title: Congenital middle ear abnormalities with absence of the oval window: diagnosis, surgery, and audiometric outcomes.
abstract: OBJECTIVE
  Congenital absence of the oval window (CAOW) is a rare condition in which the stapes footplate fails to develop, resulting in a significant conductive hearing loss in the affected ear. The purpose of this study was to describe the surgical management and outcomes of patients with CAOW undergoing the oval window drill-out (OWD) procedure.
  
  
  METHODS
  A retrospective chart review of patients with CAOW between 1996 and 2011 was performed. Clinical data of patients who underwent OWD were collected. Seventy-nine patients (103 ears) were confirmed using exploratory tympanotomy as having congenital stapes anomalies and CAOW without any anomalies of the tympanic membrane and external auditory canal. Demographic data, CT findings, operative findings, complications, and preoperative/postoperative audiometry data of patients who underwent OWD were collected. The preoperative and postoperative audiologic findings were analyzed in 42 patients (56 ears) with complete data.
  
  
  RESULTS
  Hearing restoration surgery was aborted for various reasons in 14 cases. Six patients underwent revision operations for worsening hearing after their first surgery. The average preoperative 4 tone air conduction threshold was 67 dB; the average 6-month postoperative four tone air conduction threshold was 49 dB, and the average postoperative hearing gain was 18 dB. For the 56 ears, the average 4 tone air conduction threshold 6 months after surgery was significantly lower than the preoperative threshold.
  
  
  CONCLUSIONS
  The oval window drill-out procedure is a viable operation for patients with congenital absence of the oval window, and it is important for surgeons to develop personalized treatment programs to improve patients' hearing with minimal complications.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, The PLA General Hospital, Beijing, People's Republic of China.
authors.forename: Yu
authors.initials: Y
authors.lastname: Su
authors.forename: Hu
authors.initials: H
authors.lastname: Yuan
authors.forename: Yue-shuai
authors.initials: YS
authors.lastname: Song
authors.forename: Wei-dong
authors.initials: WD
authors.lastname: Shen
authors.forename: Wei-ju
authors.initials: WJ
authors.lastname: Han
authors.forename: Jun
authors.initials: J
authors.lastname: Liu
authors.forename: Dong-yi
authors.initials: DY
authors.lastname: Han
authors.forename: Pu
authors.initials: P
authors.lastname: Dai
mesh_terms: D000293:Adolescent; D000328:Adult; D001299:Audiometry; D002648:Child; D004424:Ear Canal / Q000002:abnormalities / Q000601:surgery; D004432:Ear, Middle / Q000002:abnormalities* / Q000601:surgery*; D005260:Female; D006309:Hearing / Q000502:physiology; D006314:Hearing Loss, Conductive / Q000151:congenital / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013199:Stapes / Q000002:abnormalities*; D013201:Stapes Surgery / Q000379:methods*; D016896:Treatment Outcome; D014432:Tympanic Membrane / Q000002:abnormalities / Q000601:surgery; D055815:Young Adult