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Congenital Middle Ear Abnormalities With Absence of the Oval Window: Diagnosis, Surgery, and Audiometric Outcomes

Su, Yu; Yuan, Hu; Song, Yue-shuai; Shen, Wei-dong; Han, Wei-ju; Liu, Jun; Han, Dong-yi; Dai, Pu

doi: 10.1097/MAO.0000000000000277
Middle Ear and Mastoid Disease

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.

Materials and 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.

Conclusion 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.

Department of Otolaryngology–Head and Neck Surgery, The PLA General Hospital, Beijing, People’s Republic of China

Address correspondence and reprint requests to Dong-yi Han, M.D., Ph.D., or Pu Dai, M.D. Ph.D., Department of Otolaryngology–Head and Neck Surgery, The PLA General Hospital, 28 Fuxing Rd, Beijing 100853, People’s Republic of China;;

Y.S., H.Y., and Y.-S.S. should be considered cofirst authors.

This work was supported by Chinese National Nature Science Foundation Research Grant (81070792), the National Basic Research Program of China (973 Program) (No. 2013CB945402) to H.D.. The Project of the National Natural Science Foundation of China (Grant No. 81230020), a grant from State 863 High Technology R&D Key Project of China (2011AA02A112), a grant from Natural Science Foundation of Beijing (Grant No. 7122172), a grant from Minister of Science and Technology of China (2012BAI09B02) and a grant from Minister of Health of China (201202005) to P.D., China postdoctoral granted financial support and special financial grant (Nos. 20120481482 and 201104779) to Y.S.

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