Following the reports of a cluster of meningitis cases in recently implanted patients the FDA issued cautionary advice relating to the risk of meningitis after cochlear implantation (US Food and Drug Administration, 2002). Similar advice and a national reporting call has been issued by the Department of Health in the UK (Medical Devices Agency, 2002) and universal prophylactic pneumococcal vaccination started. We present a case of bilateral Mondini-type dysplasia associated with a defective stapes footplate and highlight the need for surgical vigilance to reduce the risks of meningitis from undiagnosed anatomical defects.
Keywords: cochlear implantation, meningitis, congenital inner-ear anomalies
Case reportA 5-year-old female was referred to the regional cochlear implant centre for consideration of implantation for profound congenital sensorineural deafness. Preoperative magnetic resonance imaging (MRI) scanning identified bilateral Mondini dysplasia (Figure 1), but failed to demonstrate a left-sided cochlear nerve. Promontory stimulation confirmed an auditory response, and having satisfied accepted audiological criteria she was offered a cochlear implant for the left ear.At surgery a profuse perilymph/cerebrospinal fluid (CSF) gusher was encountered and a defect in the stapes footplate with herniation of membranous labyrinth was seen. Partial insertion of a Cochlear Nucleus CI24 was achieved and temporalis muscle placed around the implant to secure a seal. The defect in the footplate was repaired with temporalis muscle pushed through the defect and held in place between the crura. In view of the surgical anatomy and Neural Response Te lemetry a peri-operative modified Stenvers view radiograph was taken. This showed the implant within the Mondini cochlea, although partially overfolded