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Updated: Jan 20, 2026

Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Lessons From an Analysis of Newborn Hearing Screening Data for Children With Cochlear Implants
Jeon Mi Lee1, Hyun Jin Lee2, Jinsei Jung3
1Department of Otorhinolaryngology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang.
Insights
Newborn hearing screening (NHS) can miss hearing loss, leading to delayed cochlear implants (CIs) and lower language development, especially with SLC26A4 mutations. Genetic testing and parent counseling are crucial for early intervention.
Area of Science:
- Audiology
- Genetics
- Pediatrics
Background:
- Newborn hearing screening (NHS) aims for early detection of hearing loss.
- Limitations exist in current NHS protocols, potentially leading to delayed diagnosis and intervention.
- Genetic factors, such as SLC26A4 mutations, are significant causes of congenital hearing loss.
Purpose of the Study:
- To identify limitations in current newborn hearing screening (NHS) programs.
- To provide recommendations for improved NHS protocols.
- To analyze the impact of NHS results on cochlear implant (CI) outcomes and language development.
Main Methods:
- Retrospective analysis of 185 children who received cochlear implants (CIs) at ≤5 years of age.
- Review of NHS data, screening tools, age of diagnosis, hearing thresholds, causes of hearing loss, age of CI insertion, audiogram findings, and language development.
- Statistical comparison between children who passed NHS and those referred for further evaluation.
Main Results:
- 22% of children passed NHS, but 78% were later diagnosed with hearing loss.
- Children who passed NHS had hearing loss confirmed significantly later than referred children.
- The NHS-pass group received CIs later, and those with SLC26A4 mutations showed significantly lower language development.
Conclusions:
- Parents require thorough counseling on NHS results, emphasizing that a negative screen does not preclude later-onset hearing loss.
- Genetic testing for SLC26A4 mutations is recommended in regions with high prevalence.
- Improved NHS protocols and genetic screening can enhance early intervention for hearing loss.
Objectives:
The aims of the study are to identify the limitations of the current newborn hearing screening (NHS) programs and provide recommendations for better protocols.
Study Design:
Retrospective analysis.
Setting:
Tertiary referral center.
Patients:
The study participants were 185 children who received cochlear implants (CIs) at ≤5 years of age.
Interventions:
Therapeutic and rehabilitative.
Main Outcome Measures:
The results of NHS, screening tools used, age, and hearing thresholds at which hearing loss was confirmed, causes of the hearing loss, age of CI insertion, aided pure-tone audiogram findings, and language development level were analyzed.
Results:
NHS data was available for 109 children, and 24 patients (22.0%) had passed NHS for both ears. Hearing loss was confirmed considerably later in children who had passed NHS than in children who were referred for further evaluation (p < 0.01). The most common cause for hearing loss in the NHS-pass group was SLC26A4 mutations (41.7%). Patients in the NHS-pass group received CIs considerably later than those in the NHS-referred group (p < 0.01). Among patients with SLC26A4 mutations, the language development level was significantly lower in the NHS-pass group than in the NHS-referred group (p < 0.01).
Conclusions:
Careful counseling regarding NHS results is necessary for parents to understand that the absence of hearing loss at birth does not mean that the child will not develop hearing loss later in life. Genetic testing for SLC26A4 mutations may be necessary in regions with a high incidence of these mutations, such as East Asia.
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