Introdução: A apneia obstrutiva do sono (AOS) provoca modificações na arquitetura normal do sono, fragmentando-o de forma crônica com hipóxias intermitentes levando, a longo prazo, a sérias consequências na saúde. Acredita-se que a ocorrência de eventos respiratórios durante o sono como apneia e hipopneia pode prejudicar o mecanismo de transdução da orelha interna e a transmissão de impulsos nervosos ao longo da via auditiva que são altamente dependentes do fornecimento do oxigênio. Contudo, essa associação não se encontra bem estabelecida na literatura. Adicionalmente, sujeitos com AOS poderiam apresentar alterações na função de transferência acústica da orelha média devido ao desequilíbrio pressórico existente nas vias aéreas superiores durante o sono, característico da fisiopatologia da doença. Objetivo: Comparar os achados da avaliação auditiva entre os indivíduos portadores e não portadores de AOS. Método: A casuística foi composta por 38 adultos do sexo masculino, média de idade de 35,8 (±7,2) e foram divididos em quatro grupos experimentais pareados por idade e índice da massa corpórea. Os grupos foram classificados com base na polissonografia em: controle (n=10), AOS leve (n=11), AOS moderada (n=8) e AOS grave (n=9). Todos os sujeitos do estudo negaram história pregressa de risco para perda auditiva. Todos os sujeitos foram submetidos à audiometria convencional, timpanometria, pesquisa de reflexos acústicos ipsi e contralaterais, imitância acústica de banda larga com estímulo clique de 226 a 8000 Hz para obtenção da energia de absorvância (EA), emissões otoacústicas por produto de distorção e potenciais evocados auditivos de tronco encefálico. Os resultados foram submetidos às análises estatísticas e o nível de significância adotado foi de 5% para todos os testes. Resultados: As medidas da EA não mostraram diferença significante entre os grupos quando considerados somente o fator grupo (sem AOS, AOS leve, moderada e grave). Para o efeito de interação entre os fatores grupo e frequência, o valor da média da EA do grupo com AOS de grau moderado foi significativamente maior do que do grupo com AOS de grau leve (p=0,003) em 8000 Hz. O grupo com AOS de grau grave apresentou menores valores das médias das amplitudes das EOAPD quando comparados aos dos grupos controle, AOS de grau leve e AOS de grau moderado (p=0,02, p=0,03 e p=0,01, respectivamente). Não houve diferença significante nos valores das latências absolutas das ondas I, III e V, e interpicos I-III, III-V e I-V entre os grupos. Observou-se associação entre a presença da AOS e alteração da latência absoluta da onda V (p=0,03). Foi observada associação entre AOS de grau moderado e alteração da latência da onda V (p=0,01). Conclusão: A presença da AOS está associada à presença de alteração na condução nervosa do estímulo acústico na via auditiva em tronco encefálico. A presença de AOS de grau grave prejudicou a função coclear. De maneira geral, a função de transferência acústica da orelha média é similar entre os adultos com e sem AOS / Introduction: The obstructive sleep apnea (OSA) can change the normal sleep architecture, fragmenting it chronically with intermittent hypoxias and, to the long time, inducing to serious consequences to health. It is believed that the occurrence of respiratory events during sleep with the presence of apnea and hipopneia can damage the transduction mechanism of the inner ear and nerve impulses transmission along the auditory pathways, which are highly dependent on the oxygen supply. However, this association is not well established. Moreover, the acoustic transference function of middle ear could show alterations, due to the pressure changes that occur in upper airway during the sleep, typical of OSA pathophysiology. Objective: To compare the findings of the hearing evaluations between subjects with OSA and without OSA. Method: A total of 38 subjects of the male sex, mean age 35.8 (±7.2), were divided into four groups, which were matched for age and body mass index. The groups were classified by the means of polissomnography in without OSA (n=10), mild OSA (n=11), moderate OSA (n=8) and severe OSA (n=9). All the subjects denied a history of risk for hearing loss. These subjects were submitted to: conventional audiometry, tympanometry, ipsilateral, contralateral acoustic reflex, wideband acoustic immittance for measurements of energy of absorbance (EA) with click stimulus of 226 to 8000 Hz, distortion product of otoacoustic emissions (DPOAE) and click evoked auditory brainstem responses (ABR). The recorded data of each analysis were conducted to an appropriate statistical test and was adopted the significant level of 5% for all the tests. Results: The EA data did not showed statistical differences among the groups when considering only the OSA severity factor (without, mild, moderate or severe). For the interaction between OSA severity and frequency factors, the mean value of EA of moderate OSA group was significant higher than the mild OSA group (p=0.003) in 8000 Hz. The severe OSA group presented lower mean values of amplitudes of DPOAE when compared to the control group, mild and moderate OSA groups (respectively, p=0.02, p=0.03 and p=0.01). For ABR, no differences was observed in latencies waves I, III and V, and interpeaks I-III, III-V and I-V values among the groups. Conclusion: The presence of OSA is associated with the presence of alterations in the nerve conduction of acoustic stimuli in the auditory pathway in the brainstem. The presence of severe OSA impaired the DPOAE responses. In general, the acoustic transference function of middle ear is similar between the adults with and without OSA

In this work is in details described anatomy and physiology of auditory tract, all its pathological changes included. The main part of this work concetrates on the issue of hearing disorders compensation when special emphasis comes to cochlear implants. This work characterizes every single functional part of cochlear implants from microphones to electrode array. The thesis works in details with speech processors of cochlear implants and with possibilities that provides codes strategies. In a practical part of this thesis is designed the scetch of block scheme of the cochlear implant using CIS and ACE strategy. As an outcome has been made the programme for processing acoustic signal that is based on mentioned block scheme.

Diese Arbeit beschäftigt sich mit der Simulation von Streuung und Absorption von elektromagnetischen Wellen in Gewebephantomen im Kontext optischer Cochlea-Implantate. Dabei werden wellen- und strahlenoptische Ansätze diskutiert und auf ihre Eignung für die Modellierung des Streuverhaltens in gewebeähnlichen Schichten untersucht. Im Einzelnen werden FDTD-Simulationen mit einem Mie-Streuprogramm verglichen, sowie Monte-Carlo-Simulationen durchgeführt. Die gewonnenen Daten für Gewebephantome werden mit einem experimentellem Ansatz und Literaturwerten für echtes Gewebe verglichen. Es wird gezeigt unter welchen Parametern die Lichtstreuung optimal für die gewünschte Anwendung in optischen Cochlea-Implantaten ist. Weiterhin ergibt sich, im Rahmen dieser Arbeit, die Monte-Carlo-Simulation in Verbund mit einem Mie-Streuprogramm als die praktikabelste Lösung, um mit Experiment und Literatur vergleichbare Schichtdicken zu simulieren und entsprechende Ergebnisse zu gewinnen. Die Übereinstimmung zwischen Simulationen und experimentellen Daten lies sich für Gewebephantome nachweisen. Weiterhin konnten erste Schlüsse über die Übereinstimmung des Verhaltens dieser Phantome gegenüber echter menschlicher Dermis gezogen werden. / This work addresses the simulation of scattering and absorption of electromagnetic waves in tissue phantoms in the context of optical cochlear implants. Wave-optical and ray-optical approaches are discussed and investigated for their suitability for modeling the scattering behavior in tissue-like layers. Specifically, FDTD simulations are compared with a Mie-scattering program, and Monte Carlo simulations are performed. The obtained data for tissue phantoms will be compared with an experimental approach and literature values for real tissue. It is shown under which parameters the light scattering is optimal for the desired application in optical cochlear implants. Furthermore, in the context of this work, the Monte-Carlo simulation in combination with a Mie-scattering program is most practicable to simulate layer thicknesses comparable to experiment and literature and to obtain the corresponding results. The agreement between simulations and experimental data could be demonstrated for tissue phantoms. Furthermore, first conclusions could be drawn about the agreement of the behavior of these phantoms compared to real human dermis.

Accessing the inner ear presents a significant challenge for the diagnosis and treatment of inner ear diseases. Many existing techniques to access the inner ear are invasive and can cause permanent damage to the cochlea. Recently, a novel microneedle has been fabricated to perforate the round window membrane (RWM) – a membrane sealing one of the two openings in the cochlea. These perforations enhance drug delivery into the inner ear, potentially improving the efficacy of therapeutics. Furthermore, they allow for the aspiration of perilymph samples, which is essential for diagnosing inner ear diseases. However, owing to limited knowledge about the mechanical properties of the RWM, certain technical aspects remain unexplored. Specifically, the interaction between the RWM and the microneedle during perforation is yet to be examined. This investigation is pivotal for the optimal design of microneedles — those robust enough to perforate RWMs yet delicate enough to minimize damage. In this thesis, we conduct a thorough examination of the guinea pig RWM, encompassing its geometry and its mechanical responses to pressures from the middle ear and inner ear. Additionally, we also formulate a comprehensive constitutive law for the guinea pig RWM. Our exploration begins with the creation of a U-Net model tailored to automatically segment the RWM. Despite the presence of other structures in the same image—such as bone, the basilar membrane, and ambient noise—the model proved invaluable for efficiently and automatically segmenting the RWM. To enhance accuracy, post-processing techniques like connected component analysis and majority voting were incorporated. Using this 3D model, we proceeded to study the RWM’s geometry. Recognizing the shrinkage observed in fixed RWMs, we integrated fresh RWM data to estimate the shrinkage ratio. Subsequently, we analyzed both the overall RWM thickness and that of the middle connective tissue layer—crucial metrics for future RWM modeling. Next, we proposed a method to evaluate the in-plane deformation of the RWM due to applied pressure. This involved using a bulge test system to pressurize and deform the RWM, combined with confocal microscopy to track stained nuclei or pre-introduced fluorescent beads on the RWM. We then utilized the coherent point drift (CPD) algorithm to measure the displacement of beads and nuclei. Results indicated that both markers could be successfully used to measure the RWM’s displacement. Further analysis revealed the in-plane Lagrangian strain of the RWM, with a significant observation being that the direction of maximum in-plane Lagrangian strain is perpendicular to the fiber direction. This underscores the crucial role of collagen fibers in determining the RWM’s mechanical properties. To conclude our study, we devised a constitutive law for the RWM, conceptualizing it as a combination of the ground substance and a family of dispersed fibers. This model was integrated into a FEBioStudio plugin, facilitating simulations of the RWM’s mechanical reactions to different pressures. Although our simulations closely aligned with experimental findings, some discrepancies were noted, likely stemming from an incomplete understanding of fiber dispersions. Nevertheless, our constitutive law reinforces the notion that fibers primarily govern the RWM’s mechanical characteristics.

<p>Developing embryos rely on morphogenetic signals to inform cells about where they are in space and respond to their positions through the appropriate expression of fate-determining genes. Computational and theoretical analyses are powerful tools that have proven to enhance and inform experimental work in developmental biology. In the study of positional information, mechanistic ordinary and partial differential equations are able to test and suggest hypotheses for morphogen network evolution. Information theoretic interpretations of these profiles have also been proven to be valuable towards making predictions.</p> <p>These approaches are reviewed and used here together to investigate the morphogenetic signals instructing pattern formation during the earliest phase of development in the cochlea. When the transcription factors SOX2 and pSMAD1/5/9 (two crucial carriers of positional information)<br> are quantified here for the first time, new observations, questions, and hypotheses emerge that have been out of reach otherwise. Perhaps most intriguingly is the identification of a linear pSMAD1/5/9 profile over a supermajority of the radial axis. </p> <p>This linear profile is shown to ‘set the stage’ by creating a 1:1 map between position and signal concentration. Feasible mechanisms responsible for maintaining this profile are simulated to propose the existence of a yet-unidentified BMP sink on the medial edge and suggests a role for Follistatin interaction with BMP, which there are currently doubts around. This likewise sets thestage for new experimental and simulation work to home in on the network dynamics implemented by the cochlea to turn a diffusive morphogen system into a linear signal. While BMP sets the stage of the radial axis, adding SOX2 more precisely assigns cells their places for this opening act with its steep profile that reduces positional error. The transition into subsequent phases where cell fates are assigned relies dependently on the precision encoded in this first phase in order to create the cellular pattern required to enable the sense of hearing. </p>

In unserer lautsprachlichen Welt ist Kommunikation stark auf das Hören angewiesen. Normalhörende Kinder sammeln in ihren ersten Lebensjahren Hörerfahrungen, die ihre sprachliche Entwicklung unterstützen. Früh ertaubten oder von Geburt an gehörlosen Kindern fehlt diese Möglichkeit bis zur Cochlea-Implantation. Ein Cochlea-Implantat (CI) ist eine Innenohrprothese, die geschädigte sensorische Haarzellen ersetzt und das Hören ermöglicht, wenn der Hörnerv intakt ist. Nach der Implantation beginnen CI-tragende Kinder ohne relevante Hörerfahrung mit der lautsprachlichen Entwicklung und müssen zu gleichaltrigen normalhörenden Kindern aufschließen. Die CI-Funktionen werden in mehreren Sitzungen an die Wahrnehmung der Person angepasst, doch das CI überträgt die Tonwelt nicht so exakt wie ein intaktes Gehör, was die auditive Umgebungswahrnehmung von CI-tragenden Kindern erschwert. Erwachsene CI-Nutzer können bei der Anpassung verbal kommunizieren, Kleinkinder oder Säuglinge jedoch nicht, weshalb ihre Hörwelt weitgehend unbekannt bleibt. Sprache und Musik teilen einige Wahrnehmungseigenschaften: Prosodie (Sprachmelodie) basiert auf Frequenz und Intonation, Emotionen werden durch Rhythmus und Intensität ausgedrückt, und Klangfarbe ermöglicht die Unterscheidung von Sprechstimmen und Instrumenten. Die Studie untersucht die musikalische Hörverarbeitung bei normalhörenden Säuglingen und CI-tragenden Kleinkindern mit vergleichbarem Höralter. Mithilfe der Mismatch Negativity (MMN) im EEG, die unabhängig von der Aufmerksamkeit des Probanden ausgelöst wird, wurde die Hörwahrnehmung objektiv beurteilt. Verschiedene musikalische Qualitäten wurden mit einem Multi-feature-MMN-Paradigma untersucht. Die Stimuli bestanden aus einem Tonlauf von vier Achtelnoten (Alberti-Bass) und wurden in allen Dur- und Moll-Tonarten in randomisierter Reihenfolge dargeboten, wobei der dritte Ton in Tonhöhe (pitch), Klangfarbe (timbre), Intensität (intensity), Rhythmus (rhythm), Glissando (slide) und einem zweiten Standard (standard2) variierte. Die Probanden wurden während der Stimulus-Präsentation geräuschlos abgelenkt. Es wurden Daten von 17 prälingual ertaubten CI-tragenden Kleinkindern (durchschnittlich 1 Jahr und 7 Monate alt) und 21 normalhörenden Säuglingen (6 Monate alt), beide mit 6 Monaten Hörerfahrung, ausgewertet. Für jede Bedingung wurde der Grand Average in beiden Gruppen und deren Untergruppen gebildet, und die MMN in frühen und späten Zeitfenstern analysiert. Die Ergebnisse zeigen, dass CI-tragende Kinder bei allen Devianten eine signifikante MMN zeigten. Die Untergruppe „mit Hörerfahrung“ wies bei timbre, intensity, rhythm und standard2 signifikante Ergebnisse auf, während die Untergruppe „ohne Hörerfahrung“ bei allen Devianten Signifikanzen zeigte. Dies wird jedoch aufgrund der kleinen Stichprobengröße nicht als robust betrachtet. Normalhörende Kinder zeigten bei allen Devianten außer standard2 eine signifikante MMN. CI-tragende Kinder reagierten unerwartet stark auf intensity und schwach auf rhythm, während normalhörende Kinder standard2 nicht wahrnehmen konnten. Erstmals wurde die Hörwahrnehmung von früh implantierten, bilateral CI-tragenden Kindern in ihrem frühen Höralter von sechs Monaten untersucht. CI-tragende Kinder konnten alle untersuchten Abweichungen wahrnehmen, wobei timbre und intensity besser und rhythm schwächer als erwartet wahrgenommen wurden. Normalhörende Kinder konnten standard2 aufgrund ihres Entwicklungsstandes nicht wahrnehmen, während ältere CI-tragende Kleinkinder dies kognitiv leisten konnten. Die CI-tragenden Kinder zeigten eine vergleichbare Fähigkeit zur Wahrnehmung akustischer Reize wie normalhörende Kinder, trotz fehlender oder unterbrochener Hörerfahrung.:Inhaltsverzeichnis Relevanz der Dissertation für den Forschungsbereich 1. Einleitung 1.1. Das auditive System 1.1.1. Anatomie des Ohres 1.1.2. Physiologie der akustischen Wahrnehmung 1.1.3. Embryologische Entwicklung des Innenohres: Der Beginn des Hörens 1.1.4. Kindliche Hörstörungen 1.2. Das Cochlea-Implantat (CI) 1.2.1. Definition und Entwicklung 1.2.2. Aufbau und Funktion 1.2.3. CI-Versorgung bei Kleinkindern 1.3. EEG und evozierte Potentiale 1.3.1. EEG 1.3.2. Ereignis-korrelierte Potentiale 1.3.3. CI-Artefakte 1.4. Bedeutung von Musik für die Entwicklung 1.4.1. Musik als Kulturgut 1.4.2. Psychosoziale Aspekte 1.4.3. Hörwahrnehmungen bis zum Kleinkindalter 1.4.4. Musikwahrnehmung mit CI 2. Experiment 2.1. Motivation 2.2. Stand der Wissenschaft 2.3. Fragestellung und Hypothesen 2.4. Methoden 2.4.1. Probanden 2.4.2. Versuchsdesign 2.4.3. Versuchsablauf 2.4.4. Elektrophysiologische Ableitung 2.4.5. Datenanalyse 2.5. Ergebnisse 2.5.1. Patientengruppe (CI-tragende Kinder) 2.5.2. Kontrollgruppe (normalhörende Kinder) 2.5.3. Übersicht über die CI- und die NH-Gruppe 2.5.4. Die Auswirkung des CI-Artefakts auf die MMN 3. Diskussion 3.1. Hörwahrnehmung CI-tragender Kinder 3.1.1. Pitch 3.1.2. Timbre 3.1.3. Intensity 3.1.4. Rhythm 3.1.5. Slide 3.1.6. Standard2 3.1.7. Globaler Vergleich der diskutierten Studien 3.2. Hörwahrnehmung normalhörender 6 Monate alter Säuglinge 3.3. Untergruppen „mit“ und „ohne Hörerfahrung“ 3.4. Latenzen 3.5. Fazit 3.6. Limitationen 3.7. Ausblick 4. Zusammenfassung 4.1. Deutsch 4.2. English Abbildungsverzeichnis Tabellenverzeichnis Literaturverzeichnis Anlage 1: Erklärung zur Eröffnung des Promotionsverfahrens Anlage 2: Erklärung zur Einhaltung aktueller gesetzlicher Vorgaben

Children with sensorineural hearing loss may (re)gain hearing with a cochlear implant—a device that transforms sounds into electric pulses and bypasses the dysfunctioning inner ear by stimulating the auditory nerve directly with an electrode array. Many implanted children master the acquisition of spoken language successfully, yet we still have little knowledge of the actual input they receive with the implant and specifically which language sensitive cues they hear. This would be important however, both for understanding the flexibility of the auditory system when presented with stimuli after a (life-) long phase of deprivation and for planning therapeutic intervention. In rhythmic languages the general stress pattern conveys important information about word boundaries. Infant language acquisition relies on such cues and can be severely hampered when this information is missing, as seen for dyslexic children and children with specific language impairment. Here we ask whether children with a cochlear implant perceive differences in stress patterns during their language acquisition phase and if they do, whether it is present directly following implant stimulation or if and how much time is needed for the auditory system to adapt to the new sensory modality. We performed a longitudinal ERP study, testing in bimonthly intervals the stress pattern perception of 17 young hearing impaired children (age range: 9–50 months; mean: 22 months) during their first 6 months of implant use. An additional session before the implantation served as control baseline. During a session they passively listened to an oddball paradigm featuring the disyllable “baba,” which was stressed either on the first or second syllable (trochaic vs. iambic stress pattern). A group of age-matched normal hearing children participated as controls. Our results show, that within the first 6 months of implant use the implanted children develop a negative mismatch response for iambic but not for trochaic deviants, thus showing the same result as the normal hearing controls. Even congenitally deaf children show the same developing pattern. We therefore conclude (a) that young implanted children have early access to stress pattern information and (b) that they develop ERP responses similar to those of normal hearing children.

Introdução: O implante auditivo de tronco encefálico (ABI) é indicado para pacientes com perda auditiva neurossensorial severa a profunda quando há impossibilidade de realização do implante coclear. Em pacientes com surdez por meningite e ossificação coclear total bilateral, o ABI é a opção para a reabilitação auditiva. Objetivos: O estudo tem por objetivo avaliar a contribuição do ABI para os limiares audiométricos e para a percepção de fala após no mínimo 12 meses de uso em pacientes com ossificação coclear total por surdez pós-meningite, bem como descrever as complicações do procedimento. Material e métodos: Dez pacientes com surdez pósmeningite foram submetidos ao ABI por via retrolabiríntica ampliada em um centro terciário de assistência e ensino. Todos os pacientes foram operados pela mesma equipe cirúrgica e a avaliação audiológica foi realizada pelo mesmo fonoaudiólogo. Foram realizados audiometria tonal e testes de percepção de fala no pré-operatório e no mínimo 12 meses após a ativação do implante. Foram descritas as complicações associadas ao procedimento. Resultados: Oito de dez pacientes implantados permaneceram usuários. Dois pacientes não apresentaram respostas auditivas e abandonaram o seguimento. Os oito pacientes apresentaram melhora estatisticamente significativa nos limiares audiométricos, bem como nos testes de discriminação de palavras e vogais comparando pré e pós-operatório com média de seguimento de 3,3 anos. Em dois pacientes, a discriminação de sentenças em formato fechado somente no modo auditivo foi de 30% e 40%. Todos os oito usuários referiram benefício com o uso do ABI. Não houve complicações relacionadas ao procedimento. Conclusão: O ABI via retrolabiríntica ampliada é uma opção terapêutica segura para pacientes com surdez pós-meningite e com presença de ossificação coclear total bilateral, contribuindo para melhora nos limiares audiométricos e nos testes de percepção de fala. Embora a melhora nos testes audiológicos seja inferior à do implante coclear, a maioria dos pacientes do estudo usa o ABI diariamente por um período superior a 8 horas e refere benefício em seu cotidiano / Introduction: The Auditory Brainstem Implant (ABI) is an option to auditory restoration in patients with severe to profound sensorineural hearing loss who cannot be fitted with a cochlear implant. This is the only option in patients with post meningitis hearing loss presenting with bilateral total cochlear ossification. Objectives: The main objective of the study is to evaluate the hearing contribution in audiometry and speech perception tests at least 12 months after ABI implantation in patients with post-meningitis profound hearing loss. The complications of the procedure were also described. Materials and Methods: Ten patients with post-meningitis hearing loss went an ABI through extended retrolabyrinthine approach in a tertiary center by the same surgeons. The same audiologist was responsible for audiological follow-up. Tonal audiometry and speech perception tests were made before and at least 12 months after the ABI activation. The procedure complications were described for all patients. Results: Eight of ten patients became ABI users. Two patients had no auditory response and abandoned the treatment. Eight users showed benefit in tonal audiometry, word and vowels perception tests after an average follow up of 3.3 years. Two patients were able to recognize 30 and 40% of closed sentences without lip reading. There were no complications due to the ABI procedure. Conclusion: The extended retrolabyrinthine approach for the ABI is a safe surgical option for patients with post-meningitis hearing loss and totally ossified cochleae. It contributes to hearing performance in audiometry and speech perception tests. Even though the ABI results are poorer than the cochlear implants, in this study the majority of patients use their ABI more than eight hours a day and report benefits in daily activities

Introdução: O implante auditivo de tronco encefálico (ABI) é indicado para pacientes com perda auditiva neurossensorial severa a profunda quando há impossibilidade de realização do implante coclear. Em pacientes com surdez por meningite e ossificação coclear total bilateral, o ABI é a opção para a reabilitação auditiva. Objetivos: O estudo tem por objetivo avaliar a contribuição do ABI para os limiares audiométricos e para a percepção de fala após no mínimo 12 meses de uso em pacientes com ossificação coclear total por surdez pós-meningite, bem como descrever as complicações do procedimento. Material e métodos: Dez pacientes com surdez pósmeningite foram submetidos ao ABI por via retrolabiríntica ampliada em um centro terciário de assistência e ensino. Todos os pacientes foram operados pela mesma equipe cirúrgica e a avaliação audiológica foi realizada pelo mesmo fonoaudiólogo. Foram realizados audiometria tonal e testes de percepção de fala no pré-operatório e no mínimo 12 meses após a ativação do implante. Foram descritas as complicações associadas ao procedimento. Resultados: Oito de dez pacientes implantados permaneceram usuários. Dois pacientes não apresentaram respostas auditivas e abandonaram o seguimento. Os oito pacientes apresentaram melhora estatisticamente significativa nos limiares audiométricos, bem como nos testes de discriminação de palavras e vogais comparando pré e pós-operatório com média de seguimento de 3,3 anos. Em dois pacientes, a discriminação de sentenças em formato fechado somente no modo auditivo foi de 30% e 40%. Todos os oito usuários referiram benefício com o uso do ABI. Não houve complicações relacionadas ao procedimento. Conclusão: O ABI via retrolabiríntica ampliada é uma opção terapêutica segura para pacientes com surdez pós-meningite e com presença de ossificação coclear total bilateral, contribuindo para melhora nos limiares audiométricos e nos testes de percepção de fala. Embora a melhora nos testes audiológicos seja inferior à do implante coclear, a maioria dos pacientes do estudo usa o ABI diariamente por um período superior a 8 horas e refere benefício em seu cotidiano / Introduction: The Auditory Brainstem Implant (ABI) is an option to auditory restoration in patients with severe to profound sensorineural hearing loss who cannot be fitted with a cochlear implant. This is the only option in patients with post meningitis hearing loss presenting with bilateral total cochlear ossification. Objectives: The main objective of the study is to evaluate the hearing contribution in audiometry and speech perception tests at least 12 months after ABI implantation in patients with post-meningitis profound hearing loss. The complications of the procedure were also described. Materials and Methods: Ten patients with post-meningitis hearing loss went an ABI through extended retrolabyrinthine approach in a tertiary center by the same surgeons. The same audiologist was responsible for audiological follow-up. Tonal audiometry and speech perception tests were made before and at least 12 months after the ABI activation. The procedure complications were described for all patients. Results: Eight of ten patients became ABI users. Two patients had no auditory response and abandoned the treatment. Eight users showed benefit in tonal audiometry, word and vowels perception tests after an average follow up of 3.3 years. Two patients were able to recognize 30 and 40% of closed sentences without lip reading. There were no complications due to the ABI procedure. Conclusion: The extended retrolabyrinthine approach for the ABI is a safe surgical option for patients with post-meningitis hearing loss and totally ossified cochleae. It contributes to hearing performance in audiometry and speech perception tests. Even though the ABI results are poorer than the cochlear implants, in this study the majority of patients use their ABI more than eight hours a day and report benefits in daily activities

This dissertation based on a case study explores the acquisition and the guidance of Swedish Sign Language and spoken Swedish over a span of seven years. Interactions between a pair of fraternal twins, one deaf and one hearing, and their Deaf[1] family were video-observed within the home setting. The thesis consists of a frame which provides an overview of the relationship between four studies. These describe and analyze mainly storytime sessions over time. The first article addresses attentional expressions between the participants; the second article studies the mediation of the deaf twin’s first language acquisition; the third article analyses the hearing twins acquisition of parallel bimodal bilingualism; the fourth article concerns second language acquisition, sequential bimodal bilingualism following a cochlear implant (CI). In the frame, theoretical underpinnings such as mediation and language acquisition were compiled, within a sociocultural frame. This synthesis of results provides important information; in the 12- and 13-month sessions simultaneous-tactile-looking was noted in interchanges between the twins and their mother; mediation of bilingualism was scaffolded by the caregivers with the hearing twin by inserting single vocal words or signs into the language base used at that time, a finding that differs from other reported studies; a third finding is the simultaneousness in which the deaf child’s Swedish Sign Language skill worked as a cultural tool, to build a second and spoken language. The findings over time revealed actions that included all the family members. Irrespective of the number of modes and varied types of communication with more than one child, mediation included following-in the child’s initiation, intersubjective meaningfulness and encouragement. In accordance with previous research, these factors seem to promote the acquisition of languages. In conclusion, these findings should also prove useful in the more general educational field. [1] Deaf with a capital ‘D’ is commonly used for cultural affiliation whereas lower case ‘d’, as in deaf, refers to audiological status (Monaghan, Schmaling, Nakamura &amp; Turner, 2003). / <p>Disputationen tolkas till svensk teckenspråk, hörselslinga finns.</p><p>At the time of the doctoral defense, the following papers were unpublished and had a status as follows: Paper 2: Submitted. Paper 3: Accepted. Paper 4: Submitted.</p>