Cochlear implantation via the middle cranial fossa is a safe approach, although it is a challenging procedure, even for experienced surgeons.

Introduction  The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective  To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods  A questionnaire was applied to 14 families of multiple handicapped children, aging from 2–12 years old and having from 11 months to 11 years of implant use. Results  The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion  The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.

Summary Introduction: The most relevant clinical symptom in Waardenburg syndrome is profound bilateral sensorioneural hearing loss. Aim: To characterize and describe hearing outcomes after cochlear implantation in patients with Waardenburg syndrome to improve preoperative expectations. Method: This was an observational and retrospective study of a series of cases. Children who were diagnosed with Waardenburg syndrome and who received a multichannel cochlear implant between March 1999 and July 2012 were included in the study. Intraoperative neural response telemetry, hearing evaluation, speech perception, and speech production data before and after surgery were assessed. Results: During this period, 806 patients received a cochlear implant and 10 of these (1.2%) were diagnosed with Waardenburg syndrome. Eight of the children received a Nucleus 24® implant and 1 child and 1 adult received a DigiSonic SP implant. The mean age at implantation was 44 months among the children. The average duration of use of a cochlear implant at the time of the study was 43 months. Intraoperative neural responses were present in all cases. Patients who could use the speech processor effectively had a pure tone average of 31 dB in free-field conditions. In addition, the MUSS and MAIS questionnaires revealed improvements in speech perception and production. Four patients did not have a good outcome, which might have been associated with ineffective use of the speech processor. Conclusion: Despite the heterogeneity of the group, patients with Waardenburg syndrome who received cochlear implants were found to have hearing thresholds that allowed access to speech sounds. However, patients who received early intervention and rehabilitation showed better evolution of auditory perception.

The mean changes in the low-frequency PTA comparing postoperative against preoperative thresholds were 28.03 ± 6.77 dB in group 1, 30 ± 14.53 dB in group 2, and 7.23 ± 6.12 dB in group 3. There was statistical difference when comparing group 3 with groups 1 and 2 using one-way ANOVA (p = 0.002) followed by Scheffé post hoc test.

RESUMO Objetivo: verificar a aquisição de vocabulário em crianças surdas, usuárias de implante coclear, bem como os fatores que influenciam esse desenvolvimento. Métodos: foi aplicada a parte de vocabulário do teste de linguagem infantil ABFW em 20 crianças usuárias de implante coclear por no mínimo três anos. Além disso, foi avaliada a participação familiar no desenvolvimento dessas crianças. Resultados: foi observado que as crianças implantadas apresentam possibilidade de alcançar o desenvolvimento normal de vocabulário, quando comparadas às crianças ouvintes, a depender de diversos fatores. O fator que apresentou influência estatisticamente significante no vocabulário foi a participação familiar, sendo que quanto maior o envolvimento da família no processo terapêutico, melhores os resultados no teste de vocabulário. Conclusão: as crianças implantadas podem apresentar desempenho similar às crianças ouvintes no teste de vocabulário, a depender das variáveis que transcendem a idade à implantação ou mesmo o tempo de uso do implante coclear. A estimulação/ participação familiar no desenvolvimento das crianças se mostrou de extrema importância no desenvolvimento da linguagem oral.