Milestone in Paediatric ENT at Kokilaben Dhirubhai Ambani Hospital Navi Mumbai as 11-month infant with Rare Genetic Disorder receives Cochlear Implants
~ Baraitser Winter Syndrome is a rare disorder with less than 100 cases reported around the world.
~ The cochlear implants will treat the sensorineural deafness that is a characteristic of the condition and restore the child’s hearing.
Mumbai, March 19, 2025, (GNI) : Kokilaben Dhirubhai Ambani Hospital Navi Mumbai is pleased to announce a significant milestone in paediatric ENT care with the successful bilateral cochlear implant in an 11-month-old infant diagnosed with Baraitser Winter Cerebrofrontofacial Syndrome (BWCS). A rare genetic disorder, with less than 100 cases reported worldwide, the neurological and physical signs of the syndrome include heart defects, brain abnormalities, sensorineural deafness among other anomalies. Early intervention with cochlear implants enhances speech development, auditory perception, and overall quality of life in young children. For an infant with a rare condition like Baraitser Winter Syndrome, restoring hearing at an early age helps in ensuring timely developmental milestones in speech and communication.
Dr. Varun Dave, Consulting ENT Surgeon and Otorhinolaryngologist, Kokilaben Dhirubhai Ambani Hospital Navi Mumbai said, “Baraitser Winter Cerebrofrontofacial Syndrome is a rare and complex condition with distinctive facial features, developmental delays, seizures, and other systemic complications. The multiple neurological and physical anomalies added difficulties to the surgical and anaesthetic processes and required us to take a personalised approach. Every aspect of the procedure was meticulously planned and executed to address the unique needs of the patient. Every step, from anaesthesia to electrode placement had to be handled with extra care.”
The complexity of the surgery due to the infant’s condition required careful navigation through potential complications with continuous monitoring. The procedure involved placement of the bilateral cochlear implants supported by real-time confirmation of the proper functioning of all electrodes. Smart Nav technology by Cochlearwas used intra-op which is state of the art technology that provided real time update on the functioning of the implant.This was a crucial check to ensure that that the device was correctly positioned to maximize the infant’s potential auditory development.
Dr. Varun Dave added, “The surgery went as planned and the functionality of the cochlear implants was confirmed during the procedure. The little patient had a swift recovery and was discharged in less than 24 hours following the surgery. This rapid recovery is a result of the hospital’s efficient care protocols and the commitment of the entire medical team. Early discharge not only reduced the stress on the family but also allowed the infant to recover in a familiar environment.”
Dr. Shashikant Pawar, Chief Operating Officer of Kokilaben Hospital Navi Mumbai said, “Every child deserves the opportunity to experience the world of sound, and this case reinforces our dedication to providing patient-focused care that adapts to even the most challenging conditions. Our team’s commitment to innovation and quality care is reflected in this success, which shows that with careful planning and collaboration, even the most complex medical cases can be managed effectively. We hope to continue to support children facing rare conditions.”ends Gni
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