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Reversible axonal swelling and brainstem auditory evoked potential (BAEP) changes were observed in standard chronic (9-month) toxicology studies in dogs treated with ritlecitinib, an oral Janus kinase 3/tyrosine kinase expressed in hepatocellular carcinoma family kinase inhibitor, at exposures higher than the approved 50-mg human dose. To evaluate the clinical relevance of the dog toxicity finding, this phase 2a, double-blind study assessed BAEP changes and intraepidermal nerve fiber (IENF) histology in adults with alopecia areata treated with ritlecitinib. Patients were randomized to receive oral ritlecitinib 50 mg once daily (QD) with a 4-week loading dose of 200 mg QD or placebo for 9 months (placebo-controlled phase); they then entered the active-therapy extension and received ritlecitinib 50 mg QD (with a 4-week loading dose of 200 mg in patients switching from placebo). Among the 71 patients, no notable mean differences in change from baseline (CFB) in Waves I–V interwave latency (primary outcome) or Wave V amplitude on BAEP at a stimulus intensity of 80 dB nHL were observed in the ritlecitinib or placebo group at Month 9, with no notable differences in interwave latency or Wave V amplitude between groups. The CFB in mean or median IENF density and in percentage of IENFs with axonal swellings was minimal and similar between groups at Month 9. Ritlecitinib treatment was also not associated with an imbalanced incidence of neurological and audiological adverse events. These results provide evidence that the BAEP and axonal swelling finding in dogs are not clinically relevant in humans.

IMPORTANCE: Age-related hearing loss is common in an aging population, affecting communication and contributing to a worsened quality of life. It occurs as a result of cochlear degeneration and may be further exacerbated by inflammation and microvascular changes, as observed in animal models. OBJECTIVE: To compare the effect of daily low-dose aspirin vs placebo on the progression of age-related hearing loss in healthy older adults. DESIGN, SETTING, AND PARTICIPANTS: A prespecified secondary analysis was conducted of the Aspirin in Reducing Events in the Elderly (ASPREE) randomized clinical trial. Participants were 279 healthy community-dwelling individuals living in Australia who were aged 70 years or older and free of overt cardiovascular diseases, dementia, and life-limiting illnesses. Participants were recruited between January 1, 2010, and December 31, 2014, and followed up over 3 years. Statistical analysis was completed from June to December 2023. INTERVENTION: A 100-mg daily dose of enteric-coated aspirin or matching placebo. MAIN OUTCOMES AND MEASURES: Hearing measures were air conduction audiometry and binaural speech perception in noise. Assessments were conducted at baseline, 18 months, and 3 years. The change from baseline hearing measures were analyzed using an intention to treat approach. Aspirin and placebo were compared using mixed linear regression models adjusting for age, sex, diabetes, and smoking. RESULTS: Of 279 participants, 154 (55%) were male, and the median age at baseline was 73.1 years (IQR, 71.5-76.2 years). A total of 98 of 138 participants (71%) in the aspirin group and 94 of 141 participants (67%) in the placebo group reported experiencing hearing loss at baseline. Compared with placebo, aspirin did not affect the changes in mean (SD) 4-frequency average hearing threshold from baseline to year 3 (aspirin: baseline, 27.8 [13.3] dB; year 3, 30.7 [13.7] dB; difference, 3.3 [3.9] dB; placebo: baseline, 27.5 [12.6] dB; year 3, 30.9 [13.8]

BACKGROUND: A high proportion of patients with neurofibromatosis type 1 (NF1) present with functional hearing deficiency as a result of neural abnormality in the late auditory brainstem. METHODS: In this randomized, two-period crossover study, we investigated the hypothesis that remote-microphone listening devices can ameliorate hearing and communication deficits in affected school-aged children (7-17 years). Speech perception ability in background noise was evaluated in device-active and inactive conditions using the CNC-word test. Participants were then randomized to one of two treatment sequences: (1) inactive device for two weeks (placebo), followed by active device use for two weeks, or (2) active device for 2 weeks, followed by inactive device for 2 weeks. Listening and communication ratings (LIFE-R Questionnaire) were obtained at baseline and at the end of each treatment phase. RESULTS: Each participant demonstrated functional hearing benefits with remote-microphone use. All showed a speech perception in noise increase when the device was activated with a mean phoneme-score difference of 16.4% (p < 0.001) and reported improved listening/communication abilities in the school classroom (mean difference: 23.4%; p = 0.017). DISCUSSION: Conventional hearing aids are typically ineffective as a treatment for auditory neural dysfunction, making sounds louder, but not clearer for affected individuals. In this study, we demonstrate that remote-microphone technologies are acceptable/tolerable in pediatric patients with NF1 and can ameliorate their hearing deficits. CONCLUSION: Remote-microphone listening systems offer a viable treatment option for children with auditory deficits associated with NF1.

IMPORTANCE: Neurofibromatosis type 1 (NF1) affects hearing through disruption of central auditory processing. The mechanisms, functional severity, and management implications are unclear. OBJECTIVE: To investigate auditory neural dysfunction and its perceptual consequences in individuals with NF1. DESIGN, SETTING, AND PARTICIPANTS: This case-control study included children and adults with NF1 and control participants matched on age, sex, and hearing level. Patients were recruited through specialist neurofibromatosis and neurogenetic outpatient clinics between April and September 2019. An evaluation of auditory neural activity, monaural/binaural processing, and functional hearing was conducted. Diffusion-weighted magnetic resonance imaging (MRI) data were collected from a subset of participants (10 children with NF1 and 10 matched control participants) and evaluated using a fixel-based analysis of apparent fiber density. MAIN OUTCOMES AND MEASURES: Type and severity of auditory dysfunction evaluated via laboratory testing and questionnaire data. RESULTS: A total of 44 participants (18 [41%] female individuals) with NF1 with a mean (SD) age of 16.9 (10.7) years and 44 control participants (18 [41%] female individuals) with a mean (SD) age of 17.2 (10.2) years were included in the study. Overall, 11 participants (25%) with NF1 presented with evidence of auditory neural dysfunction, including absent, delayed, or low amplitude electrophysiological responses from the auditory nerve and/or brainstem, compared with 1 participant (2%) in the control group (odds ratio [OR], 13.03; 95% CI, 1.59-106.95). Furthermore, 14 participants (32%) with NF1 showed clinically abnormal speech perception in background noise compared with 1 participant (2%) in the control group (OR, 20.07; 95% CI, 2.50-160.89). Analysis of diffusion-weighted MRI data of participants with NF1 showed significantly lower apparent fiber density within the ascending auditory brainstem pathways. The regions identi

BACKGROUND: Universal newborn hearing screening (UNHS) targets moderate or greater hearing loss. However, UNHS also frequently detects children with mild loss that results in many receiving early treatment. The benefits of this approach are not yet established. We aimed to (i) compare language and psychosocial outcomes between four hearing loss detection systems for children aged 5-8 years with congenital mild-moderate hearing loss; (ii) determine whether age of detection predicts outcomes; and (iii) compare outcomes between children identified via well-established UNHS and the general population. METHODS: Linear regression adjusted for potential confounding factors was used throughout. Via a quasi-experimental design, language and psychosocial outcomes were compared across four population-based Australian systems of hearing loss detection: opportunistic detection, born 1991-1993, n = 50; universal risk factor referral, born 2003-2005, n = 34; newly established UNHS, born 2003-2005, n = 41; and well-established UNHS, born 2007-2010, n = 21. In pooled analyses, we examined whether age of detection predicted outcomes. Outcomes were similarly compared between the current well-established UNHS system and typically developing children in the Early Language in Victoria Study, born 2003, n = 1217. RESULTS: Age at diagnosis and hearing aid fitting fell steadily across the four systems. For moderate losses, mean expressive language (P for trend .05) and receptive vocabulary (P for trend .06) improved across the four systems, but benefit was not obvious for mild losses. In pooled analyses, diagnosis before age six months predicted better language outcomes for moderate losses. Children with mild-moderate losses exposed to well-established UNHS continue to experience expressive language scores well below children in the general population (adjusted mean difference -8.9 points, 95% CI -14.7 to -3.1). CONCLUSIONS: Treatment arising from UNHS appears to be clearly benefitting chil

High levels of stress and anxiety are common in children with Autism Spectrum Disorder (ASD). Within this study of school-aged children (20 male, 6 female) we hypothesised that functional hearing deficits (also pervasive in ASD) could be ameliorated by auditory interventions and that, as a consequence, stress levels would be reduced. The use of Ear-Level Remote Microphone devices and Classroom Amplification systems resulted in significantly improved listening, communication and social interaction and a reduction in physiologic stress levels (salivary cortisol) in both one-on-one and group listening situations.

OBJECTIVE: To evaluate the auditory function of an individual with genetically confirmed hemochromatosis. METHODS: A 57 year old male with mildly impaired sound detection thresholds underwent a range of behavioural, electroacoustic and electrophysiologic assessments. These included the recording of otoacoustic emissions and auditory brainstem responses, measurement of monaural temporal resolution and evaluation of binaural speech processing. Findings for this patient were subsequently compared with those of 80 healthy controls with similar audiometric thresholds. RESULTS: The patient showed the three cardinal features of auditory neuropathy, presenting with evidence of normal cochlear outer hair cell function, disrupted neural activity in the auditory nerve/brainstem and impaired temporal processing. His functional hearing ability (speech perception) was significantly affected and suggested a reduced capacity to use localization cues to segregate signals in the presence of background noise. CONCLUSION: We present the first case of an individual with hemochromatosis and auditory neuropathy. The findings for this patient highlight the need for careful evaluation of auditory function in individuals with the disorder.

Big data appearing in health domain bring great opportunities for the health information system development. To effectively utilize the big health data, three challenges: data heterogeneity, huge data volume and high velocity of data generation, and various kinds of user requirements, need to be dealt with. To solve the problem, this paper proposes a cloud-based comprehensive health information system framework, which uses cloud computing techniques to manage and process the big health data, and provides several data analysis and recommendation services to explore the data and extract values from them.

© 2015. Background: Age-related hearing loss (ARHL) is a leading cause of disability in the elderly. Low-grade inflammation and microvessel pathology may be responsible for initiating or exacerbating some of the hearing loss associated with aging. A growing body of evidence demonstrates an association of hearing loss with cognitive decline. A shared etiological pathway may include a role of inflammation, alongside vascular determinants. The ASPREE-HEARING study aims to determine whether low-dose aspirin decreases the progression of ARHL, and if so, whether this decrease in progression is also associated with retinal microvascular changes and/or greater preservation of cognitive function. Design and methods: A three year double-blind, randomized controlled trial of oral 100. mg enteric-coated aspirin or matching placebo, enrolling 1262 Australians aged =. 70. years with normal cognitive function and no overt cardiovascular disease. The primary outcome is the change in mean pure tone average hearing threshold (decibels) in the better ear, over a 3-year period. Secondary outcomes consist of changes in retinal microvascular indicators, and changes in cognitive function. Participants are recruited from a larger trial, ASPirin in Reducing Events in the Elderly (ASPREE), which is designed to assess whether daily low dose aspirin will extend disability-free life. Discussion: ASPREE-HEARING will determine whether aspirin slows development or progression of ARHL, and will interrogate the relationship between inflammatory and microvascular mechanisms that may underlie the effects of aspirin on ARHL. This study will improve understanding of the patterns of comorbidity with, and the relationships between, aging and ARHL, alongside modeling the impacts of ARHL.

Friedreich ataxia (FRDA) is due to a triplet repeat expansion in FXN, resulting in deficiency of the mitochondrial protein frataxin. Resveratrol is a naturally occurring polyphenol, identified to increase frataxin expression in cellular and mouse models of FRDA and has anti-oxidant properties. This open-label, non-randomized trial evaluated the effect of two different doses of resveratrol on peripheral blood mononuclear cell (PBMC) frataxin levels over a 12-week period in individuals with FRDA. Secondary outcome measures included PMBC FXN mRNA, oxidative stress markers, and clinical measures of disease severity. Safety and tolerability were studied. Twenty-four participants completed the study; 12 received low-dose resveratrol (1 g daily) and 12 high-dose resveratrol (5 g daily). PBMC frataxin levels did not change in either dosage group [low-dose group change: 0.08 pg/mug protein (95 % CI -0.05, 0.21, p = 0.21); high-dose group change: 0.03 pg/mug protein (95 % CI -0.10, 0.15, p = 0.62)]. Improvement in neurologic function was evident in the high-dose group [change in Friedreich Ataxia Rating Scale -3.4 points, 95 % CI (-6.6, -0.3), p = 0.036], but not the low-dose group. Significant improvements in audiologic and speech measures, and in the oxidative stress marker plasma F2-isoprostane were demonstrated in the high-dose group only. There were no improvements in cardiac measures or patient-reported outcome measures. No serious adverse events were recorded. Gastrointestinal side-effects were a common, dose-related adverse event. This open-label study shows no effect of resveratrol on frataxin levels in FRDA, but suggests that independent positive clinical and biologic effects of high-dose resveratrol may exist. Further assessment of efficacy is warranted in a randomized placebo-controlled trial.Copyright © 2015, Springer-Verlag Berlin Heidelberg.

Friedreich ataxia (FRDA) is due to a triplet repeat expansion in FXN, resulting in deficiency of the mitochondrial protein frataxin. Resveratrol is a naturally occurring polyphenol, identified to increase frataxin expression in cellular and mouse models of FRDA and has anti-oxidant properties. This open-label, non-randomized trial evaluated the effect of two different doses of resveratrol on peripheral blood mononuclear cell (PBMC) frataxin levels over a 12-week period in individuals with FRDA. Secondary outcome measures included PMBC FXN mRNA, oxidative stress markers, and clinical measures of disease severity. Safety and tolerability were studied. Twenty-four participants completed the study; 12 received low-dose resveratrol (1 g daily) and 12 high-dose resveratrol (5 g daily). PBMC frataxin levels did not change in either dosage group [low-dose group change: 0.08 pg/mug protein (95 % CI -0.05, 0.21, p = 0.21); high-dose group change: 0.03 pg/mug protein (95 % CI -0.10, 0.15, p = 0.62)]. Improvement in neurologic function was evident in the high-dose group [change in Friedreich Ataxia Rating Scale -3.4 points, 95 % CI (-6.6, -0.3), p = 0.036], but not the low-dose group. Significant improvements in audiologic and speech measures, and in the oxidative stress marker plasma F2-isoprostane were demonstrated in the high-dose group only. There were no improvements in cardiac measures or patient-reported outcome measures. No serious adverse events were recorded. Gastrointestinal side-effects were a common, dose-related adverse event. This open-label study shows no effect of resveratrol on frataxin levels in FRDA, but suggests that independent positive clinical and biologic effects of high-dose resveratrol may exist. Further assessment of efficacy is warranted in a randomized placebo-controlled trial.Copyright © 2015, Springer-Verlag Berlin Heidelberg.

Thesis (M.C.P.)--Massachusetts Institute of Technology, Department of Urban Studies and Planning, 2013., Cataloged from PDF version of thesis., Includes bibliographical references (pages 132-138)., The social ecosystem approach explores the individuals and organizations that help effect a particular social outcome and the factors within their environment that contribute to or hinder their success. Applied to the particular housing and community development challenge facing 2-4 unit properties in the Chicago neighborhood of Woodlawn, the standard social ecosystem model is augmented with an explicit incorporation of a development process to emphasize the importance of the entrepreneurial, financial, technical and undeniably social aspects of real estate in revitalizing urban communities. The research sets out to explore the historical causes and processes that led to vacancy and market decline; the current condition of the market and neighborhood; lessons learned from previous efforts; and the parameters of action imposed by important stakeholder interests. This analysis reveals how population loss, speculation fueled by unrealized hopes of gentrification and accumulated property-level deliquencies combine to sustain high vacancies in Woodlawn. Recent declines in homeownership, depressed property values, a shortage of local amenities and structural barriers lowering investor margins also inhibit sustainable building uses in favor of landlords who "milk" properties and target Housing Choice Voucher Recipients. Due to the lack of social capital, the neighborhood struggles to control violence and maintain the public realm. The consequence is a diminished ability to attract prospective residents despite considerable local assets. The proposed response advances five strategic outcomes: stabilization of the market; advocacy for both better policy and internal structure; expansion of loans and financial assistance for homeowners; expansion of technical assistance and counseling for homeowners; and the prioritization of local development approaches that spread benefits more equitaby than is typical of gentrification. The associated recommendations, which broadly conside, by Rance G.D. Graham-Bailey., M.C.P.

info:eu-repo/semantics/published

info:eu-repo/semantics/published

Friedreich ataxia (FRDA), the most common hereditary ataxia, is due to a triplet repeat expansion in the FXN gene, resulting in deficiency of the mitochondrial protein frataxin. There is no treatment proven to alter its natural history. Resveratrol is a plant-derived polyphenol. It was identified to increase frataxin expression in cellular and mouse models of FRDA, and has anti-oxidant properties. This open-label sequential-dose clinical pilot trial evaluated the effect of two different doses of resveratrol on lymphocyte frataxin levels over a 12-week period in individuals with FRDA. Secondary aims included evaluating the effect of resveratrol on FXN mRNA, oxidative stress markers and clinical measures of disease severity. Safety and tolerability were also evaluated. 24 participants completed the study 12 received low-dose resveratrol (1 g daily) and 12 high-dose resveratrol (5 g daily). Therewas no significant change in lymphocyte frataxin levels in either dosage group [low dose group change: 0.08 pg/mug protein (95% CI -0.05, 0.21, p=0.21); high dose group change: 0.03 pg/mug protein (95% CI -0.10, 0.15, p=0.62)]. Lymphocyte mRNA levels also did not change significantly. Improvement in ataxia was evident in the high-dose group (change in International Cooperative Ataxia Rating Scale, ICARS -1.9 points, 95% CI -3.1, -0.8, p=0.004) but not the low-dose group (change in ICARS -0.3 points, 95% CI -3.2, 2.6, p=0.80). Significant improvements in hearing, measured as 'percentage phonemes correct' in a speech perception task was demonstrated in the high-dose group (improvement in phoneme score 4.6%, 95% CI 1.0, 8.2, p=0.02). Speech efficiency (reflected by a decreased mean pause length) was also significantly improved in the high dose group only. Echocardiographic parameters remained unchanged. A significant decrease in the oxidative stress marker plasma F2-isoprostanes occurred in the high-dose group (-216.9 pmol/L plasma, 95% CI -301.4, -132.2, p<0.001) but not the low

Friedreich ataxia (FRDA), the most common hereditary ataxia, is due to a triplet repeat expansion in the FXN gene, resulting in deficiency of the mitochondrial protein frataxin. There is no treatment proven to alter its natural history. Resveratrol is a plant-derived polyphenol. It was identified to increase frataxin expression in cellular and mouse models of FRDA, and has anti-oxidant properties. This open-label sequential-dose clinical pilot trial evaluated the effect of two different doses of resveratrol on lymphocyte frataxin levels over a 12-week period in individuals with FRDA. Secondary aims included evaluating the effect of resveratrol on FXN mRNA, oxidative stress markers and clinical measures of disease severity. Safety and tolerability were also evaluated. 24 participants completed the study 12 received low-dose resveratrol (1 g daily) and 12 high-dose resveratrol (5 g daily). Therewas no significant change in lymphocyte frataxin levels in either dosage group [low dose group change: 0.08 pg/mug protein (95% CI -0.05, 0.21, p=0.21); high dose group change: 0.03 pg/mug protein (95% CI -0.10, 0.15, p=0.62)]. Lymphocyte mRNA levels also did not change significantly. Improvement in ataxia was evident in the high-dose group (change in International Cooperative Ataxia Rating Scale, ICARS -1.9 points, 95% CI -3.1, -0.8, p=0.004) but not the low-dose group (change in ICARS -0.3 points, 95% CI -3.2, 2.6, p=0.80). Significant improvements in hearing, measured as 'percentage phonemes correct' in a speech perception task was demonstrated in the high-dose group (improvement in phoneme score 4.6%, 95% CI 1.0, 8.2, p=0.02). Speech efficiency (reflected by a decreased mean pause length) was also significantly improved in the high dose group only. Echocardiographic parameters remained unchanged. A significant decrease in the oxidative stress marker plasma F2-isoprostanes occurred in the high-dose group (-216.9 pmol/L plasma, 95% CI -301.4, -132.2, p<0.001) but not the low

Central auditory processing has been reported to be impaired in schizophrenia patients who experience auditory hallucinations, and interhemispheric transfer in auditory circuits may be compromised. In this study, we used EEG spectral coherence to examine interhemispheric connectivity between cortical areas known to be important in the processing of auditory information. Coherence was compared across three subject groups: schizophrenia patients with a recent history of auditory hallucinations (AH), schizophrenia patients who did not experience auditory hallucinations (nonAH), and healthy controls (HC). Subjects listened to pure tone and word stimuli while EEG was recorded continuously. Upper alpha and upper beta band coherence was calculated from six pairs of electrodes located over homologous auditory areas in the left and right cerebral hemispheres. Significant between-group differences were found on four electrode pairs (C3-C4, C5-C6, Ft7-Ft8 and Cp5-Cp6) in the upper alpha band. Relative to both the HC and nonAH groups, coherence was lower in the AH patients, consistent with the hypothesis that interhemispheric connectivity is reduced in these patients.

Objective: 1) To examine the distribution of behavioral hearing thresholds in a group of children who had shown no click-evoked auditory brain stem response (ABR) at maximum presentation levels. 2) To describe the relationship between the 90 Hz steady-state evoked potential (SSEP) and behavioral thresholds in these subjects. Design(s): A retrospective study based on clinical findings obtained from 108 infants and young children. Each of these children had shown no recordable ABR to clicks presented at maximum levels (100 dB nHL). SSEP audiograms were obtained using AM/FM tones at the octave frequencies 250 to 4000 Hz. The results of these evoked potential assessments were compared with hearing thresholds established behaviorally. Result(s): Click-ABR assessment could not differentiate between the subjects in our sample with total hearing losses and those with useful residual hearing. Although some of the ears were anacusic, more than a quarter showed residual hearing at each of the audiometric frequencies. Furthermore, at least 10% of the behavioral thresholds at each frequency fell within the moderate/severe hearing loss range. A far closer relationship was observed between SSEP and hearing thresholds. On occasions where the SSEP was absent at maximum levels, 99.5% of the ears showed either a total loss or a behavioral threshold within 10 dB of that level. When an SSEP was obtained, the hearing threshold was typically within 5 dB of the SSEP threshold. Conclusion(s): The results suggested that in our group of selected subjects, the SSEP technique was able to assess ears with only minimal amounts of residual hearing. Where the brevity of the acoustic click limits both its frequency specificity and its presentation level, the modulated tones used for SSEP testing allow accurate, frequency specific assessment at high presentation levels.

Successful early intervention in children with permanent hearing loss requires assessment techniques that can accurately reflect the behavioral audiogram in infancy. This retrospective study compared auditory steady-state response (ASSR) findings from subjects tested in the first three months of life with subsequently obtained behavioral hearing levels. ASSR audiograms were established using amplitude and frequency modulated tones at octave frequencies (500 Hz to 4 kHz). Results obtained from 575 subjects including 285 with normal hearing, 271 with sensorineural hearing loss, and 19 with auditory neuropathy-type hearing loss are presented. ASSR and behavioral hearing thresholds for subjects in the normal and sensorineural groups were highly correlated, with Pearson r values exceeding 0.95 at each of the test frequencies. In contrast, ASSR thresholds in children with AN-type hearing loss did not accurately reflect the behavioral audiogram. Overall, the findings indicate that ASSR testing can offer useful insights into the hearing acuity of children tested in infancy.

Objective: 1) To examine the distribution of behavioral hearing thresholds in a group of children who had shown no click-evoked auditory brain stem response (ABR) at maximum presentation levels. 2) To describe the relationship between the 90 Hz steady-state evoked potential (SSEP) and behavioral thresholds in these subjects. Design(s): A retrospective study based on clinical findings obtained from 108 infants and young children. Each of these children had shown no recordable ABR to clicks presented at maximum levels (100 dB nHL). SSEP audiograms were obtained using AM/FM tones at the octave frequencies 250 to 4000 Hz. The results of these evoked potential assessments were compared with hearing thresholds established behaviorally. Result(s): Click-ABR assessment could not differentiate between the subjects in our sample with total hearing losses and those with useful residual hearing. Although some of the ears were anacusic, more than a quarter showed residual hearing at each of the audiometric frequencies. Furthermore, at least 10% of the behavioral thresholds at each frequency fell within the moderate/severe hearing loss range. A far closer relationship was observed between SSEP and hearing thresholds. On occasions where the SSEP was absent at maximum levels, 99.5% of the ears showed either a total loss or a behavioral threshold within 10 dB of that level. When an SSEP was obtained, the hearing threshold was typically within 5 dB of the SSEP threshold. Conclusion(s): The results suggested that in our group of selected subjects, the SSEP technique was able to assess ears with only minimal amounts of residual hearing. Where the brevity of the acoustic click limits both its frequency specificity and its presentation level, the modulated tones used for SSEP testing allow accurate, frequency specific assessment at high presentation levels.

Successful early intervention in children with permanent hearing loss requires assessment techniques that can accurately reflect the behavioral audiogram in infancy. This retrospective study compared auditory steady-state response (ASSR) findings from subjects tested in the first three months of life with subsequently obtained behavioral hearing levels. ASSR audiograms were established using amplitude and frequency modulated tones at octave frequencies (500 Hz to 4 kHz). Results obtained from 575 subjects including 285 with normal hearing, 271 with sensorineural hearing loss, and 19 with auditory neuropathy-type hearing loss are presented. ASSR and behavioral hearing thresholds for subjects in the normal and sensorineural groups were highly correlated, with Pearson r values exceeding 0.95 at each of the test frequencies. In contrast, ASSR thresholds in children with AN-type hearing loss did not accurately reflect the behavioral audiogram. Overall, the findings indicate that ASSR testing can offer useful insights into the hearing acuity of children tested in infancy.

OBJECTIVE: Many reports have established that hearing-impaired children using the Nucleus 22-channel cochlear implant may show both significant benefits to lipreading and significant scores on open-set words and sentences using electrical stimulation only. These findings have raised questions about whether severely or severely-to-profoundly deaf children should be candidates for cochlear implants. To study this question, postoperative results for implanted children with different levels of preoperative residual hearing were evaluated in terms of speech perception benefits. STUDY DESIGN/SETTING: A retrospective study of the first 117 children, sequentially, to undergo implantation in the Melbourne and Sydney Cochlear Implant Clinics was undertaken. All children had been assessed by and received their implants in a tertiary referral centre. MAIN OUTCOME MEASURES: To assess aided residual hearing, the children were grouped into four categories of hearing on the basis of their aided residual hearing thresholds measured preoperatively. To assess benefits, the scores of children on standard speech perception tests were reviewed. As different tests were used for children with different ages and language skills, children were grouped into categories according to the level of postoperative speech perception benefit. RESULTS: The results showed that children in the higher categories of aided preoperative residual hearing showed significant scores on open-set word and sentence perception tests using the implant alone. For children in lower categories of aided residual hearing, results were variable within the groups. More than 90% of children with implants with aided residual hearing thresholds in the speech range above 1 kHz achieved open-set understanding of words and sentences. CONCLUSION: While the results of this preliminary study confirm previous findings of differential outcomes for children with different levels of preoperative residual hearing, they suggest that child