The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma

Purpose: Congenital middle ear cholesteatoma (CMEC) is less common than the acquired variety. Many theories have been put forward to explain the pathophysiology of CMEC; however, none of them have been convincingly proven thus far. This clinical study was performed to analyze the case reviews; assess the characteristic features of CMEC, and evaluate the correlation between those features and the pathophysiology of CMEC. Methods: The medical records of patients who underwent otologic procedures at the hospitals of the Catholic University, Seoul, Korea, from January 1993 to November 1998 have been reviewed. Results: Of the 14 patients, 3 had cystic lesions isolated to the anterosuperior quadrant of the mesotympanum. The lesions were easy to remove and did not affect the patients' hearing. Eleven patients had more extensive disease with posterior mesotympanum involvement; the lesions were large, were often too extensive to indicate a formative site, and caused ossicular damage. Conclusion: CMEC manifests in 2 distinctive forms according to the formation site. This distinctive formation site suggests that the pathophysiology of posterior lesions may be different from that of anterior ones. For early diagnosis of CMEC, a screening program should be established for children to prevent more extensive disease. (Am J Otolaryngol 2001;22:184-189. Copyright © 2001 by W.B. Saunders Company)