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Updated: Jun 5, 2026
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Robotic Cochlear Implantation for Direct Cochlear Access
Published on: June 16, 2022
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Cochlear implantation in syndromic patients: difficulties and lessons learnt.
Mina Fayez Saleeb1, Lobna El Fiky1, Badr Eldin Mostafa1
1Otorhinolaryngology Department Faculty of Medicine, Ain Shams University, Ramses Street, Abasseyia Square, Cairo, 11566, Egypt.
Summary
This study found that 4.3% of pediatric cochlear implant patients had syndromes, with Jervell and Lange Nielsen syndrome being most common. While most syndromic cases had normal inner ear anatomy, careful evaluation is crucial for successful cochlear implantation.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Syndromic hearing loss (HL) is a significant factor in pediatric cochlear implantation.
- Understanding the prevalence of specific syndromes and associated malformations is crucial for surgical planning and patient management.
Purpose of the Study:
- To determine the prevalence of syndromes in children undergoing cochlear implantation.
- To identify inner and middle ear malformations in syndromic patients.
- To document surgical difficulties encountered during cochlear implantation in this cohort.
Main Methods:
- Retrospective chart review of pediatric patients who underwent cochlear implantation between 2018 and 2023.
- Analysis of preoperative imaging for inner and middle ear malformations in syndromic cases.
- Documentation of intraoperative findings and surgical challenges.
Main Results:
- Out of 1024 children, 45 (4.3%) had associated syndromes, most commonly Jervell and Lange Nielsen (34%) and Waardenberg syndrome (32%).
- Inner ear malformations (IEM) were present in 9 cases (20%), including 6 cases of perilymph gusher. Middle ear anomalies and facial nerve abnormalities were also noted.
- Surgical outcomes for syndromic patients were comparable to non-syndromic controls.
Conclusions:
- Syndromic hearing loss requires individualized assessment for inner and middle ear malformations.
- Associated disabilities in syndromic patients can impact rehabilitation.
- Comprehensive medical evaluations are recommended for all children with congenital hearing loss to identify syndromic etiologies.
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