<p>Sensorineural hearing loss imparts two serious hearing deficits in hearing-impaired people: reduced dynamic range of hearing and reduced frequency selectivity. Psychophysically, these deficits render loss of speech audibility and speech intelligibility to a hearing-impaired person. Studies of an impaired cochlea in cats have shown that the hearing loss originates from damage to or complete loss of inner and outer hair cells. Neurophysiology of an impaired cochlea in cats shows that the tuning curves of the auditory nerve fibers become elevated and broadened. Amplification in hearing aids has been used to restore audibility in hearing-impaired people. Multiband compression has been commercially available in conventional hearing aids to compensate for the reduced dynamic range of hearing. However, little has been achieved to improve the intelligibility of speech in the hearing-impaired people. The aim of this thesis is to restore not only the speech audibility in a hearing-impaired person, but also to improve their speech intelligibility through some hearing-aid signal processing. The compensation technique used in this thesis for speech intelligibility is based on the hypothesis that a narrowband response of the auditory nerve fibers to speech signals ensure phonemic discriminability in the central nervous system.</p><p>Miller et al. [1999] have proposed contrast-enhancing frequency shaping ( CEFS) to compensate for the broadband responses of the fibers to first and second formants (Fl and F2) of a speech stimulus. Bruce [2004] has shown that the multiband compression can be combined with CEFS without counteracting each other. In Bruce's algorithm, a multiband compressor is serially combined with a time-domain CEFS filter. The MICEFS algorithm, herein presented, is a combination of multiband compression and an improved version of CEFS implemented in the frequency domain. The frequency domain implementation of MICEFS has improved the time delay response of the algorithm by 10 ms as compared to series implementation proposed by Bruce. The total time delay of the MICEFS algorithm is 16 ms, which is still longer than the standard time delay of 10 ms in hearing aids. The MICEFS algorithm was tested on a computational model of auditory periphery [Bruce et al., 2003] using a synthetic vowel and a synthetic sentence. The testing paradigm consisted of five conditions: 1) unmodified speech presented to a normal cochlea; 2) speech modified with halfgain rule presented to an impaired cochlea; 3) CEFS modified speech presented to the impaired cochlea; 4) speech modified with MICEFS presented to the impaired cochlea, and; 5) MICEFS-modified speech with some added noise in the formant estimation presented to an impaired cochlea. The spectral enhancement filter used in MICEFS has improved the synchrony capture of the fibers to the first three formants of a speech stimulus. MICEFS has also restored the correct tonotopic representation in the average discharge rate of the fibers at the first three formants of the speech.</p> / Thesis / Master of Applied Science (MASc)

Objective: Twenty-two million Americans or roughly 8% of the population are hearing impaired. This affects more than just the ability for one to hear. In the last three decades, younger Americans have shown a sharp increase in hearing impairment. Hearing loss affects social and emotional well being and thus has the ability to decrease one's quality of life. This study was designed to examine the association between smoking, workplace chemical exposure, and hearing loss.Methods: This cross-sectional population-based study was conducted in an occupational health clinic setting in Virginia. The study population consisted of employees from local businesses who visited the clinic during the period of 1/1/03 to 1/1/05 for pure tone audiometry. Chart reviews supplemented by telephone interviews were conducted to extract data for analysis. All study subjects were eligible to participate except for those who were unable to communicate due to a language barrier. Prevalence and crude prevalence ratios were calculated. Adjusted prevalence ratios and prevalence odds ratios were calculated using Cox and logistic regression models. Data entry and statistical analysis were accomplished with the SPSS Data Builder and the SPSS 13.0 statistical software. SAS statistical software was also used for a portion of the statistical analysis.Results: In a Cox regression model controlling for many potential confounding factors (age, race, smoking, chemical exposure, military service, as well as others) cigarette smoking was not associated with hearing loss PR(95% CI), 1.0 (0.7, 1.3). Similarly, exposure to chemicals was not associated with hearing loss 1.1 (0.7, 1.6).Conclusion: This study did not find an association between cigarette smoking and hearing loss and exposure to industrial chemicals and hearing loss in persons who worked in a noisy occupational environment.

Introdução: O potencial evocado auditivo de estado estável (PEAEE) é um exame eletrofisiológico que permite a testagem simultânea e binaural do limiar auditivo por frequência específica. Por seu caráter objetivo vem sendo recomendada sua inclusão na bateria de testes para diagnóstico de crianças com deficiência auditiva. A possibilidade de diagnóstico precoce da perda de audição em crianças é fundamental para a redução dos agravos decorrentes da privação sensorial e a tomada de decisão quanto à intervenção adequada, como o uso do implante coclear (IC). A determinação do sucesso do uso do IC é dependente de múltiplos cofatores, entre eles, sugere-se que a presença de audição residual prévia ao implante poderia possibilitar a preservação das vias auditivas e áreas auditivas corticais, proporcionando melhor desenvolvimento das habilidades auditivas e de produção oral da linguagem com o reestabelecimento da entrada sensorial. Objetivo: Verificar a relação entre o PEAEE pré-operatório e a resposta auditiva pós-implante de crianças submetidas ao IC. Casuística e Método: Trata-se de um estudo observacional retrospectivo, desenvolvido a partir da análise de prontuários de pacientes do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, submetidos à cirurgia de IC. Foram incluídas 47 crianças menores de 6 anos à data de realização da cirurgia, diagnosticadas com perda auditiva neurossensorial pré-lingual de grau profundo. Foram coletadas informações audiológicas pré-implantação - PEAEE, PEATE, audiometria comportamental e demais aspectos de caracterização da perda auditiva; a categoria de percepção auditiva e de expressão oral da linguagem antes e após a implantação; além de informações socioeconômicas e da intervenção terapêutica. Resultados: O registro do PEAEE esteve presente em 34 (72,3%) crianças com limiar médio de 89,7 ± 10,2 dB NA na orelha implantada, consideradas as frequências de 0,5; 1; 2 e 4 kHz. A reavaliação foi conduzida em média 7,2 ± 2,1 meses após a cirurgia de IC, quando 23 (49%) sujeitos apresentaram avanço de uma categoria no desenvolvimento da percepção de fala e 4 (8,5%) alcançaram um desempenho dois níveis superior ao da avaliação anterior. O limiar obtido na audiometria em campo com o uso de IC teve média de 44,4 ± 12,4 dB NA, consideradas as frequências de 0,5; 1; 2 e 4 kHz. Quanto à expressão oral da linguagem, 13 (27,6%) crianças tiveram melhora em seu desempenho. Não houve correlação entre os resultados do PEAEE pré-implante e o limiar auditivo (p-valor=0,338) ou a categoria de linguagem (p-valor=0,358) com uso de IC quando controlado pela idade; nem em relação à melhora no desempenho de linguagem oral (p-valor= 0,095). Conclusão: Não houve correlação direta entre o limiar eletrofisiológico mensurado por meio do PEAEE e a resposta auditiva com uso de IC, quando analisados o limiar auditivo comportamental com IC ou a categoria de percepção de fala. / Background: The Auditory Steady State Response (ASSR) is an electrophysiological exam that allows simultaneous and binaural frequency specific testing of hearing thresholds. Considering its objectivity, the test has been recommended for hearing loss diagnosis in young children. This possibility of early identification of hearing impairment is fundamental for minimizing handicaps caused by sensory deprivation and for planning proper intervention, for example, suggesting the use of cochlear implants (CI). The determination of the success of CI prognosis depends on several cofactors. Researchers suggest that residual hearing and hearing thresholds before CI surgery could allow auditory pathways preservation and enable the development of hearing abilities, as well as speech production skills, after the reestablishment of sensory input. Objective: To verify the relation between pre-operatory ASSR and post-CI auditory response in young children. Casuistic and Method: An observational retrospective study was conducted. Medical records were assessed in 47 children, younger than six years old at the CI surgery, diagnosed with sensorineural pre-linguistic hearing loss of profound degree. Information was collected about (a) audiological data - ASSR, ABR, behavioral audiometry, and other hearing loss aspects; (b) category of speech perception and oral language production before and after CI; (c) socioeconomics data and therapy attendance. Results: ASSR record was present in 34 (72.4%) children with mean threshold of 89.7 (10.2 SD) dB HL in the implanted ear, considering the frequencies of 0.5; 1; 2 and 4 kHz. Post-implant evaluation was conducted after a mean of 7.2 (2.1 SD) months of surgery. Then, 23 (49%) subjects enhanced one category of speech perception and four (8.5%) improved two categories, compared to previous evaluation. Field auditory threshold with CI was measure at 44.4 (12.4 SD) dB HL in mean, considering the frequencies of 0.5; 1; 2 and 4 kHz. Analyzing language expression development, 13 (27.6%) children improved their performance. To the age at CI surgery there was no correlation between ASSR thresholds and CI thresholds (p-value=0.338) or speech perception category (p-value=0.358) after CI. No correlation has been found between ASSR and expressive language development (p-value=0.095). Conclusion: There was no direct correlation between electrophysiological thresholds measured by ASSR and hearing response after CI, when analyzed behavioral CI threshold or speech perception.

Introdução: O potencial evocado auditivo de estado estável (PEAEE) é um exame eletrofisiológico que permite a testagem simultânea e binaural do limiar auditivo por frequência específica. Por seu caráter objetivo vem sendo recomendada sua inclusão na bateria de testes para diagnóstico de crianças com deficiência auditiva. A possibilidade de diagnóstico precoce da perda de audição em crianças é fundamental para a redução dos agravos decorrentes da privação sensorial e a tomada de decisão quanto à intervenção adequada, como o uso do implante coclear (IC). A determinação do sucesso do uso do IC é dependente de múltiplos cofatores, entre eles, sugere-se que a presença de audição residual prévia ao implante poderia possibilitar a preservação das vias auditivas e áreas auditivas corticais, proporcionando melhor desenvolvimento das habilidades auditivas e de produção oral da linguagem com o reestabelecimento da entrada sensorial. Objetivo: Verificar a relação entre o PEAEE pré-operatório e a resposta auditiva pós-implante de crianças submetidas ao IC. Casuística e Método: Trata-se de um estudo observacional retrospectivo, desenvolvido a partir da análise de prontuários de pacientes do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, submetidos à cirurgia de IC. Foram incluídas 47 crianças menores de 6 anos à data de realização da cirurgia, diagnosticadas com perda auditiva neurossensorial pré-lingual de grau profundo. Foram coletadas informações audiológicas pré-implantação - PEAEE, PEATE, audiometria comportamental e demais aspectos de caracterização da perda auditiva; a categoria de percepção auditiva e de expressão oral da linguagem antes e após a implantação; além de informações socioeconômicas e da intervenção terapêutica. Resultados: O registro do PEAEE esteve presente em 34 (72,3%) crianças com limiar médio de 89,7 ± 10,2 dB NA na orelha implantada, consideradas as frequências de 0,5; 1; 2 e 4 kHz. A reavaliação foi conduzida em média 7,2 ± 2,1 meses após a cirurgia de IC, quando 23 (49%) sujeitos apresentaram avanço de uma categoria no desenvolvimento da percepção de fala e 4 (8,5%) alcançaram um desempenho dois níveis superior ao da avaliação anterior. O limiar obtido na audiometria em campo com o uso de IC teve média de 44,4 ± 12,4 dB NA, consideradas as frequências de 0,5; 1; 2 e 4 kHz. Quanto à expressão oral da linguagem, 13 (27,6%) crianças tiveram melhora em seu desempenho. Não houve correlação entre os resultados do PEAEE pré-implante e o limiar auditivo (p-valor=0,338) ou a categoria de linguagem (p-valor=0,358) com uso de IC quando controlado pela idade; nem em relação à melhora no desempenho de linguagem oral (p-valor= 0,095). Conclusão: Não houve correlação direta entre o limiar eletrofisiológico mensurado por meio do PEAEE e a resposta auditiva com uso de IC, quando analisados o limiar auditivo comportamental com IC ou a categoria de percepção de fala. / Background: The Auditory Steady State Response (ASSR) is an electrophysiological exam that allows simultaneous and binaural frequency specific testing of hearing thresholds. Considering its objectivity, the test has been recommended for hearing loss diagnosis in young children. This possibility of early identification of hearing impairment is fundamental for minimizing handicaps caused by sensory deprivation and for planning proper intervention, for example, suggesting the use of cochlear implants (CI). The determination of the success of CI prognosis depends on several cofactors. Researchers suggest that residual hearing and hearing thresholds before CI surgery could allow auditory pathways preservation and enable the development of hearing abilities, as well as speech production skills, after the reestablishment of sensory input. Objective: To verify the relation between pre-operatory ASSR and post-CI auditory response in young children. Casuistic and Method: An observational retrospective study was conducted. Medical records were assessed in 47 children, younger than six years old at the CI surgery, diagnosed with sensorineural pre-linguistic hearing loss of profound degree. Information was collected about (a) audiological data - ASSR, ABR, behavioral audiometry, and other hearing loss aspects; (b) category of speech perception and oral language production before and after CI; (c) socioeconomics data and therapy attendance. Results: ASSR record was present in 34 (72.4%) children with mean threshold of 89.7 (10.2 SD) dB HL in the implanted ear, considering the frequencies of 0.5; 1; 2 and 4 kHz. Post-implant evaluation was conducted after a mean of 7.2 (2.1 SD) months of surgery. Then, 23 (49%) subjects enhanced one category of speech perception and four (8.5%) improved two categories, compared to previous evaluation. Field auditory threshold with CI was measure at 44.4 (12.4 SD) dB HL in mean, considering the frequencies of 0.5; 1; 2 and 4 kHz. Analyzing language expression development, 13 (27.6%) children improved their performance. To the age at CI surgery there was no correlation between ASSR thresholds and CI thresholds (p-value=0.338) or speech perception category (p-value=0.358) after CI. No correlation has been found between ASSR and expressive language development (p-value=0.095). Conclusion: There was no direct correlation between electrophysiological thresholds measured by ASSR and hearing response after CI, when analyzed behavioral CI threshold or speech perception.

INTRODUÇÃO: A identificação e preservação da audição residual em candidatos a implante coclear vêm assumindo maior importância clínica. A resposta auditiva de estado estável (RAEE) pode fornecer informação frequência-específica sobre o limiar auditivo em níveis de intensidade máximos de 120 dB NA, possibilitando a investigação da audição residual. Os objetivos deste estudo são avaliar a audição residual em candidatos a implante coclear, comparando os limiares eletrofisiológicos da RAEE com os limiares psicoacústicos da audiometria nas frequências de 500, 1 000, 2 000 e 4 000 Hz. MÉTODO: Foram avaliados 40 candidatos a implante coclear (80 orelhas) com perda auditiva neurossensorial severa a profunda bilateral. A audiometria foi realizada com o tom \"warble\" nas frequências de 500, 1 000, 2 000 e 4 000 Hz com intensidade máxima de estimulação de 120 dB NA. A RAEE foi obtida através da estimulação dicótica de uma frequência de tons contínuos sinusoidais modulados 100% em amplitude exponencial e 20% em frequência, nas frequências portadoras de 500, 1 000, 2 000 e 4 000 Hz, com estimulação máxima de 117, 120, 119 e 118 dB NA, respectivamente. RESULTADOS: Foram obtidos limiares mensuráveis em 62,5% de todas as frequências estudadas na audiometria tonal e em 63,1% na RAEE. A RAEE apresentou sensibilidade de 96% e especificidade de 91,6% na detecção da audição residual. As diferenças médias entre os limiares da audiometria tonal e da RAEE não apresentaram significância estatística em nenhuma das frequências. As correlações entre os limiares comportamentais e da RAEE foram significantes em todas as frequências avaliadas, sendo fortes em 500, 1 000 e 2 000 Hz e moderada em 4 000 Hz, com coeficiente de correlação de Pearson entre 0,65 e 0,81. Em 90% dos casos, os limiares da RAEE foram adquiridos nos limites de 10 dB dos limiares comportamentais. CONCLUSÕES: As correlações entre os limiares tonais e da RAEE foram significantes nas frequências de 500, 1 000, 2 000 e 4 000 Hz. A RAEE apresentou alta sensibilidade e especificidade na detecção da audição residual em candidatos a implante coclear, em comparação com a audiometria tonal / INTRODUCTION: Identification and preservation of residual hearing in cochlear implantation are becoming more important lately. Auditory steadystate response (ASSR) can provide frequency-specific information regarding the auditory thresholds at maximum intensity levels of 120 dB HL, allowing investigation of residual hearing. The study objectives are to assess residual hearing in cochlear implant candidates by comparing the electrophysiological thresholds obtained in ASSR with psychoacoustic thresholds of audiometry at 500, 1 000, 2 000 and 4 000 Hz. METHOD: Forty cochlear implant candidates (80 ears) with bilateral severe-to-profound sensorineural hearing loss were studied. Warble-tone audiometry was performed at the frequencies 500, 1 000, 2 000 e 4 000 Hz, with stimuli up to 120 dB HL. ASSR was obtained with dichotic single-frequency stimulation of sinusoidal continuous tones modulated in exponential amplitude of 100% and in frequency of 20%, at the carrier frequencies of 500, 1 000, 2 000 and 4 000 Hz at maximum stimulation levels of 117, 120, 119 and 118 dB HL, respectively. RESULTS: Thresholds were obtained in 62,5% of all frequencies evaluated in warbletone audiometry and in 63,1% in the ASSR. ASSR showed sensitivity of 96% and specificity of 91,6% in the detection of residual hearing. The mean difference between the thresholds of behavioral audiometry and ASSR were not statistically significant in any of the frequencies. Strong correlations between behavioral and ASSR thresholds were observed in 500, 1 000 and 2 000 Hz and moderate in 4 000 Hz, with Pearson correlation coefficient between 0,65 and 0,81. In 90% of the occasions, ASSR thresholds were within 10 dB of behavioral thresholds. CONCLUSIONS: The correlations between behavioral and ASSR thresholds were significant at 500, 1 000, 2 000 and 4 000 Hz. ASSR showed high sensitivity and specificity in the detection of residual hearing in cochlear implant candidates, compared to warble-tone audiometry

Made available in DSpace on 2016-04-27T18:11:56Z (GMT). No. of bitstreams: 1 Gabriela Ribeiro Ivo Rodrigues.pdf: 4015764 bytes, checksum: 07311235963cd8c282288b7d688c3e0f (MD5) Previous issue date: 2012-05-24 / Fundação de Amparo a Pesquisa do Estado de São Paulo / The auditory steady-state response (ASSR) has been suggested as an alternative to the auditory brainstem response (ABR) to estimate the hearing threshold in children who are unable to carry out the tests with conditioned behavioral hearing procedures. The purpose of this study was to verify the applicability of ASSR to estimate the hearing thresholds in children with sensorineural hearing loss, comparing them to other procedures available for this assessment. The study included 15 children ages between 2 months and 3 years old, with sensorineural hearing loss. The ASSR obtained in 1, 2 and 4 kHz were compared with click ABR; the ASSR at 0.5, 1, 2 and 4 kHz were compared with the tone-ABR and with the visual reinforcement audiometry (VRA). The results showed good concordance between the ASSR at high frequencies with the responses of click ABR (0.63 - 0.70), being the best correlation for 1 kHz (0.70). When compared to tone-ABR it could be seen good concordance between the techniques, with coefficients of 0.77, 0.60, 0.66 and 0.50 for the frequencies of 0.5, 1, 2 and 4 kHz. However, the best coefficients were comparing the ASSR with the VRA (0.89 - 0.93), indicating strong correlation between the techniques. The results showed that when compared to other procedures available to estimate the hearing, the ASSR provided similar findings, proving to be a viable technique in order to estimate the hearing thresholds in a child when the VRA may not be possible / Os Potencias Evocados Auditivos de Estado Estável (PEAEE) têm sido apontados como uma alternativa aos Potenciais Evocados Auditivos de Tronco Encefálico (PEATE) para estimar os limiares auditivos em crianças que não conseguem realizar a avaliação auditiva comportamental com procedimentos condicionados. O objetivo deste estudo foi verificar a aplicabilidade dos PEAEE para estimar os limiares auditivos em crianças com perda auditiva neurossensorial, comparando-os os outros procedimentos disponíveis para esta avaliação. Participaram deste estudo 15 crianças com idades entre 2 meses e 3 anos, todas com perda auditiva neurossensorial. As respostas obtidas nos PEAEE nas freqüências de 1, 2 e 4 kHz foram comparadas com as do PEATE-clique; as respostas dos PEAEE nas freqüências de 0.5, 1, 2 e 4 kHz foram comparadas com as dos PEATE por freqüência específica (PEATE-FE) e com a audiometria de reforço visual (VRA). Os resultados encontrados mostraram boas concordâncias entre as respostas dos PEAEE nas altas freqüências e as respostas dos PEATE-clique (0.63 - 0.70), sendo a melhor correlação para a freqüência de 1 kHz (0.70). Quando os PEAEE foram comparados aos PEATE-FE também foram observadas boas concordâncias, com coeficientes de 0.77, 0.60, 0.66 e 0.50 para as freqüências de 0.5, 1, 2 e 4 kHz. Todavia, os melhores coeficientes obtidos foram da comparação dos PEAEE com a VRA (0.89 0.93), indicando forte concordância entre as técnicas. Os resultados mostraram que, quando comparados aos outros procedimentos eletrofisiológicos disponíveis para estimar a audição, os PEAEE forneceram medidas bem semelhantes, assim como quando comparados a VRA; demonstrando ser uma técnica viável para estimar os limiares auditivos de forma objetiva quando a VRA pode não ser possível

INTRODUÇÃO: A literatura evidencia as diferenças anatômicas do osso temporal em crianças quando comparadas à adultos. Mais e mais a literatura enfatiza a importância de que as crianças com surdez congênita devem ser implantadas cada vez mais precocemente. Estudos anátomo-radiológicos são importantes para o desenvolvimento de técnicas cirúrgicas que possibilitem implantar crianças recém natas. Técnicas de implante coclear percutâneo (através de robótica) tem sido desenvolvidas na Universidade de Vanderbilt. Criamos então nossa principal pergunta \"Seria possível implantar crianças recém natas através de implante percutâneo?\" e procuraremos respondê-la no curso desse trabalho. OBJETIVO: Aferir as medidas do recesso do nervo facial e sua relação com a cóclea visando simular o trajeto da broca através desse recesso, para realização da cocleostomia, objetivando o implante coclear percutâneo. CASUÍSTICA E METODOLOGIA: Estudo experimental realizado com 9 espécimes de natimortos com idade gestacional entre 32 e 40 semanas, submetidos à tomografia computadorizada com individualização e reconstrução do nervo facial, cadeia ossicular, membrana timpânica, cóclea e labirinto, seguido da definição da trajetória da broca até a escala timpânica, utilizando o software Improvise. RESULTADOS: As medidas da trajetória da broca até o nervo facial, variaram de 0.58 a mais próxima e, 1.71 a mais distante; quando analisamos os resultados obtidos para cadeia ossicular, temos uma variação que vai de 0.38 até 1.49. A membrana timpânica se encontra entre 0.85 e 1.96 de distância da trajetória simulada da broca. A trajetória da cortical do osso temporal até a escala timpânica, variou de 5.92 a 12.65. CONCLUSÃO: As medidas da relação, entre a broca e as estruturas anatômicas da orelha média e, a simulação da trajetória, mostraram que é possível executar com segurança a técnica de implante coclear percutâneo em crianças a partir de 32 semanas de gestação / INTRODUCTION: Literature shows changes in the temporal bone anatomy in children when compared with adults. More and more literature emphasizes the importance of children with congenital deafness should be implanted increasingly early. Anatomical radiologic studies are important for the development of surgical techniques that allow implanting new born. Percutaneous cochlear implant technique (through robotics) was developed in Vanderbilt University. So we created our main question \" Is it possible to implant new born children using the percutaneous technique? \" and we\'ll try to answer it in the course of this work. PATIENTS AND METHODS: Experimental study done in 9 stillbirth specimens with 32 and 40 weeks of age, submitted to CT study followed by reconstruction of the facial nerve, ossicular chain, tympanic membrane, labyrinth and cochlea. The CT evaluation and reconstruction was done with Improvise software, also used for measurements of the distances and in the simulation of the drill\'s trajectory (mm). RESULTS: The drill trajectory measurements to the facial nerve, ranged from 0.58 the nearest and the farthest 1.71, when we analyzed the results for ossicular chain, we have a range that goes from 0.38 to 1.49. The tympanic membrane is between 0.85 and 1.96 away from the simulated drill path. Cortical trajectory of the temporal bone to the scala tympani, ranged from 5.92 to 12.65. CONCLUSION: The measures of the relationship between the drill and the anatomical structures of the middle ear and the simulated trajectory, showed that it is possible to use safely the percutaneous cochlear implant technique in children of 32 weeks/old

Objective: The purpose of this study was to investigate the effects of noise exposure on the cervical vestibular evoked myogenic potential (cVEMP) in individuals with asymmetric noise-induced sensorineural hearing loss (NIHL). Design: A cross-sectional observational study was used to compare cVEMP characteristics in 43 individuals with a history of noise exposure greater in one ear (e.g., the left ear of a right-handed rifle shooter) and asymmetric sensorineural hearing loss consistent with the history of noise exposure and in 14 age-matched controls. The characteristics of hearing loss were examined further for the noise-exposed participants with abnormal cVEMPs and the noise-exposed participants with normal cVEMPs. Results: Thirty-three percent of the noise-exposed participants had abnormal cVEMPs, whereas cVEMPs were present and symmetrical in 100% of the age-matched controls, and cVEMP threshold was greater in the noise-exposed group than in the control group. Abnormal cVEMPs occurred most often in the ears with poorer hearing (or greater NIHL), and the noise-exposed participants who had abnormal cVEMPs had poorer high-frequency pure-tone thresholds (greater NIHL) and greater interaural high-frequency pure-tone threshold differences than the noise-exposed participants with normal cVEMPs. Conclusions: These findings are consistent with previous studies that suggest that the sacculocollic pathway may be susceptible to noise-related damage. There is emerging evidence that the severity of NIHL is associated with the presence or absence of cVEMPs.

Intratympanic injection of various otoprotectants through the round window membrane (RWM) might become available in the near future as an alternative to the currently available medical and surgical methods used to treat several inner ear diseases. The most common outcome of such diseases is sensorineural hearing loss (SNHL). Two examples of  these otoprotectants are Edaravone and Brain-Derived Neurotrophic Factor (BDNF), both of which have already proved effective against  noise-induced hair cell loss, barotrauma  and ototoxicity caused by cisplatin. In four different studies we used two electrophysiological methods, auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE), to study the effects of tobramycin and Pseudomonas aeruginosa exotoxin A (PaExoA) on the inner ears of 129 male Sprague-Dawley rats. In two investigations, not only the otoprotective effects of Edaravone on tobramycin-induced ABR threshold shifts and PaExoA-induced DPOAE  threshold changes, were studied but even different application times, in order to establish in which interval it was still possible to achieve effective otoprotection.We found that Edaravone gave otoprotection from tobramycin when injected simultaneously or within 7 days, but it had only a limited effect on the changes in DPOAE thresholds caused by PaExoA when injected 1, 2, or 4 hours after the exotoxin. The effect of BDNF on PaExoA-induced ABR threshold shifts was investigated in two studies, where different doses of intratympanically injected PaExoA were used and where BDNF was applied simultaneously, 12 or 72 hours efter exotoxin instillation. We found that BDNF had an otoprotective effect on SNHL induced by different doses PaExoA when injected simultaneously or with no more than 12 hours delay.

Auditorische Synaptopathien verursachen sensorineurale Schwerhörigkeit unterschiedlicher Ausprägung. Dabei sind sowohl erworbene, als auch angeborene Formen bekannt. Eine häufige Form ist die rezessive, nichtsyndromale Taubheitsform DFNB9, die auf einer Mutation des für Otoferlin kodierenden Gens beruht. Otoferlin ist ein Transmembranprotein, das essentiell für die stimulusabhängige Transmitterausschüttung an der Bändersynapse der Haarzellen ist . Um die Prognose von Patienten mit auditorischen Synaptopathien einschätzen zu können, ist es wichtig, den zeitlichen Verlauf der neuronalen Degeneration zu kennen. Aufgrund des Fehlens von protektiven Mittelohr und olivocochleären Reflexen könnte zusätzlich eine erhöhte Lärmempfindlichkeit bestehen, die einen frühen Verlust der Funktion der äußeren Haarzellen begünstigen wurde. Wir haben Otoferlin-Knockout-Mäuse im Alter von 6 Tagen bis 12 Monaten untersucht und die Funktion des Corti-Organs mittels Hirnstammaudiometrie und Messung der otoakustischen Emissionen getestet. In immunhistochemischen Färbungen von Corti-Organ Präparaten wurde die Degeneration von inneren und äußeren Haarzellen, Bändersynapsen und Spiralganglienneuronen beobachtet. Weiterhin wurden bei 2 Monate alten Otoferlin-Knockout-Mäusen die Auswirkungen eines leichten oder schweren Lärmtraumas quantifiziert. Wir beobachteten bei den Mutanten eine frühere und schnellere Degeneration der inneren und äußeren Haarzellen, insbesondere in der basalen Windung der Kochlea. Die Lärmtraumaversuche zeigten keinen Unterschied in der Empfindlichkeit von Otoferlin-Knockout-Mäusen und Wildtyp-Kontrollen, so dass die Ursache des Haarzellverlustes ungeklärt bleibt. Die Zahl der Bändersynapsen war ab Beginn des Hörens auf die Hälfte reduziert und blieb dann konstant, dennoch waren Spiralganglienneurone auch bei 12 Monate alten Tieren kaum reduziert. Das lässt auf einen relativ langen Erhalt des Hörnerven schließen Wir beschrieben hier zum ersten Mal die Degeneration des Corti-Organs in Otoferlin-Knockout-Mäusen. Gleichzeitig erfolgte die systematische Quantifizierung der synaptischen Degeneration von C57BL/6-Mäusen im Zusammenhang mit deren Altersschwerhörigkeit.