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Updated: Jun 13, 2025
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear
Jay R Maturi1, Kimberley S Noij, Vidya Babu
1Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Insights
Cochlear implantation (CI) offers hearing and speech benefits for most pediatric patients with bilateral cochlear nerve deficiency (CND). However, outcomes vary, with some patients experiencing no measurable benefit from CI.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Neurosurgery
Background:
- Bilateral cochlear nerve deficiency (CND) presents unique challenges for hearing rehabilitation in pediatric patients.
- Cochlear implantation (CI) is a potential treatment, but outcomes can be variable.
Purpose of the Study:
- To evaluate hearing and speech outcomes in pediatric patients with bilateral CND following cochlear implantation (CI).
- To identify factors influencing the success of CI in this patient population.
Main Methods:
- A systematic review and meta-analysis of studies reporting on pediatric patients with bilateral CND who underwent CI.
- Data extraction included demographics, comorbidities, inner ear abnormalities, CND classification, and auditory performance level (APL) scores.
- Cochran-Armitage tests were used for meta-analysis of patient-level data.
Main Results:
- The meta-analysis included 339 pediatric patients with bilateral CND who received CI.
- 31% of patients achieved open-set speech perception, while 19% had no measurable stimulation.
- Cochlear nerve aplasia and presence of syndromes were associated with significantly worse APL scores.
Conclusions:
- Cochlear implantation (CI) provides significant benefit for the majority of pediatric patients with bilateral CND.
- Outcomes are heterogeneous, with nearly one-third achieving open-set speech perception.
- A significant minority (19%) did not achieve measurable benefit, highlighting the need for personalized treatment strategies.
Objective:
To assess hearing and speech outcomes in pediatric patients with bilateral cochlear nerve deficiency (CND) who underwent cochlear implantation (CI) and to identify factors associated with improved outcomes.
Databases Reviewed:
PubMed, Embase, Web of Science, and Cochran databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
Methods:
Studies that reported hearing and speech outcomes of pediatric patients with bilateral CND who underwent CI were included. Demographics, comorbidities, inner ear abnormalities, CND classification (aplasia or hypoplasia), details of diagnostic workup, and outcomes data were extracted. Outcomes were assessed using the four-level auditory performance level (APL) scale. Meta-analysis, using Cochran-Armitage tests, was performed on patients with individual data to assess factors associated with performance.
Results:
A total of 314 papers were screened, and 40 papers with 378 total patients met inclusion criteria. A total of 339 patients had patient-level data and were included in the meta-analysis. Of the 339 patients, 19% (n = 63) of patients had no measurable stimulation, 28% (n = 95) had improved detection, 22% (n = 76) achieved closed-set speech perception, and 31% (n = 105) achieved open-set speech perception. Patients with cochlear nerve aplasia (p = 0.016) and syndromes (p < 0.001) had significantly worse APL scores relative to patients with cochlear nerve hypoplasia and patients without syndromes, respectively.
Conclusions:
While most patients with bilateral CND benefit from CI and almost one-third of patients achieved open-set speech perception, outcomes were heterogenous and one-fifth of patients did not experience measurable benefit from CI.
