Cochlear implants (CIs) and auditory brainstem implants (ABIs) are prostheses for hearing used in patients with profound hearing impairment. A CI requires an operational cochlear nerve to function in contrast to an ABI. ABIs were initially designed for adult patients with neurofibromatosis type 2 (NF2), suffering from bilateral vestibular schwannomas. Now ABIs are also used for patients, both adults and children, with congenital cochlear malformations, cochlear nerve hypoplasia/aplasia, and cochlear ossification. The aims of this thesis are to evaluate hearing outcome in patients implanted with a CI after long-term deafness. An extended period of deafness has earlier been considered as a contraindication for CI surgery. Further, we analyzed if electrically evoked auditory brainstem responses (eABRs) can predict CI outcome and pinpoint the optimal selection of treatment such as CI or ABI. We also disclose our experiences from ABI surgery in Uppsala, such as implant use, hearing outcome, complications, and satisfaction among the patients. Finally, we evaluated the results and benefits of ABIs in non-NF2 pediatric patients. Results show that patients with an extended deafness period and durations over 20 years can achieve speech understanding and benefit from CIs. Patients with long-term deafness and limited years of hearing before deafness did not perform as well as those with shorter deafness duration and longer hearing experience did. eABR seems to have a definite role in the diagnostic armamentarium, to better consider alternative surgical strategies such as ABI. No eABR waveform predicted a poor CI outcome. There was no correlation between speech perception and eABR waveform latencies or eABR waveform quality. A majority of the ABI patients used their ABIs and benefited from them for at least some period. ABI assisted voice control in a majority of the full-time users and they reported improved understanding of speech with the implant switched on. No severe complications from ABI surgery or ABI stimulation were noted. The patients were generally satisfied, even if their hearing remained very limited. All pediatric patients but one used the implant continuously and benefited from it.

Objective: This study aimed to systematically review and analyse the available peer-reviewed literature reporting on the results of distortion product otoacoustic emissions (DPOAEs), transient evoked otoacoustic emissions (TEOAEs) and click auditory brainstem responses (c-ABRs) in adults with type 1 and type 2 diabetes mellitus (T1DM and T2DM). Method: A comprehensive literature search was conducted across three electronic databases to identify English; peer-reviewed articles that included results of OAEs (DPOAEs and TEOAEs) and c-ABR tests in adult subjects with DM. Articles were selected according to predetermined selection criteria and critically reviewed independently by two researchers. Results: 15 studies met the inclusion criteria for the systematic review while nine articles qualified for inclusion in the meta-analysis. DPOAE studies reported significantly reduced amplitudes with only one study reporting larger amplitudes. Abnormal TEOAEs were reported in all TEOAE studies, although these abnormalities were not always significant. Significantly delayed c-ABRs were reported in all ABR studies. Analysis of c-ABR mean wave latencies identified longer latencies for DM subjects, particularly for wave III and V, as well as for IPL I-III and I-V. Conclusions: Subjects with T1DM and T2DM may present with clinical or subclinical impairment of the cochlear outer hair cells and both the peripheral and central auditory pathway. / Dissertation (MA)--University of Pretoria, 2016. / Speech-Language Pathology and Audiology / MA / Unrestricted

After the findings were reported for the Women’s Health Initiative (WHI) study in the past decade, there has been a significant decline in the overall use of hormone replacement therapy (HRT) among women. However, there are still millions of middle-aged, menopausal women in the U.S. who are currently undergoing hormone therapy. Their reasons for continuing treatment include relief of severe menopausal symptoms, aid in the management of osteoporosis and reduction in the risk of colon cancer (Ness et al., 2005). The purpose of the following investigation was to evaluate the impact of HRT on the central and peripheral auditory systems both during and after treatment. Over the course of the study, hormone treatments were administered to female aging CBA/CaJ mice to observe what effects estrogen (E) and progestin (P) have on the peripheral and central auditory systems. Female CBA/CaJ middle age mice were ovariectomized and placed into 4 HRT groups (E, P, E+P and Placebo [Pb]). Hormone treatment lasted 6 months followed by a recovery/washout period of 1 month. During this time, electrophysiology tests such as auditory brainstem responses (ABR) and ABR gap in noise (GIN) were used to measure neural activity for the auditory nerve and brainstem. Distortion product otoacoustic emission (DPOAE) testing was also implemented to assess the functional status of the outer hair cells (OHC) and their ability to amplify sound in the cochlea. After 6 months of treatment, animals treated with E exhibited the least amount of changes in ABR thresholds and ABR GIN amplitudes than any other subject groups. Interestingly, P animals exhibited an abrupt increase in ABR thresholds only 3 months after treatment; however, for ABR GIN amplitude levels a progressive reduction observed throughout the study. E+P and Pb animals showed signs of accelerated age-related hearing loss (ARHL) with significantly elevated ABR thresholds and dwindling ABR GIN amplitude levels. No significant signs of recovery were observed for any of the hormone groups. Therefore, in the present murine investigation, the effects of HRT were long lasting. To further expand on the results obtained for the electrophysiology tests, molecular biology experiments were performed to evaluate the expression of IGF-1R and FoxO3 in the cochlea during hormone therapy, from both in vitro and in vivo perspectives. Both genes play significant roles in the PI3K/AKT pathway and were specifically chosen because of their role in anti-apoptotic responses and cell survival. It was hypothesized that E attenuates the effects of ARHL via the PI3K/AKT pathway by up-regulating IGF-1R and FoxO3 to counteract the effects of oxidative stress in the aging mammalian cochlea. qPCR experiments were performed with stria vascularis (SV) lateral wall cells extracted from the cochlea of each animal in the hormone groups post-treatment (in vivo) and in SVK-1 cells treated with HRT over various lengths of time (in vitro) to evaluate the expression levels of IGF-1R and FoxO3. In-vivo experiments showed that the E-treated animals had significantly higher IG-1R levels compared to the other subject groups after treatment was discontinued. Similarly, IGF-1R levels steadily increased for E-treated SVK-1 cells over the course of hormone therapy, compared to P and E+P cells. FoxO3 expression, on the other hand, declined for all of the hormone-treated cells groups, relative to control SVK-1 cells (in vitro), and no statistical differences were detected for FoxO3 levels among the post-treatment animals (in vivo). These findings indicate that there is cross talk between E and IGF-1R involving the PI3K/AKT pathway, which contributes to the delayed onset of ARHL observed during HRT with E. Meanwhile, FoxO3 may not play a role in neuro-protective properties in the cochlea during HRT, as initially hypothesized.

Background: Iron deficiency, anemia, and iron excess have been associated with altered hearing functions in children and adults. Animal studies suggest that iron deficiency during pregnancy negatively affect fetal auditory development. The relationship between maternal iron status and auditory functions in healthy term newborns has not been clearly elucidated among humans. The goal of this pilot study was to determine the relationship between markers of iron status during pregnancy and brainstem auditory function in healthy neonates. Methods: Pregnant women who gave birth at the Montfort Hospital were recruited to take part in this study (n=6). Within two weeks after birth, their newborn’s hearing function was assessed by wave amplitude, latency and inter-latency from the Auditory Brainstem Response (ABR) test. Markers of iron status, namely hemoglobin (Hb) and mean corpuscular volume (MCV), were collected retrospectively for the first and second trimester from the women’s medical chart. Results: Overall, no significant relationship was observed between maternal Hb and MCV concentrations and newborns auditory function. Although two significant Spearman correlations were observed (MCV and inter-latency I-V; r=0.87; p=0.005 and Hb and amplitude V; r=0.89; p=0.04), these findings may be due to chance because of multiple testing and the small sample size. Conclusion: Although iron is a key nutrient involved in the brain and auditory system development, we were not able to demonstrate a relationship between iron status during pregnancy and newborn hearing function. Prospective or intervention studies with a larger sample size and with more specific iron markers (ex. ferritin) are required to confirm these findings.

Ce travail s’est intéressé au traitement sous cortical de la parole dégradée par le bruit, notamment par la caractérisation première de l’importance de certains traits acoustiques dans la perception de la parole normale. Pour cela, nous avons d’abord participé à la mise au point de la technique électrophysiologique de potentiels évoqués auditifs obtenus en réponse à des sons de parole, technique proche de celle des potentiels évoqués auditifs précoces, mais qui a des exigences propres en matière de traitement du signal et de techniques de recueil, qui nécessitent une adaptation importante de part la nature différente des stimuli français par rapport aux stimuli anglais utilisés par l’équipe de référence américaine. Les différents axes de notre recherche ont, par ailleurs, permis de mettre en évidence l’importance de l’encodage sous cortical de certaines caractéristiques acoustiques telles que l’enveloppe temporelle, le voisement, mettant par là même en évidence un possible effet corticofuge sur l’encodage de celui-ci. Ces différentes expériences nous ont amenés à nous poser la question des conditions idéales de recueil des PEASP, et notamment l’effet de l’intensité sur le recueil de ceux-ci, mettant en évidence une relation non-linéaire entre l’intensité de stimulation, et les caractéristiques des PEAPSP. Si une intensité de 20 dB SL semble nécessaire au recueil d’un PEAPSP, les réponses montrent une variabilité qui reste très grande à l’échelon individuel, ce qui rend l’utilisation de l’outil PEAPSP à visée diagnostique, que ce soit dans les troubles du langage chez l’enfant, ou dans les troubles de l’audition dans le bruit, difficile. / The major purpose of my thesis was the investigation of brainstem structures implications into speech in noise processing, particularly by identifying the impact of acoustic cues on normal speech perception. Firstly, we were involved in the engineering of the speech auditory brainstem responses (SABR) recording system. SABR are similar to brainstem auditory evoked responses to clicks, but require different acquisition and signal processing set-ups, due to the difference between the French and the American stimuli used by the American reference team. The different studies presented here, permitted to emphasize the role of brainstem structures into the subcortical processing of acoustical cues, such as the temporal enveloppe, or the voicing, with a possible evidence of a corticofugal effect on SABR. These experimentations lead us to a more fundamental question on the best conditions required for PEASP collection, in particular, the best stimulation intensity needed. The results of the experiment on intensity effect showed a non linear relation between the stimulation intensity and PEASP characteristics. Even if an intensity of only 20 dB SL seems enough for SABR recording, individual results are still highly variable so that diagnostic application of SABR on, for example, children with language learning problems or subject suffering from speech in noise perception impairment remains difficult.

Depuis quelques années, des recherches décrivent des effets alarmants de l’exposition à des environnements acoustiques artificiels sur les propriétés fonctionnelles des neurones du système auditif. L’objectif de ce projet était de déterminer si une exposition à très long terme à une intensité sonore, qui n’est pas reconnue par les législations pour provoquer des pertes permanentes ou temporaires (80dB SLP 8h/jour), induisait ou pas des changements au niveau des audiogrammes et des propriétés fonctionnelles des neurones du cortex auditif primaire.Des rattes adultes (Sprague Dawley) ont été exposées entre 3 mois à 18 mois (selon les groupes) à un milieu acoustique mimant les environnements sonores quotidiens de la majorité de la population et dont les effets n’ont jamais été étudiés sur de telles durées. L’originalité de ce projet réside dans l’analyse des effets à tous les niveaux du système auditif depuis le niveau périphérique (ABRs) jusqu’au niveau central (électrophysiologie corticale) ainsi que les conséquences possibles au niveau comportemental. Une tâche d’apprentissage perceptif inédite a été mise au point afin d’évaluer les effets de l’exposition. Au cours du vieillissement, nos données montrent une baisse des performances comportementales, une atteinte progressive des seuils ABRs et des atteintes de certains paramètres des réponses neuronales comme (i) la latence, (ii) la durée, (iii) la détection de silence dans une vocalisation, (iv) le suivit d’une modulation d’amplitude, (v) la reproductibilité des réponses à une vocalisation. Le principal effet de l’exposition à un environnement bruité est l’apparition d’un TTS après 6 à 12 mois d’exposition (qui disparait complètement en 3 semaines), sans que cela ait, de façon très surprenante, la moindre conséquence notable sur les seuils ABRs, l’activité évoquée corticale, ou les performances de discrimination des animaux. Ces résultats nous incitent à la prudence sur la généralisation des conclusions à tirer des expositions à des environnements bruités artificiels. / Over the last few years, studies have described alarming effects of exposure to artificial acoustic environments on the functional properties of neurons in the auditory system. The aim of this project was to determine if long-lasting exposure at a sound intensity which is not recognized by the legislation to cause permanent or temporary hearing loss (80 dB SLP 8h/ day) induced, or not, changes in the audiograms and functional properties of neurons in theprimary auditory cortex. Adult female rats (Sprague Dawley) were exposed over 3 to 18 months (depending on the group) to an acoustic environment mimicking daily sound environments surrounding a large part of the population, and whose effects have never been studied on such durations. The originality of this project lies in analyzing the effects at alllevels of the auditory system from peripheral (via ABRs) to central levels (cortical electrophysiology) and also the possible consequences at the behavioral level. A new perceptual learning task has been developed to assess the effects of exposure. During aging, our data showed a decrease in behavioral performance, a gradual impairment of ABRs thresholds as well as an impairment in parameters of the neural responses such as (i) the response latency, (ii) response duration, (iii) the ability to detect silence in a vocalization (iv) or to follow an amplitude modulation, (v) the reproducibility of response to vocalization. The main effect of exposure to a noisy environment is the appearance of a Temporary Threshold Shift (TTS) after 6 to 12 months of exposure (which completely disappears in three weeks). Surprisingly, this long lasting TTS had apparently no e ffect on ABRs thresholds, the evokedcortical activity, or the animal’s discrimination performance. These results encourage us to be quite cautious in generalizing the conclusions to be drawn from exposures to artificial noisyenvironments.

No description available.

Infant hearing screening (IHS) programs are not yet widespread in developing countries, such as South Africa. In order to ensure that the benefits of early hearing detection and intervention (EHDI) programs reach all infants, initial recommendations for the implementation of IHS programs in South Africa have been made by the Year 2002 Hearing Screening Position Statement by the Health Professions Council of South Africa. One of the platforms recommended for IHS in South Africa is the neonatal intensive care unit (NICU). South African NICU infants are at an increased risk for hearing loss, resultant of their high-risk birth histories, as well as the prevalence of context-specific environmental risk factors for hearing loss. There is currently a general scarcity of contextual data regarding the prevalence of risk indicators for hearing loss, and the prevalence of auditory impairment in the South African NICU population. The objective of this study was to describe an IHS program for NICU infants at a secondary hospital in Gauteng, South Africa. A quantitative descriptive research design was used to report on a cohort of 129 NICU infants followed up during a 29 month period. The objective of the study was achieved by describing the sample of infants in terms of the presence of specific risk indicators for hearing loss, the efficiency of the IHS program, and the incidence of auditory pathologies. Infants received their initial hearing screening as part of their medical and developmental follow-up visit at the hospital at three months of age. Routine rescreening visits were scheduled three monthly, whilst infants who failed the hearing screening were requested to return after three weeks for a follow-up. A data collection sheet was used to collect biographical information and risk indicators for hearing loss. Immittance measurements were recorded in the form of high-frequency and low-frequency tympanometry. Distortion product otoacoustic emissions (DPOAE) and automated auditory brainstem responses (AABR) were recorded, as well as diagnostic auditory brainstem responses (ABR) in cases where infants referred the screening protocol. Results revealed that environmental risk factors present in this sample included poor maternal education levels and prenatal HIV/AIDS exposure. At least 32% of mothers participating in this study did not complete high school. Prenatal HIV/AIDS exposure was present in at least 21% of the current sample of infants. The screening coverage rate fell short of the 95% benchmark set by the Joint Committee on Infant Hearing (JCIH, 2000). A 67% coverage rate was achieved with AABR screening, and an 88% coverage rate was achieved with DPOAE screening. 93% of infants had immittance screening performed on their initial visit to the IHS program. According to the Fisher’s two-sided exact test and the logistic regression procedure, high frequency tympanometry proved to be more effective than low frequency tympanometry, when assessing the middle ear functioning of infants younger than seven months when compared with DPOAE results. Normative pressure and admittance data was compiled for the use of high frequency tympanometry in NICU infants. Poor follow-up rates were recorded for both routine and non-routine visits, but are expected to improve over time. Furthermore, results indicated a high incidence of hearing impairment. Permanent congenital hearing loss was identified in 3% (n=4) of the sample. Half of these presented with sensorineural hearing loss, whilst the other half had auditory neuropathy. The incidence of auditory impairment is estimated to be 3.75% if the percentage of infants who did not return for follow-up is taken into account. A high incidence of middle ear pathology was recorded, with an incidence rate of 60.4%, including bilateral and unilateral middle ear pathology. The high prevalence of auditory impairment in South African NICU infants, and the lack of widespread IHS programs, indicates that many vulnerable infants are being the denied the benefits of early identification of and intervention for hearing loss. The implementation of widespread IHS programs in South Africa is therefore essential, in order to ensure that all South African infants receive the benefits of EHDI programs. / Dissertation (MCommunication Pathology)--University of Pretoria, 2007. / Speech-Language Pathology and Audiology / unrestricted