RESUMO
Waardenburg syndrome (WS) is a rare autosomal-dominant syndrome that can be presented with sensorineural hearing loss. In this report, we describe the outcomes of three children with WS at zero, three, nine, twelve and sixty months after cochlear implant (CI) fitting. The outcomes were assessed using IT-MAIS (Infant-Toddler Meaningful Auditory Integration Scale - younger than 5 year), MAIS (Meaningful Auditory Integration Scale - older than 5 year), MUSS (Meaningful Use of Speech Scale), and categories of auditory performance and speech intelligibility. The results showed an improvement in auditory and language performance over time, two patients who used CI for 5 years achieved 100% in IT-MAIS and MUSS tests. In addition, both were able to understand sentences in open set and achieve fluent speech. Moreover, both reached fluency on auditory and language performance scale. The third patient with 50 months of follow-up and in the 48 months evaluation, is in category 5 of auditory performance and 3 of speech intelligibility. We concluded that all children who had low levels of hearing and language before cochlear implant have improved hearing and language skills after implantation and rehabilitation.
A Síndrome de Waardenburg (SW) é uma condição genética rara de padrão autossômico dominante e que pode cursar com perda auditiva neurossensorial. Nesse relato, apresentamos o desempenho da audição e linguagem de três crianças com SW, com zero, três, nove, doze e sessenta meses de seguimento após implante coclear (IC). As crianças foram avaliadas através dos questionários Escala de Integração Auditiva IT-MAIS (até 5 anos), MAIS (maiores de 5 anos), Escala de Utilização da Fala (MUSS) e categorizados quanto à audição e linguagem. Os resultados demonstraram uma melhora no desempenho da audição e linguagem ao longo do tempo, sendo que os dois pacientes que usaram o IC por 5 anos alcançaram 100% no IT-MAIS, MAIS e MUSS. Além disso, ambos foram capazes de compreender sentenças em conjunto aberto e possuem fala fluente, considerando-se as categorias de audição e linguagem. O terceiro paciente, com 50 meses de seguimento, encontra-se, na avaliação aos 48 meses, na categoria 5 de audição e 3 de linguagem. Conclui-se assim que as crianças da pesquisa, que apresentavam níveis baixos de audição e linguagem antes do implante coclear, passaram a apresentar melhores respostas auditivas e linguísticas após a implantação e reabilitação.
Assuntos
Implante Coclear , Implantes Cocleares , Percepção da Fala , Síndrome de Waardenburg , Pré-Escolar , Audição , Humanos , Lactente , Resultado do TratamentoRESUMO
OBJECTIVE: To present the protocol called the Brazilian Scale of Hearing and Language Development. MATERIALS AND METHODS: This is a protocol developed in order to assess and record the development of hearing and language skills in young children who have undergone cochlear implantation during the first two years of hearing age. It was applied to the parents of 41 children over a period of 12 months at the cochlear implant clinic in the Pequeno Príncipe Hospital in Curitiba, Brazil. Registered were: the time taken to implement the protocol; ease of understanding the questions; numerical results obtained by the children evaluated. RESULTS: In general, the questions were considered easy to understand. The minimum time taken for application of the protocol was two minutes and the maximum was 11 minutes. At the end of the evaluation, it was possible to quantify the development in 100% of the cases. CONCLUSIONS: EDAL proved an agile, quick and easy, instrument to be used in the first two years of hearing age.
Assuntos
Linguagem Infantil , Implantes Cocleares , Testes Auditivos/métodos , Audição/fisiologia , Brasil , Humanos , Lactente , PaisRESUMO
Several studies have shown that cochlear implants may reduce or even eliminate tinnitus in patients with bilateral profound hearing loss. However, there are not consistent references regarding ipsilateral tinnitus compared to unilateral profound hearing loss. The aim of this paper is to describe audiological results of a patient with asymmetrical hearing loss with incapacitating ipsilateral tinnitus in the ear subjected to cochlear implant surgery. Audiological exams and responses to perception protocols for tinnitus before and after surgery were analyzed. The tests showed improvements in the hearing threshold on the side with the implant, improvements in speech perception and a significant reduction in tinnitus perception, which consequently led to an improvement in the patient's quality of life.
