Bilateral Cochlear Implantation in a Child With Galloway-Mowat Syndrome: A Case Report.

BACKGROUND This report describes the surgical management and early auditory outcomes of bilateral cochlear implantation in a child with Galloway-Mowat syndrome (GAMOS). To the best of our knowledge, this is the first reported case of such surgery. GAMOS is an exceedingly rare genetic disorder characterized by microcephaly, early onset of steroid-resistant nephrotic syndrome, and brain anomalies. It is inherited in an autosomal recessive pattern and has a genetically heterogeneous basis. CASE REPORT We report the case of a young boy diagnosed with GAMOS who had profound bilateral deafness, cerebellar hypoplasia, hypotonia, epilepsy, and visual impairment likely due to optic nerve dysgenesis. Due to the deafness, he underwent bilateral cochlear implantation in 2 stages (at ages 2 and 3 years). No significant difficulties were encountered: appropriate surgical access was achieved despite partial bony overgrowth of the round window area, and soft electrodes were used to adapt to the dysplastic cochlear anatomy. Ethics committee approval was obtained and written informed parental consent was provided. Preliminary results at 6 months after the second implant were satisfactory. As a result of the cochlear implantation, the boy was able to perceive sounds and began using them to communicate with those around him. CONCLUSIONS Cochlear implantation can be a safe and effective solution for treating deafness in patients with GAMOS, at least in the short term, even if there are congenital defects such as cochlear dysplasia. Good surgical access and the use of soft electrodes are important in minimizing the risk of damage and maximizing auditory outcomes.