Virus infection is one of the most common etiologies of hearing loss. Hearing loss associated with viral infection can be unilateral or bilateral, mild or severe, sudden or progressive, and permanent or recoverable. Many viruses cause hearing loss in adults and children; however, the pathogenesis of hearing loss caused by viral infection is not fully understood. This review describes cytomegalovirus, the most common virus causing hearing loss, and other reported hearing loss-related viruses, along with their pathogenic characteristics and research progress on their pathology, hearing phenotypes, possible associated mechanisms, treatment and prevention measures, aiming to provide diagnostic and treatment assistance to clinical workers.

Many aging adults experience some form of hearing problems that may arise from auditory peripheral damage. However, it has been increasingly acknowledged that hearing loss is not only a dysfunction of the auditory periphery but results from changes within the entire auditory system, from periphery to cortex. Damage to the auditory periphery is associated with an increase in neural activity at various stages throughout the auditory pathway. Here, we review neurophysiological evidence of hyperactivity, auditory perceptual difficulties that may result from hyperactivity, and outline open conceptual and methodological questions related to the study of hyperactivity. We suggest that hyperactivity alters all aspects of hearing – including spectral, temporal, spatial hearing – and, in turn, impairs speech comprehension when background sound is present. By focusing on the perceptual consequences of hyperactivity and the potential challenges of investigating hyperactivity in humans, we hope to bring animal and human electrophysiologists closer together to better understand hearing problems in older adulthood.

Although congenital sensorineural hearing loss (SHL) in the bilateral cochleae mainly results from genetic abnormalities, chronic SHL progressing in later life is often influenced by systemic immune disturbances, including autoimmunity, chronic inflammation, and immunosenescence. We have investigated the relationship between the inner ear and systemic immunity and reviewed the possibilities to prevent SHL, including autoimmune SHL and age-related SHL. We also demonstrated two lymphocyte populations, interleukin 1 receptor type II (IL-1R2)-positive T cells (T1R2) and naturally occurring regulatory T cells (nTregs) in CD4⁺ T cells, which increase with aging, suppress host immune function and promote organ degeneration. Alterations in systemic immunity by fewer microbial antigen challenges in the living environment, elimination of immune suppressive lymphocytes, or immune rejuvenation with a reconstituted thymus may contribute not only to renew the cochlear function in SHL, but also to extend the healthy life of functional organs in a vigorous and youthful body, one of humanity’s greatest dreams.

Conductive hearing losses are typically present in disorders of the external/middle ear. However, there is a rare group of inner ear conditions called third windows that can also generate a conductive hearing loss. This is due to an abnormal connection between the middle and the inner ear or between the inner ear and the cranial cavity. X-linked gusher disorder is an extremely rare congenital inner ear dysplastic syndrome with such an abnormal connection due to a characteristic incomplete cochlear partition type 3 and an incomplete internal auditory meatus fundus. The disorder is inherited in an X-linked fashion due to the mutation of the POU3F4 gene. We present 2 siblings diagnosed with the condition and their long term follow ups. They both presented with audiovestibular symptoms and showed progressive mixed losses and bilateral vestibular weakness. They were treated with cochlear implant, digital amplification and with vestibular rehabilitation. Significant others around them were involved in their journey with the medical team and in both, a very favourable outcome was achieved. This is the first time that we are reporting evolving audiovestibular function with vestibular quantification in X-linked gusher disorder and emphasize on the multidisciplinary holistic approach to manage these children effectively.

Age-related hearing loss (ARHL) is the most common sensory disorder in the elderly. It is associated with aging and hair cell death due to oxidative stress-induced mitochondrial dysfunction. Although transgenic mice and long-term cultures for induction of aging have been used to study ARHL, there are presently no ARHL animal models stimulated by intermittent environmental change for aging. In this study, an ARHL animal model was established by inducing continuous oxidative stress to promote short-term aging of cells, determined based on the expression of the hearing loss-induced phenotype and aging related factors in the short term. The incidence of hearing loss was significantly different among the groups subjected to intermittent hypoxic environment, high-fat diet (HFD), and injection with D-galactose. Continuous oxidative stress and HFD were factors that accelerated cellular aging. Increase in UCP2 affected oxidative stress and mitochondrial dysfunction. CDH23, SLC26A4, KCNQ4, Myo7a, and Myo6, which are ARHL-related factors, were modified by oxidative stress in cells of the hearing organ. We found that intermittent hypoxic, HFD, and galactose injection accelerated cellular aging in the short term. Thus, we anticipate that the development of this hearing loss animal model, which reflects intermittent environmental changes, will benefit future research on ARHL.

Hearing loss in infancy leads to preventable speech, language, and cognitive developmental delay [1, 2]. Sensorineural hearing loss (SNHL) is caused by damages, problems, or issues related to the inner ear such as the cochlea with or without the auditory nerve; cranial nerve VIII, involvement. There are three anatomic areas which include the outer ear: composed of the auricle and external auditory canal and the middle ear: which includes the tympanic membrane, ossicles, and the middle ear space, the inner ear: composed of the cochlea, semi-circular canals, and internal auditory canals. The unique anatomical shape of the auricle catches the incoming sound waves to send them down the external auditory canal. Hearing risk assessment should be part of all health visits while regular hearing screening checks are done for all children from 4 to 21 years [1, 2]. Assessment of hearing loss includes history, physical examination and specific hearing assessment tests.

This study tests the utilization of Bluetooth noise-canceling headphones in improving the quality of eye exams in patients with hearing loss. University of Texas Medical Branch patients above 55 years of age were screened for hearing loss using SoundCheck audiometry. All 29 subjects (18 red-zone and 11 yellow-zone) answered pre-recorded Amsler grid and patient education questions that solicited precise responses. As controls, subjects were randomly administered half of the questions via headphones and half via a smartphone at normal speech volume (60 decibels). Points were awarded for responses demonstrating comprehension, and a post-exam survey was collected. Collectively, the mean score was 1.79 with headphones versus 0.96 with control on the Amsler grid segment and 1.90 with headphones versus 0.97 with control on education questions (p=0.001). Between the red and yellow zone hearing loss patients, the red-zone group answered significantly better in both Amsler (1.78 versus 0.50; p=0.0003) and education questions (1.88 versus 0.44; p<0.0001) with headphones. The yellow zone group answered better with headphones overall but failed to reach significance. Post-exam survey indicated that 28 of 29 patients (97%) preferred the headphones during ophthalmic exams. Patients with severe hearing loss demonstrated better comprehension with the noise-canceling headphones; thus these devices show great promise in improving effective, compassionate communication between providers and hearing-loss patients.

Hearing dysfunctions can be classified by type, degree, configuration, time of onset, aetiology, and finally, consequences on speech development. They can be divided into conductive, mixed, central types and sensorineural. Conductive hearing loss (CHL) results from interference with the mechanical transmission of sound through the external and middle ear; it can be congenital, as a consequence of anatomic abnormalities, but it can commonly be acquired following middle ear inflammatory pathologies. Sensorineural hearing loss (SNHL) results from failure to transduce vibrations to neural impulses in the cochlean and is a consequence of an irreversible damage to the differentiated cells which make up the organ of hearing and the acoustic paths at various levels. Mixed hearing loss involves a combination of these two types in the same ear. Studies in neuroscience field have shown that the prevention of cell degeneration is only possible if all the factors taken at the different stages of stem cells’ multiplication and differentiation are administered together. We have demonstrated this in a recent study on the ability of SCDSFs to prevent neurodegeneration in hippocampal cells of the CA1 zone in mice. This study confirms previous findings demonstrating that early developmental zebrafish embryo extracts could act as a modulator of senescence in human mesenchymal stem cells (hMSC) isolated from many adult tissues. These findings have open a promising way for the approaches promoting the rejuvenation and regeneration of different tissues, by-passing stem cell transplantation. In the present clinical trial we have used SCDSFs to study the possible reversion of neurosensory hearing loss, until now considered an irreversible condition.

The aim of this study was to explore perspectives of occupation health personnels (OHPs) regarding education and training of mineworkers on occupational noise induced hearing loss (ONIHL) and its impact on mineworkers’ hearing. Qualitative, in-depth telephonic and face-to-face interviews were conducted with 16 OHPs comprising representatives from the state, employer and labour as well as audiologists and occupational health hygienists. Purposive and snowball sampling were utilized to recruit participants. Data were analysed using inductive thematic analysis. Findings revealed that mineworkers have a superficial awareness and knowledge of the impact of noise on their hearing and health. Moreover, OHPs are not knowledgeable on how mineworkers are educated on ONIHL and its latent consequences. Furthermore, language, low levels of education and literacy as well as the financial constraints were factors that had a negative impact on raising awareness and training mineworkers. There is a need to prioritize health literacy among mineworkers. Additionally, audiologists need to play an active role in educating mineworkers about the effects of excessive exposure to noise. There is a need to take into account literacy levels and language barriers in planning training material for mineworkers.

Genetic sensorineural hearing loss and Meniere disease have been associated with rare variations in the coding and non-coding region of the human genome. Most of these variants are classified as likely pathogenic or variants of unknown significance and require functional validation in cellular or animal models. Given the difficulties to obtain human samples and the raising concerns about animal experimentation, human induced pluripotent stem cells emerge as cellular models to investigate the interaction of genetic and environmental factors in the pathogenesis of inner ear disorders. The generation of human sensory epithelia and neuron-like cells carrying the variants of interest may facilitate a better understanding of their role during differentiation. These cellular models will allow us to explore new strategies for restoring hearing and vestibular sensory epithelia as well as neurons. This review summarizes the use of human induced pluripotent stem cells in sensorineural hearing loss and Meniere disease and proposes some strategies for its application in clinical practice.

: Forty-nine children who started wearing cartilage conduction hearing aids (CC-HA) before elementary school graduation (including 17 cases of bilateral hearing loss and 32 cases of unilateral hearing loss) were followed up and examined. The wearing and utilization status of CC-HA, as well as the progress to date, were evaluated. In addition, 33 participants who purchased CC-Has were interviewed to assess the wearing effect. Eleven of the 17 children with bilateral hearing loss and 25 of the 32 children with unilateral hearing loss have continued using CC-HA. In terms of wearing effect, a good wearing effect was reported, even by those with one-sided hearing loss. In cases where it is difficult to wear CC-HAs stably with pasting or ear tips, it is possible to fix them stably using commercially available hair bands and eyeglass vines. In two cases, CC-HAs were worn from the age of 0. With ingenuity and appropriate educational and medical support, it is possible to wear CC-HA from infancy.

Hearing impairment (HI) is a common sensory disorder that is defined as the partial or complete inability to detect sound in one or both ears. This diverse pathology is associated with a myriad of phenotypic expressions and/or syndromes. HI can be caused by various intrinsic, environmental and/or unknown factors. Some ontologies capture some relevant HI forms, phenotypes and syndromes, but there is no comprehensive knowledge portal which includes aspects specific to the HI disease state. This hampers inter-study comparability, integration and interoperability within and across disciplines. This work describes the HI Ontology (HIO) that was developed based on the Sickle Cell Disease Ontology (SCDO) model. This is a collaboratively developed resource built around the 'Hearing Impairment' concept by a group of experts in different aspects of HI and ontologies. HIO is the first comprehensive, standardized, hierarchical and logical representation of existing HI knowledge. HIO allows researchers and clinicians alike to readily access standardized HI-related knowledge in a single location and promote collaborations and HI information sharing, including epidemiological, socio-environmental, biomedical, genetic and phenotypic information. Furthermore, this ontology illustrates the adaptability of the SCDO framework for use in developing a disease-specific ontology.

Second-level hospitals face peculiarities that hinder the implementation of the hearing rescreening protocol, which are not uncommon in other settings. This study analyzes the hearing rescreening process in this kind of hospital. A total of 1130 individuals were included. In this cohort, 61.07% were newborns in the hospital who failed their first otoemission test after birth (n=679) or were unable to have the test performed (n=11), then being referred to outpatient clinic. The remaining 38.93% were individuals who were born in another hospital with their first test conducted in the outpatient clinic (n=440). A high amount of rescreenings were made outside the recommended time frame, mainly in children referred from another hospital. There was a high rate of lost to follow-up especially with otolaryngologist referrals. Neonatal Hearing screening in second level hospitals is difficult because of the staffing and time constraints. This results in longer than recommended turnaround times and interferes with the timely detection of hearing loss. This is particularly serious in outpatients. Referral of children with impaired screening to out-of-town centers leads to unacceptable loss of follow-up. A legislative support for all these rescreening issues is necessary. We discuss these findings and propose some solutions.

A single paragraph of about 200 words maximum. For research articles, abstracts should give a pertinent overview of the work. We strongly encourage authors to use the following style of structured abstracts, but without headings: (1) Background: Place the question addressed in a broad context and highlight the purpose of the study; (2) Methods: briefly describe the main methods or treatments applied; (3) Results: summarize the article's main findings; (4) Conclusions: indicate the main conclusions or interpretations. The abstract should be an objective representation of the article and it must not contain results that are not presented and substantiated in the main text and should not exaggerate the main conclusions.

Objective: This study assessed hearing aid benefits for people with a normal audiogram but hearing-in-noise problems in everyday listening situations. Design: Exploratory double-blinded case control study whereby participants completed retrospective questionnaires, ecological momentary assessments, speech-in-noise testing, and mental effort testing with and without hearing aids. Twenty-seven adults reporting speech-in-noise problems but normal air-conduction pure-tone audiometry took part in the study. They were randomly separated into an experimental group who trialled mild-gain hearing aids with advanced directional processing and a control group fitted with hearing aids with no gain or directionality. Results: Self-reports showed mild-gain hearing aids reduce hearing-in-noise difficulties and provide a better hearing experience (i.e., improved understanding, participation, and mood). Despite the self-reported benefits, the laboratory tests did not reveal a benefit from the mild-gain hearing aids, with no group differences on speech-in-noise tests or mental effort measures. Further, participants found the elevated cost of hearing aids to be a barrier for their adoption. Conclusions: Hearing aids benefit the listening experience in some listening situations for people with normal audiogram who report hearing difficulties in noise. Decreasing the price of hearing aids may lead to greater accessibility to those seeking remediation for their communication needs.

A relatively loud sound is audible when a vibrator is attached to the aural cartilage. This form of conduction is referred to as cartilage conduction (CC). In Japan, a new type of hearing aid has been developed using CC and is available in clinical practice since 2017. A clinical study conducted prior to its launch demonstrated its benefits, particularly in patients with aural atresia who were unable to use air-conduction hearing aids. Several studies have been published on the benefits of CC hearing aids since their introduction in clinical practice. Most of the patients included in these studies had canal stenosis or aural atresia, and the purchase rates of CC hearing aids in these patients were relatively high. However, the number of patients with open ears was small, with overall poor results in the trials, with the exception in patients with continuous otorrhea. CC hearing aids are considered a good option for compensating hearing loss in ears with canal stenosis or atresia in both bilateral and unilateral cases. However, CC hearing aids are not currently considered the first choice for patients with an open ear,.

The twelve cranial nerves play a crucial role in nervous system, orchestrating a myriad of functions vital for our everyday life. These nerves are each specialized for particular tasks. Cranial Nerve I, known as the olfactory nerve, is responsible for our sense of smell, allowing us to perceive and distinguish various scents. Cranial Nerve II, or the optic nerve, is dedicated to vision, transmitting visual information from the eyes to the brain. Eye movements are governed by Cranial Nerves III, IV, and VI, ensuring our ability to track objects and focus. Cranial Nerve V controls facial sensation and jaw movement, while Cranial Nerve VII, the facial nerve, facilitates facial expressions and taste perception. Cranial Nerve VIII, or the vestibulocochlear nerve, plays a critical role in hearing and balance. Cranial Nerve IX, the glossopharyngeal nerve, affects throat sensation and taste perception. Cranial Nerve X, the vagus nerve, is a far-reaching nerve, influencing numerous internal organs such as the heart, lungs, and digestive system. Cranial Nerve XI, the accessory nerve, is responsible for neck muscle control, contributing to head movement. Finally, Cranial Nerve XII, the hypoglossal nerve, manages tongue movement, essential for speaking, swallowing, and breathing. Understanding these cranial nerves is fundamental in comprehending the intricate workings of our nervous system and the functions that sustain our daily lives.

Better auditory processing of musicians is observed in previous research. As musicians differentiate their practice method and performance environment, we aimed to assess auditory perception in Greek musicians with respect to their musical specialization. If there are differences, this may provide a basis for better shaping auditory training in individuals with auditory processing disorder. The auditory tests administered were speech in noise (Speech in Babble), with and without rhythmic advantage (Word Recognition—Rhythm Component), short-term and working memory (Digit Span - Forward and Backwards), temporal resolution (Gaps In Noise) and detection of frequency discrimination threshold (DFL). Groups consisted of classical musicians, Byzantine chanters, percussionists, and non-musicians (12 participants/group). Statistical analysis revealed significant difference in: (i) word recognition in noise with precursor synchronized pulse between classical musicians compared to Byzantine musicians, (ii) better frequency discrimination of Byzantine musicians compared to non-musicians for the 2000Hz region and (iii) working memory, an advantage detected in musicians. Considering all the above, we conclude that musicians have a superior auditory perception, regardless of musical specialization. Musical training enhances elements of auditory processing and may be used as an additional rehabilitation during auditory training, focusing on specific types of music for specific auditory processing deficits.

Background: In order to achieve the best possible hearing and understanding with a cochlear implant (CI), regular hearing speech therapy treatment is necessary after implantation. This treatment should also be accessible to the growing proportion of hearing-impaired people with a migration background. This requires an alternative to therapy in the therapist's native language. The aim of this study was to evaluate six multilingual conversation applications with regard to their usefulness for therapy. Material and Methods: The six most commonly used applications were reviewed in terms of accuracy of content and grammatical translation, as well as pronunciation for English, Spanish, Arabic, Turkish, and Russian by native speakers. The number of available languages, availability, cost, and additional features were also analyzed. The accuracy of the content and grammatical translation as well as the pronunciation were statistically evaluated, and the differences were highlighted. The results of the different applications were compared with the performance of a native speaker. Results: All applications tested differed significantly from the native speaker level, with Google Translator showing the closest approximation to the native speaker level. All apps offer translations for multiple languages and, with exceptions, are available in both app stores. Furthermore, all apps have additional therapist-facilitating features. Conclusion: Multilingual conversation apps can make speech therapy in a foreign language much easier when used with patients. An adaptation of the software to the specific requirements of a hearing speech therapy is necessary to achieve a linguistic level that corresponds to the native language of the therapist and to enable an easy use in the therapy.

Meniere Disease (MD) is a multifactorial disorder of the inner ear characterized by vertigo attacks associated with sensorineural hearing loss and tinnitus with a significant heritability. Although MD has been associated with several genes, no epigenetic studies have been performed in MD. Here we performed whole genome bisulfite sequencing in 14 MD patients and 6 healthy controls, with the aim of identifying a MD methylation signature and potential disease mechanisms. We observed a high number of differentially methylated CpGs (DMC) when comparing MD patients to controls (N= 9,545), several of them in hearing loss genes such as PCDH15, ADGRV1 and CDH23. Bioinformatic analyses of DMCs and cis-regulatory regions predicted phenotypes related to abnormal excitatory postsynaptic currents, abnormal NMDA-mediated receptor currents and abnormal glutamate-mediated receptor currents when comparing MD to controls. Moreover, we identified various DMCs in genes previously associated with cochleovestibular phenotypes in mice. We have also found 12 undermethylated regions (UMR) that were exclusive to MD, including 2 UMR in an inter CpG island in the PHB gene. We suggest that the DNA methylation signature allows to distinguish between MD patients and controls. The enrichment analysis confirms previous findings of a chronic inflammatory process underlying MD.

Congenital deafness is a major pediatric problem, affecting about 1.5-3 per 1000 newborns. The early treatment through cochlear implantation and auditory rehabilitation has been a historic milestone. Early diagnosis of congenital deafness is an essential requirement to obtain the best results, which is achieved through neonatal screening, a diagnostic practice that we began systematically at the Hospital Clínico in Valencia (Spain) 30 years ago.

Background: Meniere disease (MD) is an inner ear disorder associated with comorbidities such as autoimmune diseases or migraine. This study describes clinical and cytokine profile in MD according to the age of onset of the condition. Methods: A cross-sectional study including 83 MD patients: 44 with early onset MD (EOMD, <35 years old), and 39 with late onset MD (LOMD, > 50 years old), 64 patients with migraine and 55 controls was carried out. Clinical variables and cytokines levels of CCL3, CCL4, CCL18, CCL22, CXCL1 and IL-1β were compared among the different groups. Results: CCL18 levels were higher in patients with migraine or MD than in controls. Elevated levels of IL-1β were observed in 11.4% EOMD and in 10.3% LOMD patients and these levels were not dependent on the age of individuals. EOMD had a longer duration of the disease (p=0.004) and a higher prevalence of migraine than LOMD (p=0.045). Conclusions: Patients with EOMD have a higher prevalence of migraine than LOMD, but migraine is not associated with any cytokine profile in patients with MD. The levels of CCL18, CCL3 and CXCL4 were different between patients with MD or migraine and controls.

Zebrafish is an excellent model for observing human genetic disorders. Hearing impairment is the most common genetic disorder including syndromic & non-syndromic hearing loss. Stem cell therapies are considered a new hope in case of hearing impairment. Stem cells are the master cells of the human body and having the capability to differentiate into any other form of cells in more than 200 types of cells (regeneration). This review article describes zebrafish as a biomedical model for stem cell research in hearing impairment, which revolutionized the biomedical arena to compete for the challenges.

The prevalence of congenital hearing impairment (HI) is highest in Africa. Estimates evaluated genetic causes to account for 31% of HI cases in Africa, but the identification of associated causative genes mutations have been challenging. In this study, we reviewed the potential roles, in humans, of 38 novel genes identified in a murine study. We gathered information from various genomic annotation databases and performed functional enrichment analysis using online resources i.e. genemania and g.proflier. Results revealed that 27/38 genes are express mostly in the brain, suggesting additional cognitive roles. Indeed, HERC1- R3250X had been associated with intellectual disability in a Moroccan family. A homozygous 216-bp deletion in KLC2 was found in two siblings of Egyptian descent with spastic paraplegia. Up to 27/38 murine genes have link to at least a disease, and the commonest mode of inheritance is autosomal recessive (n=8). Network analysis indicates that 20 other genes have intermediate and biological links to the novel genes, suggesting their possible roles in HI. This study will contribute to advance our knowledge in unravelling the biological roles of novel murine HI genes in humans and could enhance the understanding of the genetic causes of HI in Africans.

Background: Autoimmune inner ear disease (AIED) is a rare autoimmune condition characterized by progressive sensorineural hearing loss with hearing fluctuations over weeks to months, presenting significant challenges in both diagnosis and treatment. Due to its rarity, treatment responses and prognosis in AIED remain poorly understood. Methods: This study reviewed medical records from Taipei Medical University Hospital documented between August 2016 and August 2021. Among 67 patients experiencing fluctuating hearing loss, 11 met the diagnostic criteria for AIED and underwent regular follow-up assessments within a 5-year timeframe. ROC curve was used to discriminate the power of these prognostic parameters. Results: In this study, several factors including younger age, milder initial hearing loss, meeting criteria for bilateral Meniere's disease (MD), having an ascending audiogram pattern, longer time interval between hearing impairment attacks in contralateral ears and responding well to steroids were associated with better prognosis. ROC curve demonstrated the diagnostic value of specific parameters such as the presence of bilateral MD (AUC 0.825, 95% CI 0.640–1.000), pretreatment HI ≤ 45 dB HL (AUC 0.808, 95% CI 0.610–1.000), response to steroids (AUC 0.800, 95% CI 0.595–1.000), and ascending audiogram (AUC 0.858, 95% CI 0.683–0.858). Conclusions: This study identified key prognostic factors for AIED, providing valuable insights for predicting AIED prognosis and facilitating personalized patient management.

Background: this study aimed to describe the distribution of the genotype and allele frequencies of GJB2 variants in the Chinese population of the Dongfeng Tongji cohort and to analyze the features of the hearing phenotype. Methods: we used data from 9910 participants in the Dongfeng Tongji cohort in 2013 and selected nine GJB2 variants. Pure-tone audiometry was employed to measure hearing. Differences in genotype and allele frequencies were analyzed by chi-squared test or Fisher's exact test. Results: of the 9910 participants, 5742 had hearing loss. The genotype frequency of the GJB2 variant p.V37I was statistically significantly distributed between the normal and impaired hearing groups, but not for the variant c.235delC. A higher frequency of the p.V37I homozygous genotype was found in the hearing loss group (0.5%) than in the normal hearing group (0.1%). Patients with p.V37I and c.235delC homozygous mutations exhibited varying degrees of hearing loss, mainly presenting sloping and flat audiogram shapes. Conclusions: a significant difference was found in the genotype frequency of the GJB2 variant p.V37I between the case and control groups, but not for the variant c.235delC. Different degrees of hearing loss and various audiogram shapes were observed in patients with p.V37I and c.235delC homozygous mutations.

The capacity of 3D printing (3DP) technologies to initiate speedy polymerization of solvent free resins accounts for their utility in the manufacturing of medical devices. Nonetheless, independent biological evaluation of 3D printed materials is recommended due to the unique parameters of the manufacturing process, which can influence their physical, chemical, and biological properties. In this study, E-Shell 450 material indicated for 3DP of hearing aid shells and inner ear devices was examined for biological safety using zebrafish bioassays adapted to OECD fish embryo test. In addition, the proprietary material was characterized for composition using headspace gas-chromatography mass spectrometry (GC-MS). To initiate test, newly fertilized zebrafish eggs were cultured on non-treated and ethanol-treated materials in glass petri dishes with ultrapure water, incubated at 28.5°C and assessed for developmental endpoints of toxicity at 24h interval until 96h. Data confirmed non-treated material was extremely toxic in bioassays within 24h whereas ethanol-treated material showed a relative lower toxicity possibly due to ethanoic-aqueous interactions as observed by GC-MS. With the current influx of 3D printing materials, users are urged to exercise caution. Operators must also take cognizance of the potential toxicity of the chemicals used in 3DP and implement safety measures to limit their exposure.

Cochlear implantation (CI) allows rehabilitation for patients with severe to profound hearing impairment. Although the use of a robotic assistant provides technical assistance to the surgeon, the assessment of the impact of its use on auditory outcomes remains uncertain. We aim to com-pare the hearing results of patients who underwent bilateral cochlear implantation, one side was performed with manual insertion and the other side with robot-assisted insertion. The electrode array intrascalar positioning and the surgery duration were also studied. This retrospective intra-individual study involved 10 patients who underwent bilateral cochlear implantation. The study included 2 infants and 8 adults. The unique composition of this cohort enabled us to utilize each patient as their own control. Regarding speech disyllabic recognition, pure tone average, ECAP, ratio of array translocation, basilar membrane rupture, percentage of translocated electrodes there was no difference between manual and robot-assisted CI groups. This study is the first to compare intra-individual hearing performance after cochlear implantation either manually or robot-assisted. The number of patients and the time delay between manual and robotic implantation may have led to a lack of power, but there was no apparent difference in hearing performance between manual and robotic implantation.

Titanium middle ear prostheses are widely used in surgical practice due to their acoustic properties. However, they present a significant drawback shared by all synthetic materials currently in use for middle ear reconstruction: they can be rejected by the organism of the host. In this study, we aim to review the current literature on titanium TORP and PORP extrusion and dislocation. Eighty-one articles were analysed after full article review based on the inclusion and exclusion criteria. The most common indication for reconstruction was chronic otitis media with cholesteatoma. The average extrusion or dislocation rate was 5.1%, ranging from 0 to 35%. The average improvement in air-bone gap was 12.3 dB (1.6 dB to 25.1 dB) and 13.7 (-0,5 dB to 22.7 dB) for PORP ad TORP groups, respectively. The data reported on this topic are highly variable, demonstrating the functional outcomes are difficult to predict in clinical practice. We believe the current limitations could be overcome with technological developments, including bioengineering research focused on promoting the prothesis adaptation to the ME environment.

Objectives: Understanding the intersection of age, ethnicity, and disability will become increasingly important as the global population ages and becomes more diverse. By 2060, Hispanics will comprise 28% of the U.S. population. This study examines critical associations between sensory impairment, social isolation, and cognitive functioning among Hispanic older adults. Methods: Our sample consisted of 557 Hispanic older adults that participated in Rounds 1-3 or Rounds 5-7 of the National Health and Aging Trends Study. Longitudinal mediation models across a three-year span were estimated using Mplus with vision, hearing, and dual sensory impairment predicting cognitive functioning directly and indirectly through social isolation. Results: Findings indicated that cognitive functioning was concurrently and, in certain cases, longitudinally predicted by vision and dual sensory impairment, and by social isolation. Contrary to expectations, vision and hearing impairment were not predictive of social isolation. Dual sensory impairment was associated with social isolation, yet no significant indirect associations were found for sensory impairments predicting cognitive functioning through social isolation. Discussion: The finding that social isolation did not mediate the relationship between sensory impairment and cognitive decline among Hispanic older adults in the U.S. is contrary to findings from other studies that were not specifically focused on this population. This finding may be evidence that culturally motivated family support and intergenerational living buffer the impact of sensory impairments in later life. Findings suggest that Hispanic older adults experiencing dual sensory impairments may benefit from interventions that foster social support and include family members.

In patients with unilateral tinnitus with normal hearing, several studies have compared the ipsilateral and contralateral ears; however, few studies have investigated its relationship with the duration of tinnitus. We compared the auditory brainstem response and otoacoustic emission parameters between ipsilateral and contralateral ears in adults with unilateral tinnitus and normal hearing. This retrospective review included 84 patients with unilateral tinnitus and normal hearing who underwent auditory brainstem response and otoacoustic emission; they were categorized according to the duration of tinnitus. The latencies and amplitudes of waves I, III, and V, and V/I ratio of both ears in auditory brainstem response, and the results of distortion-product otoacoustic emission and transient evoked otoacoustic emission were examined. The auditory brainstem response parameters, distortion-product otoacoustic emission parameters, and transient evoked otoacoustic emission parameters between the ipsilateral and contralateral ears along the duration of tinnitus were analyzed. Moreover, the failure rates of both distortion-product otoacoustic emission and transient evoked otoacoustic emission between the ears along with the duration and the effects of the variables on the amplitude and latency of each wave were examined. Although there was little significant difference between the ipsilateral and contralateral ears, laterality seemed to have an effect on wave I latency in the multiple linear regression analysis. The distortion-product otoacoustic emission failure rate of the ipsilateral ear was higher than that of the contralateral ear in all patients. However, there was no remarkable difference between the ears in the distortion-product otoacoustic emission and transient evoked otoacoustic emission parameters throughout the duration. We found that outer hair cells and the distal portion of the cochlear nerve are possible pathologic lesions in tinnitus with normal hearing and cochlear synaptopathy could be suspected. Further studies, including those on inner hair cells and higher central cortex, are needed.

A Tangible User Interface (TUI) is a new interaction option that uses nontraditional input and output elements. A tangible interface thus allows the manipulation of physical objects using digital information. The exploration and manipulation of physical objects is a factor to be considered in learning in children, especially those with some kind of disability such as hearing, who maximize the use of other senses such as vision and touch. In a tangible interface, three elements are related - physical, digital and social. The potential of IoT for children is growing. This technology IoT integrated with TUI, can help for that parents or teachers can monitoring activities of the child. Also to identify behavior patterns in the child with hearing impairment. This article shows four case studies, where had been designed different products of Internet of Things Tangible applied a several contexts and with products of low cost.

Noise pollution is an environmental problem due to its effect on hearing and other related health issues. The elevated noise levels above standard limits cause hearing loss and other attendant problems. In Nigeria, viewing centers where football matches are watched via satellite are known as ‘Mini Stadium’. In this study, the noise pollution of a viewing center was assessed. To do this, five top teams each from the English Premier League (EPL), La Liga, Bundesliga, League 1, and Serie A were selected for the assessment. A sound level meter (GB: 2266204, made in China) was used for the monitoring. The range of noise levels in dBA showed as follow: EPL (56-108); La Liga (46-106); Bundesliga (54-102); Serie A (49-101); and Ligue 1 (54-101). The results when compared with the standard limits, it was observed that part of the results exceeded the limits, this means that the viewers and others within the viewing center may be prone to the hearing problem. It is recommended that acoustic insulators and hearing aids should be used to reduce the attendant problems associated with noise.

Tissue bioengineering has been assuming a pivotal role in implementing alternatives to conven-tional ossicular middle ear replacement prostheses to overcome extrusion while preserving acoustic properties. This in vitro study aims to explore for the first time in current literature the feasibility of a biohybrid middle ear prosthesis composed of titanium surrounded by bone ex-tracellular matrix as bio-coating. We have hereby studied the adhesion and proliferation of human adipose-derived mesenchymal stem cells (hASC) on titanium scaffolds in vitro. Moreover, we identified the osteogenic differentiation of hASC using immunofluorescence assay to analyze osteoblasts gene expression profile (Alp, Runx2, Col1a1, Osx, and Bglap) and we accounted the presence of collagen as a marker of hASC ability to secrete extracellular matrix. We utilized scanning electron microscopy to evaluate the presence of extracellular matrix on the scaffolds. Our preliminary data demonstrated the titanium ability to support human adipose-derived mesenchymal stem cell colonization, proliferation, and osteoblastic differentiation, to obtain a biohybrid device. Our experience seems encouraging, we advocate further in vivo research to corroborate our results towards bone transplantation.

The automatic classification of bird sounds is an ongoing research topic and several results have been reported for the classification of selected bird species. In this contribution we use an artificial neural network fed with pre-computed sound features to study the robustness of bird sound classification. We investigate in detail if and how classification results are dependent on the number of species and the selection of species in the subsets presented to the classifier. In more detail, a bag-of-birds approach is employed to randomly create balanced subsets of sounds from different species for repeated classification runs. The number of species present in each subset is varied between 10 and 300, randomly drawing sounds of species from a dataset of 659 bird species taken from Xeno-Canto database. We observe that the shallow artificial neural network trained on pre-computed sound features is able to classify the bird sounds relatively well. The classification performance is evaluated using several common measures such as precision, recall, accuracy, mean average precision and area under the receiver operator characteristics curve. All of these measures indicate a decrease in classification success as the number of species present in the subsets is increased. We analyze this dependence in detail and compare the computed results to an analytic explanation assuming dependencies for an idealized perfect classifier. Moreover, we observe that the classification performance depends on the individual composition of the subset and varies across 20 randomly drawn subsets.

The KARS gene encodes the aminoacyl-tRNA synthetase (aaRS) which activates and joins the lysin with its corresponding transfer RNA (tRNA), through the ATP-dependent aminoacylation of the amino acid. The KARS gene mutations have been linked to diverse neurologic phenotypes such as: neurosensorial hearing loss, leukodistrophy, microcephaly, developmental delay or regression, peripheral neuropathy, cardiomyopathy, impairment of the mitochondrial respiratory chain, hyperlactatemia, among others. This article presents the case of a Colombian pediatric patient with two pathological missense variants in a compound heterozygous state in the KARS gene.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic necrotizing vasculitis, affecting small-to-medium sized vessels. EGPA’s clinical manifestations are heterogeneous affecting different organs and systems and the upper respiratory tract can be affected with Ear, Nose and Throat (ENT) involvement. The aim of our study was to assess type manifestations at time of diagnosis in a cohort of EGPA patients and correlate findings with baseline variables (sex, age, antineutrophil cytoplasmic antibodies –ANCA- status) and literature reports. Main ENT manifestations in our patients at time of diagnosis were: nasal polyposis (CRSwNP) (52%), turbinate hypertrophy (48%), nasal swelling (40%), rhinorrhoea (40%), chronic rhinosinusitis without nasal polyposis (CRSsNP) (32%), nasal bone deformities (32%), nasal crusts (20%), nasal mucosal ulcers (12%), corditis (12%), hoarseness/dysphonia (12%), hearing loss (12%), mucoceles(4%) eosinophilic rhinitis (4%). No correlations were found between sex, age, ANCA status and ENT clinical manifestations. A polymorphic ENT involvement is often observed at early stages of EGPA. The presence of nasal, sinus, ear and/or laryngeal manifestations in patients with asthma and hypereosinophilia, independently of sex, age or ANCA status, should raise allert for further investigation and differential diagnosis for EGPA. ENT specialists should be aware of their leading position in this diagnostic race.

Background. In the last decade, it was concluded that glaucomatous optic neuropathy is part of the neurodegenerative diseases in which destructive neuronal lesions are located not only in the structure of the retina, but they are also present at the level of the central visual and nervous pathways. Patients with Primitive Open Angle Glaucoma may have experienced changes such as sensorineural hearing loss as well as balance changes. The purpose of this study is to evaluate the degree of involvement of the auditory system in patients with glaucoma, the structural changes in the optic nerve and, to establish correlation between the hearing loss and structural changes in the optic nerve. The subjects included in the study are grouped as follows: a group of patients with glaucoma that will be compared to a group of patients without glaucoma only as well as identi-fy-ing the evolution of structural changes in optic nerve to patients with glaucoma and to normal patients. Material and methods. It is a prospective, cross-sectional study on 32 eyes of 16 subjects with Primitive Open Angle Glaucoma in a study group and 24 eyes of 12 healthy subjects in con-trol group with a mean age of 62 years in both groups. Both groups underwent ophthalmo-logical, audiological and radio imaging evaluation: Pure-tone audiometry (PTA) were recorded, oph-thalmologic evaluations according to a standardized protocol and MRI cerebral examination was performed. Results: In the Study group patients, compared to the Control group, the average lev-els of the left PTA, the cup/disc (C/D) area in both eyes, the C/D ratio in both eyes are in-creased. The mean level of Retinal nerve fiber layer (RNFL) mean thickness was slightly lower in patients with glaucoma. Multivariate analysis demonstrates that gender can be a good predictor of C/D area, gender and age can be good predictors of PTA both eyes and C/D ratio in the right eye, and gender, age and background can be good predictors of disc area in the left eye. Conclu-sions: Pa-tients with Primitive Open Angle Glaucoma show changes in audiometry test in correla-tion with ophthalmological parameters demonstrating that the auditory system can be affected in Primitive Open Angle Glaucoma. This study emphasizes the problem of interdisciplinarity in medicine with the aim of ensuring the quality of life of patients with glaucoma. A good collabora-tion between ophthalmologist and otolaryngologist will be very useful for these patients.

This study measures the noise levels in a baseball stadium and analyzes baseball fans’ attitude of effect of recreational noise exposure on their hearing. In the baseball stadium, noise levels were measured in four seating sections using a sound level meter during the games. The LAeq average of the 16 measures produced 91.7 dBA, showing a significantly high noise level in the red and navy sections. As a function of frequency by LZeq analysis, the noise levels were significantly higher in low frequencies than other frequencies. For the survey sample, 688 randomly selected participants completed a 16-question survey on their noise exposure during the game and on the potential risk of hearing loss. Despite the very high noise levels, 70% of the respondents preferred sitting in either the red or the navy section to be closer to the cheerleaders and to obtain a good view. Most respondents reported that they did not consider wearing earplugs, and one-third experienced hearing muffled speech after the game. We conclude that the noise levels in baseball stadiums are high enough to cause hearing damage and/or tinnitus later, but expect these results to improve public education regarding safe noise exposure during popular sports activities.

Wearable assistant devices play an important role in daily life for people with disabilities. Those who are hearing impaired may face dangers while walking or driving on the road. The major danger is their inability to hear warning sounds from cars or ambulances. Thus, the goal of this study is to develop a wearable assistant device for the hearing impaired to recognize emergency vehicle sirens on the road using edge computing. An EfficientNet-based fuzzy rank-based ensemble model was proposed to classify seven audio sounds, including human vocalizations and emergency vehicle sirens. This model was embedded in an Arduino Nano 33 BLE Sense development board. The audio files were respectively obtained from the CREMA-D dataset and Large Scale Audio dataset of emergency vehicle sirens on the road, with a total number of 8756 files. The seven audio sounds included neutral vocalization, anger vocalization, fear vocalization, happy vocalization, car horn sound, siren sound, and ambulance siren sound. The audio signal was converted into a spectrogram by the short-time Fourier transform as the feature. When one of the car horns, sirens, or ambulance sirens was detected, the wearable assistant device presented alarms through vibration and messages on the OLED panel. The performances of the EfficientNet-based fuzzy rank-based ensemble model in offline computing achieved an accuracy of 97.1%, precision of 97.79%, sensitivity of 96.8%, and specificity of 97.04%. In edge computing, the results were an accuracy of 95.2%, precision of 93.2%, sensitivity of 95.3%, and specificity of 95.1%. Thus, the proposed wearable assistant device has the potential benefit of helping the hearing impaired avoid traffic accidents.

Purpose: Hearing assistive technology (HAT) has been shown to be a viable solution to the speech-in-noise perception (SPIN) issue in children with autism spectrum disorder (ASD); however, little is known about its efficacy in tonal language speakers. This study compared sentence-level SPIN performance between Chinese children with ASD and neurotypical (NT) children and evaluated HAT use in improving SPIN performance and easing SPIN difficulty. Methods: Children with ASD (n=26) and NT children (n=19) aged 6-12 performed two adaptive tests in steady-state noise and three fixed-level tests in quiet and steady-state noise with and without using HAT. Speech recognition thresholds (SRT) and accuracy rates were assessed using adaptive and fixed-level tests, respectively. Parents or teachers of the ASD group completed a questionnaire regarding children’s listening difficulty under six circumstances before and after a ten-day trial period of HAT use. Results: Although the two groups of children had comparable SRTs, the ASD group showed a significantly lower SPIN accuracy rate than the NT group. Also, a significant impact of noise was found in the ASD group’s accuracy rate, but not in the NT group’s. There was a general improvement in the ASD group’s SPIN performance with HAT and a decrease in their listening difficulty ratings across all conditions after the device trial. Conclusion: The findings indicated inadequate SPIN in the ASD group using a relatively sensitive measure to gauge SPIN performance among children. The markedly increased accuracy rate in noise during HAT-on sessions for the ASD group confirmed the feasibility of HAT for improving SPIN performance in controlled laboratory settings, and the reduced post-use ratings of listening difficulty further confirmed the benefits of HAT use in daily scenarios.

This article propose a didactic, through games, tool based on information and communication technologies, in order to eliminate possible communication barriers and to promote the inclusion of students with hearing impairment in Physical Education classes. To this end, a dossier of traditional games has been developed. These are structured in turn into objectives, materials, organisation, graphic description and a QR code for each game. These codes are linked to different videos hosted on the YouTube platform, in which the explanation of the games, mentioned above, can be visualised graphically using sign language. The whole creative process is described in the article, as well as possible pedagogical applications of the use of the tools created for this purpose in other educational contexts.

Directed movement towards a target requires spatial working memory, including processing of sensory inputs and motivational drive. In a stimulus-driven, operant conditioning paradigm designed to train zebrafish, we present a pulse of light via LED’s and/or sounds via an underwater transducer. A webcam placed below a glass tank records fish swimming behavior. During operant conditioning, a fish must interrupt an infrared beam at one location to obtain a small food reward at the same or different location. A timing-gated interrupt activates robotic-arm and feeder stepper motors via custom software controlling a microprocessor (Arduino). “Ardulink”, a JAVA facility, implements Arduino-computer communication protocols. In this way, full automation of stimulus-conditioned directional swimming is achieved. Precise multi­day scheduling of training, including timing, location and intensity of stimulus parameters, and feeder control is accomplished via a user-friendly interface. Our training paradigm permits tracking of learning by monitoring, turning, location, response times and directional swimming of individual fish. This facilitates comparison of performance within and across a cohort of animals. We demonstrate the ability to train and test zebrafish using visual and auditory stimuli. Current methods used for associative conditioning often involve human intervention, which is labor intensive, stressful to animals, and introduces noise in the data. Our relatively simple yet flexible paradigm requires a simple apparatus and minimal human intervention. Our scheduling and control software and apparatus (NemoTrainer) can be used to screen neurologic drugs and test the effects of CRISPR­-based and optogenetic modification of neural circuits on sensation, locomotion, learning and memory.

There are numerous hazards found on the farms. Most of them are ignored, which might cause the farmer to pay later in terms of his ill health, potential injuries or death. The current article discusses some of the common issues such as dust and air quality concerns; environmental (weather) stressors and psychological stressors; noise and hearing protection; and tractor safety and seatbelt use. And finally, the recommendations to overcome the hazards are discussed.

Until the 1950s, developmental dyslexia was defined as a hereditary visual disability, selectively affecting reading without compromising oral or non-verbal reasoning skills. This changed radically after the development of the phonological theory of dyslexia; this not only ruled out any role for visual processing in its aetiology, but also cast doubt on the use of discrepancy between reading and reasoning skills as a criterion for diagnosing it. Here I argue that this theory is set at too high a cognitive level to be explanatory; we need to understand the pathophysiological visual and auditory mechanisms that cause children’s phonological problems. I discuss how the ‘magnocellular theory’ attempts to do this in terms of slowed and error prone temporal processing which leads to dyslexics’ defective visual and auditory sequencing when attempting to read. I attempt to deal with the criticisms of this theory and show how it leads to a number of successful ways of helping dyslexic children to overcome their reading difficulties.

Tightly regulated and robust mitochondrial activities are critical for normal hearing. Previously, we demonstrated that Fus1 KO mice with mitochondrial dysfunction exhibit premature hearing loss. Molecular analysis of the cochlea revealed hyperactivation of the mTOR pathway, oxidative stress, altered mitochondrial morphology and quantity, suggesting compromised energy sensing and production. Here, we investigated whether pharmacological modulation of metabolic pathways using rapamycin (RAPA) or 2-deoxy-D-glucose (2-DG) supplementation can protect against hearing loss in female Fus1 KO mice. Additionally, we aimed to identify mitochondria- and Fus1-dependent molecular pathways and processes critical for hearing. We found that inhibiting mTOR or activating alternative mitochondrial energetic pathways to glycolysis protected hearing in the mice. Comparative gene expression analysis revealed dysregulation of critical biological processes in the KO cochlea, including mitochondrial metabolism, neural and immune responses, and cochlear hypothalamic–pituitary–adrenal axis signaling system. RAPA and 2-DG mostly normalized these processes, although some genes showed a drug-specific response or no response at all. Interestingly, both drugs resulted in a pronounced upregulation of critical hearing-related genes not altered in the non-treated KO cochlea, including cytoskeletal and motor proteins, and Ca2+-linked transporters and voltage-gated channels. These findings suggest that pharmacological modulation of mitochondrial metabolism and bioenergetics may restore and activate processes critical for hearing, thereby protecting against hearing loss