Surgery for congenital stapes ankylosis with an associated congenital ossicular chain anomaly

The surgical findings and results are presented on 32 ears with congenital stapes ankylosis with an associated congenital anomaly of the ossicular chain. One third of the patients had a syndromal diagnosis. In 26 ears, stapedectomy could be performed. In 2 other ears, stapes ankylosis to the bony facial canal was mobilized successfully. In the 4 remaining ears, surgical intervention had to be limited to an exploratory tympanotomy for various reasons. The average hearing gain was 23 dB for the 28 ears on which stapes surgery had been performed. A substantial hearing gain of at least 15 dB was achieved in 19 of these 28 ears (68%). The end result was limited to a small extent by an average preoperative sensorineural component of 16 dB in the hearing loss. A review of the findings and results from other larger series in the literature are presented.