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Laryngo- Rhino- Otologie May 2023The human brain shows extensive development of the cerebral cortex after birth. This is extensively altered by the absence of auditory input: the development of cortical...
The human brain shows extensive development of the cerebral cortex after birth. This is extensively altered by the absence of auditory input: the development of cortical synapses in the auditory system is delayed and their degradation is increased. Recent work shows that the synapses responsible for corticocortical processing of stimuli and their embedding into multisensory interactions and cognition are particularly affected. Since the brain is heavily reciprocally interconnected, inborn deafness manifests not only in deficits in auditory processing, but also in cognitive (non-auditory) functions that are affected differently between individuals. It requires individualized approaches in therapy of deafness in childhood.
Topics: Humans; Auditory Cortex; Auditory Perception; Cognition; Deafness; Hearing
PubMed: 37130527
DOI: 10.1055/a-1973-5087 -
Canadian Family Physician Medecin de... Oct 2022A young infant seen in our practice was diagnosed with profound congenital hearing loss. Their parents want to pursue cochlear implant surgery for their child, but they...
QUESTION
A young infant seen in our practice was diagnosed with profound congenital hearing loss. Their parents want to pursue cochlear implant surgery for their child, but they are concerned about language acquisition before and after the surgery. What should they know about the procedure, and how can they improve language outcomes?
ANSWER
Congenital hearing loss is often identified on newborn screening hearing tests. Cochlear implants may lead to overall improved spoken language skills among children with profound hearing loss. Some factors associated with successful language acquisition in children after cochlear implant surgery include having the procedure at an earlier age and family engagement in early intervention programs. Learning sign language before cochlear implant surgery may improve subsequent language outcomes and support the child's cognitive and socioemotional success.
Topics: Child; Cochlear Implantation; Cochlear Implants; Deafness; Hearing Aids; Humans; Infant; Infant, Newborn; Language Development
PubMed: 36241402
DOI: 10.46747/cfp.6810737 -
Aging Cell Nov 2023The GJB2 gene, encoding Connexin26 (Cx26), is one of the most common causes of inherited deafness. Clinically, mutations in GJB2 cause congenital deafness or late-onset...
The GJB2 gene, encoding Connexin26 (Cx26), is one of the most common causes of inherited deafness. Clinically, mutations in GJB2 cause congenital deafness or late-onset progressive hearing loss. Recently, it has been reported that Cx26 haploid deficiency accelerates the development of age-related hearing loss (ARHL). However, the roles of cochlear Cx26 in the hearing function of aged animals remain unclear. In this study, we revealed that the Cx26 expression was significantly reduced in the cochleae of aged mice, and further explored the underlying molecular mechanism for Cx26 degradation. Immunofluorescence co-localization results showed that Cx26 was internalized and degraded by lysosomes, which might be one of the important ways for Cx26 degradation in the cochlea of aged mice. Currently, whether the degradation of Cx26 in the cochlea leads directly to ARHL, as well as the mechanism of Cx26 degradation-related hearing loss are still unclear. To address these questions, we generated mice with Cx26 knockout in the adult cochlea as a model for the natural degradation of Cx26. Auditory brainstem response (ABR) results showed that Cx26 knockout mice exhibited high-frequency hearing loss, which gradually progressed over time. Pathological examination also revealed the degeneration of hair cells and spiral ganglions, which is similar to the phenotype of ARHL. In summary, our findings suggest that degradation of Cx26 in the cochlea accelerates the occurrence of ARHL, which may be a novel mechanism of ARHL.
Topics: Animals; Mice; Cochlea; Connexins; Deafness; Mice, Knockout; Presbycusis; Connexin 26
PubMed: 37681746
DOI: 10.1111/acel.13973 -
American Annals of the Deaf 2023
Topics: Humans; Multilingualism; Education of Hearing Disabled; Deafness; Persons With Hearing Impairments; Sign Language; Child; Learning
PubMed: 38661778
DOI: 10.1353/aad.2023.0006 -
American Annals of the Deaf 2023During the COVID-19 pandemic, many children experienced multiple challenges while transitioning from traditional to online schooling. Teachers, administrators, and...
During the COVID-19 pandemic, many children experienced multiple challenges while transitioning from traditional to online schooling. Teachers, administrators, and parents were expected to work together to provide students an optimal educational experience through those turbulent times. This experience generated new insights into how to teach deaf and hard of hearing (DHH) students and assess their knowledge. New tools were invented and used during the pandemic, and though teachers and students are slowly returning to traditional learning environments, educators now know that alternative forms of teaching and learning exist. We acknowledge the ways teachers, administrators, and parents can innovate and bring new knowledge to the table. The present article introduces an American Annals of the Deaf Special Issue that offers further research and discussion in the event that another, comparable challenge occurs. Multiple issues must be considered in DHH education, from academic rigor to social-emotional wellness.
Topics: Child; Humans; Education, Special; Pandemics; Deafness; Education of Hearing Disabled; Curriculum
PubMed: 38588106
DOI: 10.1353/aad.2023.a917253 -
Cochlear Implants International Nov 2023Identify and evaluate the effectiveness of methods for improving postoperative cochlear implant (CI) hearing performance in subjects with single-sided deafness (SSD) and... (Review)
Review
OBJECTIVE
Identify and evaluate the effectiveness of methods for improving postoperative cochlear implant (CI) hearing performance in subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL).
DATA SOURCES
Embase, PubMed, Scopus.
REVIEW METHODS
Systematic review and narrative synthesis. English language studies of adult CI recipients with SSD and AHL reporting a postoperative intervention and comparative audiometric data pertaining to speech in noise, speech in quiet and sound localization were included.
RESULTS
32 studies met criteria for full text review and 6 (n = 81) met final inclusion criteria. Interventions were categorized as: formal auditory training, programming techniques, or hardware optimization. Formal auditory training (n = 10) found no objective improvement in hearing outcomes. Experimental CI maps did not improve audiologic outcomes (n = 9). Programed CI signal delays to improve synchronization demonstrated improved sound localization (n = 12). Hardware optimization, including multidirectional (n = 29) and remote (n = 11) microphones, improved sound localization and speech in noise, respectively.
CONCLUSION
Few studies meeting inclusion criteria and small sample sizes highlight the need for further study. Formal auditory training did not appear to improve hearing outcomes. Programming techniques, such as CI signal delay, and hardware optimization, such as multidirectional and remote microphones, show promise to improve outcomes for SSD and AHL CI users.
Topics: Adult; Humans; Cochlear Implantation; Hearing Loss, Unilateral; Speech Perception; Treatment Outcome; Hearing Loss; Cochlear Implants; Sound Localization; Deafness
PubMed: 37490782
DOI: 10.1080/14670100.2023.2239512 -
Human Genetics Apr 2022
Topics: Deafness; Hearing; Hearing Tests; Humans; Molecular Biology
PubMed: 35376981
DOI: 10.1007/s00439-022-02451-y -
Cell Calcium Mar 2023Deafness is a highly heterogeneous disorder which stems, for 50%, from genetic origins. Sensory transduction relies mainly on sensory hair cells of the cochlea, in the... (Review)
Review
Deafness is a highly heterogeneous disorder which stems, for 50%, from genetic origins. Sensory transduction relies mainly on sensory hair cells of the cochlea, in the inner ear. Calcium is key for the function of these cells and acts as a fundamental signal transduction. Its homeostasis depends on three factors: the calcium influx, through the mechanotransduction channel at the apical pole of the hair cell as well as the voltage-gated calcium channel at the base of the cells; the calcium buffering via Ca-binding proteins in the cytoplasm, but also in organelles such as mitochondria and the reticulum endoplasmic mitochondria-associated membranes with specialized proteins; and the calcium extrusion through the Ca-ATPase pump, located all over the plasma membrane. In addition, the synaptic transmission to the central nervous system is also controlled by calcium. Genetic studies of inherited deafness have tremendously helped understand the underlying molecular pathways of calcium signaling. In this review, we discuss these different factors in light of the associated genetic diseases (syndromic and non-syndromic deafness) and the causative genes.
Topics: Humans; Calcium Signaling; Mechanotransduction, Cellular; Calcium; Rare Diseases; Deafness
PubMed: 36791536
DOI: 10.1016/j.ceca.2023.102702 -
JAMA Otolaryngology-- Head & Neck... Jul 2020
Topics: Age Factors; Audiometry, Pure-Tone; Cochlear Implants; Deafness; Humans; Patient Selection; Speech Perception; Time-to-Treatment
PubMed: 32407470
DOI: 10.1001/jamaoto.2020.0678 -
American Annals of the Deaf 2021
Topics: Deafness; Human Rights; Humans; Persons With Hearing Impairments; Social Justice
PubMed: 33678715
DOI: 10.1353/aad.2021.0002