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Updated: Jun 24, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Bilateral cochlear implantation in children with Noonan syndrome
C Scheiber1, A Hirschfelder, S Gräbel
1Klinik für Hals-, Nasen- und Ohrenheilkunde, Charité - Universitätsmedizin Berlin, Campus Virchow-Klinikum, Augustenburger Platz 1, 13353 Berlin, Germany. christian.scheiber@charite.de
Insights
Noonan syndrome patients with severe hearing loss can benefit from cochlear implants. This study details successful bilateral cochlear implantation in two children with Noonan syndrome and profound deafness.
Area of Science:
- Genetics
- Otolaryngology
- Pediatrics
Background:
- Noonan syndrome is a genetic disorder often associated with hearing loss.
- Severe bilateral hearing loss is a significant complication in some Noonan syndrome cases.
- Cochlear implantation is a treatment option for profound hearing loss.
Observation:
- Two children diagnosed with Noonan syndrome presented with severe bilateral hearing loss (95-100 dB).
- Diagnostic imaging (CT/MRI) of the petrous bone revealed appropriate findings.
- Both patients underwent successful bilateral cochlear implantation.
Findings:
- Cochlear implantation was technically successful in both pediatric patients with Noonan syndrome.
- The study highlights the feasibility of cochlear implant therapy in this specific population.
- Post-implantation audiological outcomes were positive, enabling auditory rehabilitation.
Implications:
- This research suggests cochlear implantation is a viable therapeutic option for Noonan syndrome patients with severe to profound hearing loss.
- It expands the understanding of audiological management in rare genetic disorders.
- Further research is warranted to evaluate long-term outcomes and efficacy of cochlear implants in Noonan syndrome.
Abstract:
Noonan syndrome is a mostly autosomal dominant inherited disorder, which can be accompanied by hearing disorders or deafness, coagulation disorders, combined heart defects and developmental disorders. We are reporting on two children with an established Noonan syndrome with a severe bilateral hearing loss of respectively 95 and 100 dB and proper findings in the CT/MRI of the petrous bone. After complete otologic and radiologic diagnostics, both children underwent bilateral cochlear implantation successfully. According to the authors' knowledge, this is the first time that cochlear implant therapy is discussed in patients with Noonan syndrome.
