Learning Objectives:
Objective: To investigate the clinical features of Congenital Cholesteatoma (CC) of middle ear in children and provide methods for early diagnosis.
Methods: 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 Pariser, and were divided into two groups according to the course of disease. The main complains, diagnostic methods and Potsic's stage of temporal bone CT findings were recorded.
Results:
1) 11/14 (78.57%) patients with CC were boys, with a median age of 7.20 years (from 3.33 to 10.17 years).
2) Hearing loss (13/14, 92.86%) was the most common complain. Finding methods included hearing test and CT scan (11/14, 78.57%), tympanotomy (2/14, 14.28%) and otoscopic examination(1/14,7.14%).
3) The preoperative Air-Bone conduction threshold Gap (ABG) was 40.46 ± 8.86 dB. According to the course of disease, patients with CC were divided into two groups (A 6/14, B 8/14). There was no significant difference in preoperative ABGs (38.10 ± 7.43 dB & 42.09 ± 9.96 dB, P = 0.427).
4) According to Potsic's stage among A, B groups(P = 0.043).
5) Modified canal wall up mastoidectomy was the preferred procedure and 11/14 (78.57%) patients had this surgery done. In 6 patients followed up, the difference between pre-ABG and post-ABG(36.26 ± 5.56 dB & 21.70 ± 3.80 dB, P = 0.004) was significant.
Conclusion: CC of middle ear in children happened more in boys than in girls. Hearing loss was the most common complaint. The shorter was the course of disease , the less damage of the structure of middle ear would be. Hearing test, CT, tympanotomy and otoscopic examination were usually used for diagnosis.
