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Updated: May 24, 2026

Intrathecal Application of a Fluorescent Dye for the Identification of Cerebrospinal Fluid Leaks in Cochlear Malformation
Published on: February 29, 2020
A postmeningitic cochlear implant patient who was postoperatively diagnosed as having X-linked agammaglobulinemia
Ryosei Minoda1, Haruo Takahashi, Satoru Miyamaru
1Department of Otolaryngology Head and Neck Surgery, Graduate School of Medicine, Kumamoto University, 1-1-1 Honjo, Kumamoto, Japan. minoda@gpo.kumamoto-u.ac.jp
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.
