Cochlear implantation in children with anomalous cochleovestibular anatomy.

OBJECTIVE: The aim of the study is to assess the audiological and surgical outcome after cochlear implantation in children with inner ear malformation and to compare them with a group of congenitally deaf children implantees without inner ear malformation. INTRODUCTION: Children with profound sensorineural deafness with malformations of the inner ear represent a challenge, accounting to 5-15% of congenital sensorineural deafness. These cases were originally regarded as a contraindication for cochlear implant surgery. METHODS: Retrospective study of 26 patients with congenital inner ear malformation, from a total population of 329 cochlear implant patients. Radiological evaluation with high resolution computed tomography and magnet resonance was performed to all patients in order to evaluate all the preoperative conditions. All patients were tested using European Portuguese word discrimination tests (monosyllabic test, number test and sentences test), capacity of auditory performance (CAP) and speech intelligibility rating (SIR). RESULTS: In all 7.9% of deaf children in our center study have inner ear abnormalities. All children underwent successful implantation. CAP yielded an average 7.1 (+/-1.7), SIR 4.3 (+/-1.0). The children without inner ear abnormalities did not achieve statistically significant better scores. Two children had a perilymph gusher, and there were no other complications. CONCLUSION: Cochlear implantation can be successfully performed in children with inner ear malformation. Audiological performance after cochlear implantation in malformed inner ears is comparable to that found in other congenitally deaf patients. The risk of CSF leak is associated with inner ear abnormalities and should be anticipated during surgery.

Cochlear implant rehabilitation outcomes in Waardenburg syndrome children.

OBJECTIVES: The purpose of this study was to review the outcomes of children with documented Waardenburg syndrome implanted in the ENT Department of Centro Hospitalar de Coimbra, concerning postoperative speech perception and production, in comparison to the rest of non-syndromic implanted children. METHODS: A retrospective chart review was performed for children congenitally deaf who had undergone cochlear implantation with multichannel implants, diagnosed as having Waardenburg syndrome, between 1992 and 2011. Postoperative performance outcomes were assessed and confronted with results obtained by children with non-syndromic congenital deafness also implanted in our department. Open-set auditory perception skills were evaluated by using European Portuguese speech discrimination tests (vowels test, monosyllabic word test, number word test and words in sentence test). Meaningful auditory integration scales (MAIS) and categories of auditory performance (CAP) were also measured. Speech production was further assessed and included results on meaningful use of speech Scale (MUSS) and speech intelligibility rating (SIR). RESULTS: To date, 6 implanted children were clinically identified as having WS type I, and one met the diagnosis of type II. All WS children received multichannel cochlear implants, with a mean age at implantation of 30.6±9.7months (ranging from 19 to 42months). Postoperative outcomes in WS children were similar to other nonsyndromic children. In addition, in number word and vowels discrimination test WS group showed slightly better performances, as well as in MUSS and MAIS assessment. CONCLUSIONS: Our study has shown that cochlear implantation should be considered a rehabilitative option for Waardenburg syndrome children with profound deafness, enabling the development and improvement of speech perception and production abilities in this group of patients, reinforcing their candidacy for this audio-oral rehabilitation method.