title: A postmeningitic cochlear implant patient who was postoperatively diagnosed as having X-linked agammaglobulinemia.
abstract: X-linked agammaglobulinemia (XLA) is caused by a mutation in the Bruton tyrosine kinase, leading to an arrest in B cell development. Consequently, patients with XLA show significant decreases in gammaglobulin. Here, we describe a child with postmeningitic deafness and XLA who underwent a cochlear implantation. His psychomotor development had been normal and his congenital immunodeficiency was noticed only postoperatively. Immunoglobulin replacement treatment was started, but he still suffered repeated infections. Eventually, his cochlear implant was removed. A preoperative check of immunological status might be advisable in postmeningitic patients undergoing cochlear implantation to reduce the risk of postoperative infectious complications.
authors.affiliation: Department of Otolaryngology Head and Neck Surgery, Graduate School of Medicine, Kumamoto University, 1-1-1 Honjo, Kumamoto, Japan. minoda@gpo.kumamoto-u.ac.jp
authors.forename: Ryosei
authors.initials: R
authors.lastname: Minoda
authors.forename: Haruo
authors.initials: H
authors.lastname: Takahashi
authors.forename: Satoru
authors.initials: S
authors.lastname: Miyamaru
authors.forename: Masako
authors.initials: M
authors.lastname: Masuda
authors.forename: Toru
authors.initials: T
authors.lastname: Miwa
authors.forename: Tetsuji
authors.initials: T
authors.lastname: Sanuki
authors.forename: Toshinori
authors.initials: T
authors.lastname: Hirai
authors.forename: Eiji
authors.initials: E
authors.lastname: Yumoto
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications* / Q000628:therapy; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000628:therapy; D006319:Hearing Loss, Sensorineural / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008297:Male; D016920:Meningitis, Bacterial / Q000150:complications; D016459:Prosthesis-Related Infections / Q000209:etiology*