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Annual Review of Neuroscience Jul 2019The modern cochlear implant (CI) is the most successful neural prosthesis developed to date. CIs provide hearing to the profoundly hearing impaired and allow the... (Review)
Review
The modern cochlear implant (CI) is the most successful neural prosthesis developed to date. CIs provide hearing to the profoundly hearing impaired and allow the acquisition of spoken language in children born deaf. Results from studies enabled by the CI have provided new insights into () minimal representations at the periphery for speech reception, () brain mechanisms for decoding speech presented in quiet and in acoustically adverse conditions, () the developmental neuroscience of language and hearing, and () the mechanisms and time courses of intramodal and cross-modal plasticity. Additionally, the results have underscored the interconnectedness of brain functions and the importance of top-down processes in perception and learning. The findings are described in this review with emphasis on the developing brain and the acquisition of hearing and spoken language.
Topics: Animals; Auditory Perception; Auditory Perceptual Disorders; Brain; Cochlear Implantation; Cochlear Implants; Comprehension; Critical Period, Psychological; Cues; Deafness; Equipment Design; Humans; Language Development; Language Development Disorders; Learning; Neuronal Plasticity; Photic Stimulation
PubMed: 30699049
DOI: 10.1146/annurev-neuro-080317-061513 -
American Annals of the Deaf 2021The authors employed a scoping review to examine peer-reviewed journal articles published 2002-2020 focusing on adolescents who are deaf or hard of hearing and their... (Review)
Review
The authors employed a scoping review to examine peer-reviewed journal articles published 2002-2020 focusing on adolescents who are deaf or hard of hearing and their self-reported identities. d/Deaf identity theory was explored in light of recent advances in developed countries, e.g., universal newborn hearing screening, increased access to mainstream school placements, and rising rates of cochlear implantation. Key themes were explored, including deafness acculturation, competing and complementary identities, and flexibility and fluidity. The results demonstrated that the way adolescents perceive their identity is evolving, in what is often a flexible and fluid process dependent on the adolescents' context. Other identities, such as those relating to ethnicity and culture, were often seen as equal in importance to one's d/Deaf identity. These findings are contrasted with those of wider research on d/Deaf identity, and further research investigating adolescents' perceptions of themselves is recommended.
Topics: Adolescent; Cochlear Implantation; Deafness; Hearing; Hearing Loss; Humans; Infant, Newborn; Persons With Hearing Impairments
PubMed: 34053942
DOI: 10.1353/aad.2021.0008 -
Advances in Experimental Medicine and... 2019It is estimated that at least 50% of congenital or childhood hearing loss is attributable to genetic causes. In non-syndromic hearing loss, which accounts for 70% of... (Review)
Review
It is estimated that at least 50% of congenital or childhood hearing loss is attributable to genetic causes. In non-syndromic hearing loss, which accounts for 70% of genetic hearing loss, approximately 80% of cases are autosomal recessive, 15% autosomal dominant, and 1-2% mitochondrial or X-linked. In addition, 30% of genetic hearing loss is syndromic. The genetic causes of hearing loss are highly heterogeneous. So far, more than 140 deafness-related genes have been discovered. Studies on those genes tremendously increased our understanding of the inner ear functions at the molecular level. It also offers important information for the patients and allows personalized and accurate genetic counseling. In many cases, genetic diagnosis of hearing loss can help to avoid unnecessary and costly clinical testing, offer prognostic information, and guide future medical management. On the other hand, a variety of gene therapeutic approaches have been developed aiming to relieve or converse the hearing loss due to genetic causes. Prevention of genetic hearing loss is feasible through prepregnancy and prenatal genetic diagnosis and counseling.
Topics: Deafness; Ear, Inner; Female; Genetic Counseling; Hearing Loss; Humans; Pregnancy; Prenatal Diagnosis
PubMed: 30915702
DOI: 10.1007/978-981-13-6123-4_5 -
International Journal of Pediatric... Apr 2022To examine the results of health-related quality of life (HRQoL) questionnaire scores from deaf children fitted with at least one cochlear implant (CI) and compare...
OBJECTIVES
To examine the results of health-related quality of life (HRQoL) questionnaire scores from deaf children fitted with at least one cochlear implant (CI) and compare responses to normal-hearing age-peers (NHP) and to their parents.
METHODS
This cross-sectional study included 33 pediatric patients with a hearing experience of at least 1 year with CI and a control group of 21 NHP. The KINDL questionnaire for measuring HRQoL (generic) in children (7-12years) and adolescents (13-17years) and a CI questionnaire (specific) were completed by CI users and their parents. Scores were transformed to a 100-point scale with 100 representing the most positive response.
RESULTS
The group of children and adolescents with CI reached a similar score in the generic HRQoL (Children:78,6; Adolescents:80,4; p = 0,35) and in the overall CI questionnaire (Children:77,6; Adolescents:75,6; p = 0,53). CI users in both age groups scored generic HRQoL similarly to their NHP (NH children:82,2, p = 0,18; NH adolescents:77,5; p = 0,07) and higher than CI users from other centers (OC) (OC children:75,4; OC adolescents:70,3). CI users scored their school domain lower than their NHP (p = 0,04). Generic and CI questionnaire scores of parents and their children correlated positively (r = 0,66, p = 0,00 and r = 0,73, p = 0,00). The total scores of HRQoL in the self-rating and parent rating correlate with none of the variables at study (gender, cause of deafness, age at implantation, years with a CI, chronological age) except with speech progression (p = 0,007).
CONCLUSIONS
Children with CI experience similar quality of life as NHP. Parents are reliable reporters on the status of their child's overall quality of life.
Topics: Adolescent; Child; Cochlear Implantation; Cochlear Implants; Cross-Sectional Studies; Deafness; Humans; Quality of Life; Surveys and Questionnaires
PubMed: 35202900
DOI: 10.1016/j.ijporl.2022.111087 -
American Annals of the Deaf 2016The authors address considerations and promising practices relating to assessment of d/Deaf and Hard of Hearing Multilingual Learners. DMLs' unique culture(s),... (Review)
Review
The authors address considerations and promising practices relating to assessment of d/Deaf and Hard of Hearing Multilingual Learners. DMLs' unique culture(s), language(s), and learning needs must be considered when assessments of this population are being planned, conducted, and interpreted. The authors address theory and research on (a) general considerations for the overarching assessment process, (b) specific assessment approaches used to assess DMLs, and (c) assessment of language proficiency for diverse language learners. In addition, basic recommendations for the assessment of DMLs are made, including increased availability of assessments in various languages, use of multiple sources of individual and family data, assessment of all languages, and incorporation of a strong assessment component (that includes nondiscrimination practices) into teacher preparation programs.
Topics: Adolescent; Adolescent Development; Child; Child Development; Cultural Diversity; Curriculum; Deafness; Education of Hearing Disabled; Educational Measurement; Educational Status; Humans; Learning; Multilingualism; Persons With Hearing Impairments; Sign Language; Teaching; Terminology as Topic
PubMed: 27156918
DOI: 10.1353/aad.2016.0011 -
Anais Brasileiros de Dermatologia 2015Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical...
Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness.
Topics: Child; Deafness; Early Diagnosis; Female; Humans; Phenotype; Waardenburg Syndrome
PubMed: 26375234
DOI: 10.1590/abd1806-4841.20153343 -
International Journal of... Feb 2015Cortical development is dependent on extrinsic stimulation. As such, sensory deprivation, as in congenital deafness, can dramatically alter functional connectivity and... (Review)
Review
Cortical development is dependent on extrinsic stimulation. As such, sensory deprivation, as in congenital deafness, can dramatically alter functional connectivity and growth in the auditory system. Cochlear implants ameliorate deprivation-induced delays in maturation by directly stimulating the central nervous system, and thereby restoring auditory input. The scenario in which hearing is lost due to deafness and then reestablished via a cochlear implant provides a window into the development of the central auditory system. Converging evidence from electrophysiologic and brain imaging studies of deaf animals and children fitted with cochlear implants has allowed us to elucidate the details of the time course for auditory cortical maturation under conditions of deprivation. Here, we review how the P1 cortical auditory evoked potential (CAEP) provides useful insight into sensitive period cut-offs for development of the primary auditory cortex in deaf children fitted with cochlear implants. Additionally, we present new data on similar sensitive period dynamics in higher-order auditory cortices, as measured by the N1 CAEP in cochlear implant recipients. Furthermore, cortical re-organization, secondary to sensory deprivation, may take the form of compensatory cross-modal plasticity. We provide new case-study evidence that cross-modal re-organization, in which intact sensory modalities (i.e., vision and somatosensation) recruit cortical regions associated with deficient sensory modalities (i.e., auditory) in cochlear implanted children may influence their behavioral outcomes with the implant. Improvements in our understanding of developmental neuroplasticity in the auditory system should lead to harnessing central auditory plasticity for superior clinical technique.
Topics: Auditory Cortex; Child; Cochlear Implants; Deafness; Evoked Potentials, Auditory; Humans; Models, Biological; Neuronal Plasticity
PubMed: 24780192
DOI: 10.1016/j.ijpsycho.2014.04.007 -
Bulletin of the World Health... Oct 2019The lack of an appropriately trained global hearing-care workforce is recognized as a barrier to developing and implementing services to treat ear and hearing disorders....
The lack of an appropriately trained global hearing-care workforce is recognized as a barrier to developing and implementing services to treat ear and hearing disorders. In this article we examine some of the published literature on the current global workforce for ear and hearing care. We outline the status of both the primary-care workforce, including community health workers, and specialist services, including audiologists, ear, nose and throat specialists, speech and language therapists, and teachers of the deaf. We discuss models of training health workers in ear and hearing care, including the role of task-sharing and the challenges of training in low and middle-income countries. We structure the article by the components of ear and hearing care that may be delivered in isolation or in integrated models of care: primary care assessment and intervention; screening; hearing tests; hearing rehabilitation; middle-ear surgery; deaf services; and cochlear implant programmes. We highlight important knowledge gaps and areas for future research and reporting.
Topics: Community Health Workers; Deafness; Health Personnel; Hearing; Hearing Loss; Hearing Tests; Humans; Primary Health Care
PubMed: 31656334
DOI: 10.2471/BLT.18.224659 -
Journal of Deaf Studies and Deaf... Apr 2020Navigating a phonocentric and audistic society can be challenging for Deaf people, yet some of them adapt and exhibit resilience. Although there is a plethora of... (Review)
Review
Navigating a phonocentric and audistic society can be challenging for Deaf people, yet some of them adapt and exhibit resilience. Although there is a plethora of resilience studies, Deaf people's narratives are largely unexplored. This phenomenological study explored from a Deaf cultural-linguistic lens 10 Deaf professionals who use American Sign Language in the United States and their observation of other Deaf people's lived experience. The study focused particularly on their experiences in overcoming significant barriers in their lives and the factors that strengthen Deaf people's resilience. Based on qualitative data gathered from narrative interviews, three main themes emerged from this study: (a) Barriers to Incidental Learning: The Dinner Table Syndrome; (b) Importance of Family Involvement; and (c) Protective Role of the Deaf Community as a Second Family. The stories in this study highlight how Deaf professionals and other Deaf people navigate barriers and manifest resilience.
Topics: Communication; Deafness; Humans; Persons With Hearing Impairments; Sign Language
PubMed: 32091592
DOI: 10.1093/deafed/enz045 -
Audiology & Neuro-otology 2023Hearing loss is known to play a fundamental role in voice production due to a lack of auditory feedback. In this study, we evaluated both fundamental frequency (F0) and...
INTRODUCTION
Hearing loss is known to play a fundamental role in voice production due to a lack of auditory feedback. In this study, we evaluated both fundamental frequency (F0) and loudness of voice on adult deaf patients subjected to cochlear implantation, and we analyzed these results according to the prelingual or postlingual onset of the deafness.
METHODS
The study population, balanced in terms of sex, consisted of 32 adults who had undergone cochlear implantation due to severe or profound bilateral hearing loss (16 with prelingual deafness and 16 with postlingual deafness) and their outcomes were compared with a control group of 32 normal hearing (NH) subjects. All subjects were asked to utter the sustained vowel /a/ for at least 5 s and then to read an Italian phonetically balanced text. Voice recordings were performed by means of an ambulatory phonation monitoring (APM 3200). Measurements were performed without cochlear implant (CI), then with CI switched on, both in quiet condition and with background noise.
RESULTS
Compared to NH subjects, deaf individuals were overall characterized by higher F0 and loudness values, especially in the vowel task than the reading. In the sustained vowel task, no patients demonstrated significant voice changes after switching on the CI; contrarily, in the reading task, the use of the CI reduced both loudness and F0 up to values comparable to NH subjects, although only in males. There was no significant difference in speech parameters between prelingual and postlingual deafness, although overall lower values were evident in case of postlingual deafness. The use of the CI showed a significant reduction of F0 in males with postlingual deafness and of loudness, both for patients with prelingual and postlingual deafness. Finally, there was a positive correlation between postoperative hearing thresholds and overall speech loudness, highlighting how subjects with better hearing outcomes after CI positioning generally speak with a lower loudness and therefore a reduced vocal effort and load.
DISCUSSION/CONCLUSION
We found similar speech performances between prelingual and postlingual deafness, both in the vowel /a/ phonation and in the reading, providing a further suggestion that prelingual adult patients may benefit from cochlear implantation in phonation as well, in addition to the known excellent hearing outcomes. Overall, these results highlight the ability of the CI to adjust in everyday speech certain phonatory aspects such as F0 and loudness by restoring the auditory feedback.
Topics: Adult; Male; Humans; Cochlear Implantation; Phonation; Deafness; Cochlear Implants; Hearing; Speech Perception
PubMed: 36195076
DOI: 10.1159/000526936