Diagnosis and Treatment of Congenital Temporal Bone Cholesteatoma

CHEN Xihang; CHENG Jinmei; LIN Chang; YE Shengnan; LIN Gongbiao; YI Zixiang; ZHANG Rong

doi:10.3969/j.issn.1672-2922.2014.03.031

Abstract：Objective To investigate the location, range of involvement,size,clinicial symptoms and imaging data of congenital cholesteatoma in temporal bone, to report authors’experiences with its differential diagnosis and surgical treat-ment, as well as complications including damage to function of the facial nerve . Methods This was a retrospective review of ten cases of congenital temporal bone cholesteatoma treated from January 1999 to April 2013. Clinical data and treatment evaluate were evaluationed. Results The age of the 10 patients (7 males and 3 females) ranged from 1 to 57 years. The lesion involved the left ear in 5 cases and right ear in 5 cases. The lesion was located in the mastoid area in 6 cases (including 1 showing external auditory canal wall damage), and the petrous bone in 4 cases (with also facial paralysis). Intraoperative cere-brospinal fluid leakage in 1 case, which was repaired during surgery. Modified canal wall down tympanomastoidectomy was performed in 9 cases, including 1 with simultaneous facial nerve decompression and tympanoplasty. Canal wall up tympano-mastoidectomy with simultaneous cochlear implantation was performed in 1 case, with satisfactory postoperative training out-comes. No residual or recurrent cholesteatoma was found on CT scan 1 year after operation. Conclusions For congenital cho-lesteatoma, early detection through history, audiology and imaging improves differential diagnosis and makes early diagnosis and selection of appropriate surgical approach possible. Particularly in congenital petrous bone cholesteatoma with facial pa-ralysis or other complications,CT,MRI and other imaging data improve early diagnosis and treatment, and help the surgeon avoid wrong or missed diagnosis.