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Published on: October 11, 2024
Cochlear Implantation in Children with Enlarged Vestibular Aqueduct: A Systematic Review of Surgical Implications and
Marie-Louise Uhre Hansen1,2, Eva Rye Rasmussen3, Per Cayé-Thomasen1
1Department of Otorhinolaryngology, Head and Neck Surgery and Audiology Rigshospitalet, Denmark.
Insights
Cochlear implantation significantly improves hearing and speech in children with Pendred Syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA). While implantation ages have decreased, they remain higher than in the general pediatric population, with perilymph gusher/oozing being the most common complication.
Area of Science:
- Otolaryngology
- Pediatric audiology
- Genetics
Background:
- Pendred Syndrome (PS) and non-syndromic enlarged vestibular aqueduct (NSEVA) are genetic conditions causing hearing loss in children.
- Cochlear implantation is a common intervention for pediatric hearing loss, but outcomes in PS/NSEVA require specific evaluation.
Approach:
- A systematic review of studies published between 1984 and 2021 was conducted.
- 55 studies were analyzed, focusing on pediatric cochlear implant recipients with PS or NSEVA.
- Data on age at implantation, audiological outcomes, speech perception, and surgical complications were extracted and assessed.
Key Points:
- Cochlear implantation improved the four-frequency pure-tone audiogram average by 60–78 dB HL.
- Auditory performance and speech intelligibility scores increased by an average of 44%.
- The average age of implantation decreased by 21 months in studies post-2000 compared to earlier studies, with an overall average of 60 months.
- Perilymph gusher/oozing was the most frequent surgical complication, reported in 187 of 1572 implantations.
Conclusions:
- Cochlear implantation offers significant audiological and speech benefits for children with PS/NSEVA.
- Despite a trend towards earlier implantation, the average age remains higher than for unselected pediatric cases.
- Understanding surgical complication rates, particularly perilymph gusher/oozing, is crucial for managing these patients.
Objectives:
This study investigated age at implantation, improvement in hearing and speech perception outcomes, as well as surgical complications in pediatric cochlear implant recipients with Pendred Syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA).
Design:
A systematic review of the literature between 1984 and 2021 was performed. Two independent reviewers performed abstract and full-text screening using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The inclusion criteria were: English language, cochlear implant, age at implantation available, age <18 years, PS, Mondini malformation, and enlarged vestibular aqueduct. Full-text analysis was completed using the National Institute of Health assessment tool for case series and case-control studies. Studies were also graded according to the Oxford Centre for Evidence-Based Medicine grading system.
Results:
In total, 198 studies were identified and screened, and 55 studies were included for analysis. Audiological outcomes were available in 46 studies, and the four-frequency pure-tone audiogram average improved by 60 to 78 dB HL due to cochlear implantation. Auditory performance and speech intelligibility scores increased by 44%. The overall average implantation age was 60 months. The implantation age was 21 months lower in the studies where individuals were implanted after the year 2000 compared with those implanted before the year 2000. Perilymph gusher/oozing was the most common surgical incident reported, occurring in 187 of 1572 implantations.
Conclusions:
In children with PS/NSEVA, cochlear implantation improves pure-tone average by 60 to 78 dB HL and capacity of auditory performance/speech intelligibility by 44%. The implantation age for these children has decreased during the last two decades but is still somewhat higher than reported for unselected pediatric cochlear implantation. Perilymph gusher/oozing is the most common surgical complication.
