A novel mutation in the gene in a Chinese family with auditory neuropathy.

Gene therapy for monogenic auditory neuropathy (AN) has successfully improved hearing function in target gene-deficient mice. Accurate genetic diagnosis can not only clarify the etiology but also accurately locate the lesion site, providing a basis for gene therapy and guiding patient intervention and management strategies. In this study, we collected data from a family with a pair of sisters with prelingual deafness.

Coping with alpine habitats: genomic insights into the adaptation strategies of (Caprifoliaceae).

How plants find a way to thrive in alpine habitats remains largely unknown. Here we present a chromosome-level genome assembly for an alpine medicinal herb, (Caprifoliaceae), and 13 transcriptomes from other species of Dipsacales. We detected a whole-genome duplication event in that occurred prior to the diversification of Dipsacales.

A new active bone-conduction implant: surgical experiences and audiological outcomes in patients with bilateral congenital microtia.

Purpose: First-generation bone bridges (BBs) have demonstrated favorable safety and audiological benefits in patients with conductive hearing loss. However, studies on the effects of second-generation BBs are limited, especially among children. In this study, we aimed to explore the surgical and audiological effects of second-generation BBs in patients with bilateral congenital microtia.

Effects of Age on the Auditory Cortex During Speech Perception in Noise: Evidence From Functional Near-Infrared Spectroscopy.

Objectives: Age-related speech perception difficulties may be related to a decline in central auditory processing abilities, particularly in noisy or challenging environments. However, how the activation patterns related to speech stimulation in different noise situations change with normal aging has yet to be elucidated. In this study, we aimed to investigate the effects of noisy environments and aging on patterns of auditory cortical activation.

Aging effects on dual-route speech processing networks during speech perception in noise.

Healthy aging leads to complex changes in the functional network of speech processing in a noisy environment. The dual-route neural architecture has been applied to the study of speech processing. Although evidence suggests that senescent increases activity in the brain regions across the dorsal and ventral stream regions to offset reduced periphery, the regulatory mechanism of dual-route functional networks underlying such compensation remains largely unknown.

Comparisons of air-conduction hearing thresholds between manual and automated methods in a commercial audiometer.

Objective: To investigate the correlation of air-conduction thresholds between automated audiometry in a non-isolated environment and manual audiometry in participants with normal hearing and different degrees of hearing loss.

Methods: Eighty-three participants aged 11-88 years old underwent automated pure-tone audiometry in a non-acoustically isolated environment, and the results were compared with those of manual pure-tone audiometry performed in a standard acoustically isolated booth, with the order of testing randomised. Six frequencies of 250, 500, 1,000, 2000, 4,000 and 8,000 Hz were tested.

Features of the speech processing network in post- and prelingually deaf cochlear implant users.

The onset of hearing loss can lead to altered brain structure and functions. However, hearing restoration may also result in distinct cortical reorganization. A differential pattern of functional remodeling was observed between post- and prelingual cochlear implant users, but it remains unclear how these speech processing networks are reorganized after cochlear implantation.

Analysis of audiological outcomes of children referred from a universal newborn hearing screening program over 9 years in Beijing, China.

Universal newborn hearing screening (UNHS) and audiological diagnosis are crucial for children with congenital hearing loss (HL). The objective of this study was to analyze hearing screening techniques, audiological outcomes and risk factors among children referred from a UNHS program in Beijing. A retrospective analysis was performed in children who were referred to our hospital after failing UNHS during a 9-year period.

Abnormal white and gray matter functional connectivity is associated with cognitive dysfunction in presbycusis.

Presbycusis is characterized by high-frequency hearing loss and is closely associated with cognitive decline. Previous studies have observed functional reorganization of gray matter in presbycusis, but the information transmission between gray matter and white matter remains ill-defined. Using resting-state functional magnetic resonance imaging, we investigated differences in functional connectivity (GM-GM, WM-WM, and GM-WM) between 60 patients with presbycusis and 57 healthy controls.

Optimized concurrent hearing and genetic screening in Beijing, China: A cross-sectional study.

Concurrent screening has been proven to provide a comprehensive approach for management of congenital deafness and prevention of ototoxicity. The SLC26A4 gene is associated with late-onset hearing loss and is of great clinical concern. For much earlier detection of newborns with deafness-causing mutations in the SLC26A4 gene, the Beijing Municipal Government launched a chip for optimized genetic screening of 15 variants of 4 genes causing deafness based on a chip to screen for 9 variants of 4 genes, and 6 variants of the SLC26A4 gene have now been added.

The longitudinal relationship between hearing loss and cognitive decline in tonal language-speaking older adults in China.

Introduction: Previous longitudinal studies indicate that hearing loss and cognitive impairment are associated in non-tonal language-speaking older adults. This study aimed to investigate whether there is a longitudinal association between hearing loss and cognitive decline in older adults who speak a tonal language.

Methods: Chinese-speaking older adults aged 60 years and above were recruited for baseline and 12 month follow-up measurements.

The Relationship of Age-Related Hearing Loss with Cognitive Decline and Dementia in a Sinitic Language-Speaking Adult Population: A Systematic Review and Meta-Analysis.

Background And Objectives: Substantial evidence supports the association between untreated hearing loss, cognitive decline, and dementia in the non-tonal language-speaking population. Whether a similar association between hearing loss and cognitive decline and dementia exists in Sinitic tonal language-speaking people is yet to be elucidated. We aimed to systematically review the current evidence on the association between hearing loss and cognitive impairment/decline, and dementia in older adults who speak a Sinitic tonal language.

[Analysis of clinical audiological characteristics in 868 children referred from maternal and child institutions].

To investigate the clinical audiological characteristics of children referred from maternal and child institutions and analyze the high risk factors of hearing loss, so as to provide scientific basis for further improvement of children's ear and hearing care. The subjects of this study were 868 children who were referred by maternal and child institutions in Beijing to the otology outpatient of Beijing Tongren Hospital, Capital Medical University for hearing diagnosis. All subjects underwent acoustic immittance, auditory brainstem response, distortion products otoacoustic emission and other audiological tests.

Features of beta-gamma phase-amplitude coupling in cochlear implant users derived from EEG.

Cochlear implants (CIs) allow patients with severe to profound hearing loss to gain or regain their sense of hearing. However, the objective assessment of auditory rehabilitation in CI users remains a challenge. In particular, the utility of phase-amplitude coupling (PAC) for evaluating postoperative rehabilitation of CI users remains unknown.

The impact of untreated hearing loss on depression, anxiety, stress, and loneliness in tonal language-speaking older adults in China.

Background: Age-related hearing loss, mental health conditions, and loneliness commonly affect older adults. This study aimed to determine whether untreated hearing loss is independently associated with depression, anxiety, stress, and loneliness in tonal language-speaking older adults in China.

Study Design: Observational, cross-sectional study.

ENT characteristics and therapeutic results in multisystemic disorders of mitochondrial encephalomyopathy.

Here we report the evaluation of the frequency of subjective and objective otolaryngologic findings and therapeutic results in 32 patients with mitochondrial encephalomyopathy (MEM) from September 2001 to June 2021. Our analysis included studying the patients' family histories, the clinical manifestations of MEM, and the therapeutic effects of treatments. The patients' ages ranged from 2 to 77 years, with a median age of 12.

Clinical comparison of two automated audiometry procedures.

Objective: Automated pure-tone audiometry has been shown to provide similar hearing threshold estimates to conventional audiometry, but lower correlations were reported at high and low frequencies in audiometric tests than those of manual tests, while the correlations were better in the middle frequencies. In this paper, we used the same equipment and different test procedures for automated testing, and compared the results with manual test results.

Design: One hundred subjects aged 18-36 years were randomly divided into two groups to perform air-conduction pure-tone audiometry (0.

Aging Affects Subcortical Pitch Information Encoding Differently in Humans With Different Language Backgrounds.

Aging and language background have been shown to affect pitch information encoding at the subcortical level. To study the individual and compounded effects on subcortical pitch information encoding, Frequency Following Responses were recorded from subjects across various ages and language backgrounds. Differences were found in pitch information encoding strength and accuracy among the groups, indicating that language experience and aging affect accuracy and magnitude of pitch information encoding ability at the subcortical level.

Relationship of age-related hearing loss with cognitive decline and dementia in Sinitic tonal language-speaking populations: protocol for a systematic review and meta-analysis.

Introduction: Existing research on the potential association between age-related hearing loss (ARHL) and cognitive decline and dementia in tonal language-speaking populations is limited and inconsistent. There is psychophysiological evidence suggesting a tonal language background may be linked to enhanced general cognitive functions. We aim to systematically review the current evidence on the association between hearing loss and cognitive impairment/decline and dementia in older adults who speak a Sinitic tonal language, the most commonly spoken tonal language.

High-Frequency Cochlear Amplifier Dysfunction: A Dominating Contribution to the Cognitive-Ear Link.

The objective of this study was to investigate the role of the high-frequency cochlear dysfunction in the cognitive-ear link. Seventy-four presbycusis patients (PC group) and seventy-one age-, sex-, and education-level matched normal hearing controls (NH group) were recruited in this study. Participants underwent a battery of cognitive tests estimated by Montreal Cognitive Assessment (MoCA), Stroop Color-Word Interference Test (Stroop), Symbol Digit Modalities Test (SDMT), Auditory Verbal Learning Test (AVLT), and Trail-Making Test (TMT-A and B), as well as auditory tests including distortion product otoacoustic emission (DPOAE), pure tone (PT) thresholds, and speech reception thresholds (SRT).

The Relationship Between Hearing Loss and Cognitive Impairment in a Chinese Elderly Population: The Baseline Analysis.

The objective of the study was to investigate the association between untreated age-related hearing loss and cognitive impairment in Mandarin-speaking older adults living in China. Older adults (293; 111 males, = 70.33 ± 4.

[Mitochondrial encephalomyopathy involves ophthalmology otorhinolaryngology neurology and their clinical features].

This study aimed to provide better understanding of the otolaryngologic features, combined with ophthalmologic and neurologic characteristics in mitochondrial encephalomyopathy（MEM）, and to help ENT and auditory practitioner making correct diagnosis as well. Twenty-eight patients with MEM were enrolled between September 2001 and January 2020. Information about family histories and clinical symptoms was retrospectively analyzed.

Effects of Wireless Remote Microphone on Speech Recognition in Noise for Hearing Aid Users in China.

This study was aimed at evaluating improvements in speech-in-noise recognition ability as measured by signal-to-noise ratio (SNR) with the use of wireless remote microphone technology. These microphones transmit digital signals via radio frequency directly to hearing aids and may be a valuable assistive listening device for the hearing-impaired population of Mandarin speakers in China. Twenty-three adults (aged 19-80 years old) and fourteen children (aged 8-17 years old) with bilateral sensorineural hearing loss were recruited.

Neural Mechanisms of Hearing Recovery for Cochlear-Implanted Patients: An Electroencephalogram Follow-Up Study.

Background: Patients with severe profound hearing loss could benefit from cochlear implantation (CI). However, the neural mechanism of such benefit is still unclear. Therefore, we analyzed the electroencephalogram (EEG) and behavioral indicators of auditory function remodeling in patients with CI.