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Original Investigation |

Surgical and Audiometric Outcomes for Repair of Congenital Aural Atresia and Hypoplasia

William J. Moss, MD1; Harrison W. Lin, MD2; Roberto A. Cueva, MD3
[+] Author Affiliations
1Division of Otolaryngology–Head and Neck Surgery, University of California, San Diego
2Department of Otolaryngology–Head and Neck Surgery, University of California, Irvine
3Department of Otolaryngology–Head and Neck Surgery, Southern California Permanente Medical Group, San Diego
JAMA Otolaryngol Head Neck Surg. 2016;142(1):52-57. doi:10.1001/jamaoto.2015.2713.
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Importance  Surgical repair of congenital aural atresia and hypoplasia (CAAH) is technically challenging. Long-term surgical and audiologic outcomes of atresiaplasty are incompletely understood.

Objectives  To review the surgical outcomes for CAAH and analyze the hearing results.

Design, Setting, and Participants  A retrospective medical record review of CAAH outcomes was performed during an 11-year period from January 1, 2004, through December 31, 2014. The data analysis was undertaken from December 1, 2014, through January 31, 2015. The mean clinic follow-up time was 3.9 years, and the mean audiologic follow-time was 2.8 years. The study included 98 patients aged 5 to 66 years (mean age, 16.6 years) with CAAH who underwent a total of 104 operations.

Interventions  Surgical repair of CAAH.

Main Outcomes and Measures  Preoperative and postoperative pure-tone averages (PTAs), speech reception thresholds (SRTs), air-bone gaps (ABGs), and interaural PTA and SRT differences were compared. Factors that affect hearing outcomes were analyzed. The complication rates were reviewed and compared with results from similar studies.

Results  In the 98 patients with CAAH, the mean improvement in ABGs and SRTs was 26.7 and 25.9 dB, respectively, resulting in a postoperative ABG of 30 dB or less in 4 of 5 cases. The mean postoperative PTAs and SRTs were 36.9 and 34.3 dB, respectively. Patients with a functional native ossicular chain (36 of 104 [34.6%]) had significantly superior audiometric outcomes when compared with patients in whom a reconstruction prosthesis was required during primary or revision operations. Audiometric results from hypoplasia surgery were not significantly different from those of atresia surgery; results in patients with craniofacial syndromes were similarly not significantly different from those in patients with sporadic CAAH. We report a low incidence of meatal stenosis.

Conclusions and Relevance  The mean hearing outcomes for this group compared favorably with other series. The need for ossicular chain reconstruction was associated with poorer audiometric outcomes. The safety profile and the demonstrated hearing improvement of CAAH surgery suggest that it remains a favorable option for patients.

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