Cochlear implantation in children with CHARGE association

Area of Science:

• Otolaryngology
• Pediatric audiology
• Medical imaging

Background:

• CHARGE association is a rare genetic disorder associated with significant congenital anomalies.
• Sensorineural hearing loss is common in children with CHARGE association, often necessitating cochlear implantation.
• Temporal bone abnormalities are frequently observed in CHARGE association, posing unique surgical considerations.

Purpose of the Study:

• To investigate temporal bone anomalies in children with CHARGE association using radiologic imaging.
• To identify technical challenges encountered during cochlear implantation in this patient group.
• To evaluate the audiologic outcomes and benefits following cochlear implantation in children with CHARGE association.

Main Methods:

• A case series design was employed, analyzing data from six children with CHARGE association and sensorineural hearing loss.
• Computed tomographic (CT) scans were reviewed to assess temporal bone anatomy.
• Cochlear implantation operative records, audiometric data, and speech perception data were analyzed pre- and post-implantation.

Main Results:

• All six children exhibited temporal bone abnormalities on CT scans and during surgery.
• Cochlear implantation was successfully performed in five children; one child could not undergo surgery due to aberrant facial nerve anatomy.
• The five children who received cochlear implants demonstrated varying degrees of audiological and speech perception benefit.

Conclusions:

• Anatomical variations in the temporal bone associated with CHARGE association increase surgical complexity and facial nerve risks during cochlear implantation.
• While outcomes vary, cochlear implantation can provide significant audiological benefits for children with CHARGE association.
• Comprehensive parental counseling regarding potential challenges and realistic expectations is crucial before proceeding with cochlear implantation.