Arthur Menino Castilho

Resultados auditivos com o implante coclear multicanal em pacientes submetidos a cirurgia no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

Complete hearing loss is a very important handicap resulting in sensory deprivation and affecting an individuals personality, relationships and life style. Individuals with profound deafness are unable to hear environmental sounds as doorbells, telephones, traffic noises and sirens that are an important alert for dangerous situations in the daily life. They are also unable to hear their own voices and for this people with profound deaf do not modulate their voices. The hearing aids are the first choice to treat deafness, but there are patients with severe damage of the auditory systems who cannot receive a useful hearing from normal hearing aids. The cochlear implant is the alternative for patients that cannot have good discriminatory ability wearing a powerful hearing aid and wish or need a better hearing. AIM: To study the hearing results of the adult patients who are using a multichannel cochlear implant system at the Department of Otolaryngology - University of Sao Paulo. STUDY DESIGN: Series study. MATERIAL AND METHOD: We selected 61 patients with a severe bilateral hearing loss that have been using the cochlear implant for at least six months. The hearing evaluation performed after using the device for six months included word and sentence recognition tests. RESULTS: The PTA of four patients reached 38.7 dB. Speech perception tests showed 713% recognition of open set sentences, 86.5% of vowels and 52.60% of monosyllabic. Most of our patients are able to speak on the telephone. CONCLUSION: Most of patients showed excellent results in open-set sentence and word tests.A surdez profunda e uma deficiencia que afeta a personalidade, o relacionamento e todo o estilo de vida do paciente. Incapacita os individuos de escutarem sons ambientes como sirenes e alarmes que constituem alerta em situacoes da vida diaria e nao permite a modulacao vocal, tornando a voz esteticamente ruim. A protese auditiva convencional e eficiente no tratamento de grande parte das deficiencias auditivas, porem existem pacientes que nao conseguem obter discriminacao de palavras e sentencas mesmo com uma protese auditiva potente. O implante coclear e a alternativa atual para estes pacientes. OBJETIVO: Avaliar o resultado auditivo dos pacientes adultos implantados com o implante coclear multicanal pelo Grupo de Implante Coclear da Disciplina de Otorrinolaringologia da Faculdade de medicina da Universidade de Sao Paulo. FORMA DE ESTUDO: Estudo de serie. CASUISTICA E METODO: Foram selecionados 61 pacientes com surdez profunda bilateral que utilizam o implante coclear multicanal por pelo menos seis meses e estudados os resultados auditivos atraves de testes de reconhecimento de palavras e sentencas. RESULTADOS: O PTA medio obtido pelos pacientes foi 38.7 dB NPS. A media em reconhecimento de sentencas em formato aberto foi de 71.3%, vogais em 86.5%, monossilabos em 52.60% e consoante medial em 52.6%. A maioria dos pacientes esta apta ao uso do telefone. CONCLUSAO: A avaliacao auditiva obtida seis meses apos a primeira programacao do implante demonstra que pacientes de lingua portuguesa obtem excelentes resultados em testes de reconhecimento de palavras e sentencas em apresentacao aberta, readquirindo uma audicao util.

Telemetria de resposta neural intra-operatória em usuários de implante coclear

A possibilidade de realizar o implante coclear em criancas pequenas torna necessario o uso de medidas objetivas para auxiliar a programacao do processador de fala. Telemetria e a propriedade que permite, no Nucleus 24®, a obtencao do potencial de acao composto evocado do VIII par (EAP) utilizando o implante como instrumento de estimulacao e gravacao para o estudo das propriedades neurais remanescentes. OBJETIVO: Descrever a utilizacao do sistema de telemetria para a gravacao do EAP, caracterizando as respostas obtidas e a sua prevalencia na condicao intraoperatoria. FORMA DE ESTUDO: clinico com coorte transversal. MATERIAL E METODO: Medidas das impedâncias dos eletrodos e do EAP em um grupo de 17 individuos usuarios do implante Nucleus 24® durante a cirurgia. Analise das respostas de acordo com a etiologia, o tempo de duracao da surdez e a posicao dos eletrodos dentro da coclea. RESULTADOS: Maior prevalencia nos eletrodos apicais e limiares mais elevados nos casos de meningite e otosclerose. CONCLUSAO: A telemetria e eficiente para a verificacao da integridade dos eletrodos na condicao intraoperatoria e para a gravacao do EAP, apresentando alta prevalencia na populacao estudada.

Neural response telemetry measures in patients implanted with Nucleus 24

UNLABELLED Cochlear implantation has been recommended for children under 24 months of age. The use of objective measures is needed to help speech processor programming. The electrically evoked compound potential (EAP), which can be assessed by neural response telemetry (NRT), is one of those objective measures. AIM to determine how often the EAP can be recorded by NRT system during surgery and to describe the responses. STUDY DESIGN Clinical with transversal cohort. MATERIAL AND METHOD The impedances and NRT were measured in a group of 17 Nucleus 24 implant users. The responses were analyzed and compared to the etiology, hearing loss duration and electrode array position. RESULTS The EAP was easily recorded in the apical electrodes and, in otosclerosis and meningitis cases the EAP threshold was higher than in the other etiology cases. CONCLUSIONS The NRT can be found in 82% of the cases during surgery. The responses obtained may vary according to etiology and the position of electrodes along the cochlea.

Molecular analysis of SLC26A4 gene in patients with nonsyndromic hearing loss and EVA: Identification of two novel mutations in Brazilian patients

UNLABELLED The SLC26A4 gene has been described as the second gene involved in most cases of sensorineural non-syndromic hearing loss, since the first is the GJB2 gene. Recessive mutations in the SLC26A4 gene encoding pendrin, an anion transporter, are responsible for non-syndromic hearing loss associated with an enlarged vestibular aqueduct (EVA) and Pendred syndrome, which causes early hearing loss and affects the thyroid gland. Typically, the hearing loss is profound and prelingual. However, in some individuals, hearing impairment may develop later in childhood and then progress. Over 200 different SLC26A4 mutations have been reported, with each ethnic population having its own distinctive mutant allele series including a few prevalent founder mutations. OBJECTIVE Perform the screening of the 20 coding exons of SLC26A4 gene in Brazilian deaf individuals with EVA. PATIENTS AND METHODS Among the 23 unrelated non-syndromic hearing loss Brazilian patients with EVA, in whom no deafness-causing mutations of the GJB2 gene, the direct sequencing was performed to screen the 20 exons and their flanking regions of the SLC26A4 gene. RESULTS The sequencing results revealed 9 cases (39%) carrying 13 different SLC26A4 mutations, including 11 known mutations (279delT, V138F, T193I, IVS8+1G>A, T410M, Q413R, R409H, L445W, IVS15+5G>A, V609G, and R776C) and 2 novel mutation (G149R and P142L). CONCLUSION The SLC26A4 mutations have a high carrying rate in non-syndromic hearing loss Brazilian patients. The identification of a disease-causing mutation can be used to establish a genotypic diagnosis and provide important information to the patients and their families.

Etiologic and diagnostic evaluation: algorithm for severe to profound sensorineural hearing loss in Brazil.

Abstract Objective: Evaluation of the effectiveness of imaging and genetic testing, and establishment of a cost-effective diagnostic protocol for the etiologic diagnosis of sensorineural hearing loss (SNHL) in Brazil. Design: Prospective cohort study. Study sample: Analysis of 100 unrelated Brazilian patients with severe to profound bilateral SNHL submitted to cochlear implant (CI) between 2002 and 2010 at the University of Campinas hospital. The study was based upon three groups: individuals with congenital, progressive, and sudden SNHL. Results: After the diagnostic investigation, the number of cases with unknown etiology was reduced from 72 to 42 (a 42% reduction); 25% of cases were due to environmental factors, 19% to genetic causes, and 14% to inner-ear abnormalities or other clinical features. The genetic and imaging findings contributed to the diagnosis of SNHL in 19% and 20% of the cases analysed, respectively. Molecular testing mainly contributed to the diagnosis of patients with congenital SNHL, while the contribution of radiologic examination was higher for individuals with progressive or sudden SNHL. A sequential diagnostic protocol was proposed based on these data. Conclusions: The proposed diagnostic workup algorithm could provide better optimization of etiologic diagnosis, as well as reduced costs, compared to a simultaneous testing approach.

Hearing preservation and cochlear implants according to inner ear approach: multicentric evaluation.

INTRODUCTION Electroacoustic stimulation is an excellent option for people with residual hearing in the low frequencies, who obtain insufficient benefit with hearing aids. To be effective, the subjects residual hearing should be preserved during cochlear implant surgery. OBJECTIVES To evaluate the hearing preservation in patients that underwent implant placement and to compare the results in accordance with the approach to the inner ear. METHODS 19 subjects underwent a soft surgical technique, and the electrode MED-EL FLEX™ EAS, designed to be atraumatic, was used. We evaluated pre- and postoperative tonal audiometric tests with an average of 18.4 months after implantation, to measure the rate of hearing preservation. RESULTS 17 patients had total or partial preservation of residual hearing; 5 had total hearing preservation and two individuals had no preservation of hearing. The insertion of the electrode occurred through a cochleostomy in 3 patients, and in 2 of these there was no hearing preservation; the other 16 patients experienced electrode insertion through a round window approach. All patients benefited from the cochlear implant, even those who are only using electrical stimulation. CONCLUSION The hearing preservation occurred in 89.4% of cases. There was no significant difference between the forms of inner ear approach.

Digisonic SP® Binaural cochlear implant: the coronal tunneled approach.

UNLABELLED Cochlear implants represent a significant breakthrough in the treatment of hearing loss. Evidence indicates bilateral hearing brings significant benefits to patients, particularly when binaural hearing is offered. OBJECTIVE To describe the first case of implantation of a Digisonic SP® Binaural Neurelec device in Brazil (the third implant placed in the Americas, after Mexico and Colombia) and the chosen surgical approach. METHOD Description of a surgical approach. RESULTS The procedure was successfully completed. DISCUSSION The squelch effect, binaural summation, location of the sound source, and the shadow effect of the head are listed among the reasons to explain the superiority of binaural rehabilitation. Cost of treatment must be considered in the development of public health policies. CONCLUSION The cost of cochlear implants has been one of the main impediments to bilateral rehabilitation. The Digisonic SP® Binaural Neurelec device addresses this issue and exposes patients to less risk through a minimally invasive implantation procedure.

Electro acoustic stimulation of the auditory system: UNICAMP's surgical approach

UNLABELLED A new era has arrived in auditory rehabilitation with the introduction of new technologies such as electroacoustic stimulation (EAS). EAS is indicated for patients with residual hearing at low frequencies and severe or profound hearing loss at high frequencies. These patients have no indication for conventional cochlear implant and have difficulties in adapting to individual sound amplification devices. Preservation of hearing is vital in this process; the surgical technique must be based on this concept. OBJECTIVES To present the cochlear implant surgical technique with MED-EL Mand FlexEAS to preserve hearing in patients with hearing loss at high frequencies and to maintain low frequency hearing. We are the first institution to carry out this treatment in Brazil. METHODS A case report of the surgical technique carried out in four patients; the procedure was carried out by the cochlear implant group of a specialized clinical hospital. RESULTS The procedures were successful and uneventful. CONCLUSION We described the technique used at our institution for implants using EAS; the surgical technique is complex and includes steps for preservation of hearing.

Audiological and genetics studies in high-risk infants

UNLABELLED Hearing is one of the main ways with which one person can contact the external world; it plays a key role in their integration with society. AIM The objective of this study was to analyze the results of the hearing, medical and genetic evaluation of high-risk infants who failed the newborn hearing screening. MATERIALS AND METHODS Clinical and experimental study. We assessed thirty-eight neonates, with ages between one and six months. The infants underwent the following procedures: medical interview; immittance testing; Brainstem Auditory Evoked Potential; Transient Evoked Otoacoustic Emission and otorhinolaryngological evaluation. DNA extraction from the oral mucosa was performed for genetic studies using the protocol method adapted from the Human Genetics Lab of the CBMEG/UNICAMP. RESULTS Regarding gender and presence of risk factors, significant statistically differences were not found in normal hearing infants and in those with hearing loss. Concerning gestational age, term infants were more affected by hearing loss. Hearing loss was identified in 58% of the sample, conduction hearing loss represented 31.5% (12/38) and neurossensory 28.9% of cases. There were none of the genetic mutations most commonly seen in cases with a genetic etiology. CONCLUSION Hearing loss was identified in the majority of High-risk infants.

Advanced Bionics Cochlear Implants in Patients with Prelingual Hearing Loss

Introduction: Cochlear Implants (CI) have become standard in the treatment of prelingual, postlingual and perilingual deafness in children. Bilateral implants are considered standard for bilaterally affected children. Studies also find that the CI provides better access to speech for most children, and this access results in improved speech perception. In earlier times children who did not react to acoustic stimuli and were neither able to understand speech nor to acquire it spontaneously encountered severe discrimination, being dismissed as simple-minded or worse. Different studies broadly agree that one or two of every 1000 newborns have a hearing impairment that on current evidence warrants treatment or observation, i.e., permanent hearing loss with a lowering of the absolute threshold of hearing for speech perception by at least 35 dB. Approximately 50% of severe hearing impairments arising in the inner ear are thought to be hereditary in origin. When new Cochlear Implant (CI) sound processors are being introduced by the manufacturers, usually the newest generation implants benefit first from the new technology in order to release the full potential of the new hardware. Objective: Evaluate the improvement of speech language and sound perception in patients with prelingual deafness that underwent cochlear implant using Advanced Bionics® device. Method: Retrospective study of the medical records of the patients fitted with Advanced Bionics® cochlear implant in our institution between 2011 and 2012. Results: Sixteen patients underwent to cochlear implantation using Advanced Bionics® devices. There were 43,75% prelingual and 43,75% postlingual patients with bilateral hearing loss. Mean age at implantation in the prelingual group was 3.6 years (ranged from 2 to 6 years). There was one case with medical history of deafness in family. All prelingual patients used hearing devices before the cochlear implant. The hearing levels improved after CI in all patients. Conclusion: This study evaluated patients with pre-lingual deafness using the Advanced Bionics® cochlear implants demonstrated significant gains in neural stimulation and language development in children.