title: Open-type congenital cholesteatoma: differential diagnosis for conductive hearing loss with a normal tympanic membrane.
abstract: CONCLUSIONS
  In patients with progressive conductive hearing loss and a normal tympanic membrane (TM), and with soft tissue density in the middle ear cavity (MEC) on temporal bone computed tomography (TBCT) scan, open-type congenital cholesteatoma (OCC) should be highly suspected and a proper surgical plan that includes mastoid exploration and second-stage operation is required.
  
  
  OBJECTIVE
  The clinical presentation of OCC is very similar to congenital ossicular anomaly (COA) presenting with a conductive hearing loss with intact TM. Therefore, it is challenging to make a correct preoperative diagnosis in patients with OCC. We evaluated the clinical characteristics of OCC compared with those of COA to find diagnostic clues useful in diagnosis of OCC.
  
  
  METHODS
  The medical records of 12 patients with surgically proven OCC and 14 patients with surgically proven COA were reviewed for demographic data, otologic history, preoperative TBCT findings, intraoperative findings, and pre- and postoperative audiologic data.
  
  
  RESULTS
  There was no difference between OCC and COA based on demographic data, preoperative hearing, and ossicular status on TBCT. However, the presence of progressive hearing loss, soft tissue density in the MEC on TBCT scan, and the need for mastoid surgery and second-stage operation were significantly more frequent in OCC patients.
authors.affiliation: Department of Otorhinolaryngology - Head and Neck Surgery, Jeju National University School of Medicine, Jeju National University Hospital, Jeju, South Korea.
authors.forename: Se-Hyung
authors.initials: SH
authors.lastname: Kim
authors.forename: Yang-Sun
authors.initials: YS
authors.lastname: Cho
authors.forename: Ho-Suk
authors.initials: HS
authors.lastname: Chu
authors.forename: Jeon-Yeob
authors.initials: JY
authors.lastname: Jang
authors.forename: Won-Ho
authors.initials: WH
authors.lastname: Chung
authors.forename: Sung Hwa
authors.initials: SH
authors.lastname: Hong
mesh_terms: D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D002648:Child; D018424:Cholesteatoma, Middle Ear / Q000150:complications / Q000151:congenital / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004429:Ear Ossicles; D005260:Female; D005500:Follow-Up Studies; D006309:Hearing; D006314:Hearing Loss, Conductive / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014432:Tympanic Membrane / Q000033:anatomy & histology*; D055815:Young Adult