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

Ali Khurram, Cynthia Wang, Charles Saadeh, Kenneth Lee

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Article number100319
JournalOtolaryngology Case Reports
StatePublished - Sep 2021


  • Branchial fistula
  • Brancho-oto-renal syndrome
  • Cochlear Implant

ASJC Scopus subject areas

  • Otorhinolaryngology


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