Background: Waardenburg syndrome (WS) is the consequence of an inherited autosomal dominant mutation which causes the early degeneration of intermediate cells of cochlear stria vascularis (SV) and profound hearing loss. Patients with WS may also experience primary vestibular symptoms. Most of the current WS studies did not discuss the relationship between WS and abnormal vestibular function. Our study found that a spontaneous mutant pig showed profound hearing loss and depigmentation. MITF-M, a common gene mutation causes type WS which affect the development of the intermediate cell of SV, was then identified for animal modeling. Results: In this study, the degeneration of vestibular hair cells was found in pigs with MITF-M. The morphology of hair cells in vestibular organs of pigs was examined using electron microscopy from embryonic day E70 to postnatal two weeks. Significant hair cell loss in the mutant saccule was found in this study through E95 to P14. Conversely, there was no hair cell loss in either utricle or semi-circular canals. Conclusions: Our study suggested that MITF-M gene mutation only affects hair cells of the saccule, but has no effect on other vestibular organs. The study also indicated that the survival of cochlear and saccular hair cells was dependent on the potassium release from the cochlear SV, but hair cells of the utricle and semi-circular canals were independent on SV.

Conclusions: Cochlear microphonics (CMs) play an important role in the diagnosis of auditory neuropathy (AN). It is necessary and helpful to diagnose the sites-of-lesion in infants and children with AN by analyzing the patterns of CM amplitudes and I/O functions together. Objectives: To investigate the characteristics and clinical significance of CMs in the diagnosis of AN among infants and children. Methods: A total of 36 infants and children (16 males and 20 females) were divided into two groups. Group A included 15 children (30 ears) with auditory brainstem response (ABR) absent and distortion product otoacoustic emissions (DPOAEs) present and group B included 21 children (30 ears) with ABR absent and DPOAEs absent. Fifteen normal-hearing infants (30 ears) made up the control group. Click eliciting CMs were recorded at stimulus levels of 100, 90, 80, and 70 dB nHL for each ear using a button electrode placed at the top of the forehead. A tube-clamping method was used to distinguish CMs from artifacts, and an averaging algorithm was used to obtain a clear CM waveform. The time delay and amplitude of CMs were measured in both children with AN and normal-hearing infants on (C–R)/2 waveforms, and an I/O function curve for each group was plotted with the stimulating level as input and the CM amplitude as output. Results: The largest identifiable CMs were generally found between 0.5 and 0.8 ms after stimulation with mean delay of 0.63 ± 0.04 ms in both group A and the control group, and 0.63 ± 0.07 ms in group B. There was no significant difference between the AN group and the control group in CM time delay. There was no significant difference (p > 0.05) between group A (AN with OAEs present, 0.47 ± 0.15 μV) and the control group (0.45 ± 0.13 μV) in CM amplitude, while CM amplitudes in children with AN with DPOAEs absent (0.24 ± 0.08 μV) were significantly lower than those in either the control group or group A (p < 0.01). The amplitude of CMs reduced with stimulus intensity in all the subjects. There was obvious nonlinearity in group A and the control group, while there was a more linear tendency in amplitude increasing on the I/O function curve in group B.

The prevention and treatment of presbycusis becomes a serious health challenge and socio-economic problem.We performed a cross-sectional analysis of the audiometry results of all elderly patients w... 背景：老年性耳聋的防治已经成为一种严峻的健康和社会经济问题。目的：我们对所有在整个 2018 年期间来我科看病的老年患者的听力测量结果进行了横断面分析。材料与方法：共纳入60岁及以上的老年患者5520人, 分为三组：低龄老年组（60-74岁）、高龄老年组（75-89岁）、长寿组（90 岁以上）。分析所有患者的纯音测听结果。结果：5520例患者中, 低龄老年组4445例, 高龄老年组1029例...

Wide dynamic range compression (WDRC) and noise reduction both play important roles in hearing aids. WDRC provides level-dependent amplification so that the level of sound produced by the hearing aid falls between the hearing threshold and the highest comfortable level of the listener, while noise reduction reduces ambient noise with the goal of improving intelligibility and listening comfort and reducing effort. In most current hearing aids, noise reduction and WDRC are implemented sequentially, but this may lead to distortion of the amplitude modulation patterns of both the speech and the noise. This paper describes a deep learning method, called Neural-WDRC, for implementing both noise reduction and WDRC, employing a two-stage low-complexity network. The network initially estimates the noise alone and the speech alone. Fast-acting compression is applied to the estimated speech and slow-acting compression to the estimated noise, but with a controllable residual noise level to help the user to perceive natural environmental sounds. Neural-WDRC is frame-based, and the output of the current frame is determined only by the current and preceding frames. Neural-WDRC was compared with conventional slow- and fast-acting compression and with signal-to-noise ratio (SNR)-aware compression using objective measures and listening tests based on normal-hearing participants listening to signals processed to simulate the effects of hearing loss and hearing-impaired participants. The objective measures demonstrated that Neural-WDRC effectively reduced negative interactions of speech and noise in highly non-stationary noise scenarios. The listening tests showed that Neural-WDRC was preferred over the other compression methods for speech in non-stationary noises.

ECAPs are the summary of multiple neurons’ spikes which could be recorded by a bidirectional stimulation-recording system via the cochlear implant, with the artifact elimination paradigms of forward-masking subtraction paradigm or alternating polarity paradigm. Three kinds of FDA approved cochlear implants support ECAP testing. This article is to summarize the clinical application of ECAP test. ECAP test after insertion of electrode during implant operation has been widely used during cochlear implant surgery. In recent years, ECAP thresholds are also used to estimate the T levels and C levels helping programming. However, correlation between ECAP thresholds and psychophysical thresholds is affected by many factors. So far, ECAPs cannot yet be a good indicator of post-operative hearing and speech performance

Background: The SCN11A gene, encoded Nav1.9 TTX resistant sodium channels, is a main effector in peripheral inflammation related pain in nociceptive neurons. The role of SCN11A gene in the auditory system has not been well characterized. We therefore examined the expression of SCN11A in the murine cochlea, the morphological and physiological features of Nav1.9 knockout (KO) ICR mice. Results: Nav1.9 expression was found in the primary afferent endings beneath the inner hair cells (IHCs). The relative quantitative expression of Nav1.9 mRNA in modiolus of wild-type (WT) mice remains unchanged from P0 to P60. The number of presynaptic CtBP2 puncta in Nav1.9 KO mice was significantly lower than WT. In addition, the number of SGNs in Nav1.9 KO mice was also less than WT in the basal turn, but not in the apical and middle turns. There was no lesion in the somas and stereocilia of hair cells in Nav1.9 KO mice. Furthermore, Nav1.9 KO mice showed higher and progressive elevated ABR threshold at 16 kHz, and a significant increase in CAP thresholds. Conclusions: These data suggest a role of Nav1.9 in regulating the function of ribbon synapses and the auditory nerves. The impairment induced by Nav1.9 gene deletion mimics the characters of cochlear synaptopathy.