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Robotic Cochlear Implantation for Direct Cochlear Access
Published on: June 16, 2022
Cochlear implantation in Donnai-Barrow syndrome
Iain A Bruce1, Stephen J Broomfield, Lise Henderson
1Manchester Royal Infirmary, UK. ia.bruce@tiscali.co.uk
Cochlear Implants International
|July 16, 2011
Summary
Cochlear implantation successfully rehabilitated severe hearing loss in a child with Donnai-Barrow syndrome, a rare genetic disorder. This case highlights the potential benefits of cochlear implants for hearing restoration in this condition.
Area of Science:
- Genetics and rare diseases
- Otolaryngology
- Audiology
Background:
- Donnai-Barrow syndrome is a rare autosomal recessive disorder.
- It is characterized by severe sensorineural hearing loss (SNHL) and various ocular abnormalities.
- Associated conditions include diaphragmatic hernia, exomphalos, absent corpus callosum, and developmental delay.
Observation:
- A case of Donnai-Barrow syndrome was identified from a pediatric cochlear implant database.
- The patient initially received a right cochlear implant at 39 months, which failed 4 years later.
- Bilateral simultaneous cochlear implantation was subsequently performed.
Findings:
- Post-operatively, bilateral cochlear implant use yielded a 98% BKB score at 2 years.
- Individual ear testing showed 94% for the right and 80% for the left.
- The child achieved 100% on sound localization testing with three speakers.
Implications:
- This is the first reported case of cochlear implantation for hearing loss rehabilitation in Donnai-Barrow syndrome.
- Cochlear implantation can be an effective treatment for SNHL in this rare disorder.
- Further research is warranted to explore the long-term efficacy and outcomes of cochlear implantation in patients with Donnai-Barrow syndrome.
