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Anatomical Record (Hoboken, N.J. : 2007) Mar 2020This review presents many but not all the major historical events that have led to our current understanding of gene and stem cell therapies for the treatment of hearing... (Review)
Review
This review presents many but not all the major historical events that have led to our current understanding of gene and stem cell therapies for the treatment of hearing and balance disorders in animal models of these disorders. In order to better understand the application of these emerging therapies to the treatment of inner ear disorders in a clinical setting, it has been necessary to provide some genetic and pathobiology backgrounds from both animal models and clinical disorders. The current focus and goal of gene and stem cell therapies are directed toward understanding the effective treatment of animal models that mimic human disorders of hearing and balance. This approach not only addresses the most effective ways to deliver the gene or stem cell therapies to affected inner ears, it also provides an assessment of the efficacy of the applied therapy(s) in achieving either partial or full restoration of either hearing and/or balance within the animal models receiving these therapeutic interventions. This review also attempts to present a realistic assessment of how close the research fields of gene and stem cell therapies are to application for the treatment of human disorders in a clinical setting. Progress made in developing these novel therapies toward clinical applications would not have been possible without the many pioneering studies and discoveries achieved by the investigators cited in this review. There were also many other excellent studies performed by gifted investigators that were not able to be included within this review. Anat Rec, 303:390-407, 2020. © 2019 American Association for Anatomy.
Topics: Animals; Genetic Therapy; Hearing Disorders; History, 20th Century; History, 21st Century; Humans; Stem Cell Transplantation; Vestibular Diseases
PubMed: 31994336
DOI: 10.1002/ar.24332 -
Current Opinion in Otolaryngology &... Dec 2012Over the last decade, the selection criteria for cochlear implantation have expanded to include children with special auditory, otologic, and medical problems. Included... (Review)
Review
PURPOSE OF REVIEW
Over the last decade, the selection criteria for cochlear implantation have expanded to include children with special auditory, otologic, and medical problems. Included within this expanded group of candidates are those children with auditory neuropathy spectrum disorder, cochleovestibular malformations, cochlear nerve deficiency, associated syndromes, as well as multiple medical and developmental disorders. Definitive indications for cochlear implantation in these unique pediatric populations are in evolution. This review will provide an overview of managing and habilitating hearing loss within these populations with specific focus on cochlear implantation as a treatment option.
RECENT FINDINGS
Cochlear implants have been successfully implanted in children within unique populations with variable results. Evaluation for cochlear implant candidacy includes the core components of a full medical, audiologic, and speech and language evaluations. When considering candidacy in these children, additional aspects to consider include disorder-specific surgical considerations and child/caregiver counseling regarding reasonable postimplantation outcome expectations.
SUMMARY
Cochlear implants are accepted as the standard of care for improving hearing and speech development in children with severe-to-profound hearing loss. However, children with sensorineural hearing loss who meet established audiologic criteria for cochlear implantation may have unique audiologic, medical, and anatomic characteristics that necessitate special consideration regarding cochlear implantation candidacy and outcome. Individualized preoperative candidacy and counseling, surgical evaluation, and reasonable postoperative outcome expectations should be taken into account in the management of these children.
Topics: Audiometry, Pure-Tone; CHARGE Syndrome; Cochlear Implantation; Electrodes, Implanted; Goiter, Nodular; Hearing Loss; Hearing Loss, Central; Hearing Loss, Sensorineural; Hearing Tests; Humans; Magnetic Resonance Imaging; Usher Syndromes; Vestibular Diseases; Vestibulocochlear Nerve Diseases
PubMed: 23128686
DOI: 10.1097/MOO.0b013e328359eea4 -
RoFo : Fortschritte Auf Dem Gebiete Der... Oct 2023Since the introduction of hearing screening in Germany in 2009, pediatric hearing disorders are detected at an early stage. Early therapy is essential for language...
BACKGROUND
Since the introduction of hearing screening in Germany in 2009, pediatric hearing disorders are detected at an early stage. Early therapy is essential for language development. Imaging plays a central role in diagnosis and therapy planning.
METHOD
Imaging findings of the most relevant causes of pediatric hearing disorders are presented. Specific attention is given to the method used in each case - CT or MRI.
RESULTS AND CONCLUSIONS
While CT is the method of choice for conductive hearing loss, a combination of CT and MRI with high-resolution T2-3D sequences has been established as the best diagnostic method for sensorineural hearing loss. The most common causes of conductive hearing loss in childhood are chronic inflammation and cholesteatoma. Congenital malformations of the outer or middle ear are less frequent. In the case of sensorineural hearing loss, the cause is located in the inner ear and/or the cochlear nerve or the cerebrum. In these cases, congenital malformations are the most common cause.
KEY POINTS
· CT and MRI are necessary to identify morphological causes of hearing disorders and to clarify the possibility of hearing-improving ear surgery or cochlear implantation.. · Contraindications for surgical procedures must be excluded.. · Anatomical variants that may be risk factors for surgery must be described..
CITATION FORMAT
· Sorge I, Hirsch F, Fuchs M et al. Imaging diagnostics for childhood hearing loss. Fortschr Röntgenstr 2023; 195: 896 - 904.
Topics: Humans; Child; Hearing Loss, Conductive; Tomography, X-Ray Computed; Hearing Loss; Hearing Loss, Sensorineural; Ear, Inner; Magnetic Resonance Imaging
PubMed: 37348530
DOI: 10.1055/a-2081-4119 -
Frontiers in Neural Circuits 2021Neuronal hyperexcitability in the central auditory pathway linked to reduced inhibitory activity is associated with numerous forms of hearing loss, including noise...
Neuronal hyperexcitability in the central auditory pathway linked to reduced inhibitory activity is associated with numerous forms of hearing loss, including noise damage, age-dependent hearing loss, and deafness, as well as tinnitus or auditory processing deficits in autism spectrum disorder (ASD). In most cases, the reduced central inhibitory activity and the accompanying hyperexcitability are interpreted as an active compensatory response to the absence of synaptic activity, linked to increased central neural gain control (increased output activity relative to reduced input). We here suggest that hyperexcitability also could be related to an immaturity or impairment of tonic inhibitory strength that typically develops in an activity-dependent process in the ascending auditory pathway with auditory experience. In these cases, high-SR auditory nerve fibers, which are critical for the shortest latencies and lowest sound thresholds, may have either not matured (possibly in congenital deafness or autism) or are dysfunctional (possibly after sudden, stressful auditory trauma or age-dependent hearing loss linked with cognitive decline). Fast auditory processing deficits can occur despite maintained basal hearing. In that case, tonic inhibitory strength is reduced in ascending auditory nuclei, and fast inhibitory parvalbumin positive interneuron (PV-IN) dendrites are diminished in auditory and frontal brain regions. This leads to deficits in central neural gain control linked to hippocampal LTP/LTD deficiencies, cognitive deficits, and unbalanced extra-hypothalamic stress control. Under these conditions, a diminished inhibitory strength may weaken local neuronal coupling to homeostatic vascular responses required for the metabolic support of auditory adjustment processes. We emphasize the need to distinguish these two states of excitatory/inhibitory imbalance in hearing disorders: (i) Under conditions of preserved fast auditory processing and sustained tonic inhibitory strength, an excitatory/inhibitory imbalance following auditory deprivation can maintain precise hearing through a memory linked, transient disinhibition that leads to enhanced spiking fidelity (central neural gain⇑) (ii) Under conditions of critically diminished fast auditory processing and reduced tonic inhibitory strength, hyperexcitability can be part of an increased synchronization over a broader frequency range, linked to reduced spiking reliability (central neural gain⇓). This latter stage mutually reinforces diminished metabolic support for auditory adjustment processes, increasing the risks for canonical dementia syndromes.
Topics: Autism Spectrum Disorder; Cognition; Hearing Loss; Humans; Reproducibility of Results; Tinnitus
PubMed: 35069123
DOI: 10.3389/fncir.2021.785603 -
Nature Communications Jan 2024Tinnitus is a heritable, highly prevalent auditory disorder treated by multiple medical specialties. Previous GWAS indicated high genetic correlations between tinnitus... (Meta-Analysis)
Meta-Analysis
Tinnitus is a heritable, highly prevalent auditory disorder treated by multiple medical specialties. Previous GWAS indicated high genetic correlations between tinnitus and hearing loss, with little indication of differentiating signals. We present a GWAS meta-analysis, triple previous sample sizes, and expand to non-European ancestries. GWAS in 596,905 Million Veteran Program subjects identified 39 tinnitus loci, and identified genes related to neuronal synapses and cochlear structural support. Applying state-of-the-art analytic tools, we confirm a large number of shared variants, but also a distinct genetic architecture of tinnitus, with higher polygenicity and large proportion of variants not shared with hearing difficulty. Tissue-expression analysis for tinnitus infers broad enrichment across most brain tissues, in contrast to hearing difficulty. Finally, tinnitus is not only correlated with hearing loss, but also with a spectrum of psychiatric disorders, providing potential new avenues for treatment. This study establishes tinnitus as a distinct disorder separate from hearing difficulties.
Topics: Humans; Tinnitus; Cochlea; Deafness; Hearing Loss, Noise-Induced
PubMed: 38242899
DOI: 10.1038/s41467-024-44842-x -
The Clinical Neuropsychologist Jul 2022There is higher risk for autism spectrum disorder (ASD) across many pediatric neurological conditions characterized by vision impairment or hearing loss. Early and... (Review)
Review
Considerations for the identification of autism spectrum disorder in children with vision or hearing impairment: A critical review of the literature and recommendations for practice.
OBJECTIVE
There is higher risk for autism spectrum disorder (ASD) across many pediatric neurological conditions characterized by vision impairment or hearing loss. Early and accurate identification of ASD is imperative in promoting access to appropriate and early evidenced-based intervention; however, differential diagnosis can be particularly challenging in children with sensory impairment given the heterogeneity of ASD combined with the impact of vision impairment or hearing loss/deafness on development and behavior. A neuropsychologist's unique expertise and appreciation of the interplay between sensory and behavioral manifestations can be valuable for making an early and accurate ASD diagnosis in children who are blind/visually impaired or deaf/hard-of-hearing. This article highlights clinical considerations when identifying ASD within the context of vision impairment or hearing loss/deafness.
METHOD
We discuss clinical considerations for the early identification of ASD in children who are blind/visually impaired and deaf/hard-of-hearing. Information presented in the article is based on a critical review of the literature and the expertise of the author group.
CONCLUSION
Ongoing development of clinical expertise and evidence-based assessment methods are important when informing the early differential diagnosis of ASD in individuals with sensory impairment. Accurate identification is also vital for the development of targeted interventions across the lifespan.
Topics: Autism Spectrum Disorder; Child; Deafness; Hearing Loss; Humans; Neuropsychological Tests; Vision, Ocular
PubMed: 34889701
DOI: 10.1080/13854046.2021.2002933 -
Hawai'i Journal of Medicine & Public... May 2017Hearing and balance disorders affect people of all ages. Among children, hearing loss affects speech and language development, academic performance, and psychosocial...
Hearing and balance disorders affect people of all ages. Among children, hearing loss affects speech and language development, academic performance, and psychosocial development. Hearing loss in adults negatively impacts work productivity, cognitive function, and psychosocial status. Prevalence of hearing loss in children in Hawai'i is higher than the national average. Research indicates that hearing loss is a prevalent condition among veterans and advanced age adults. This is of particular concern in Hawai'i as the state is home to many military training facilities and has a large elderly population. In contrast to the higher than average prevalence of hearing loss in Hawai'i, there is a relatively small number of practicing audiologists in the state. Audiologists are independent doctoral level professionals responsible for the assessment and non-medical management of hearing impairment, vestibular disorders, auditory processing disorder, auditory neuropathy, tinnitus, and related disorders. Currently, there is no formal audiology training program in Hawai'i to meet the needs for current or future hearing health professionals. The Department of Communication Sciences and Disorders at the University of Hawai'i at Mānoa is developing a proposal for a Doctor of Audiology (Au.D.) program to provide a comprehensive doctoral-level audiology curriculum and clinical training in order to graduate audiologists with knowledge and skills needed to serve people with hearing and balance disorders in Hawai'i. This review article describes the current status of hearing and balance disorders and services in Hawai'i and stresses the importance of early identification and intervention in remediating their effects in all ages, with a focus on the need for expanding hearing health services in Hawai'i.
Topics: Adolescent; Adult; Aged; Audiology; Child; Child, Preschool; Female; Hawaii; Health Services Needs and Demand; Hearing Disorders; Humans; Male; Middle Aged; Population Surveillance; Postural Balance; Prevalence
PubMed: 28484666
DOI: No ID Found -
Journal of Clinical Pharmacy and... Nov 2022Evidence on whether the coronavirus disease 2019 (COVID-19) vaccination could cause hearing-related adverse events is still conflicting. This study aims to access the...
WHAT IS KNOWN AND OBJECTIVE
Evidence on whether the coronavirus disease 2019 (COVID-19) vaccination could cause hearing-related adverse events is still conflicting. This study aims to access the association between COVID-19 vaccine and hearing disorder.
METHODS
The Vaccine Adverse Event Reporting System (VAERS) was queried between January 2020 to November 2021. The disproportionality pattern for hearing impairment of COVID-19 vaccine was accessed by calculating the reporting odds ratio (ROR) and proportional reporting ratio (PRR). A further subgroup analysis based on the type of COVID-19 vaccine and the doses administered was performed. In addition, the disproportionalities for hearing dysfunction between COVID-19 and influenza vaccines were compared.
RESULTS AND DISCUSSION
A total of 14,956 reports of hearing-related adverse events were identified with COVID-19 vaccination and 151 with influenza vaccine during the analytic period in VAERS. The incidence of hearing disorder following COVID-19 vaccination was 6.66 per 100,000. The results of disproportionality analysis revealed that the adverse events of hearing impairment, after administration of COVID-19 vaccine, was significantly highly reported (ROR 2.38, 95% confidence interval [CI] 2.20-2.56; PRR: 2.35, χ 537.58), for both mRNA (ROR 2.37, 95% CI 2.20-2.55; PRR 2.34, χ 529.75) and virus vector vaccines (ROR 2.50, 95% CI 2.28-2.73; PRR 2.56, χ 418.57). While the disproportional level for hearing dysfunction was quite lower in influenza vaccine (ROR 0.36, 95% CI 0.30-0.42; PRR 0.36, χ 172.24).
WHAT IS NEW AND CONCLUSION
This study identified increased risk for hearing disorder following administration of both mRNA and virus vector COVID-19 vaccines compared to influenza vaccination in real-world settings.
Topics: Humans; Pharmacovigilance; COVID-19 Vaccines; Adverse Drug Reaction Reporting Systems; Influenza Vaccines; COVID-19; Vaccination; Hearing Disorders; RNA, Messenger
PubMed: 36089844
DOI: 10.1111/jcpt.13767 -
Hearing Research Dec 2011Normal blood supply to the cochlea is critically important for establishing the endocochlear potential and sustaining production of endolymph. Abnormal cochlear... (Review)
Review
Normal blood supply to the cochlea is critically important for establishing the endocochlear potential and sustaining production of endolymph. Abnormal cochlear microcirculation has long been considered an etiologic factor in noise-induced hearing loss, age-related hearing loss (presbycusis), sudden hearing loss or vestibular function, and Meniere's disease. Knowledge of the mechanisms underlying the pathophysiology of cochlear microcirculation is of fundamental clinical importance. A better understanding of cochlear blood flow (CoBF) will enable more effective management of hearing disorders resulting from aberrant blood flow. This review focuses on recent discoveries and findings related to the physiopathology of the cochlear microvasculature.
Topics: Animals; Cochlea; Hearing Disorders; Humans; Microcirculation; Microvessels; Regional Blood Flow
PubMed: 21875658
DOI: 10.1016/j.heares.2011.08.006 -
The Journal of International Advanced... Dec 2017Hearing loss in children represents a relevant topic, which needs an increasing attention by clinicians and researchers. Unfortunately, most cases of hearing loss still... (Review)
Review
Hearing loss in children represents a relevant topic, which needs an increasing attention by clinicians and researchers. Unfortunately, most cases of hearing loss still remain idiopathic (most frequently reported causes are genetic, infectious, toxic). An early diagnosis is crucial, as if not properly recognized, hearing disorders may impact negatively on children development and on quality of life. Literature data show that also children with mild-moderate, or even monolateral, hearing loss may present learning or verbal language disorders, if not adequately managed. The diagnostic work-up of hearing loss in children, which starts with universal neonatal hearing screening at birth, is complex, has to define hearing threshold, and, when possible, its etiology, often by multidisciplinary approach. At the same time, the audiological follow-up of those affected by moderate-severe hearing loss must be tight, as it is necessary to verify constantly: (i) hearing threshold levels, (ii) adequateness of hearing aids fitting, and (iii) language development. This review focuses specifically on the diagnostic work-up and the rehabilitative features of moderate-severe hearing loss in children.
Topics: Genetic Predisposition to Disease; Hearing Loss; Humans; Language Development
PubMed: 29360094
DOI: 10.5152/iao.2017.4162