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1. Implantable Devices for Single-Sided Deafness and Conductive or Mixed Hearing Loss

Implantable Devices for Single-Sided Deafness and Conductive or Mixed Hearing Loss Published March 2020 Volume 20, Number 1 ONTARIO HEALTH TECHNOLOGY ASSESSMENT SERIES Implantable Devices for Single-Sided Deafness and Conductive or Mixed Hearing Loss: A Health Technology Assessment KEY MESSAGES What Is This Health Technology Assessment About? Single-sided deafness is profound sensorineural hearing loss (damage to the hearing organ or hearing nerve in the inner ear) or non-functional hearing (...) be. It also looked at the experiences, preferences, and values of people with single-sided deafness or conductive or mixed hearing loss. What Did This Health Technology Assessment Find? The best available evidence shows that cochlear and bone-conduction implants helped people with single-sided deafness or conductive or mixed hearing loss hear better and improved their hearing-specific quality of life. For people with single-sided deafness, cochlear implants may be cost-effective compared with no hearing

2020 Health Quality Ontario

2. The effect of hearing loss on the use of lexical categories by Hebrew-speaking mothers of deaf children with cochlear implants. (Abstract)

The effect of hearing loss on the use of lexical categories by Hebrew-speaking mothers of deaf children with cochlear implants. The frequency of use of nouns versus verbs in child-directed speech (CDS) of mothers to their normal hearing (NH) children has been investigated in various languages. Recent studies have shown that CDS to deaf children is affected by hearing loss. Thus, the main aim of the present study was to examine the effect of hearing loss on the use of content words by NH Hebrew (...) -speaking mothers to their deaf children using CIs. The second aim was to compare the use of content words by mothers speaking to CI children to that of NH children of the same chronological age and NH children with the same hearing experience.Three groups of mother-child dyads participated: Ten mothers of deaf children with bilateral CIs (CIs) (age range 20-48 months), ten mothers of NH children matched to the deaf children by their chronological age (NCA), and ten mothers of NH children matched

2020 International Journal of Pediatric Otorhinolaryngology

3. Development of a new Health Assembly resolution and action plan for prevention of deafness and hearing loss

Development of a new Health Assembly resolution and action plan for prevention of deafness and hearing loss Development of a new Health Assembly resolution and action plan for prevention of deafness and hearing loss JavaScript is disabled for your browser. Some features of this site may not work without it. Toggle navigation Toggle navigation Search Browse Statistics Related Links Development of a new Health Assembly resolution and action plan for prevention of deafness and hearing loss View (...) / Open View Statistics Altmetrics Share Citation Executive Board, 139 . (‎2016)‎. Development of a new Health Assembly resolution and action plan for prevention of deafness and hearing loss. World Health Organization. Gov't Doc # EB139.R1 Collections Language English Metadata View Item

2016 WHO

4. Imaging findings in pediatric single-sided deafness and asymmetric hearing loss. (Abstract)

Imaging findings in pediatric single-sided deafness and asymmetric hearing loss. To examine the imaging findings on computer tomography (CT) and magnetic resonance imaging (MRI) in pediatric single-sided deafness (SSD) and asymmetric hearing loss (ASH).The medical records of 189 pediatric patients with SSD and ASH were retrospectively reviewed, and imaging findings were compared. SSD was defined as unilateral profound hearing loss and contralateral normal hearing ear. In the ASH group, ASHw (...) was defined as the worse hearing ear with profound hearing loss, while ASHb was defined as the better hearing ear with mild-moderate hearing loss.There were 170 patients with SSD and 19 patients with ASH. In the SSD group, 83 patients (48.8%) had imaging findings associated with hearing loss. In the ASH group, such imaging findings were found in six (31.6%) of the ASHw and in five (26.3%) of the ASHb ears. The most common finding in the SSD group was cochlear nerve deficiency (50.6%), followed by cochlear

2019 Laryngoscope

5. Progressive Dominant Hearing Loss (Autosomal Dominant Deafness-41) and P2RX2 Gene Mutations: A Phenotype-Genotype Study. (Abstract)

Progressive Dominant Hearing Loss (Autosomal Dominant Deafness-41) and P2RX2 Gene Mutations: A Phenotype-Genotype Study. P2RX2 encoding P2X purinoreceptor 2 has been identified as the gene responsible for autosomal dominant deafness-41 (DFNA41) as well as mediating vulnerability to noise-induced hearing loss (NIHL). The objective of this study was to investigate the audiological and molecular characteristics of P2RX2-related deafness, with emphasis on its role in NIHL by determining (...) examinations of the family members were performed, and audiograms were obtained to assess the hearing thresholds. Clinical follow-up features in this DFNA41 family are presented along with correlation analyses of phenotype-genotype in all reported families with P2RX2-related deafness.Progressive hearing impairment was confirmed by history and by audiological follow-up testing in all the patients. The onset of hearing loss was between age 25 and 35 years. All affected subjects had bilateral sensorineural

2019 Laryngoscope

6. Cochlear implantation as a treatment for single-sided deafness and asymmetric hearing loss: a randomized controlled evaluation of cost-utility. Full Text available with Trip Pro

Cochlear implantation as a treatment for single-sided deafness and asymmetric hearing loss: a randomized controlled evaluation of cost-utility. Single-sided deafness (SSD) and asymmetric hearing loss (AHL) have recently been proposed as a new indication for cochlear implantation. There is still no recommended treatment for these hearing deficits, and most options considered rely on the transfer of sound from the poor ear to the better ear, using Contralateral Routing of the Signal (CROS (...) ) hearing aids or bone conduction (BC) devices. In contrast, cochlear implantation allows the poor ear to be stimulated and binaural hearing abilities to be partially restored. Indeed, most recently published studies have reported an improvement in the spatial localisation of an incoming sound and better speech recognition in noisy environments after cochlear implantation in SSD/AHL subjects. It also provides consistent relief of tinnitus when associated. These encouraging hearing outcomes raise

2019 BMC Ear, Nose and Throat Disorders Controlled trial quality: uncertain

7. Mutation analysis of common deafness-causing genes among 506 patients with nonsyndromic hearing loss from Wenzhou city, China. (Abstract)

Mutation analysis of common deafness-causing genes among 506 patients with nonsyndromic hearing loss from Wenzhou city, China. The frequency and spectrum of mutations in deafness-causing genes differs significantly according to the ethnic population and region under investigation. The molecular etiology of nonsyndromic hearing loss (NSHL) in Wenzhou, China, has not yet been systematically elucidated. To provide accurate genetic testing and counseling in this area, we investigated the molecular (...) etiology of NSHL in a deaf population from Wenzhou.A total 506 unrelated patients with NSHL were enrolled in this study. Nine hotspot mutations in four major deafness genes were investigated by sequencing (Group I: 187 patients enrolled between 2011 and 2015) or allele-specific PCR-based universal array (Group II: 319 patients enrolled between 2016 and 2017). The investigated genes included GJB2 (c.35delG, c.176_191del16, c.235delC, c.299-300delAT), SLC26A4 (c.2168A > G, c.919-2A > G), mtDNA 12SrRNA (m

2019 International Journal of Pediatric Otorhinolaryngology

8. The association between auditory nerve neurovascular conflict and sudden unilateral sensorineural hearing loss Full Text available with Trip Pro

The association between auditory nerve neurovascular conflict and sudden unilateral sensorineural hearing loss There may be an association between a neurovascular conflict (NVC) of the auditory nerve and unilateral sudden sensorineural hearing loss (SSNHL).Compression of cranial nerves by vascular structures can lead to significant symptomatology that may require surgical decompression. Notable examples are trigeminal neuralgia and hemifacial spasm. Magnetic resonance imaging (MRI) is part (...) of the workup for SSNHL, and it may depict an NVC of the auditory nerve. Here we look into the association between this NVC and unilateral SSNHL.A retrospective analysis was performed on all consecutive patients with unilateral SSNHL who underwent an MRI scan in our medical center. The data collected included age, gender, side and severity of hearing loss, and accompanying complaints. Each MRI scan was reviewed by a neuroradiologist who was unaware of hearing loss laterality. The presence, side, extent

2018 Laryngoscope investigative otolaryngology

9. Cochlear implants for children and adults with severe to profound deafness

from the outer to the inner ear (conductive hearing loss) or damage within the cochlea, the auditory nerve or auditory centres in the brain (sensorineural hearing loss). In adults the most common cause of sensorineural hearing loss is presbycusis. This is a progressive condition caused by the loss of function of hair cells in the inner ear, leading to deafness. Hearing loss in adults may also be caused by excessive exposure to noise, or by ototoxic drugs, metabolic disorders, infections or genetic (...) in a language in which a person is sufficiently fluent for the tests to be appropriate, other methods of assessment should be considered. [2009] [2009] Cochlear implants for children and adults with severe to profound deafness (TA566) © NICE 2019. All rights reserved. Subject to Notice of rights (https://www.nice.org.uk/terms-and- conditions#notice-of-rights). Page 5 of 302 2 Clinical need and pr Clinical need and practice actice 2.1 Hearing loss may be caused by interference with the transmission of sound

2019 National Institute for Health and Clinical Excellence - Technology Appraisals

10. Evaluation of Speed and Accuracy of Next-Generation Auditory Steady State Response and Auditory Brainstem Response Audiometry in Children With Normal Hearing and Hearing Loss. (Abstract)

Evaluation of Speed and Accuracy of Next-Generation Auditory Steady State Response and Auditory Brainstem Response Audiometry in Children With Normal Hearing and Hearing Loss. The first objective of this study was to compare the predicted audiometric thresholds obtained by auditory steady state response (ASSR) and auditory brainstem response (ABR) in infants and toddlers when both techniques use optimal stimuli and detection algorithms. This information will aid in determining the basis (...) for ASSR than ABR. The ABR-ASSR discrepancy at 500 Hz was 14.39 dB, at 1000 Hz was 10.12 dB, at 2000 Hz was 3.73 dB, and at 4000 Hz was 3.67 dB. The average test time for ASSR of 19.93 min (for 8 thresholds) was found to be significantly lower (p < 0.001) than the ABR test time of 32.15 min. One half of the subjects were found to have normal hearing. ASSR thresholds plotted in dB nHL for normal-hearing children in this study were found to be the lowest yet described except for one study which used

2018 Ear and hearing

11. Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function Full Text available with Trip Pro

Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function Mouse Tmc1 and Tmc2 are required for sensory transduction in cochlear and vestibular hair cells. Homozygous Tmc1∆/∆ mice are deaf, Tmc2∆/∆ mice have normal hearing, and double homozygous Tmc1∆/∆; Tmc2∆/∆ mice have deafness and profound vestibular dysfunction. These phenotypes are consistent with their different spatiotemporal expression patterns. Tmc1 expression (...) ] transgene slightly but significantly restored hearing in young Tmc1∆/∆ mice, though hearing thresholds were elevated with age. The elevation of hearing thresholds was associated with deterioration of sensory transduction in inner hair cells and loss of outer hair cell function. Although sensory transduction was retained in outer hair cells, their stereocilia eventually degenerated. These results indicate distinct roles and requirements for Tmc1 and Tmc2 in mature cochlear hair cells.

2018 Scientific reports

12. Effects of Directional Microphone and Noise Reduction on Subcortical and Cortical Auditory-Evoked Potentials in Older Listeners With Hearing Loss. (Abstract)

Effects of Directional Microphone and Noise Reduction on Subcortical and Cortical Auditory-Evoked Potentials in Older Listeners With Hearing Loss. Understanding how signal processing influences neural activity in the brain with hearing loss is relevant to the design and evaluation of features intended to alleviate speech-in-noise deficits faced by many hearing aid wearers. Here, we examine whether hearing aid processing schemes that are designed to improve speech-in-noise intelligibility (i.e (...) ., directional microphone and noise reduction) also improve electrophysiological indices of speech processing in older listeners with hearing loss.The study followed a double-blind within-subjects design. A sample of 19 older adults (8 females; mean age = 73.6 years, range = 56-86 years; 17 experienced hearing aid users) with a moderate to severe sensorineural hearing impairment participated in the experiment. Auditory-evoked potentials associated with processing in cortex (P1-N1-P2) and subcortex (frequency

2020 Ear and hearing

13. Effects of auditory and socio-demographic variables on discontinuation of hearing aid use among older adults with hearing loss fitted in the Chilean public health sector. Full Text available with Trip Pro

Effects of auditory and socio-demographic variables on discontinuation of hearing aid use among older adults with hearing loss fitted in the Chilean public health sector. The percentage of older adults with hearing loss who stop using their hearing aids and the variables associated with this phenomenon have not been systematically investigated in South America. This problem is relevant to the region since countries such as Colombia, Brazil and Chile have public programmes that provide hearing (...) aids to older adults. The aims of this study were to determine the percentage of older adults fitted with a hearing aid at a public hospital in Chile who subsequently stop using it and the auditory and socio-demographic variables associated with the hazard of discontinuing hearing aid use.A group that included 355 older adults who had been fitted with a hearing aid was studied retrospectively. In a structured interview, participants were asked about socio-demographic variables and answered part

2019 BMC Geriatrics

14. A Comparison of Word-Recognition Performances on the Auditec and VA Recorded Versions of Northwestern University Auditory Test No. 6 by Young Listeners with Normal Hearing and by Older Listeners with Sensorineural Hearing Loss Using a Randomized Presentat (Abstract)

A Comparison of Word-Recognition Performances on the Auditec and VA Recorded Versions of Northwestern University Auditory Test No. 6 by Young Listeners with Normal Hearing and by Older Listeners with Sensorineural Hearing Loss Using a Randomized Presentat The Auditec of St. Louis and the Department of Veterans Affairs (VA) recorded versions of the Northwestern University Auditory Test No. 6 (NU-6) are in common usage. Data on young adults with normal hearing for pure tones (YNH) demonstrate (...) equal recognition performances on the two versions when the VA version is presented 5 dB higher but similar data on older listeners with sensorineural hearing loss (OHL) are lacking.To compare word-recognition performances on the Auditec and VA versions of NU-6 presented at six presentation levels with YNH and OHL listeners.A quasi-experimental, repeated-measures design was used.Twelve YNH (M = 24.0 years; PTA = 9.9-dB HL) and 36 OHL listeners (M = 71.6 years; PTA = 26.7-dB HL) participated in three

2019 Journal of the American Academy of Audiology Controlled trial quality: uncertain

15. The cognitive and psychosocial effects of auditory training and hearing aids in adults with hearing loss. Full Text available with Trip Pro

The cognitive and psychosocial effects of auditory training and hearing aids in adults with hearing loss. Our study assessed the efficacy of the simultaneous use of hearing aids and auditory training for improving cognition and psychosocial function in adults with hearing loss, and the relationships between hearing loss, speech perception and cognition.A 40-person (aged 50-90 years) pilot study in Melbourne, Australia, was conducted. Participants with hearing impairment completed the Geriatric (...) Depression Scale-Short Form, questions about social activity participation, a wide range of cognitive tasks and a speech perception test at baseline, 3 and 6 months. Participants underwent auditory training for 6 months and used hearing aids for 3 months.Correlations and structural equation modeling suggested that several cognitive domains were associated with speech perception at baseline, but only the Incongruent Stroop cognition measure was associated with hearing loss. Hearing aid use reduced

2019 Clinical interventions in aging

16. Auditory Evoked Responses in Older Adults With Normal Hearing, Untreated, and Treated Age-Related Hearing Loss. (Abstract)

Auditory Evoked Responses in Older Adults With Normal Hearing, Untreated, and Treated Age-Related Hearing Loss. The goal of this study was to identify the effects of auditory deprivation (age-related hearing loss) and auditory stimulation (history of hearing aid use) on the neural registration of sound across two stimulus presentation conditions: (1) equal sound pressure level and (2) equal sensation level.We used a between-groups design, involving three groups of 14 older adults (n = 42; 62 (...) to 84 years): (1) clinically defined normal hearing (≤25 dB from 250 to 8000 Hz, bilaterally), (2) bilateral mild-moderate/moderately severe sensorineural hearing loss who have never used hearing aids, and (3) bilateral mild-moderate/moderately severe sensorineural hearing loss who have worn bilateral hearing aids for at least the past 2 years.There were significant delays in the auditory P1-N1-P2 complex in older adults with hearing loss compared with their normal hearing peers when using equal

2019 Ear and hearing

17. A novel mutation of the EYA4 gene associated with post-lingual hearing loss in a proband is co-segregating with a novel PAX3 mutation in two congenitally deaf family members. (Abstract)

A novel mutation of the EYA4 gene associated with post-lingual hearing loss in a proband is co-segregating with a novel PAX3 mutation in two congenitally deaf family members. This work was aimed at establishing the molecular etiology of hearing loss in a 9-year old girl with post-lingual non-syndromic mild sensorineural hearing loss with a complex family history of clinically heterogeneous deafness.The proband's DNA was subjected to NGS analysis of a 59-targeted gene panel, with the use (...) phenotype was masked by Waardenburg syndrome. The use of NGS targeted gene-panel, in combination with an extensive clinical and audiological examination led us to identify the genetic cause of the hearing loss in members of a family in which different forms of autosomal dominant deafness segregate. These results provide precise and especially important prognostic and follow-up information for the future audiologic management in the youngest affected member.Copyright © 2017 Elsevier B.V. All rights

2018 International Journal of Pediatric Otorhinolaryngology

18. Single-Sided Deafness and cochlear implantation in congenital and acquired hearing loss in children. (Abstract)

Single-Sided Deafness and cochlear implantation in congenital and acquired hearing loss in children. To determine the audiological and clinical results of cochlear implantation in children below the age of 12 years old with congenital and acquired single-sided deafness.Observational, descriptive, transversal study.Speech reception thresholds, Cortical responses, Auditory Lateralization Test and SSQ questionnaire.Children < 12 implanted for congenital or acquired SSD.All the children (...) with congenital SSD showed positive cortical responses. Positive results were obtained in the Auditory Lateralization Test for the following modalities: 0º, 45º and 90º. With respect to the Speech Test, the children with acquired SSD showed the following results: 92% and 100% in recognition and 48% and 68% (Azimuth modalities), Signal CI side 52% and 68% and Signal normal hearing side 44% - 60% (p < 0.05). In both group the processor was used for 6-12 hours. With respect to the SSQ questionnaire results

2018 Clinical Otolaryngology

19. Whole exome sequencing in adult-onset hearing loss reveals a high load of predicted pathogenic variants in known deafness-associated genes and identifies new candidate genes Full Text available with Trip Pro

Whole exome sequencing in adult-onset hearing loss reveals a high load of predicted pathogenic variants in known deafness-associated genes and identifies new candidate genes Deafness is a highly heterogenous disorder with over 100 genes known to underlie human non-syndromic hearing impairment. However, many more remain undiscovered, particularly those involved in the most common form of deafness: adult-onset progressive hearing loss. Despite several genome-wide association studies of adult (...) purposes. We conclude that there may be a high number of pathogenic variants affecting hearing in the ageing population, including many in known deafness-associated genes. Our findings of frequent predicted-pathogenic variants in both our hearing-impaired sample and in the larger 1000 Genomes Project sample unselected for auditory function suggests that the reference population for interpreting variants for this very common disorder should be a population of people with good hearing for their age

2018 BMC medical genomics

20. Hearing children of Deaf parents: Gender and birth order in the delegation of the interpreter role in culturally Deaf families Full Text available with Trip Pro

Hearing children of Deaf parents: Gender and birth order in the delegation of the interpreter role in culturally Deaf families Culturally, hearing children born to Deaf parents may have to mediate two different positions within the hearing and Deaf cultures. However, there appears to be little written about the experiences of hearing children born to Deaf parents in the South African context.This study sought to investigate the roles of children of Deaf adults (CODAs) as interpreters in Deaf (...) of interpreting services for Deaf people in South Africa in the form of professional interpreters rather than the reliance on hearing children as interpreters in order to mediate between Deaf and hearing cultures.

2018 African journal of disability

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