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Auditory Function (auditory + function)
Selected AbstractsAuditory function and hearing loss in children and adults with Williams syndrome: Cochlear impairment in individuals with otherwise normal hearing,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 2 2010Jeffrey A. Marler Abstract Hearing loss is common in school-age individuals with Williams syndrome (WS) and extensive in adults. Prior studies with relatively small sample sizes suggest that hearing loss in WS has an early onset and may be progressive, yet the auditory phenotype and the scope of the hearing loss have not been adequately characterized. We used standard audiometric tools: Otoscopy, tympanometry, air-conduction (bone conduction when available) behavioral testing, and distortion product otoacoustic emissions (DPOAEs) to measure hearing sensitivity and outer hair cell function. We tested 81 individuals with WS aged 5.33,59.50 years. Sixty-three percent of the school-age and 92% of the adult participants had mild to moderately-severe hearing loss. The hearing loss in at least 50% was sensorineural. DPOAE testing corroborated behavioral results. Strikingly, 12 of 14 participants with hearing within normal limits bilaterally had 4,000-Hz DPOAE input/output (DPOAE IO) functions indicative of outer hair cell damage and impaired cochlear compression. Our results indicate that hearing loss is very common in WS. Furthermore, individuals with WS who have "normal" hearing as defined by behavioral thresholds may actually have sub-clinical impairments or undetected cochlear pathology. Our findings suggest outer hair cell dysfunction in otherwise normal hearing individuals. The DPOAE IO in this same group revealed growth functions typically seen in groups with noise-induced damage. Given this pattern of findings, individuals with WS may be at increased risk of noise-induced hearing loss. Recommendations regarding audiological testing for individuals with WS and accommodations for these individuals in both academic and nonacademic settings are provided. © 2010 Wiley-Liss, Inc. [source] Auditory neuropathy/dys-synchrony: Diagnosis and managementDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 4 2003Charles I. Berlin Abstract Auditory brainstem responses (ABRs) and otoacoustic emissions (OAEs) are objective measures of auditory function, but are not hearing tests. Normal OAEs reflect normal cochlear outer hair cell function, and an ABR indicates a synchronous neural response. It is quite possible for a patient to have normal OAEs but absent or grossly abnormal ABR and a behavioral audiogram that is inconsistent with either test. These patients, who may constitute as much as 10% of the diagnosed deaf population, have auditory neuropathy/dys-synchrony (AN/AD). To diagnose AN/AD accurately, ABRs are obtained in response to condensation and rarefaction clicks to distinguish cochlear microphonics (CM) from neural responses. Appropriate management is confounded by variation among patients and changes in auditory function in some patients over time. Recommendations for management include visual language exposure through methods such as American Sign Language (ASL), Cued Speech, or baby signs, and closely following patients. MRDD Research Reviews 2003;9:225,231. © 2003 Wiley-Liss, Inc. [source] Chondrocyte-specific Smad4 gene conditional knockout results in hearing loss and inner ear malformation in miceDEVELOPMENTAL DYNAMICS, Issue 8 2009Shi-Ming Yang Abstract Smad4 is the central intracellular mediator of transforming growth factor-, (TGF-,) signaling, which plays crucial roles in tissue regeneration, cell differentiation, embryonic development, and regulation of the immune system. Conventional Smad4 gene knockout results in embryonic lethality, precluding its use in studies of the role of Smad4 in inner ear development. We used chondrocyte-specific Smad4 knockout mice (Smad4Co/Co) to investigate the function of Smad4 in inner ear development. Smad4Co/Co mice were characterized by a smaller cochlear volume, bone malformation, and abnormalities of the osseous spiral lamina and basilar membrane. The development of the hair cells was also abnormal, as evidenced by the disorganized stereocilia and reduced density of the neuronal processes beneath the hair cells. Auditory function tests revealed the homozygous Smad4Co/Co mice suffered from severe sensorineural hearing loss. Our results suggest that Smad4 is required for inner ear development and normal auditory function in mammals. Developmental Dynamics, 2009. © 2009 Wiley-Liss, Inc. [source] Cortical auditory dysfunction in benign rolandic epilepsyEPILEPSIA, Issue 6 2008Dana F. Boatman Summary Purpose: To evaluate cortical auditory function, including speech recognition, in children with benign rolandic epilepsy (BRE). Methods: Fourteen children, seven patients with BRE and seven matched controls, underwent audiometric and behavioral testing, simultaneous EEG recordings, and auditory-evoked potential recordings with speech and tones. Speech recognition was tested under multiple listening conditions. Results: All participants demonstrated normal speech recognition abilities in quiet, as well as normal peripheral and subcortical auditory function. BRE patients performed significantly worse than controls when speech recognition was tested under adverse listening conditions, including background noise. Five BRE patients who were impaired on two or more tests had centrotemporal spiking on awake EEG. There were no significant group differences in the latency or amplitude of early N100 cortical responses to speech or tones. Conversely, the mismatch negativity, a preattentive index of cortical processing that is elicited passively, was absent or prolonged for speech, but not tones, in BRE patients as compared to controls. Discussion: Children with BRE demonstrated specific speech recognition impairments. Our evoked potential findings indicate that these behavioral impairments reflect dysfunction of nonprimary auditory cortex and cannot be attributed solely to attention difficulties. A possible association between auditory impairments and centrotemporal spiking (>1/min) on awake EEG was identified. The pattern of speech recognition impairments observed is a known risk factor for academic difficulties in school-age children. Our results underscore the importance of comprehensive auditory testing, using behavioral and electrophysiological measures, in children with BRE. [source] Spatio-temporal distribution of cellular retinoid binding protein gene transcripts in the developing and the adult cochlea.EUROPEAN JOURNAL OF NEUROSCIENCE, Issue 8 2000CRBPI-null mutant mice, Morphological, functional consequences in CRABP- Abstract The expression patterns of the mouse cellular retinoid binding protein genes were investigated by in situ hybridization analysis in the inner ear from 10.5 days post coďtum (dpc) up to the adult stage. The cellular retinoic acid binding protein II (CRABPII) and cellular retinol binding protein I (CRBPI) were present in a widespread and abundant pattern in cochlear structures during embryogenesis. Expression of the cellular retinoic acid binding protein I (CRABPI) is restricted during development in Kölliker's organ whilst cellular retinol binding protein II (CRBPII) is only visible after birth with a ubiquitous distribution in most regions of the cochlea including nervous components. No CRABP or CRBP transcripts were observed in the auditory receptors. Morphological observations of CRBPI- and CRABPI/CRABPII-null mutant fetus at 18.5 dpc do not show any structural modification at the level of the organ of Corti. Furthermore, electrophysiological tests performed by measuring distorsion-product otoacoustic emissions and auditory brainstem evoked responses did not present significant alteration of the auditory function for the different types of mutants. The expression of retinoid binding proteins in cochlear structures during embryogenesis could suggest important roles for these proteins during ontogenesis and morphogenesis of the inner ear. Despite these observations, morphological and functional data from mutant mice did not present obvious modifications of the cochlear structures and auditory thresholds. It is therefore unlikely that CRABPs and CRBPI are directly involved in development of the cochlea and hair cell differentiation. [source] Mild carbon monoxide exposure and auditory function in the developing ratJOURNAL OF NEUROSCIENCE RESEARCH, Issue 5 2003Janet E. Stockard-Sullivan Abstract We have examined the influence of chronic mild exposure to carbon monoxide (CO) on cognitive (learning) and auditory function in the developing rat. We have demonstrated that the auditory pathway is compromised at exposures less than 50 ppm, whereas learning was not influenced at 100 ppm. Artificially reared rat pups were exposed to CO during the brain growth spurt and onset of myelination. Spatial learning was assessed using the Morris Water Maze and three tests of auditory function: (1) auditory brainstem conduction times; (2) the amplitude of the eighth nerve's action potential; and (3) otoacoustic emissions carried out on rat pups (age 22, 24 days). The pups were gastrostomy-reared on a rat milk substitute and chronically exposed to CO at discrete concentrations in the range of 12,100 ppm from 6 days of age to post-weaning at 21,23 days of age. We found no difference in auditory brainstem conduction times at all CO concentrations in comparison to non-exposed controls. There was a difference in otoacoustic emissions for test and controls at CO concentrations of 50 ppm but not at lower concentrations. There was a consistent attenuation of the amplitude of the eighth nerve's action potential, even at the lowest CO exposure examined. The attenuation of the amplitude of the action potential of the eighth nerve at 50 ppm carbon monoxide exposure did not completely recover by 73 days of age. We conclude that prolonged mild exposure to carbon monoxide during development causes measurable functional changes at the level of the eighth cranial nerve. © 2003 Wiley-Liss, Inc. [source] Comparative gene expression analysis reveals a characteristic molecular profile of the superior olivary complexTHE ANATOMICAL RECORD : ADVANCES IN INTEGRATIVE ANATOMY AND EVOLUTIONARY BIOLOGY, Issue 4 2006Hans Gerd Nothwang Abstract The superior olivary complex (SOC) is a very conspicuous structure in the mammalian auditory brainstem. It represents the first binaural processing center and is important for sound localization in the azimuth and in feedback regulation of cochlear function. In order to define molecular determinants of the SOC, which are of potential functional relevance, we have performed a comprehensive analysis of its transcriptome by serial analysis of gene expression in adult rats. Here, we performed a detailed analysis of the SOC's gene expression profile compared to that of two other neural tissues, the striatum and the hippocampus, and with extraocular muscle tissue. This tested the hypothesis that SOC-specific or significantly upregulated transcripts provide candidates for the specific function of auditory neurons. Thirty-three genes were significantly upregulated in the SOC when compared to the two other neural tissues. Thirteen encoded proteins involved in neurotransmission, including action potential propagation, exocytosis, and myelination; five genes are important for the energy metabolism, and five transcripts are unknown or poorly characterized and have yet to be described in the nervous system. The comparison of functional gene classes indicates that the SOC has the highest energy demand of the three neural tissues, yet protein turnover is apparently not increased. This suggests a high energy demand for fueling auditory neurotransmission. Such a demand may have implications on auditory-specific tasks and relate to central auditory processing disorders. Ultimately, these data provide new avenues to foster investigations of auditory function and to advance molecular physiology in the central auditory system. Anat Rec Part A, 2006. © 2006 Wiley-Liss, Inc. [source] The Effect of CO2 and KTP laser on the cat saccule and utricleTHE LARYNGOSCOPE, Issue 8 2009Larry Lundy MD Abstract Objectives/Hypothesis: To assess the potential carbon dioxide (CO2) and potassium-titanyl-phosphate (KTP) laser-related trauma to the saccule and utricle in a cat model. Study Design: Basic science experiment utilizing cat model. Methods: Twelve adult male cats were divided into two groups,CO2 and KTP,to assess the potential saccule and/or utricle trauma with direct discharge of laser energy into the vestibule after the stapes was removed. Both groups were subdivided to assess the effects with acute sacrifice and three-month survival. Bone conduction auditory brain-stem response thresholds were used to monitor auditory function. Clinical observation was used to monitor vestibular function. The temporal bones were harvested, processed, and stained with hematoxylin and eosin (H&E) in all animals with the uninvolved side serving as the control. Results: None of the animals demonstrated changes in bone conduction auditory brain-stem responses. None of the animals in the survival group demonstrated clinical vestibular dysfunction. Saccular and utricular wall rupture was observed in all animals sacrificed acutely. None of the saccular and utricular wall ruptures were of a size and location that could be attributed to laser trauma, and none of the saccular and utricular wall ruptures were associated with neuroepithelial trauma. Conclusions: There is no evidence of a difference between the CO2 and KTP laser in potential laser-related trauma. Using bone-conducting auditory brain-stem response threshold and clinical monitoring of vestibular function, there was no evidence of clinical auditory or vestibular dysfunction. The histologic evidence of saccular and utricular wall rupture is more consistent with stapes extraction trauma than laser-related trauma. Laryngoscope, 2009 [source] Concentration Gradient Along the Scala Tympani After Local Application of Gentamicin to the Round Window Membrane,THE LARYNGOSCOPE, Issue 7 2007Stefan K. Plontke MD Abstract Objectives: The distribution of gentamicin along the fluid spaces of the cochlea after local applications has never previously been demonstrated. Computer simulations have predicted that significant basal-apical concentration gradients might be expected, and histologic studies indicate that hair cell damage is greater at the base than at the apex after local gentamicin application. In the present study, gradients of gentamicin along the cochlea were measured. Methods: A recently developed method of sampling perilymph from the cochlear apex of guinea pigs was used in which the samples represent fluid originating from different regions along the scala tympani. Gentamicin concentration was determined in sequential apical samples that were taken after up to 3 hours of local application to the round window niche. Results: Substantial gradients of gentamicin along the length of the scala tympani were demonstrated and quantified, averaging more than 4,000 times greater concentration at the base compared with the apex at the time of sampling. Peak concentrations and gradients for gentamicin varied considerably between animals, likely resulting from variations in round window membrane permeability and rates of perilymph flow. Conclusions: The large gradients for gentamicin demonstrated here in guinea pigs account for how it is possible to suppress vestibular function in some patients with a local application of gentamicin without damaging auditory function. Variations in round window membrane permeability and in perilymph flow could account for why hearing losses are observed in some patients. [source] Multichannel Cochlear Implants: Relation of Histopathology to Performance,THE LARYNGOSCOPE, Issue 8 2006Jose N. Fayad MD Abstract Objectives: To determine the relationship of surviving neural elements to auditory function in multichannel cochlear implant temporal bones. Study Design: Case series of all 14 existing multichannel cochlear implants in our temporal bone collection. Methods: Devices included Nucleus 22 (n = 11), Nucleus 24 (n = 1), Ineraid (n = 1), and Clarion (n = 1). Morphologic evaluation of structural elements including spiral ligament, stria vascularis, hair cells, peripheral processes, and spiral ganglion cells was performed. Clinical performance data were obtained from patient charts. For eight patients, nonimplanted contralateral temporal bones were available and paired comparisons were made. Results: Despite frequent absence of hair cells and peripheral processes, all bones had at least some remaining spiral ganglion cells. Percent of normal remaining structures were unrelated to auditory performance with the implant for any of the structural elements. Ganglion cell count in segment III showed significant negative correlations to speech discrimination scores for words and sentences (Rhos = ,.687 and ,.661, P , .03 and .04) as did segment IV and total ganglion cell count with word score (Rhos = ,.632 and ,.638; P , .05). Spiral ganglion cell survival did not differ between implanted and nonimplanted ears, with the exception of segment I, which had fewer cells in the implanted ear (P , .028). Conclusions: Performance variability of cochlear implants cannot be explained on the basis of cochlear neuronal survival. Although hair cells and peripheral processes were frequently absent or greatly diminished from normal, all subjects had at least some spiral ganglion cells. And, in this series, there was an inverse relationship between survival of ganglion cells and performance. [source] Nasal Provocation Testing as an International Standard for Evaluation of Allergic and Nonallergic RhinitisTHE LARYNGOSCOPE, Issue 3 2005Jan Gosepath MD Abstract Standardized nasal provocation testing (NPT) has been shown to be a safe and very useful tool in the diagnosis of allergic and nonallergic rhinitis. However, in the United States, its use has been mostly limited to scientific investigations, and it has not yet been widely accepted as a standard diagnostic procedure in clinical practice. NPT aims to identify and quantify the clinical relevance of inhalant allergens or occupational irritants. During NPT, nasal respiratory mucosa is exposed to an airborne substance suspected to cause symptoms in the respective individual. Clinical reactions are monitored in a controlled and standardized fashion. Nasal secretions, symptoms such as itching, sneezing and, most importantly, nasal obstruction are assessed as well as ocular, bronchial, cutaneous, and systemic reactions. To achieve objective data on changes in nasal airflow and patency after the challenge, anterior rhinomanometry and acoustic rhinometry have been included in the standard protocol of NPT. By monitoring changes of nasal airflow on one hand and of nasal geometry on the other hand, these methods display nasal function in a graphic way just as speech and pure tone audiometry do for auditory function. Also, by their objective nature, these methods offer a clear and internationally comparable standard. This review outlines a protocol for NPT and discusses practical applications and clinical indications. The use of rhinomanometry and acoustic rhinometry as objective diagnostic tools is emphasized. For the diagnosis of allergic and occupational rhinitis, standardized NPT should be regarded as an international diagnostic standard. [source] Central Effects of Residual Hearing: Implications for Choice of Ear for Cochlear Implantation,THE LARYNGOSCOPE, Issue 10 2004Howard W. Francis MD Abstract Objectives/Hypothesis: The study tested the hypothesis that among patients with similar levels of residual hearing in the nonimplanted ear, speech perception outcome is the same whether or not the implanted ear has profound or severe levels of hearing loss. Study Design: Retrospective. Methods: Levels of hearing loss in postlingually deafened adults who had cochlear implantation at Johns Hopkins University (Baltimore, MD) between 1991 and 2002 were classified according to pure-tone averages as bilateral severe (n = 20), severe-profound (severe in one ear and profound in the other) (n = 23), and bilateral profound (n = 43). There was no significant difference in the age at onset and duration of deafness among the three patient groups. Individuals in the bilateral severe and severe-profound groups had comparable levels of severe hearing loss in their nonimplanted ears, whereas those in severe-profound and bilateral profound groups had comparable levels of profound hearing loss in their implanted ears. Speech perception performance was evaluated using words from the Consonant Nucleus Consonant word list, Hearing in Noise Test sentences in quiet, and Central Institute for the Deaf sentences through recorded presentation at 70 dB sound pressure level (SPL). Results: Despite the profound hearing loss of the implanted ear in the asymmetrical group, there was no significant difference in mean speech perception scores compared with the bilateral severe group within the first year after implant surgery. By comparison, the bilateral profound group had lower speech perception results compared with patients with residual hearing in one or both ears. Conclusion: The study results suggest that implantation of the profoundly deafened ear does not diminish the functional advantage conferred by residual hearing in a patient with asymmetrical hearing loss. Therefore, the central auditory pathway may be the site at which persistent auditory function has its most beneficial effects. [source] Cochlear Implants in Five Cases of Auditory Neuropathy: Postoperative Findings and Progress,THE LARYNGOSCOPE, Issue 4 2001Jon K. Shallop PhD Abstract Objectives To review our experiences with some of the preoperative and postoperative findings in five children who were diagnosed with auditory neuropathy and were provided with cochlear implants. We describe changes in auditory function, which enabled these children to have significant improvement in their hearing and communication skills. Study Design Pre- and postoperatively, these children received complete medical examinations at Mayo Clinic, including related consultations in audiology, pediatrics, neurology, medical genetics, otolaryngology, psychology, speech pathology, and radiology. Methods These children typically had additional medical and audiological examinations at more than one medical center. The hearing assessments of these children included appropriate behavioral audiometric techniques, objective measures of middle ear function, acoustic reflex studies, transient (TOAE) or distortion product (DPOAE) otoacoustic emissions, auditory brainstem responses (ABR), and, in some cases, transtympanic electrocochleography (ECoG). After placement of the internal cochlear implant devices (Nucleus CI24), intraoperatively we measured electrode impedances, visually detected electrical stapedius reflexes (VESR) and neural response telemetry (NRT). These intraoperative objective measures were used to help program the speech processor for each child. Postoperatively, each child has had regular follow-up to assure complete healing of the surgical incision, to assess their general medical conditions, and for speech processor programming. Their hearing and communication skills have been assessed on a regular basis. Postoperatively, we have also repeated electrode impedance measurements, NRT measurements, otoacoustic emissions, and electrical auditory brainstem responses (EABR). We now have 1 year or more follow-up information on the five children. Results The five children implanted at Mayo Clinic Rochester have not had any postoperative medical or cochlear implant device complications. All of the children have shown significant improvements in their sound detection, speech perception abilities and communication skills. All of the children have shown evidence of good NRT results. All but case D (who was not tested) showed evidence of good postoperative EABR results. Otoacoustic emissions typically remained in the non-operated ear but, as expected, they are now absent in the operated ear. Conclusion Our experiences with cochlear implantation for children diagnosed with auditory neuropathy have been very positive. The five children we have implanted have not had any complications postoperatively, and each child has shown improved listening and communication skills that have enabled each child to take advantage of different communication and educational options. [source] Optical imaging of infants' neurocognitive development: Recent advances and perspectivesDEVELOPMENTAL NEUROBIOLOGY, Issue 6 2008Yasuyo Minagawa-Kawai Abstract Near-infrared spectroscopy (NIRS) provides a unique method of monitoring infant brain function by measuring the changes in the concentrations of oxygenated and deoxygenated hemoglobin. During the past 10 years, NIRS measurement of the developing brain has rapidly expanded. In this article, a brief discussion of the general principles of NIRS, including its technical advantages and limitations, is followed by a detailed review of the role played so far by NIRS in the study of infant perception and cognition, including language, and visual and auditory functions. Results have highlighted, in particular, the developmental changes of cerebral asymmetry associated with speech acquisition. Finally, suggestions for future studies of neurocognitive development using NIRS are presented. Although NIRS studies of the infant brain have yet to fulfill their potential, a review of the work done so far indicates that NIRS is likely to provide many unique insights in the field of developmental neuroscience. © 2008 Wiley Periodicals, Inc. Develop Neurobiol, 2008 [source] Modernizing times: UK hearing-impaired consumers at the policy crossroadsINTERNATIONAL JOURNAL OF CONSUMER STUDIES, Issue 2 2008Liz Ross Abstract Although there is now a long-standing belief in the UK that free consumer choice improves market efficiency, the supply of some consumer products and services remained controlled by the state. In the interests of consumers, it regards as vulnerable to misdirection and malpractice or unlikely to have the technical expertise to make informed decisions. Historically, the supply of hearings aids has been restricted to the National Health Service and specific licensed practitioners in the independent sector. Recent changes to both product and service provision have brought about a radical alteration to this situation, and to the framework of control. This case study of a changing healthcare system demonstrates more generally the difficulties experienced by people trying to improve or maintain auditory functions for speech communication. Access to appropriate technological solutions may be precluded by cost, distribution arrangements or lack of knowledge. Overarching these difficulties, regional health policy variations within the UK mean that consumer experiences vary according to where they live. Consumer influence over the direction and scope of changes to the hearing aid market is limited despite the rhetoric of choice. This article examines the emerging ,liberalized' market and its contradictions. [source] | |||||||||||||