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3p deletion syndrome: implications for cochlear implantation.
Daniel H Coelho1, Yula Taormina, Jaime Moore
1Department of Otolaryngology - Head and Neck Surgery, Virginia Commonwealth University, Richmond, VA 23298-0146, USA. dcoelho@mcvh-vcu.edu
Cochlear Implants International
|March 28, 2012
Summary
Cochlear implantation can improve sensorineural hearing loss (SNHL) in children with 3p deletion syndrome. Special attention to imaging is crucial due to potential mastoid abnormalities in 3pDS patients.
Area of Science:
- Otolaryngology
- Genetics
- Pediatric Audiology
Background:
- 3p deletion syndrome (3pDS) is associated with craniofacial abnormalities and sensorineural hearing loss (SNHL).
- Management of SNHL in 3pDS requires careful consideration of unique anatomical challenges.
Observation:
- A 23-month-old child with 3pDS presented with bilateral SNHL and poorly developed mastoids.
- Standard mastoid approaches for cochlear implantation may be challenging in such cases.
Findings:
- Successful cochlear implantation was achieved using a retro-auricular transmeatal approach.
- This approach bypasses the need for extensive mastoidectomy.
Implications:
- Cochlear implantation is a viable option for improving hearing in 3pDS patients with SNHL.
- Pre-operative imaging is essential to identify mastoid abnormalities and plan surgical approach.
- Alternative surgical techniques can mitigate risks and improve outcomes in complex cases.