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Published on: February 29, 2020
Case report: cochlear implant in a child with schizencephaly and cortical dysplasia
Lena Koren1, Talma Shpak, Hava Duchman
1Department of Otolaryngology, Head and Neck Surgery, Bnai-Zion Medical Center, The Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Insights
Cochlear implantation is safe for children with schizencephaly, a brain disorder. This study shows successful hearing restoration and language progress in a child with this condition, even with epilepsy.
Area of Science:
- Neuroscience
- Developmental Biology
- Audiology
Background:
- Schizencephaly is a rare congenital disorder characterized by bilateral clefts in the cerebral hemispheres, often associated with early-onset seizures.
- Profound hearing loss can co-occur with neurological conditions, posing significant challenges for development and communication.
- Evaluating the safety and efficacy of medical interventions in children with complex neurodevelopmental disorders is crucial.
Observation:
- A case study detailing pre-implantation considerations and post-implantation outcomes for a child diagnosed with schizencephaly and bilateral profound hearing loss.
- The child underwent cochlear implantation at 21 months of age.
- Epileptic patterns emerged at 4 years and 3 months, alongside mild dysesthesia, prompting careful monitoring.
Findings:
- The child demonstrated successful use of the cochlear implant by age 6, with no requirement for anticonvulsant medication.
- Language development, while initially delayed, showed steady progress.
- The observed epileptic pattern was deemed unrelated to the cochlear implant's electrical stimulation.
Implications:
- Cochlear implantation is a viable option for restoring hearing in children with schizencephaly and profound hearing loss.
- The findings suggest that cortical anomalies associated with schizencephaly do not contraindicate cochlear implantation.
- This case highlights the potential for positive neurodevelopmental outcomes, including language acquisition, following auditory rehabilitation in this patient population.
Abstract:
Schizencephaly is a developmental disorder of the cerebral cortex, usually with seizures occurring before the age of 3. We describe pre-implantation considerations and post-implantation outcome in a child with schizencephaly and bilateral profound hearing loss. Cochlear implantation was performed in the right ear when she was 21 months old. At 4 years and 3 months she developed an epileptic pattern on electroencephalogram and had mild attacks of dysesthesia. Now aged 6, she uses the implant successfully and needs no anticonvulsant medication. Language skills, though delayed, are progressing steadily. We consider that the epileptic pattern was unrelated to the electrical stimulation, and conclude that implantation is not contraindicated in children with cortical anomalies.
