Objective·To evaluate the long-term performance and influencing factors of auditory and speech abilities, and social-life abilities in congenital deaf children with cochlear nerve deficiency (CND) after cochlear implant (CI) surgery.Methods·Twenty-one CND children with CI implantation in Shanghai Ninth People′s Hospital, Shanghai Jiao Tong University School of Medicine were assigned to CND group, and twenty children with extremely severe sensorineural hearing loss with normal inner ear structure matching implantation age and CI use time of the CND group were selected as control group. The aided hearing threshold, Infant-toddler Meaningful Auditory Integration Scale (IT-MAIS), LittlEARS Auditory Questionnaire (LEAQ), Categories of Auditory Performance-Ⅱ (CAP-Ⅱ), Meaningful Use of Speech Scale (MUSS), Speech Intelligibility Rating (SIR), closed and open disyllabic speech recognition test, and speech intelligibility test were used to hierarchically evaluate the subjects′ auditory and verbal abilities, and the Infant-junior Middle School Student′s Social Life Ability Scale (S-M Scale) was used to assess the subjects′ social-life ability.Results·The average aided hearing threshold of each frequency point in the CND group was significantly worse than that of the control group (all P<0.05). In the CND group, 90.5% of children′s aided hearing threshold of all frequencies entered the Chinese banana map. The auditory and speech abilities of the CND group were worse than those of the control group, and the significant differences between the two groups were found in the results of IT-MAIS, CAP-Ⅱ, MUSS, SIR, and closed and open disyllabic speech recognition (all P<0.05), but not in LEAQ. The social-life ability of the CND group was worse than that of the control group, and the difference was statistically significant (P<0.05). There was no correlation between implantation age, usage time of CI, auditory and language rehabilitation time, and auditory and speech ability of the CND group.Conclusion·CI is an effective auditory intervention method for children with CND, but their average auditory and speech abilities and social-life abilities are significantly behind those of CI users with normal inner ear structure. Therefore, for CND children, individualized communication strategies and rehabilitation training methods should be emphasized, and the cultivation of social-life ability and psychological guidance should be strengthened.

Objectives The objective of the study was to identify the acute high-intensity recreational noise-induced effects on auditory function, especially the cochlear synaptopathy-related audiological metrics, in humans with normal hearing. Methods This prospective cohort study enrolled 32 young adults (14 males and 18 females); the mean age was 24.1 ± 2.4 years (ranging from 20 to 29). All participants with normal hearing (audiometric thresholds ≤25 dB HL at frequencies of 0.25, 0.5, 1, 2, 3, 4, 6, and 8 kHz for both ears) had already decided to participate in the outdoor music festival. Participants were asked to measure the noise exposure dose and complete auditory examinations, including the air-conduction pure-tone audiometry (PTA), distortion product otoacoustic emission (DPOAE), contralateral suppression (CS) on transient evoked otoacoustic emission (TEOAE), auditory brainstem response (ABR) test and Mandarin Hearing in Noise Test (MHINT), at baseline and 1 day and 14 days after music festival noise exposure. Results The mean time of attending the music festival was 7.34 ± 0.63 h (ranging from 6.4 to 9.5), the mean time-weighted average (TWA) of noise exposure dose was 93.2 ± 2.39 dB(A) (ranging from 87.9 to 97.7). At neither 1 day nor 14 days post exposure, there were no statistically significant effects on PTA thresholds, DPOAE amplitudes, CS on TEOAEs, or MHINT signal-to-noise ratios (SNRs) of acute outdoor music festival noise exposure, regardless of sex. While the ABR wave I amplitudes significantly decreased at 1 day after exposure and recovered at 14 days after exposure, the exposed/unexposed ABR wave I amplitude ratio was significantly correlated with MHINT SNR change at 1 day after exposure, although it was not correlated with the noise exposure dose. Conclusion In young adults with normal hearing, we found the self-compared decrement of ABR wave I amplitudes at 1 day post acute recreational noise exposure at high intensity, which also contributes to the change in speech perceptual ability in noisy backgrounds. This study indicated that auditory electrophysiological metric changes might be a more sensitive and efficient indicator of noise-induced cochlear synaptic dysfunction in humans. More attention should be paid to the recreational noise-induced cochlear synaptopathy and auditory perceptual disorder.

This study was designed to investigate the effects of the aging process on peripheral and central auditory functions in adults with normal hearing. In this study, 149 participants with normal hearing were divided into four groups: aged 20-29, 30-39, 40-49 and 50-59 years for statistical purposes. Electrocochleography (EcochG), transient evoked otoacoustic emissions (TEOAE), Mandarin Hearing in Noise Test (MHINT) and the Gap Detection Test (GDT) were used. Our study found: (1) MHINT is significantly associated with aging (left ear R2=0.29, right ear R2=0.35). (2) TEOAE amplitude, TEOAE contralateral acoustic stimulation (CS) amplitude, EcochG action potential (AP), EcochG AP latency, EcochG summating potential (SP) and GDT progressively declined with age. (3) The EcochG SP/AP has no statistically significant difference among different age groups. (4) The peripheral auditory function of the right ear declines more slowly than that of the left ear. (5) Hypofunction of the central auditory system accelerates after age 40. The results demonstrate: (1) The age-related decline in the ability of speech recognition in a noisy environment may be the most sensitive indicator that reflects auditory function. (2) The decline of central auditory function is independent of peripheral auditory function, according to the auditory characteristics of the right ear. (3) Auditory function needs to be assessed individually to allow early prevention before age 40.

Background: The definition of notched audiogram for noise-induced hearing loss (NIHL) is presently based on clinical experience, but audiometric phenotypes of NIHL are highly heterogeneous. The data-driven clustering of subtypes could provide refined characteristics of NIHL, and help identify individuals with typical NIHL at diagnosis.Methods: This cross-sectional study initially recruited 12,218 occupational noise-exposed employees aged 18–60 years from two factories of a shipyard in Eastern China. Of these, 10,307 subjects with no history of otological injurie or disease, family history of hearing loss, or history of ototoxic drug use were eventually enrolled. All these subjects completed health behavior questionnaires, cumulative noise exposure (CNE) measurement, and pure-tone audiometry. We did data-driven cluster analysis (k-means clustering) in subjects with hearing loss audiograms (n = 6,599) consist of two independent datasets (n = 4,461 and n = 2,138). Multinomial logistic regression was performed to analyze the relevant characteristics of subjects with different audiometric phenotypes compared to those subjects with normal hearing audiograms (n = 3,708).Results: A total of 10,307 subjects (9,165 males [88.9%], mean age 34.5 [8.8] years, mean CNE 91.2 [22.7] dB[A]) were included, 3,708 (36.0%) of them had completely normal hearing, the other 6,599 (64.0%) with hearing loss audiograms were clustered into four audiometric phenotypes, which were replicable in two distinct datasets. We named the four clusters as the 4–6 kHz sharp-notched, 4–6 kHz flat-notched, 3–8 kHz notched, and 1–8 kHz notched audiogram. Among them, except for the 4–6 kHz flat-notched audiogram which was not significantly related to NIHL, the other three phenotypes with different relevant characteristics were strongly associated with noise exposure. In particular, the 4–6 kHz sharp-notched audiogram might be a typical subtype of NIHL.Conclusions: By data-driven cluster analysis of the large-scale noise-exposed population, we identified three audiometric phenotypes associated with distinct NIHL subtypes. Data-driven sub-stratification of audiograms might eventually contribute to the precise diagnosis and treatment of NIHL.

AbstractBackgroundTo determine the characteristics and sex differences of auditory perception and cochlear function in individuals with long-term occupational noise exposure.MethodsYoung workers with long-term occupational noise exposure from a shipyard were recruited in the current study as the hidden hearing loss (HHL) risk group. Age-matched office workers in the same shipyard who had no occupational noise-exposure history were enrolled in the control group. The auditory processing ability of speech-in-noise (SIN) score and gap detection threshold (GDT) were further examined by sex. The cochlear function of action potential (AP) and summating potential (SP)/AP values were tested and compared by sex and side. The correlation between the SIN score and cochlear function was studied by sex. The correlation between either auditory processing ability or cochlear function and occupational-noise working length (OWL) was also analysed in the HHL risk group.ResultsSignificantly decreased SIN scores and a higher GDT of the 4 kHz gap marker were only found in men in the HHL risk group. Although the hearing thresholds of the women in the HHL risk group were slightly but significantly worse than those of the women in the control group, no significant defects in auditory processing or temporal resolution were found between the two groups. Significantly decreased cochlear function and increased SP/AP values in the left ear were only found in men in the HHL risk group. Neither the AP amplitude nor the AP latency differed significantly between the two groups by sex. A correlation study indicated that only the correlation between the SIN score and the AP amplitude of the right ear in men was significant. No significant difference was found between the SIN score and cochlear function in women. The AP latency of the right ear was only significantly correlated with OWL in men.ConclusionIn long-term occupational working exposure individuals with normal hearing, defects in auditory processing, temporal resolution and cochlear function showed sex differences, none of which were significant in women. In men, a weak correlation between the SIN score and the AP amplitude of the right ear was found. There was only a weak correlation between OWL and the AP latency of the right ear in men. Our findings indicate men are more vulnerable to occupational noise than women. Considering the noise-exposure dose differences between the control and HHL risk groups, our measures are insensitive to cochlear synaptopathy in humans.