Learning Objectives:

Objectives: To analyzed the clinical features and surgical findings in 29 cases of congenital cholesteatoma of the middle ear.

Methods: 29 patients (30 ears) who underwent surgery for congenital cholesteatoma between September 2012 and January 2016 were involved in this retrospective study. The otoscope examination, HRCT and audiogram were routinely done before surgery. The location and type of cholesteatoma, the surgical procedures, and the results were evaluated. Patient who had prior middle ear diseases were excluded.

Results: Of these 29 patients, the median age was 12 years (ranged from 3 to 54 years old). Hearing loss was complained of as a main symptom, and conductive hearing loss was found in 26 cases with the AB-gap were about 35.8 dB HL pre-operation. Two of these patients were misdiagnosed as secret otitis media in other hospital. Nine (30%) patients had closed-type cystic masses, while other 21 (70%) had open-type lesions. Canal wall down technique was performed in 20 cases, while Canal wall up in 1cases, and Trans-canal approach was applied in 9 cases of which the lesion was limited in the mesotympanum. The erosion of the ossicular chain was found in 28 cases during the surgery. Ossicular chain reconstruction were done in 21 cases(TORP13, PORP 3, Cartilage 5), and the hearing thresholds were improved satisfied after more than 1 year follow up.

Conclusions: Congenital cholesteatoma of middle ear was a rare disease and usually delayed to be diagnosed in clinical practice. Conductive hearing loss was the most common symptom of these patients. There were different clinical features between the open-type and the closed-type lesion, which reflect a distinct pathogenesis. Surgery was the best choice when the middle ear congenital cholesteatoma was identified.