Objective: Describe long-term trends and stability of hearing outcomes for patients undergoing primary congenital aural atresia (CAA) repair.
Study design: Retrospective chart review.
Setting: Single academic, tertiary referral center.
Patients: Children and adults who underwent primary CAA repair between 1980 and 2017.
Intervention: CAA repair.
Main outcome measures: Long-term (> 1 yr) postoperative three-tone (500, 1000, 2000 Hz) air conduction pure-tone average (AC PTA) compared with the immediate (within 90 d of surgery) postoperative audiogram.
Results: The mean preoperative AC PTA was 59.6 dB HL. CAA repair improved hearing an average of 30.5 dB, but hearing declined by 8.2 dB over the long-term follow-up period (mean 4.4 yr; range 1-15.7 yr), leaving a final mean improvement of 22.2 dB (final mean AC PTA 37.3 dB HL). Two-thirds (92 of 138 ears) had an AC PTA ≤ 30 dB HL recorded in the first year after surgery. At the last follow-up test, 64% had "stable" hearing defined as no more than a 10 dB decline in AC PTA compared with the immediate postoperative audiogram; 21% had a 10 to 20 dB decline, 8% a 20 to 30 dB decline, and 7% declined > 30 dB.
Conclusions: All patients enjoyed improvement in AC PTA after surgery (preoperative minus the best postoperative PTA, mean = 34 dB, range = 3.3-52 dB). Hearing declined by an average 8.2 dB over the long-term leaving a final average AC PTA of 37.4 dB HL. Sixty-four percent of patients exhibited stable (< 10 dB loss) hearing over time; 36% lost 10 dB or more over the long-term follow-up period. Hearing results typically stabilize over time with most of the change occurring in the first 3 years after CAA repair. Bone conduction thresholds remained stable over the study period.