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Sound-level coding in the auditory nerve is achieved through the progressive recruitment of auditory nerve fibers (ANFs) that differ in threshold of activation and in the stimulus level at which the spike rate saturates. To investigate the functional state of the ANFs, the electrophysiological tests routinely used in clinics only capture the first action potentials firing in synchrony at the onset of the acoustic stimulation. Assessment of other properties (e.g., spontaneous rate and adaptation time constants) requires single-fiber recordings directly from the nerve, which for ethical reasons is not allowed in humans. By combining neuronal activity measurements at the round window and signal-processing algorithms, we constructed a peristimulus time response (PSTR), with a waveform similar to the peristimulus time histograms (PSTHs) derived from single-fiber recordings in young adult female gerbils. Simultaneous recordings of round-window PSTR and single-fiber PSTH provided models to predict the adaptation kinetics and spontaneous rate of the ANFs tuned at the PSTR probe frequency. The predictive model derived from gerbils was then validated in female mice and finally applied to humans by recording PSTRs from the auditory nerve in normal-hearing patients who underwent cerebellopontine angle surgeries. A rapid adaptation time constant of â¼3 ms and a mean spontaneous rate of â¼22 spikes/s in the 4 kHz frequency range were found. This study offers a promising diagnostic tool to map the human auditory nerve, thus opening new avenues to better understanding auditory neuropathies, tinnitus, and hyperacusis.SIGNIFICANCE STATEMENT Neural adaptation in auditory nerve fibers corresponds to the reduction in the neuronal activity to prolonged or repeated sound stimulation. For obvious ethical reasons, single-fiber recordings from the auditory nerve are not feasible in humans, creating a critical gap in extending data obtained using animal models to humans. Using electrocochleography in rodents, we inferred adaptation kinetics and spontaneous discharge rates of the auditory nerve fibers in humans. Routinely used in basic and clinical laboratories, this tool will provide a better understanding of auditory disorders such as neuropathies, tinnitus, and hyperacusis, and will help to improve hearing-aid fittings.
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Nervo Coclear , Audição , Estimulação Acústica , Animais , Nervo Coclear/fisiologia , Potenciais Evocados Auditivos/fisiologia , Feminino , Gerbillinae , Audição/fisiologia , Humanos , Camundongos , Fibras Nervosas/fisiologiaRESUMO
PURPOSE: Balloon dilatation of the Eustachian tube (BDET) is an option for treating chronic obstructive Eustachian tube dysfunction (COETD). In this prospective multicentric study, the main objective was to evaluate the results of BDET in unilateral COETD refractory to medical treatment. METHODS: Adults with unilateral COETD whose Eustachian Tube Score (ETS) was less than 5 despite medical pressure therapy were included. The primary endpoint was the change in ETS measured at 2, 6, and 12 months after BDET. Secondary objectives were the evolution of clinical symptoms assessed by the Eustachian Tube Questionnaire (ETDQ-7), audiometry, tympanometry, and otoscopy changes after BDET. RESULTS: Twenty-eight patients were included between May 2014 and December 2017 and were treated with BDET without adverse effects in three different referral centers. Population's median age was 52 (Q25; Q75: 24, 82) years. The median follow-up time was 381 (Q25; Q75: 364; 418) days. The median ETS was 2 (Q25; Q75: 1; 4) before BDET. There was a significant improvement in ETS at 2 and 6 months and 1 year after BDET (score at 1 year: 6 (Q25; Q75: 2; 8) (p < 0.0001)). There was a significant, sustained improvement in the ETDQ-7 with a score of 4.21 (Q25; Q75-3.50; 4.79) before BDET and 3.43 (Q25; Q75-2.43; 4.14) (p = 0.0012) at 1 year. There was a suggestive improvement in tympanometry results at 1 year (p = 0.025). CONCLUSION: BDET provides an improvement in symptoms and objective measures assessed at 1 year in patients with COETD who have failed medical treatment. TRIAL REGISTRATION: NCT02123277 (April 25, 2014).
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Otopatias , Tuba Auditiva , Adulto , Humanos , Pessoa de Meia-Idade , Tuba Auditiva/cirurgia , Dilatação/métodos , Estudos Prospectivos , Endoscopia , Otoscopia , Otopatias/terapia , Otopatias/cirurgia , Resultado do TratamentoRESUMO
OBJECTIVE: This study evaluated the diagnostic performances of a tablet-based hearing screening test by assisted-test and self-test modes. DESIGN/METHOD: Measurements were performed with the SoTone tests in normal hearing and hearing-impaired adult participants using an Android tablet and calibrated Bluetooth headphones. The duration of assisted- and self-test modes were compared. Receiver operating characteristic (ROC) curves were plotted after calculations of sensitivity and specificity at 20, 30, and 35 dB HL cut-off values. STUDY SAMPLE: 217 participants performed the tests. The effect of test mode (assisted versus self) was compared in a sample of 103 participants. RESULTS: Self-test duration (89 s) was significantly longer than the assisted-test duration (75 s) (p = 0.003, Wilcoxon test). For the 20, 30, and 35 dB HL cut-off values, sensitivity was between 92% and 96%, and specificity was between 79 and 90%. Concordance of results between assisted-test and self-test modes was excellent (Cohen's kappa = 0.81, p < 0.001). CONCLUSIONS: The SoTone hearing screening test is accurate for identifying the presence of a suspected hearing loss at 20 dB HL or more in adults. It can be used either in assisted-test or self-test modes.
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INTRODUCTION: Cochlear implantation is a recent approach proposed to treat single-sided deafness (SSD) and asymmetric hearing loss (AHL). Several cohort studies showed its effectiveness on tinnitus and variable results on binaural hearing. The main objective of this study is to assess the outcomes of cochlear implantation and other treatment options in SSD/AHL on quality of life. METHODS: This prospective multicenter study was conducted in 7 tertiary university hospitals and included an observational cohort study of SSD/AHL adult patients treated using contralateral routing of the signal (CROS) hearing aids or bone-anchored hearing systems (BAHSs) or who declined all treatments, and a randomized controlled trial in subjects treated by cochlear implantation, after failure of CROS and BAHS trials. In total, 155 subjects with SSD or AHL, with or without associated tinnitus, were enrolled. After 2 consecutive trials with CROS hearing aids and BAHSs on headband, all subjects chose any of the 4 treatment options (abstention, CROS, BAHS, or cochlear implant [CI]). The subjects who opted for a CI were randomized between 2 arms (CI vs. initial observation). Six months after the treatment choice, quality of life was assessed using both generic (EuroQoL-5D, EQ-5D) and auditory-specific quality-of-life indices (Nijmegen Cochlear implant Questionnaire [NCIQ] and Visual Analogue Scale [VAS] for tinnitus severity). Performances for speech-in-noise recognition and localization were measured as secondary outcomes. RESULTS: CROS was chosen by 75 subjects, while 51 opted for cochlear implantation, 18 for BAHSs, and 11 for abstention. Six months after treatment, both EQ-5D VAS and auditory-specific quality-of-life indices were significantly better in the "CI" arm versus "observation" arm. The mean effect of the CI was particularly significant in subjects with associated severe tinnitus (mean improvement of 20.7 points ± 19.7 on EQ-5D VAS, 20.4 ± 12.4 on NCIQ, and 51.4 ± 35.4 on tinnitus). No significant effect of the CI was found on binaural hearing results. Before/after comparisons showed that the CROS and BAHS also improved significantly NCIQ scores (for CROS: +7.7, 95% confidence interval [95% CI] = [4.5; 10.8]; for the BAHS: +14.3, 95% CI = [7.9; 20.7]). CONCLUSION: Cochlear implantation leads to significant improvements in quality of life in SSD and AHL patients, particularly in subjects with associated severe tinnitus, who are thereby the best candidates to an extension of CI indications.
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Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Unilateral , Perda Auditiva , Percepção da Fala , Adulto , Surdez/cirurgia , Perda Auditiva Unilateral/cirurgia , Humanos , Estudos Prospectivos , Qualidade de Vida , Resultado do TratamentoRESUMO
OBJECTIVES: To describe the treatment choice in a cohort of subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). To assess the reliability of the treatment trials. DESIGN: In this national, multicentre, prospective study, the choice of subjects was made after two consecutive trials of Contralateral Routing Of the Signal (CROS) hearing aids and a Bone Conduction Device (BCD) on a headband. Subjects could proceed with one of these two options, opt for cochlear implantation or decline all treatments. SETTING: Seven tertiary university hospitals. PARTICIPANTS: One hundred fifty-five subjects with SSD or AHL fulfilling the candidacy criteria for cochlear implantation, with or without associated tinnitus. MAIN OUTCOME MEASURES: After the two trials, the number of subjects choosing each option was described. Repeated assessments of both generic and auditory-specific quality of life were conducted, as well as hearing assessments (speech recognition in noise and horizontal localization). RESULTS: CROS was chosen by 75 subjects, followed by cochlear implantation (n = 51), BCD (n = 18) and abstention (n = 11). Patients who opted for cochlear implantation had a poorer quality of life (P = .03). The improvement of quality of life indices after each trial was significantly associated with the final treatment choice (P = .008 for generic indices, P = .002 for auditory-specific indices). The follow-up showed that this improvement had been overestimated in the CROS group, with a long-term retention rate of 52.5%. CONCLUSIONS: More than one third of SSD/AHL subjects are unsatisfied after CROS and BCD trials. Repeated quality of life assessments help counselling the patient for his/her treatment choice.
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Perda Auditiva Unilateral/reabilitação , Condução Óssea , Comportamento de Escolha , Implantes Cocleares , Feminino , França , Auxiliares de Audição , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Qualidade de Vida , Teste do Limiar de Recepção da FalaRESUMO
AIM: A Phase 1 study was performed to evaluate safety, pharmacokinetics (PK) and pharmacodynamics (PD) of the selective histamine H4 receptor antagonist SENS-111, an oral small molecule. METHODS: One hundred healthy subjects were randomized in a placebo-controlled, double-blind study evaluating single-ascending doses (SAD; 100-500 mg) and multiple-ascending doses (MAD; 50-150 mg day-1 , 4 days; 200-250 mg day-1 , 7 days). Effects of SENS-111 on nystagmus and vertigo induced by modified caloric tests were measured in the MAD studies. Population PK and PK/PD models were developed using a nonlinear mixed-effects approach. RESULTS: SENS-111 was well tolerated with mild to moderate events. No sedation was reported. A maximal tolerated dose was not reached. Dose-proportional increases in concentrations were seen up to 200 mg and more than dose-proportional thereafter, with mean half-life between 24 and 56 h. The caloric test induced mild but measurable vertigo and nystagmus with large intra/inter-individual variation for all parameters. SENS-111 did not significantly impact nystagmus but significantly improved latency of vertigo appearance/disappearance, duration and European Evaluation of Vertigo questionnaire parameters vs. baseline. A two-compartment model with first-order absorption, distribution and elimination best fit the data. PK/PD indirect modelling applied to vertigo duration and latency of appearance indicated maximum activity between 100 and 500 ng ml-1 plasma concentrations, corresponding to 100 and 200 mg day-1 , which are appropriate for clinical efficacy evaluations in vestibular diseases. CONCLUSIONS: SENS-111 is a well-tolerated first-in-class H4 receptor antagonist with acceptable PK for oral daily dosing. PK/PD modelling determined plasma concentrations and doses for efficacy studies in patients with vertigo symptoms.
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Azetidinas/efeitos adversos , Testes Calóricos , Antagonistas dos Receptores Histamínicos/efeitos adversos , Pirimidinas/efeitos adversos , Receptores Histamínicos H4/antagonistas & inibidores , Adolescente , Adulto , Azetidinas/farmacocinética , Azetidinas/farmacologia , Método Duplo-Cego , Voluntários Saudáveis , Antagonistas dos Receptores Histamínicos/farmacocinética , Antagonistas dos Receptores Histamínicos/farmacologia , Humanos , Masculino , Pessoa de Meia-Idade , Modelos Biológicos , Pirimidinas/farmacocinética , Pirimidinas/farmacologia , Vertigem/tratamento farmacológico , Adulto JovemRESUMO
OBJECTIVE: Teleotoscopy requires the assistance of telehealth facilitators; but their training requirements remain to be determined. We evaluated the use of an otoscopy simulator to train facilitators to remote otoscopies sent via the Internet using a teleaudiology platform. DESIGN: Neurotologists experts were asked to identify images using the otoscopy simulator and to perform an identification task of significant anatomical landmarks. The experts were asked to repeat those tasks remotely, with the help of facilitators who either received basic training, or no training prior to the experiment. STUDY SAMPLE: Three experts, three trained facilitators and three untrained facilitators participated in this study. RESULTS: The use of an otoscopy simulator in addition to remote otoscopy yielded a good inter- and intrarater agreement (κ between 0.81-1, and 0.80-0.87, respectively). The accuracy of diagnosis was high on-site (11.7% error) and remotely (0% error). The time required for landmark identification task was not increased when performed remotely with a trained facilitator versus on-site otoscopy (9.3 versus 9.2 s/landmark). Conversely, the lack of training of facilitators increased significantly this time (15.6 s/landmark, p < 0.001). CONCLUSION: An otoscopic simulator coupled to teleaudiology software can be used to efficiently train both experts and facilitators to perform remote otoscopy.
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Audiologistas/educação , Audiologia/educação , Instrução por Computador/métodos , Otopatias/diagnóstico , Educação Médica/métodos , Otoscopia , Consulta Remota , Treinamento por Simulação/métodos , Pontos de Referência Anatômicos , Currículo , Otopatias/patologia , Otopatias/fisiopatologia , Humanos , Variações Dependentes do Observador , Valor Preditivo dos Testes , Reprodutibilidade dos TestesRESUMO
Frequency-place mismatch often occurs after cochlear implantation, yet its effect on speech perception outcome remains unclear. In this article, we propose a method, based on cochlea imaging, to determine the cochlear place-frequency map. We evaluated the effect of frequency-place mismatch on speech perception outcome in subjects implanted with 3 different lengths of electrode arrays. A deeper insertion was responsible for a larger frequency-place mismatch and a decreased and delayed speech perception improvement by comparison with a shallower insertion, for which a similar but slighter effect was noticed. Our results support the notion that selecting an electrode array length adapted to each individual's cochlear anatomy may reduce frequency-place mismatch and thus improve speech perception outcome.
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Cóclea/diagnóstico por imagem , Implantes Cocleares , Surdez/reabilitação , Percepção da Altura Sonora , Percepção da Fala , Adolescente , Adulto , Cóclea/cirurgia , Implante Coclear/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVES: To compare speech perception outcomes between bilateral implantation (cochlear implants [CIs]) and bimodal rehabilitation (one CI on one side plus one hearing aid [HA] on the other side) and to explore the clinical factors that may cause asymmetric performances in speech intelligibility between the two ears in case of bilateral implantation. DESIGN: Retrospective data from 2247 patients implanted since 2003 in 15 international centers were collected. Intelligibility scores, measured in quiet and in noise, were converted into percentile ranks to remove differences between centers. The influence of the listening mode among three independent groups, one CI alone (n = 1572), bimodal listening (CI/HA, n = 589), and bilateral CIs (CI/CI, n = 86), was compared in an analysis taking into account the influence of other factors such as duration of profound hearing loss, age, etiology, and duration of CI experience. No within-subject comparison (i.e., monitoring outcome modifications in CI/HA subjects becoming CI/CI) was possible from this dataset. Further analyses were conducted on the CI/CI subgroup to investigate a number of factors, such as implantation side, duration of hearing loss, amount of residual hearing, and use of HAs that may explain asymmetric performances of this subgroup. RESULTS: Intelligibility ranked scores in quiet and in noise were significantly greater with both CI/CI and CI/HA than with a CI-alone group, and improvement with CI/CI (+11% and +16% in quiet and in noise, respectively) was significantly better than with CI/HA (+6% and +9% in quiet and in noise, respectively). From the CI/HA group, only subjects with ranked preoperative aided speech scores >60% performed as well as CI/CI participants. Furthermore, CI/CI subjects displayed significantly lower preoperative aided speech scores on average compared with that displayed by CI/HA subjects. Routine clinical data available from the present database did not explain the asymmetrical results of bilateral implantation. CONCLUSIONS: This retrospective study, based on basic speech audiometry (no lateralization cues), indicates that, on average, a second CI is likely to provide slightly better postoperative speech outcome than an additional HA for people with very low preoperative performance. These results may be taken into consideration to refine surgical indications for CIs.
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Implante Coclear , Correção de Deficiência Auditiva/métodos , Perda Auditiva Bilateral/reabilitação , Percepção da Fala , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Audiometria da Fala , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: To demonstrate the feasibility and reliability of simultaneous binaural recording of auditory steady-state responses (ASSR) in young children using narrow-band CE-Chirps as stimuli. DESIGN: Prospective cohort study comparing ASSR thresholds to four frequency stimuli (0.5, 1, 2, and 4 kHz), with click-evoked auditory brainstem responses (ABR) and behavioral response audiometry. STUDY SAMPLE: Thirty-two young children (mean age 7.4 ± 5.2 months) referred for auditory assessment were evaluated. RESULTS: The mean duration for ABR recordings was 13.3 ± 7.2 min versus 22.9 ± 15.8 min for ASSR (p < 0.01). ASSR (means of 2 and 4 kHz thresholds) were highly correlated with ABR thresholds (R2 = 0.935, p < 0.001), though significantly different (3 ± 10.7 dB, p = 0.02). ASSR (means of 0.5, 1, 2, and 4 kHz thresholds) were highly correlated with mean behavioral response audiometry thresholds (R2 = 0.968, p < 0.001). ASSRs were highly and significantly correlated with behavioral response audiometry at 0.5, 1, 2, and 4 kHz (R2 = 0.845, 0.907, 0.929, and 0.859 respectively, p < 0.001). 87.5% and 90.7% ASSR thresholds were within a ± 10 dB range around their corresponding ABR and mean behavioral response audiometry thresholds. CONCLUSIONS: Narrow-band CE-Chirps allow a fast and reliable assessment of auditory thresholds in children, especially in the low-frequency range, by comparison with other stimuli.
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Estimulação Acústica/métodos , Audiometria de Resposta Evocada/métodos , Limiar Auditivo/fisiologia , Potenciais Evocados Auditivos/fisiologia , Estudos de Coortes , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Estudos de Viabilidade , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Reprodutibilidade dos TestesRESUMO
To evaluate the performance of the video head impulse test (VHIT) in assessing vestibular deficit in vestibular neuritis. Test validation study was conducted in Tertiary referral center. Twenty-nine patients, referred for vestibular neuritis between October 2009 and March 2012, were included. We recorded age, gender, values of caloric deficit (caloric testing), and deficits in semicircular function (VHIT) at initial presentation and at the follow-up visit (1-3 months). Multivariate linear regression analysis was performed to determine variables associated with values of caloric testing at the follow-up visit. Diagnostic values of VHIT were compared with caloric testing data using the receiver-operating characteristic (ROC) curve and subsequent statistical analysis. At the follow-up visit, complete recovery occurred in 31% of cases according to caloric evaluation, and VHIT normalized in 51.8%. Multivariate regression showed that a higher caloric deficit at the follow-up visit was associated with elevated age (p = 0.012) and high caloric deficit at initial presentation (p = 0.042). A lower caloric deficit was associated with normal VHIT results at the follow-up visit (p < 0.001). The ROC curve showed that specificity and sensitivity of VHIT were 100% when the caloric deficit was respectively lower than 40% or higher than 62.5%. At the caloric testing value of 30%, specificity was 100%, sensitivity 68.84%, positive predictive value 100% and negative predictive value 62.5%. VHIT is a fast, convenient and specific test to detect vestibular deficits in vestibular neuritis. However, VHIT lacks sensitivity by comparison with caloric testing, especially for moderate vestibular lesions.
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Teste do Impulso da Cabeça/métodos , Vertigem/diagnóstico , Neuronite Vestibular/diagnóstico , Adolescente , Adulto , Fatores Etários , Idoso , Testes Calóricos/métodos , Estudos de Coortes , Feminino , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Prospectivos , Curva ROC , Recuperação de Função Fisiológica/fisiologia , Sensibilidade e Especificidade , Vertigem/etiologia , Vertigem/fisiopatologia , Neuronite Vestibular/complicações , Neuronite Vestibular/fisiopatologia , Gravação em Vídeo/métodos , Adulto JovemRESUMO
The objective of this study was to measure performance benefits obtained by upgrading recipients of the Cochlear Nucleus CI24 cochlear implant to the new CP810 sound processor. Speech recognition in quiet and in spatially separated noise was measured in established users of the Cochlear ESPrit 3G (n = 22) and Freedom (n = 13) sound processors, using the "Everyday" listening program. Subjects were then upgraded to the CP810 processor and were re-assessed after a 3-month period, using both the "Everyday" program and the new "Noise" program, which incorporates several pre-processing features including a new directional microphone algorithm ("Zoom"). Subjective perceptions were also recorded using the abbreviated profile of hearing aid benefit (APHAB) questionnaire. Mean scores for monosyllables in quiet, presented at 50 and 60 dB SPL, increased by 11% (p < 0.0001) and 8% (p < 0.001), respectively, after upgrade, for all subjects combined. Significant increases were also recorded for both processor groups. In noise, the mean scores were 60.0 and 67.4% for the original and CP810 Everyday programs, respectively (difference not significant). With the CP810 Noise programs the mean score increased to 82.5% (p < 0.01), with significant increases in both processor groups. There was evidence of slightly greater upgrade benefit in users of the ESPrit 3G processor and in relatively poor performers. The APHAB questionnaire also indicated significant reduction in perceived difficulty in the background noise and reverberation sub-scales after upgrade. The findings of the study appear to support the expectation of increased benefit from the new CP810 sound processor.
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Implantes Cocleares , Adolescente , Adulto , Idoso , Desenho de Equipamento , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Razão Sinal-Ruído , Percepção da Fala , Interface para o Reconhecimento da Fala , Adulto JovemRESUMO
OBJECTIVE: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. STUDY DESIGN: Retrospective multicenter study. METHODS: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers. RESULTS: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss. CONCLUSIONS: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences.
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Implante Coclear , Implantes Cocleares , Perda Auditiva/cirurgia , Percepção da Fala/fisiologia , Fala/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Perda Auditiva/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Pessoas com Deficiência Auditiva , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: We describe the various molecular and cellular pathways that lead to early and delayed loss of residual hearing after cochlear implantation. METHODS: We performed a systematic review using the Medline database with the key words cochlear implant, residual hearing, inflammation, apoptosis, and necrosis. RESULTS: The mechanisms underlying the loss of residual hearing after cochlear implantation are multiple. Early hearing loss may be provoked by the surgical access to the inner ear spaces and by trauma caused by insertion of the electrode array. After the initial trauma, an acute inflammatory response promotes elevated levels of cytokines and reactive oxygen species, which in turn promote sensory cell loss by apoptosis, necrosis, and necrosis-like programmed cell death. Treatments that counteract such an inflammatory reaction, production of reactive oxygen species, and apoptosis are effective at preventing hair cell degeneration. However, delayed hearing loss appears to be a consequence of chronic inflammation with development of fibrotic tissue. The mechanisms that lead to fibrosis are poorly understood, and standard antiinflammatory drugs are insufficient for preventing its development. CONCLUSIONS: Cochlear implantation is followed by an inflammatory response involving several pathways that lead to either short-term or long-term sensory hair cell degeneration. Future studies should focus on revealing the precise molecular mechanisms induced by cochlear implantation to allow the discovery of new targets for the effective prevention and treatment of loss of residual hearing.
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Implantes Cocleares , Perda Auditiva , Apoptose , Audiometria de Tons Puros , Limiar Auditivo/fisiologia , Audição , Perda Auditiva/metabolismo , Perda Auditiva/patologia , Perda Auditiva/cirurgia , HumanosRESUMO
Transtympanic promontory stimulation test (TPST) has been suggested to be a useful tool in predicting postoperative outcomes in patients at risk of poor auditory neuron functioning, especially after a long auditory deprivation. However, only sparse data are available on this topic. This study aimed at showing correlations between the auditory nerve dynamic range, evaluated by TPST, the electrical dynamic range of the cochlear implant and speech perception outcome. We evaluated 65 patients with postlingual hearing loss and no residual hearing, implanted with a Nucleus CI24 cochlear implant device for at least 2 years and with a minimum of 17 active electrodes. Using the TPST, we measured the threshold for auditory perception (T-level) and the maximum acceptable level of stimulation (M-level) at stimulation frequencies of 50, 100 and 200 Hz. General linear regression was performed to correlate 1/speech perception, evaluated using the PBK test 1 year after surgery, and 2/cochlear implant electrical dynamic range, with the age at time of implantation, the duration of auditory deprivation, the etiology of the deafness, the duration of cochlear implant use and auditory nerve dynamic range. Postoperative speech perception outcome correlated with etiology, duration of auditory deprivation and implant use, and TPST at 100 and 200 Hz. The dynamic range of the cochlear implant map correlated with duration of auditory deprivation, speech perception outcome at 6 months and TPST at 100 and 200 Hz. TPST test can be used to predict functional outcome after cochlear implant surgery in difficult cases.
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Implantes Cocleares , Estimulação Elétrica , Perda Auditiva/fisiopatologia , Perda Auditiva/cirurgia , Percepção da Fala/fisiologia , Membrana Timpânica/fisiologia , Limiar Auditivo/fisiologia , Nervo Coclear/fisiopatologia , Eletrodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by an impaired transmission of sound from the cochlea to the brain. This defect can be due to a lesion or defect in the inner hair cell (IHC), IHC ribbon synapse (e.g., pre-synaptic release of glutamate), postsynaptic terminals of the spiral ganglion neurons, or demyelination and axonal loss within the auditory nerve. To date, the only clinical treatment options for ANSD are hearing aids and cochlear implantation. However, despite the advances in hearing-aid and cochlear-implant technologies, the quality of perceived sound still cannot match that of the normal ear. Recent advanced genetic diagnostics and clinical audiology made it possible to identify the precise site of a lesion and to characterize the specific disease mechanisms of ANSD, thus bringing renewed hope to the treatment or prevention of auditory neurodegeneration. Moreover, genetic routes involving the replacement or corrective editing of mutant sequences or defected genes to repair damaged cells for the future restoration of hearing in deaf people are showing promise. In this review, we provide an update on recent discoveries in the molecular pathophysiology of genetic lesions, auditory synaptopathy and neuropathy, and gene-therapy research towards hearing restoration in rodent models and in clinical trials.
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OBJECTIVE: In this paper, we focus on the carrying out and validation of minimally invasive three-dimensional (3D) ultrasound (US) imaging of the auditory system, which is based on a new miniaturized endoscopic 2D US transducer. METHODS: This unique probe consists of a 18 MHz 24 elements curved array transducer with a distal diameter of 4 mm so it can be inserted into the external auditory canal. Typical acquisition is achieved by rotating such a transducer around its own axis using a robotic platform. Reconstruction of a US volume from the set of acquired B-scans during the rotation is then performed using scan-conversion. The accuracy of the reconstruction procedure is evaluated using a dedicated phantom that includes a set of wires as reference geometry. RESULTS: Twelve acquisitions obtained from different probe poses are compared to a micro-computed tomographic model of the phantom, leading to a maximum error of 0.20 mm. Additionally, acquisitions with a cadaveric head highlight the clinical applicability of this set up. Structures of the auditory system such as the ossicles and the round window can be identified from the obtained 3D volumes. CONCLUSION: These results confirm that our technique enables the accurate imaging of the middle and inner ears without having to deteriorate the surrounding bone. SIGNIFICANCE: Since US is a real-time, wide available and non-ionizing imaging modality, our acquisition setup could facilitate the minimally invasive diagnosis and surgical navigation for otology in a fast, cost-effective and safe way.
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Robótica , Ultrassom , Humanos , Ultrassonografia , Tomografia Computadorizada por Raios X , Imagens de Fantasmas , Transdutores , Imageamento Tridimensional/métodosRESUMO
INTRODUCTION: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. MATERIAL AND METHODS: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of ENT physicians, audiologists, geriatricians and hearing specialists from all over France. They are classified as grade A, B, C or professional agreement according to a decreasing level of scientific evidence. RESULTS: The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its face-to-face or remote management. CONCLUSION: In the case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. Free-field speech audiometry in noise is recommended to measure intelligibility in a realistic environment. Questionnaires in addition to audiometric tests would allow the best assessment of the patient's disability. Hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time since the hearing was fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, that include different training domains to complement face-to-face sessions.
RESUMO
INTRODUCTION: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. MATERIAL AND METHODS: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors and audioprosthetists from all over France. They are graded A, B, C or expert opinion according to decreasing level of scientific evidence. RESULTS: The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its management in face-to-face or even distance learning. CONCLUSION: In case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. It is recommended to perform free-field speech audiometry in noise to measure intelligibility in an environment as close as possible to reality. Questionnaires can be used in addition to audiometry to best assess the patient's disability. It is recommended that hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time elapsed since the fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, integrating different training domains in addition to face-to-face sessions.
Assuntos
Audiologia , Geriatria , Otolaringologia , Presbiacusia , Humanos , Idoso , Presbiacusia/terapia , Presbiacusia/reabilitação , CogniçãoRESUMO
Cochlear implants are the most common and successful sensory neuroprosthetic devices. However, reimplantation can be required for medical reasons, device failure, or technological upgrading. Resolving the problem driving the intervention and offering stable or better audiological results are the main challenges. We aimed to analyze the success rate of this intervention and to identify factors influencing speech perception recovery after reimplantation in the pediatric population. We retrospectively collected the causes and the outcomes of 67 consecutive reimplantations in one cochlear implant center over 30 years. Reimplantation resolved the cause without recurrence for 94% of patients. The etiology of deafness, time since implantation, indication of reimplantation, sex, and age did not influence word discrimination test scores in silence, 3 years after surgery. However, adherence to a speech rehabilitation program was statistically associated with gain in perception scores: +8.9% [-2.2; +31.0%] versus -19.0% [-47.5; -7.6%] if no or suboptimal rehabilitation was followed (p = 0.0037). Cochlear reimplantation in children is efficient and is associated with predictable improvement in speech perception, 3 years after intervention. However, good adherence to speech rehabilitation program is necessary and should be discussed with the patient and parents, especially for the indication of reimplantation for technological upgrading.