Simultaneous cochlear implantation and ipsilateral branchial cleft fistula excision in a pediatric patient

Branchio-oto-renal syndrome (BOR), also known as Melnick-Fraser Syndrome, is an autosomal dominant genetic disorder resulting from a mutation of the EYA-1 gene and characterized by branchial cysts or fistulas, ear anomalies, hearing loss, agenesis or hypoplasia of the kidneys, and chronic renal disease. We describe a unique surgical case of a branchial cleft fistula excision and concurrent ipsilateral cochlear implantation in a pediatric patient with BOR. A 2-year-old female presented with a right draining second branchial cleft fistula and severe bilateral profound sensorineural hearing loss due to bilateral cochlear dysplasia and cochlear nerve hypoplasia. She had previously undergone a left cochlear implant and was diagnosed with BOR. Her parents were interested in a sequential right cochlear implant as she was demonstrating excellent function with her left implant. A computed tomography fistulogram with an injection of contrast dye into the external orifice of the draining structure revealed a fistula connecting the small pit in the right lateral neck to the area of the tonsillar fossa in the oropharynx suggesting the presence of a second branchial cleft anomaly. She underwent simultaneous tonsillectomy, right branchial fistula excision, and right cochlear implant surgery under one exposure to general anesthesia. This is a unique case where special consideration must be taken with the order of procedures, consolidation or separation of the surgeries, and the use of monopolar cautery.