Programming peculiarities in two cochlear implant users with superficial siderosis of the central nervous system

To report the audiological outcomes of cochlear implantation in two patients with severe to profound sensorineural hearing loss secondary to superficial siderosis of the CNS and discuss some programming peculiarities that were found in these cases. Retrospective review. Data concerning clinical presentation, diagnosis and audiological assessment pre- and post-implantation were collected of two patients with superficial siderosis of the CNS. Both patients showed good hearing thresholds but variable speech perception outcomes. One patient did not achieve open-set speech recognition, but the other achieved 70% speech recognition in quiet. Electrical compound action potentials could not be elicited in either patient. Map parameters showed the need for increased charge. Electrode impedances showed high longitudinal variability. The implants were fairly beneficial in restoring hearing and improving communication abilities although many reprogramming sessions have been required. The hurdle in programming was the need of frequent adjustments due to the physiologic variations in electrical discharges and neural conduction, besides the changes in the impedances. Patients diagnosed with superficial siderosis may achieve limited results in speech perception scores due to both cochlear and retrocochlear reasons. Careful counseling about the results must be given to the patients and their families before the cochlear implantation indication.

Cochlear implant in postlingual children: functional results 10 years after the surgery

The benefits of cochlear implants (CI) for communication skills are obtained over the years. There are but a few studies regarding the long-term outcomes in postlingual deaf children who grew up using the electronic device. Aim: To assess the functional results in a group of postlingual children, 10 years after using a CI. Methods: Ten postlingual deaf children, implanted before 18 years of age, participated in this study. We assessed: sentence recognition and speech intelligibility. We documented: device use and function and the patient's academic/occupational status. Study design: series. Results: The mean scores were 73% for sentence recognition in silence and 40% in noise. The average write-down intelligibility score was 92% and the average rating-scale intelligibility score was 4.15. There were no cases of device failure. Regarding educational/vocational status, three subjects graduated from the University. Five quit education after completing high school. Eight subjects had a professional activity. Conclusion: This study showed that cochlear implantation is a safe and reliable procedure. The postlingual profoundly hearing-impaired children after 10 years of CI use developed satisfactory levels regarding speech perception and intelligibility, and completed at least high school and were inserted in the labor market. Clinical Trials Registry: NCT01400178.

Speech perception and cortical auditory evoked potentials in cochlear implant users with auditory neuropathy spectrum disorders

Objective: To characterize the PI component of long latency auditory evoked potentials (LLAEPs) in cochlear implant users with auditory neuropathy spectrum disorder (ANSD) and determine firstly whether they correlate with speech perception performance and secondly whether they correlate with other variables related to cochlear implant use. Methods: This study was conducted at the Center for Audiological Research at the University of Sao Paulo. The sample included 14 pediatric (4-11 years of age) cochlear implant users with ANSD, of both sexes, with profound prelingual hearing loss. Patients with hypoplasia or agenesis of the auditory nerve were excluded from the study. LLAEPs produced in response to speech stimuli were recorded using a Smart EP USB Jr. system. The subjects' speech perception was evaluated using tests 5 and 6 of the Glendonald Auditory Screening Procedure (GASP). Results: The P-1 component was detected in 12/14 (85.7%) children with ANSD. Latency of the P-1 component correlated with duration of sensorial hearing deprivation (*p = 0.007, r = 0.7278), but not with duration of cochlear implant use. An analysis of groups assigned according to GASP performance (k-means clustering) revealed that aspects of prior central auditory system development reflected in the P-1 component are related to behavioral auditory skills. Conclusions: In children with ANSD using cochlear implants, the P-1 component can serve as a marker of central auditory cortical development and a predictor of the implanted child's speech perception performance. (c) 2012 Elsevier Ireland Ltd. All rights reserved.

Measures of quality of life in children with cochlear implant: systematic review

The use of cochlear implant (CI) in children enables the development of listening and communication skills, allowing the child's progress in school and to be able to obtain, maintain and carry out an occupation. However, the progress after the CI has different results in some children, because many children are able to interact and participate in society, while others develop limited ability to communicate verbally. The need for a better understanding of CI outcomes, besides hearing and language benefits, has spurred the inclusion of quality of life measurements (QOL) to assess the impact of this technology. OBJECTIVE: Identify the key aspects of quality of life assessed in children with cochlear implant. METHOD: Through a systematic literature review, we considered publications from the period of 2000 to 2011. CONCLUSION: We concluded that QOL measurements in children include several concepts and methodologies. When referring to children using CI, results showed the challenges in broadly conceptualizing which quality of life domains are important to the child and how these areas can evolve during development, considering the wide variety of instruments and aspects evaluated.

Cochlear Implantation Via the Middle Fossa Approach: Surgical and Programming Considerations

Objectives: To report the results of cochlear implantation via the middle fossa approach in 4 patients, discuss the complications, and present a detailed description of the programming specifications in these cases. Study Design: Retrospective case review. Setting: Tertiary-care referral center with a well-established cochlear implant program. Patients: Four patients with bilateral canal wall down mastoid cavities who underwent the middle fossa approach for cochlear implantation. Interventions: Cochlear implantation and subsequent rehabilitation. A middle fossa approach with cochleostomy was successfully performed on the most superficial part of the apical turn in 4 patients. A Nucleus 24 cochlear implant system was used in 3 patients and a MED-EL Sonata Medium device in 1 patient. The single electrode array was inserted through a cochleostomy from the cochlear apex and occupied the apical, middle, and basal turns. Telemetry and intraoperative impedance recordings were performed at the end of surgery. A CT scan of the temporal bones was performed to document electrode insertion for all of the patients. Main Outcome Measures: Complications, hearing thresholds, and speech perception outcomes were evaluated. Results: Neural response telemetry showed present responses in all but 1 patient, who demonstrated facial nerve stimulation during the test. Open-set speech perception varied from 30% to 100%, despite the frequency allocation order of the MAP. Conclusion: Cochlear implantation via the middle cranial fossa is a safe approach, although it is a challenging procedure, even for experienced surgeons.

Surgical complications in 550 consecutive cochlear implantation

Cochlear implantation is a safe and reliable method for auditory restoration in patients with severe to profound hearing loss. Objective: To describe the surgical complications of cochlear implantation. Materials and Methods: Information from 591 consecutive multichannel cochlear implant surgeries were retrospectively analyzed. All patients were followed-up for at least one year. Forty-one patients were excluded because of missing data, follow-up loss or middle fossa approach. Results: Of 550 cochlear implantation analyzed, 341 were performed in children or adolescents, and 209 in adults. The mean hearing loss time was 6.3 +/- 6.7 years for prelingual loss and 12.1 +/- 11.6 years for postlingual. Mean follow-up was 3.9 +/- 2.8 years. Major complications occurred in 8.9% and minor in 7.8%. Problems during electrode insertion (3.8%) were the most frequent major complication followed by flap dehiscence (1.4%). Temporary facial palsy (2.2%), canal-wall lesion (2.2%) and tympanic membrane lesion (1.8%) were the more frequent minor complications. No death occurred. Conclusion: There was a low rate of surgical complications, most of them been successfully managed. These results confirm that cochlear implant is a safe surgery and most surgical complications can be managed with conservative measures or minimal intervention.

Cochlear implanted children present vocal parameters within normal standards

Objective: to compare acoustic and perceptual parameters regarding the voice of cochlear implanted children, with normal hearing children. Method: this is a cross-sectional, quantitative and qualitative study. Methods: Thirty six cochlear implanted children aged between 3y and 3 m to 5y and 9 m and 25 children with normal hearing, aged between 3y and 11 m and 6y and 6 m, participated in this study. The recordings and the acoustics analysis of the sustained vowel/a/and spontaneous speech were performed using the PRAAT program. The parameters analyzed for the sustained vowel were the mean of the fundamental frequency, jitter, shimmer and harmonic-to-noise ratio (HNR). For the spontaneous speech, the minimum and maximum frequencies and the number of semitones were extracted. The perceptual analysis of the speech material was analyzed using visual-analogical scales of 100 points, composing the aspects related to the overall severity of the vocal deviation, roughness, breathiness, strain, pitch, loudness and resonance deviation, and instability. This last parameter was only analyzed for the sustained vowel. Results: The results demonstrated that the majority of the vocal parameters analyzed in the samples of the implanted children disclosed values similar to those obtained by the group of children with normal hearing. Conclusion: implanted children who participate in a (re) habilitation and follow-up program, can present vocal characteristics similar to those vocal characteristics of children with normal hearing. (C) 2012 Elsevier Ireland Ltd. All rights reserved.

Auditory brainstem implant outcomes and MAP parameters: Report of experiences in adults and children

The auditory brainstem implant (ABI) was first developed to help neurofibromatosis type 2 patients. Recently, its use has been recently extended to adults with non-tumor etiologies and children with profound hearing loss who were not candidates for a cochlear implant (Cl). Although the results has been extensively reported, the stimulation parameters involved behind the outcomes have received less attention. Objective: The aim of this study is to describe the audiologic outcomes and the MAP parameters in ABI adults and children at our center. Methods: Retrospective chart review. Five adults and four children were implanted with the ABI24M from September 2005 to June 2009. In the adult patients, four had Neurofibromatosis type 2, and one had postmeningitic deafness with complete ossification of both cochleae. Three of the children had cochlear malformation or dysplasia, and one had complete ossified cochlea due to meningitis. Map parameters as well as the intraoperative electrical auditory brainstem responses were collected. Evaluation was performed with at least six months of device use and included free-field hearing thresholds, speech perception tests in the adult patients and for the children, the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and (ESP) were used to evaluate the development of auditory skills, besides the MUSS to evaluate. Results: The number of active electrodes that did not cause any non-auditory sensation varied from three to nineteen. All of them were programmed with SPEAK strategy, and the pulse widths varied from 100 to 300 mu s. Free-field thresholds with warble tones varied from very soft auditory sensation of 70 dBHL at 250 Hz to a pure tone average of 45 dBHL. Speech perception varied from none to 60% open-set recognition of sentences in silence in the adult population and from no auditory sensation at all to a slight improvement in the IT-MAIS/MAIS scores. Conclusion: We observed that ABI may be a good option for offering some hearing attention to both adults and children. In children, the results might not be enough to ensure oral language development. Programming the speech processor in children demands higher care to the audiologist. (C) 2011 Elsevier Ireland Ltd. All rights reserved.

Crianças com implante coclear: habilidades comunicativas e qualidade de vida

Dado o âmbito multidimensional do implante coclear, há crescente necessidade em avaliar não somente medidas clínicas de eficácia relacionadas às habilidades comunicativas, mas também aspectos mais genéricos envolvidos na efetividade do tratamento, como a qualidade de vida. OBJETIVOS: Tradução e adaptação de questionário internacional para o Português Brasileiro; análise das correlações entre fatores relacionados à qualidade de vida; análise das correlações entre qualidade de vida e medidas clínicas de resultado. MATERIAL E MÉTODO: Estudo prospectivo realizado com pais de crianças com implante coclear consistindo na aplicação de instrumentos validados para avaliar aspectos de qualidade de vida e habilidades comunicativas. RESULTADOS: A tradução e adaptação cultural do questionário foi satisfatoriamente realizada e este estudo proporciona a disponibilização do questionário em versão para o Português Brasileiro. Pelos dados obtidos, o implante coclear apresentou efeito positivo na qualidade de vida das crianças implantadas e de suas famílias. As correlações observadas para a variável comunicação demonstram uma relação direta entre comunicação oral e outras variáveis de qualidade de vida. CONCLUSÃO: Este estudo disponibiliza o questionário em versão para o Português Brasileiro. Para os pais de crianças brasileiras usuárias de implante coclear, a habilidade lexical (aquisição e uso das palavras) é a variável de maior impacto na qualidade de vida de seus filhos.

Avaliação da qualidade de vida em crianças com implante coclear: revisão sistemática

The use of cochlear implant (CI) in children enables the development of listening and communication skills, allowing the child's progress in school and to be able to obtain, maintain and carry out an occupation. However, the progress after the CI has different results in some children, because many children are able to interact and participate in society, while others develop limited ability to communicate verbally. The need for a better understanding of CI outcomes, besides hearing and language benefits, has spurred the inclusion of quality of life measurements (QOL) to assess the impact of this technology. OBJECTIVE: Identify the key aspects of quality of life assessed in children with cochlear implant. METHOD: Through a systematic literature review, we considered publications from the period of 2000 to 2011. CONCLUSION: We concluded that QOL measurements in children include several concepts and methodologies. When referring to children using CI, results showed the challenges in broadly conceptualizing which quality of life domains are important to the child and how these areas can evolve during development, considering the wide variety of instruments and aspects evaluated.

O uso da Classificação Internacional de Funcionalidade, Incapacidade e Saúde para acompanhamento de pacientes usuários de Implante Coclear.

Purpose: To characterize the profile of patients with cochlear implant as proposed by the International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY). Methods: This is a descriptive, crosssectional retrospective study, which examined 30 medical records of patients using the cochlear implant of Centro de Pesquisas Audiológicas. To characterize the profile of the patients, the ICF-CY was used. Regarding the assessment, researchers relied on procedures performed in clinical routine, besides information registered in the medical record. After reviewing the information, it was related to codes from the ICF-CY; with the addition of a qualifier afterwards. Results: Overall, 55 codes from the ICF were related to the instruments to characterize this population. Regarding the Body Functions field, most participants did not have disabilities related to reception and expression of oral language and auditory functions, with only written language disabilities being found. These same findings were observed in the Activity and Participation Field. Regarding environmental factors, noise and the non-availability of technology resources to assist in the auditory comprehension of noise were characterized as a barrier, as well as the absence of speech therapy. Conclusion: This study concluded that most of the participating children showed no deficiency in the body functions, with difficulties being only reported in relation to school performance. Environmental factors (noise, non-availability of technological resources, absence of speech therapy) were characterized as a barrier. The need to expand assessments in the clinical routine was also noted.

Dimensão afetiva da pessoa com surdez adquirida, antes e após o implante coclear

Este estudo teve como objetivo averiguar, antes e após o uso do implante coclear, a dimensão afetiva em pacientes adultos com surdez adquirida, no que diz respeito às modalidades dos sentimentos egoicos, sentimentos em relação ao próximo, sentimentos de temporalidade e estados de ânimo. Participaram 44 adultos que realizaram o implante coclear no Centro de Pesquisas Audiológicas do Hospital de Reabilitação de Anomalias Craniofaciais da Universidade de São Paulo de Bauru. Concluiu-se que, na vivência da surdez, houve predomínio de sentimentos negativos e de um clima afetivo de tensão e depressão, que levava o sujeito a uma vinculação negativa, de assintonia com o mundo. Entretanto, na vivência com o implante coclear, houve predomínio de sentimentos positivos e de um clima afetivo de tranquilidade e contentamento, observando-se uma vinculação positiva do sujeito e sintonia com o mundo.

Avaliação das habilidades escolares de crianças com implante coclear

OBJETIVO: avaliar e caracterizar o desempenho escolar de crianças com deficiência auditiva usuárias de implante coclear, entre 9 e 12 anos, assim como, verificar o tempo que a criança leva para acessar a informação fonológica e confrontar estes resultados de acordo com o gênero e série dos participantes. MÉTODO: participaram deste estudo 32 crianças, de ambos os gêneros, freqüentando da primeira a quinta séries do ensino fundamental, regularmente matriculadas no Centro de Pesquisas Audiológicas do Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, na cidade de Bauru/SP. Os instrumentos utilizados foram o Teste de Desempenho Escolar (TDE) e o Teste de Nomeação Automática Rápida (RAN). RESULTADOS: os resultados mostraram que 74% das crianças apresentaram desempenho escolar geral abaixo do esperado, com maior dificuldade na escrita e dentro da média para o tempo que levam ao processar a informação. CONCLUSÕES: concluiu-se que 74% das crianças apresentaram desempenho escolar inferior nas avaliações e que não houve relação entre estes resultados com a nomeação rápida, já que este está dentro da média e é um pré-requisito para a leitura, e os participantes deste estudo apresentam habilidade satisfatória para a leitura.

Radiografia do implante coclear: técnica adaptada para aparelho portátil

Considerando a indisponibilidade de equipamentos avançados de aquisição de imagens nos centros cirúrgicos da maioria dos centros hospitalares e a importância fundamental que têm para o cirurgião uma visualização imediata do implante coclear logo após sua inserção, uma boa opção é a utilização da radiografia convencional. OBJETIVO: Descrever um método radiográfico rápido prático e de baixo custo, que permita avaliar não só a posição, mas também a integridade dos eletrodos, na instalação do implante coclear. MATERIAL E MÉTODO: Foram analisadas radiografias de 262 pacientes submetidos à cirurgia de implante coclear entre Março/2005 e Outubro/2008, com radiografia transoperatória, logo após a inserção dos eletrodos. As radiografias foram analisadas pelo cirurgião no transoperatório e, posteriormente, pelo médico radiologista. RESULTADOS: Foram analisadas 524 radiografias das quais, 95,61% apresentavam técnica adequada, com posicionamento do paciente dentro da técnica descrita neste estudo e boa visualização dos eletrodos, sendo consideradas satisfatórias e 4,39% apresentavam técnica inadequada e/ou visualização insatisfatória dos eletrodos, sendo consideradas insatisfatórias. CONCLUSÃO: Apesar dos aparelhos de Raios X portáteis possuírem limitações, utilizando técnicas e acessórios adequados, é possível conseguir radiografias com resultados satisfatórios para visualização dos implantes cocleares.