Surgical aspects of cochlear implantation in syndromic children

The objective of this study was to report surgical results and outcomes of cochlear implantation in a large series of children with syndromes from one centre.

All syndromic children who underwent cochlear implantation at Great Ormond Street Hospital, from January 2000 to December 2010 were included in this study. The surgical technique was analysed and audiological outcomes were collected.

Over the 10-year period of this study, a total of 88 cochleas in 67 children with syndromes were implanted. The common syndromes implanted in this study were Ushers syndrome (23 patients, 33 cochleas), Wardenburgs syndrome (8 patients, 9 cochleas), Pendreds syndrome (4 patients, 4 cochleas), Jervell-Lange-Neilsen syndrome (3 patients, 4 cochleas), Enlarged vestibular aqueduct syndrome (4 patients, 7 cochleas), Cogans syndrome (3 patients, 4 cochleas), CHARGE (5 patients, 6 cochleas), and Branchio Oto Renal syndrome (3 patients, 4 cochleas). Pre-operative radiological inner ear anatomy was found to be abnormal in 28.4% (25/88) cochleas in this study group. Full insertion of the electrode was achieved in 93.1% (82/88) of cochleas, partial insertion in three cochleas, and insertion was abandoned in three cochleas. Early complications were seen in 6.8% (6/88) of implantations. All the 64/67 children who were implanted are still using the implant.

Cochlear implantation in syndromic children is challenging in both its audiological and surgical aspects. Good surgical results and good audiological and speech outcomes were achieved in this study, and subjective improvement in quality of life was achieved in these patients.