(A809) Cochlear Implantation in Syndromic Patients with Aural Atresia: Case Series & Literature Review

Introduction: This study investigates the feasibility and outcomes of cochlear implantation (CI) in syndromic patients with aural atresia and profound sensorineural hearing loss (SNHL). We present two cases of patients with Treacher-Collins syndrome and Crouzon syndrome undergoing CI and provide a comprehensive literature review on this topic.

Methods: Two patients with syndromic aural atresia and profound SNHL were treated with cochlear implantation. Pre-operative audiologic and radiologic evaluation was conducted to assess hearing thresholds and anatomical anomalies. Intraoperative approaches were tailored to address aberrant anatomy. A literature search was performed to identify previously reported cases of cochlear implantation in aural atresia, with and without syndromic associations.

Results: Both patients achieved favorable hearing outcomes post-implantation. Case 1, a 72-year-old female with Treacher-Collins syndrome, achieved aided hearing thresholds of 25–40 dB and improved speech understanding. Case 2, an 18-year-old male with Crouzon syndrome, achieved bilateral aided thresholds of 25–35 dB with sentence recognition scores improving to 98%. Surgical challenges, including atypical facial nerve positioning, poor mastoid pneumatization, and ossicular dysplasia were overcome using modified surgical techniques, including retrofacial approaches and stereotactic guidance.

Conclusions: Cochlear implantation is a viable option for hearing rehabilitation in syndromic patients with aural atresia and SNHL. These cases underscore the need for tailored surgical approaches due to the unique anatomic variations associated with syndromes such as Treacher-Collins and Crouzon. Imaging and intraoperative guidance can improve outcomes by addressing these challenges. These findings expand the scope of CI candidacy and highlight the importance of recognizing SNHL in syndromic aural atresia populations.