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Author Spotlight: Advancements in Impedance Monitoring for Cochlear Implant Surgery
Published on: August 4, 2023
[Perioperative management of cochlear implantation for CHARGE syndrome]
Ying Lin1, Cuncun Ren1, Xiaoqin Fan1
1Department of Otolaryngology Head and Neck Surgery，First Affiliated Hospital of Military Medical University of Air Force，Xi'an，710032，China.
Insights
Cochlear implantation in children with CHARGE syndrome presents surgical and rehabilitation challenges due to complex deformities. Careful planning is essential for safe and effective auditory-speech outcomes.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Genetics
Background:
- CHARGE syndrome is a rare genetic disorder associated with multiple congenital anomalies.
- Children with CHARGE syndrome often present with profound hearing loss requiring cochlear implantation.
- Complex anatomical variations in CHARGE syndrome pose significant challenges for cochlear implantation.
Purpose of the Study:
- To investigate the perioperative characteristics of pediatric cochlear implant recipients with CHARGE syndrome.
- To analyze surgical planning, intraoperative findings, and postoperative complications.
- To evaluate auditory-speech rehabilitation outcomes following cochlear implantation.
Main Methods:
- Retrospective case series analysis of pediatric patients with CHARGE syndrome undergoing cochlear implantation.
- Review of radiological findings, intraoperative details, and surgical complications.
- Pre- and post-operative audiometric measurements and speech perception assessments.
Main Results:
- Five prelingual, profoundly deaf children with CHARGE syndrome were included, all with congenital heart disease and upper airway abnormalities.
- Cochlear abnormalities (Incomplete Partition II) and middle ear deformities were common.
- Improved audio-speech performance was observed at 12 and 24 months post-implantation.
Conclusions:
- Cochlear implantation in CHARGE syndrome is surgically and rehabilitatively challenging due to multiple abnormalities.
- Comprehensive treatment planning, addressing airway and temporal bone anatomy, is crucial for successful outcomes.
- Despite challenges, cochlear implantation can lead to improved auditory-speech function in these complex patients.
Abstract:
Objective:To explore the perioperative period characteristics of paediatric cochlear implant recipients of CHARGE syndrome with complex deformities. Methods:Retrospective case series of CHARGE syndrome were included. Radiological results, intraoperative findings, surgical planning and post-operative complications were analyzed. Routine audiometric measurements, speech perception categories and speech intelligibility ratings were performed pre and post-operatively to measure auditory speech rehabilitation outcomes. Results:Five prelingual profoundly deaf children were identified, aged from 14 months to 60 months. All patients had congenital heart disease and underwent surgery before cochlear implantation. Upper airway abnormalities were detected as choanal atresia, laryngomalacia and tracheal stenosis. All ten ears showed cochlear abnormalities（Incomplete partition Ⅱ）, eight of them combined with secretory otitis media and/or middle ear deformity. All patients underwent single side surgery using standard transmastoid facial recess approach. Full insertion of the electrode was achieved in two cochleas, while partial insertion was done in three cochleas. Three ears with absent auditory nerves in MRI showed no response in the neural remote test. All patients had improved audio-speech performance with CAP scores 3.0±0.7 and 3.6±0.9, SIR scores 1.2±0.4 and 1.8±0.8, IT-MAIS scores 18.8±9.1 and 26.2±10.0, MUSS scores 2.2±2.4 and 7.2±8.3 after twelve months and twenty-four months follow up. Conclusion:Cochlear implantation in patients with CHARGE syndrome is a challenge in both its surgical and rehabilitation aspects due to multiple abnormalities. Adequate treatment planning is necessary for safe and effective surgery, including airway structures and intricate temporal bone landmarks.
