Cochlear implants in children deafened by congenital cytomegalovirus and matched Connexin 26 peers.

Objective: To compare the long-term speech perception and production outcomes after cochlear implantation (CI) in children deafened by congenital cytomegalovirus (cCMV) with a matched group of Cx26-CI children by controlling for chronological age and magnetic resonance imaging (MRI) findings.

Methods: Retrospective review of 12 cCMV-CI children and matched Cx26-CI children for speech perception and speech production outcomes.

Results: Two trends were seen in our data. First, cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years. The majority of cCMV-CI children with normal MRI scans (5 out of 7), suffered from a delayed-onset SNHL. Their mean age at first implantation (2y9m, range 15-82m) was higher compared to their matched Cx26 peers (9m, range 7-12m). Before being implanted, the majority of these delayed-onset hearing impaired children had benefited from a certain period of normal hearing (with or without amplification of a hearing aid). Possibly, this input might have led to an advantage the first three years after CI. Second, results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormalities on MRI scans catch up for speech perception, but lag behind for speech production.

Conclusion: cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years, whereas results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormal MRI scans catch up for speech perception, but lag behind for speech production. In future, the inclusion of MRI results may assist in improved counseling of parents with cCMV deafened children seeking CI.