Major congenital ear malformations: surgical management and results.

Fifteen patients with major congenital aural atresia underwent operations using an anterior (trans-atretic bone) approach. Facial nerve monitoring was used in all cases and there were no instances of facial nerve injury. Postoperatively, two thirds of the patients had speech reception thresholds of 30 dB or better; the air conduction threshold (averaged for 500, 1,000, and 2,000 Hz) improved at least 25 dB in 80% of the patients and at least 30 dB in 60% of the patients (follow-up, 10 to 29 months). Minor revision surgery was necessary in three patients. On the basis of this series, several conclusions were made. First, one of the most important factors in proper patient selection is the degree of middle ear development on computed tomography, both in terms of size and ossicular formation. Second, every attempt should be made to keep the ossicular chain intact (versus use of a prosthesis), as this appears to optimize hearing results. Third, embryologic considerations and surgical experience predict a mobile stapes in the majority of major atresias. Lastly, facial nerve abnormalities are to be expected, especially in patients with significant microtia, but facial nerve monitoring will help minimize the risk of facial nerve injury.