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ENT management of a child with CHARGE association
Dennis K K Au1, Yau Hui, Amy W C Tsang
1Division of Otorhinolaryngology, Department of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Block S3, 102 Pokfulam Road, Hong Kong SAR, China. kinkwau@hkucc.hku.hk
Insights
CHARGE association, a condition with multiple birth defects, often includes hearing loss. This study details a unique cochlear implantation surgery for a child with CHARGE syndrome, discussing outcomes for hearing and speech development.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Genetics
Background:
- CHARGE association is a complex genetic disorder characterized by multiple congenital anomalies, frequently involving hearing impairment.
- Hearing loss in CHARGE association presents significant challenges for otolaryngologists and audiologists.
- Cochlear implantation is a potential intervention for severe hearing loss in affected children.
Observation:
- A case report of a pediatric patient diagnosed with CHARGE association and severe hearing loss.
- The patient underwent cochlear implantation utilizing an unconventional surgical technique.
- Postoperative audiological and speech perception outcomes were meticulously reviewed.
Findings:
- The unconventional surgical approach for cochlear implantation in this CHARGE association case was successfully performed.
- Postoperative speech perception results demonstrated notable improvements, indicating the efficacy of the intervention.
- The study highlights the feasibility and benefits of cochlear implantation in children with complex congenital defects.
Implications:
- This case suggests that cochlear implantation can be a viable option for improving hearing and communication in children with CHARGE association.
- The unconventional surgical approach may offer benefits in managing complex anatomical challenges associated with CHARGE syndrome.
- Further research is warranted to explore the long-term benefits and optimal surgical strategies for cochlear implantation in this patient population.
Abstract:
The acronym CHARGE is used to describe specific congenital birth defects in children: colobomata, heart defect, atresia of the choanae, retarded growth or development, genital hypoplasia, and ear anomalies or deafness. CHARGE association with hearing impairment is a challenge to ENT surgeons. We report the case of a child with CHARGE association who underwent cochlear implantation using an unconventional surgical approach and review the postoperative speech perception results. The benefits of cochlear implantation in children with multiple congenital defects are discussed.
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