Clinical analysis of missed diagnostic congenital cholesteatoma

Objective:The clinical symptoms, imaging features and surgical treatment of congenital cholesteatoma are reported. Method:The clinical data of 20 patients with congenital cholesteatoma diagnosed and treated in our hospital from January 2016 to May 2018 were retrospectively analyzed, including the age of onset, clinical symptoms, signs, audiology, HRCT of temporal bone, surgical methods and so on. Result:In 20 patients with congenital cholesteatoma, the clinical manifestations were hearing loss in the affected ears, including 5 cases with ear fullness and 4 cases with tinnitus. There were 13 cases of local yellow-white shadow of tympanic membrane, 5 cases of normal tympanic membrane and 2 cases of tympanic membrane bombe. Pure tone audiometry showed conductive deafness in 14 cases and mixed deafness in 6 cases. CT scan of temporal bone showed that 15 cases had pneumatic type of mastoid, 4 cases had diploectic type and 1 case had sclerostic type. There were 15 cases of interspersed flocculent shadow and 5 cases of mass shadow. Among the 20 cases of chronic otitis media with cholesteatoma, 6 have cholesteatoma in epitympanum, 4 in tympanic cavity, 3 in tympanic cavity and epitympanum, 2 in mastoid and tympanic antrum, 1 in mastoid, tympanic antrum and tympanic cavity, 1 in tympanic antrum, tympanic cavity and petrous apex, 1 in tympanic cavity, hypotympanum, posterior tympanum and ostium tympanicum tubae auditivae, 1 in aditus ad antrum, epitympanum and posterior tympanum, 1 in tympanic cavity, epitympanum and posterior tympanum. Intact canal wall mastoidectomy and tympanoplasty were performed in 7 cases, open mastoidectomy and tympanoplasty in 5 cases, middle ear exploration and tympanoplasty in 4 cases, atticotomy with reconstruction and tympanoplasty in 3 cases and subtotal temporal bone resection in 1 case. Conclusion:For patients with intact tympanic membrane presenting with hearing loss, the tympanic membranes should be carefully examined, and thin-section CT and MRI of temporal bone should be performed in time to avoid missed diagnosis of congenital cholesteatoma. Once diagnosed, surgery should be performed as soon as possible.