Describe long-term trends and stability of hearing outcomes for patients undergoing primary congenital aural atresia
Retrospective chart review.
Single academic, tertiary referral center.
Children and adults who underwent primary CAA repair between 1980 and 2017.
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.
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.
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.