 (mean age 18).
RESULTS
Three of the 14 patients had the lesions isolated to the anterosuperior quadrant of the mesotympanum which were cystic, easily removed and did not affect hearing. The others had more serious condition with extension into the posterior mesotympanum, which were large, often too extensive to indicate a formative site, and causing ossicular damage.
CONCLUSION
CMEC presents in two distinctive forms according to the site of formation: the anterosuperior and posterior mesotympanum. The review suggest that the pathophysiology of posterior lesions may be different from anterior ones. For early diagnosis of CMEC, screening program should be carried out in children to prevent the more extensive diseases.