By Helen Albert
Children with Waardenburg syndrome (WS) respond well to cochlear implantation and achieve equally good speech and hearing outcomes as nonsyndromic children undergoing the same procedure, report researchers.
WS, an inherited genetic disorder, is characterized by abnormal skin, hair, and iris pigmentation, as well as varying degrees of sensorineural hearing loss.
Initial research suggests that children with this syndrome have good results after cochlear implantation for profound deafness.
Building on previous research, Susana de Sousa Andrade (Centro Hospitalar de Coimbra, Portugal) and colleagues compared the speech perception and production outcomes between seven children with WS and 372 children without WS who all had multichannel cochlear implants fitted in a Portuguese medical center during 1992 to 2011. The children were aged 30.6 months on average when their implants were fitted.
As reported in the International Journal of Pediatric Otolaryngology, postoperative outcomes in WS children were similar to those of nonsyndromic children.
After a mean follow-up period of 57.7 months, open-set speech recognition skills of WS children were slightly lower than those of nonsyndromic implanted children overall. However, WS children were better than the other children at recognizing vowels and numbers.
On the classification of auditory receptive abilities scale, the WS children scored a median of 5.5 compared with 6.0 for the nonsyndromic children. On the meaningful auditory integration scale, the WS children scored 37.4 and the nonsyndromic children 36.5 out of 40.0.
Regarding speech intelligibility, the median rating (Speech Intelligibility Rating [SIR]) was 4 in WS versus 4.5 in nonsyndromic implanted children, with 71.4% of WS children reaching the maximum SIR category. The meaningful use of speech scale questionnaire resulted in a mean score of 33.2 in WS versus 32.3 in nonsyndromic children out of a total score of 40.0.
Overall, no statistically significant differences between nonsyndromic and WS children were observed for the outcomes measured, notes the team.
"Although the current study reports on a relatively small cohort, similarly to other published studies that look into cochlear implantation on WS children, we have encountered a significant benefit obtained by this auditory rehabilitation method comparable to that achieved by the general population of implanted children," write de Sousa Andrade and team.
"Our data confirm that prelingually deafened WS children, with prior non-significant or marginal benefit from acoustic amplification and normal inner ear anatomy, are potentially good candidates for audio-oral rehabilitation with cochlear implant," they conclude.
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