RESUMO
[ABSTRACT]OBJECTIVETo investigate the methods of diagnosing and treating congenital middle ear cholesteatoma and causes of misdiagnosis.METHODSThis was a retrospective review of 9 cases of congenital middle ear cholesteatoma in this hospital.RESULTS9 patients all received operative treatment and definite diagnosis was made by histopathologic investigation. The ossicular reconstruction was performed to 6 patients. All of the cases were followed-up for 3 months-2.5 years. A-B gap was 20 dB. After 1 year, no residual or recurrence of cholesteatoma was found in CT scan. All patients had been misdiagnosed. Misdiagnosis rate was 100%.CONCLUSIONCongenital middle ear cholesteatoma is a clinically rare and diagnosis is usually delayed in clinical practice due to the silent nature of the disease in its early stage. Preoperative diagnosis may be based on Levenson diagnostic criteria and CT imaging. Early surgical treatment can obtain a good effect of hearing reconstruction. To avoid misdiagnosis and incorrect treatment, radiological evaluation and careful local examination are really important, which may also avoid serious complications.