Neuropatia auditiva: avaliação clínica e abordagem diagnóstica

Auditory neuropathy is a condition in which there is a change in the neuronal transmission of the auditory stimuli. Our objective was to describe the patients’ series within the clinical spectrum of auditory neuropathy. We designed a transversal, retrospective study, with a description of a consecutive case series. Auditory neuropathy was defined by the presence of acoustic otoemissions plus absent/abnormal auditory brainstem responses with cochlear microphonism. 34 patients with bilateral hearing loss, 23 males and 11 females, were included in the study. Eighty percent of the cases had congenital onset of hearing loss. Acoustic otoemissions were absent in 67% of them. Cochlear microfonism was present in 79% of all cases. Prenatal, perinatal or ambiental factors were present in 35.2% of the cases. Medical literature shows great variability in findings related to auditory neuropathy, both in its etiology and epidemiological data. Auditory neuropathy presents a broad spectrum of changes that may result from mild to severe changes in the functioning of the auditory pathway, and in our sample we observed that 80% of Auditory neuropathy have congenital onset of hearing loss and/or with cochlear microphonism identified. 91% of patients experience significant hearing impairment and 53% suffer from severe or profound deafness6353359CONSELHO NACIONAL DE DESENVOLVIMENTO CIENTÍFICO E TECNOLÓGICO - CNPQCOORDENAÇÃO DE APERFEIÇOAMENTO DE PESSOAL DE NÍVEL SUPERIOR - CAPESnão temnão temA neuropatia auditiva é uma condição na qual há alteração na condução neuronal do estímulo sonoro. Este trabalho pretende descrever e caracterizar a casuística de doentes com neuropatia auditiva. Realizámos um estudo transversal, retrospetivo, com descrição de uma série de casos consecutivos. O diagnóstico da neuropatia auditiva foi definido nas seguintes situações: Presença de otoemissões acústicas com potenciais auditivos de tronco encefálico ausente ou anormal e presença do microfonismo coclear independentemente da presença de otoemissões acústicas. Foram avaliados 34 doentes com perda auditiva bilateral, 67% deles do sexo masculino. O aparecimento dos sintomas foi congênito em 80% dos casos. Na pesquisa das otoemissões acústicas, a resposta foi ausente em 67% dos doentes. O microfonismo coclear foi detetado em 79% dos doentes. Antecedentes gestacionais, perinatais ou ambientais relevantes estiveram presentes em 35,3% dos casos. A literatura médica ainda apresenta grande variabilidade nos achados relacionados com a neuropatia auditiva, tanto na sua etiologia quanto nos dados epidemiológicos. A neuropatia auditiva apresenta um amplo espectro de alterações que podem resultar em disfunções leves a severas no funcionamento da via auditiva. Na nossa amostra, observámos que 80% das neuropatias auditivas terão tido origem congênita e/ou apresenta microfonismo coclear, 91% dos doentes apresenta défice auditivo significativo e 53% sofrem de surdez severa ou profunda

Evaluation of the Digisonic® SP cochlear implant: patient outcomes and fixation system with titanium screws

Cochlear implants have revolutionized the way patients affected by severe hearing loss experience the world. Neurelec developed a fixation system with two titanium screws that requires no skull bone drilling. OBJECTIVE: To describe the outcomes and procedure-related details of a series of patients implanted with the Digisonic® SP cochlear implant. METHOD: This retrospective study analyzed patients submitted to cochlear implant placement within a period of 18 months. All patients had postlingual hearing impairment. Data was collected from patient charts and standard questionnaires answered by the surgeons in charge of carrying out the procedures. RESULTS: The six patients offered the Digisonic® SP cochlear implants were operated by experienced surgeons. The procedures took 95 to 203 minutes (mean = 135') to be completed, which is less time than what has been described for other fixation approaches. No complications were recorded and hearing improvement was satisfactory. CONCLUSION: The Digisonic® SP cochlear implant developed by Neurelec offered good audiological results for adult patients, shorter surgery time, and no surgical or postoperative complications.O implante coclear para pacientes com disacusia grave/profunda revolucionou o modo com que estes interagem com o meio ambiente. A Neurelec desenvolveu um sistema que permite fixação com uso de dois simples parafusos de titânio, sem necessidade de broqueamento do osso do crânio do paciente. Este artigo teve o objetivo de descrever uma casuística de pacientes submetidos a implante coclear com o Digisonic® SP, para mostrar os resultados cirúrgicos e detalhes do procedimento. MÉTODO: Estudo retrospectivo que avaliou os pacientes, no período de 18 meses, submetidos a implante coclear com o Digisonic® SP. Todos os pacientes eram pós-linguais. Coleta dos dados realizada por meio da análise dos prontuários dos pacientes, além de questionário padronizado aplicado nos cirurgiões que realizaram o procedimento. RESULTADOS: Os seis casos implantados com o Digisonic® SP foram realizados por cirurgiões experientes e o tempo cirúrgico variou de 95 a 203 minutos (média de 135 minutos), que é menor do que o descrito com outras formas de fixação. Não houve nenhum tipo de complicação e o ganho auditivo foi satisfatório. CONCLUSÃO: O implante Digisonic® SP desenvolvido pela Neurelec apresentou bons resultados audiológicos em adultos, tempo de cirurgia menor e não ocorreram complicações cirúrgicas ou pós-operatórias.566

SARS-CoV-2 infection with bilateral intralabyrinthine hemorrhage

A 47-year-old woman presented with the complaint of sudden hearing loss associated with vertigo. Serological testing was positive for IgM and negative for IgG COVID-19 antibodies, with no other associated factors. Magnetic resonance imaging of the brain showed bilateral intralabyrinthine hemorrhage

Neural response telemetry measures in patients implanted with Nucleus 24®

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.A possibilidade de realizar o implante coclear em crianças pequenas torna necessário o uso de medidas objetivas para auxiliar a programação do processador de fala. Telemetria é a propriedade que permite, no Nucleus 24®, a obtenção do potencial de ação composto evocado do VIII par (EAP) utilizando o implante como instrumento de estimulação e gravação para o estudo das propriedades neurais remanescentes. OBJETIVO: Descrever a utilização do sistema de telemetria para a gravação do EAP, caracterizando as respostas obtidas e a sua prevalência na condição intraoperatória. FORMA DE ESTUDO: clínico com coorte transversal. MATERIAL E MÉTODO: Medidas das impedâncias dos eletrodos e do EAP em um grupo de 17 indivíduos usuários do implante Nucleus 24® durante a cirurgia. Análise das respostas de acordo com a etiologia, o tempo de duração da surdez e a posição dos eletrodos dentro da cóclea. RESULTADOS: Maior prevalência nos eletrodos apicais e limiares mais elevados nos casos de meningite e otosclerose. CONCLUSÃO: A telemetria é eficiente para a verificação da integridade dos eletrodos na condição intraoperatória e para a gravação do EAP, apresentando alta prevalência na população estudada.HC-FMUSP Santa Casa de Misericórdia de SPHC-FMUSPUNIFESPPUC-SPUNIFESPSciEL

Screening Of Genetic Alterations Related To Non-syndromic Hearing Loss Using Massarray Iplex® Technology.

Recent advances in molecular genetics have enabled to determine the genetic causes of non-syndromic hearing loss, and more than 100 genes have been related to the phenotype. Due to this extraordinary genetic heterogeneity, a large percentage of patients remain without any molecular diagnosis. This condition imply the need for new methodological strategies in order to detect a greater number of mutations in multiple genes. In this work, we optimized and tested a panel of 86 mutations in 17 different genes screened using a high-throughput genotyping technology to determine the molecular etiology of hearing loss. The technology used in this work was the MassARRAY iPLEX® platform. This technology uses silicon chips and DNA amplification products for accurate genotyping by mass spectrometry of previous reported mutations. The generated results were validated using conventional techniques, as direct sequencing, multiplex PCR and RFLP-PCR. An initial genotyping of control subjects, showed failures in 20 % of the selected alterations. To optimize these results, the failed tests were re-designed and new primers were synthesized. Then, the specificity and sensitivity of the panel demonstrated values above 97 %. Additionally, a group of 180 individuals with NSHL without a molecular diagnosis was screened to test the diagnostic value of our panel, and mutations were identified in 30 % of the cases. In 20 % of the individuals, it was possible to explain the etiology of the HL. Mutations in GJB2 gene were the most prevalent, followed by other mutations in in SLC26A4, CDH23, MT-RNR1, MYO15A, and OTOF genes. The MassARRAY technology has the potential for high-throughput identification of genetic variations. However, we demonstrated that optimization is required to increase the genotyping success and accuracy. The developed panel proved to be efficient and cost-effective, being suitable for applications involving the molecular diagnosis of hearing loss.168

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

AbstractIntroductionElectroacoustic 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 subject's residual hearing should be preserved during cochlear implant surgery.ObjectivesTo evaluate the hearing preservation in patients that underwent implant placement and to compare the results in accordance with the approach to the inner ear.Methods19 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.Results17 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.ConclusionThe hearing preservation occurred in 89.4% of cases. There was no significant difference between the forms of inner ear approach

Hifocus Helix™ Electrode Insertion: Surgical Approach.

Cochlear implants have been used for almost 30 years as a device for the rehabilitation of individuals with severe-to-profound hearing loss. One of the important aspects of cochlear implantation is the type of electrode selected and proper insertion of the electrode array in scala tympani to minimize cochlear damage. The HiFocus Helix™ electrode is a precurved design aimed at placing the electrode contacts close to the spiral ganglion cells in the modiolus. The prescribed insertion techniques are intended to minimize the likelihood of damage to the basilar membrane or lateral wall of the cochlea. To describe the first insertion of a HiFocus Helix™ electrode in Brazil exposing surgical particularities and device details in a patient with profound hearing loss, due to Mondini's dysplasia. No problems were encountered during the surgical procedure. The patient experienced improvement in hearing thresholds and speech perception. The HiFocus Helix™ electrode proved easy to insert and provided expected hearing benefits for the patient. This manuscript indicates that the HiResolution™ Bionic Ear System with HiFocus Helix™ electrode comprise a cochlear implant system that is practical and beneficial for the treatment of severe-to-profound hearing loss.830