[Clinical treatment of congenital middle ear cholesteatoma in children]

Objective:To summarize the clinical characteristics and clinical treatment of congenital cholesteatoma (CC) of the middle ear in children, provide early diagnosis methods and explore standardized diagnosis and treatment plan.Method:A retrospective chart review of 94 patients with a diagnosis of middle ear cholesteatoma, in Beijing Children's Hospital, between 2009 and 2015 was carried on. 14 patients with CC were identified using the criteria proposed by Levenson, and were divided into two groups according to the course of disease. The course of disease in group A was less than 3 months, and group B was more than 3 months. The main complains, diagnostic methods and Potsic's stage of temporal bone CT findings were recorded.Result:①The age of 14 cases of congenital cholesteatoma of the middle ear ranged from 3.33 to 10.17 years, with the median age of 7.20 years. ②Hearing loss (13/14, 92.86%) was the most common complain. Finding methods included hearing screening and CT scan (11/14, 78.57%), tympanotomy (2/14, 14.28%) and otoscopic examination (1/14, 7.14%). ③There were 6 people in group A and 8 in group B. According to Potsic's grading standard, the difference between the two groups was statistically significant (P=0.043). ④The preoperative Air-Bone conduction threshold Gap (ABG) in A and B two groups were (38.10±7.43) dB and (42.09±9.96) dB, respectively, and there was no significant difference in analysis (P=0.427). ⑤The difference between pre-ABG and post-ABG ［(36.26±5.56）dB and （21.70±3.80）dB, P=0.004］ was significant. Canal wall up mastoidectomy was the preferred procedure and 11/14 (78.57%) patients had this surgery done.Conclusion:The shorter the course of disease, the lower the stage of cholesteatoma of the middle ear indicates the importance of early detection. But congenital cholesteatoma is more occult, and even within 3 months, cholesteatoma can cause severe damage to the hearing and middle ear structure. Early screening programs can recommend hearing screening and CT scan to facilitate early intervention.