Cochlear Implantation in Children with Charge Syndrome: Surgical Approach and Outcomes

Background: CHARGE syndrome is a rare congenital condition that manifests with coloboma, heart defects, choanal atresia, mental retardation, genitourinary and ear anomalies that can affect almost any part of the auditory pathway. Bilateral, severe-profound, sensorineural hearing loss is common in CHARGE syndrome. The anatomical inner ear malformations and the co-morbidities make cochlear implantation (CI) and hearing rehabilitation challenging in these children.

Objective: To describe the special aspects in CI surgery in these patients and to assess their audiological outcomes.

Methods: Eight CI surgeries in 6 children with CHARGE were conducted in the Shaare Zedek Medical Center between the years 2011-2017. The average age was 4.5 years (range 2-12). Computed tomography (CT) and magnetic resonance imaging (MRI) were carried out preoperatively in all children to look for anatomical ear malformations. Audiological outcomes were measured using age appropriate speech perception tests and the CAP (category of Auditory Perception) scale.

Results: The main anatomic variations seen on CT and MRI were: absent/aplastic lateral semicircular canals and aberrant course of the facial nerve. All CI surgeries were performed using the standard posterior-tympanotomy approach. This approach included removal of the incus for better visualization of the course of the facial nerve. In addition, the posterior canal wall was moved forward in cases of very anterior facial nerve. A full insertion of the electrode array in the cochlea and normal function of the facial nerve were achieved in all patients. Audiological follow-up ranged from 1 to 6 years. 4 patients demonstrated benefit from their implants as measured by speech-perception measures. One patient is currently a non-user and a second (without a cochlear nerve) is candidate for an auditory brainstem implant.

Conclusion: The approach to the cochlea in patients with CHARGE association is difficult but possible. Cochlear implant surgery can be recommended in these cases but outcomes can be highly variable.