Cochlear Implant - CHARGE Syndrome (IFOS 2017)

This video offers a tutorial on a technique for cochlear implantation in patients with CHARGE syndrome or significant inner ear malformations. The accompanying abstract is listed below.

Objective/Hypothesis: CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. The aim of this study is to describe a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess.

Study Design: Retrospective chart review from a tertiary care pediatric hospital.

Methods: Three children with severe to profound sensorineural hearing loss associated with CHARGE syndrome underwent a transcanal cochlear implantation with closure of the external auditory canal via a modified Rambo meatoplasty. An analysis of the temporal bone anomalies and technical nuances were assessed.

Results: The mean patient age was 2.5 years (range 1.5 – 3.8 years). Two were males and two were left ears. All patients had a hypoplastic mastoid, lacked development of all three semicircular canals, and had some degree of cochlear dysplasia. A full cochlear implant insertion via a round window cochleostomy was achieved in all cases, even in the presence of grossly abnormal middle ear and facial nerve anatomy. There were no intraoperative or postoperative complications. The mean follow-up was 12.4 months (range 3.9 – 25.2 months). All three patients use their device daily. Their guardians report improved vocalization and environmental awareness.

Conclusions: The transcanal approach with modified Rambo canal closure provides a safe corridor for cochlear implantation in patients with CHARGE syndrome. This approach minimizes much of the anatomical variations associated with the syndrome and protects against electrode extrusion. Implant outcomes in this patient population are still variable and largely dependent on the patient’s global cognitive capacity.