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Hearing Disorders: HELP
Articles by Wei Sun
Based on 25 articles published since 2010
(Why 25 articles?)
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Between 2010 and 2020, Wei Sun wrote the following 25 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Editorial Hearing loss and auditory plasticity. 2017

Sun, Wei / Yang, Shiming / Liu, Ke / Salvi, Richard J. ·Center for Hearing & Deafness, Department of Communicative Disorders and Science, State University of New York at Buffalo, Buffalo, NY, USA. · Department of Otolaryngology, Head and Neck Surgery, Chinese PLA General Hospital, Beijing, PR China. · Department of Otolaryngology, Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, PR China. ·Hear Res · Pubmed #28434581.

ABSTRACT: -- No abstract --

2 Review Effects of Non-traumatic Noise and Conductive Hearing Loss on Auditory System Function. 2019

Lauer, Amanda M / Dent, Micheal L / Sun, Wei / Xu-Friedman, Matthew A. ·Dept of Otolaryngology-HNS, Center for Hearing and Balance, Johns Hopkins University School of Medicine, United States. · Dept. Psychology, University at Buffalo, SUNY, United States. · Dept. Communicative Disorders and Sciences, University at Buffalo, SUNY, United States. · Dept. Biological Sciences, University at Buffalo, SUNY, United States. Electronic address: mx@buffalo.edu. ·Neuroscience · Pubmed #30685543.

ABSTRACT: The effects of traumatic noise-exposure and deafening on auditory system function have received a great deal of attention. However, lower levels of noise as well as temporary conductive hearing loss also have consequences on auditory physiology and hearing. Here we review how abnormal acoustic experience at early ages affects the ascending and descending auditory pathways, as well as hearing behavior.

3 Review Salicylate-induced cochlear impairments, cortical hyperactivity and re-tuning, and tinnitus. 2013

Chen, Guang-Di / Stolzberg, Daniel / Lobarinas, Edward / Sun, Wei / Ding, Dalian / Salvi, Richard. ·Center for Hearing & Deafness, SUNY at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. gchen7@buffalo.edu ·Hear Res · Pubmed #23201030.

ABSTRACT: High doses of sodium salicylate (SS) have long been known to induce temporary hearing loss and tinnitus, effects attributed to cochlear dysfunction. However, our recent publications reviewed here show that SS can induce profound, permanent, and unexpected changes in the cochlea and central nervous system. Prolonged treatment with SS permanently decreased the cochlear compound action potential (CAP) amplitude in vivo. In vitro, high dose SS resulted in a permanent loss of spiral ganglion neurons and nerve fibers, but did not damage hair cells. Acute treatment with high-dose SS produced a frequency-dependent decrease in the amplitude of distortion product otoacoustic emissions and CAP. Losses were greatest at low and high frequencies, but least at the mid-frequencies (10-20 kHz), the mid-frequency band that corresponds to the tinnitus pitch measured behaviorally. In the auditory cortex, medial geniculate body and amygdala, high-dose SS enhanced sound-evoked neural responses at high stimulus levels, but it suppressed activity at low intensities and elevated response threshold. When SS was applied directly to the auditory cortex or amygdala, it only enhanced sound evoked activity, but did not elevate response threshold. Current source density analysis revealed enhanced current flow into the supragranular layer of auditory cortex following systemic SS treatment. Systemic SS treatment also altered tuning in auditory cortex and amygdala; low frequency and high frequency multiunit clusters up-shifted or down-shifted their characteristic frequency into the 10-20 kHz range thereby altering auditory cortex tonotopy and enhancing neural activity at mid-frequencies corresponding to the tinnitus pitch. These results suggest that SS-induced hyperactivity in auditory cortex originates in the central nervous system, that the amygdala potentiates these effects and that the SS-induced tonotopic shifts in auditory cortex, the putative neural correlate of tinnitus, arises from the interaction between the frequency-dependent losses in the cochlea and hyperactivity in the central nervous system.

4 Article Hidden Age-Related Hearing Loss and Hearing Disorders: Current Knowledge and Future Directions. 2018

Salvi, Richard / Ding, Dalian / Jiang, Haiyan / Chen, Guang-Di / Greco, Antonio / Manohar, Senthilvelan / Sun, Wei / Ralli, Massimo. ·Center for Hearing and Deafness, 137 Cary Hall, University at Buffalo, Buffalo, NY, 14214 USA. · Department of Sense Organs, Sapienza University of Rome, Rome, Italy. · Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Rome, Italy. ·Hearing Balance Commun · Pubmed #30931204.

ABSTRACT: Age-related hearing loss, which affects roughly 35% of those over the age of 70, is the second most common disorder among the elderly. The severity of age related hearing loss may actually be worse if assessments are made under more realistic conditions, such as communicating in noise. Emerging data from humans and animal models suggest that damage to the inner hair cells and/or type I neurons, that relay sound information to the brain may contribute to hearing deficits in a noisy background. Data obtained from carboplatin-treated chinchillas suggest that tone-in-noise thresholds are a sensitive and frequency dependent method of detecting damage to the IHC/type I system. Therefore, tone detection thresholds measured in broadband noise may provide an efficient method of detecting the deficits in specific frequency regions. Preliminary data obtained in elderly subject with normal thresholds in quiet compared to young subjects illustrate the importance of repeating these measurements in broadband noise because thresholds in noise were worse for our elderly subjects than young subjects, even though both groups had similar hearing thresholds in quiet. N-acetyl cysteine supplementation which protects against inner hair cell loss in animal models, may represent a viable therapy for protecting the inner hair cell/type I neurons.

5 Article Temporary conductive hearing loss in early life impairs spatial memory of rats in adulthood. 2018

Zhao, Han / Wang, Li / Chen, Liang / Zhang, Jinsheng / Sun, Wei / Salvi, Richard J / Huang, Yi-Na / Wang, Ming / Chen, Lin. ·Hefei National Laboratory for Physical Sciences at the Microscale, School of Life Sciences, University of Science and Technology of China, Hefei, China. · Auditory Research Laboratory, University of Science and Technology of China, Hefei, China. · Department of Otolaryngology-Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan. · Center for Hearing and Deafness, State University of New York at Buffalo, Buffalo, New York. ·Brain Behav · Pubmed #29855161.

ABSTRACT: INTRODUCTION: It is known that an interruption of acoustic input in early life will result in abnormal development of the auditory system. Here, we further show that this negative impact actually spans beyond the auditory system to the hippocampus, a system critical for spatial memory. METHODS: We induced a temporary conductive hearing loss (TCHL) in P14 rats by perforating the eardrum and allowing it to heal. The Morris water maze and Y-maze tests were deployed to evaluate spatial memory of the rats. Electrophysiological recordings and anatomical analysis were made to evaluate functional and structural changes in the hippocampus following TCHL. RESULTS: The rats with the TCHL had nearly normal hearing at P42, but had a decreased performance with the Morris water maze and Y-maze tests compared with the control group. A functional deficit in the hippocampus of the rats with the TCHL was found as revealed by the depressed long-term potentiation and the reduced NMDA receptor-mediated postsynaptic current. A structural deficit in the hippocampus of those animals was also found as revealed the abnormal expression of the NMDA receptors, the decreased number of dendritic spines, the reduced postsynaptic density and the reduced level of neurogenesis. CONCLUSIONS: Our study demonstrates that even temporary auditory sensory deprivation in early life of rats results in abnormal development of the hippocampus and consequently impairs spatial memory in adulthood.

6 Article Functional Change in the Caudal Pontine Reticular Nucleus Induced by Age-Related Hearing Loss. 2018

Zhao, Ning / Alkharabsheh, Ana'am / Xu, Fei / Sun, Wei. ·Department of Otolaryngology-Head and Neck Surgery, The First Affiliated Hospital of China Medical University, Shenyang 110001, China. · Center for Hearing & Deafness, Department of Communicative Disorders and Science, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, USA. · Department of Hearing and Speech Sciences, Zhejiang Chinese Medical University, Hangzhou 310053, China. ·Neural Plast · Pubmed #29853848.

ABSTRACT: Increased acoustic startle responses (ASR), which represent reduced uncomfortable loudness level in humans, have been reported in middle-aged C57BL/6J mice with sensorineural hearing loss. Although neural plasticity changes in the central auditory system after the peripheral lesions were suggested to underlie this phenomenon, the neurological cause of exaggerated ASR is still not clear. In this study, the local field potentials and firing rates of the caudal pontine reticular nucleus (PnC), which plays a major role in the ASR pathway, were recorded in 2-month- and 6-month-old C57BL/6 J mice. Consistent with our previous studies, the amplitude of ASR increased, and the threshold of ASR decreased in the 6-month-old mice after developing 20-40 dB hearing loss. The PnC response induced by high-frequency stimuli (>20 kHz) decreased in the 6-month group, whereas the PnC response induced by low-frequency stimuli (<12 kHz) showed a significant increase in the 6-month group compared to the 2-month group. The enhancement of PnC response is similar to the ASR increase found in the 6-month-old C57 mice. Our results suggest that the high-frequency hearing loss caused an increase in PnC sensitivity in the C57 mice which may enhance ASRs.

7 Article Familial nonsyndromic hearing loss with incomplete partition type II caused by novel DSPP gene mutations. 2018

Li, Wan-Xin / Peng, Hong / Yang, Le / Hao, Qing-Qing / Sun, Wei / Ji, Fei / Guo, Wei-Wei / Yang, Shi-Ming. ·a Department of Otolaryngology, Head and Neck Surgery , Institute of Otolaryngology, Chinese PLA General Hospital , Beijing , China. · b Department of Otolaryngology Head and Neck Surgery , The First Affiliated Hospital of Soochow University , Suzhou , China. · c Department of Otolaryngology-Head and Neck Surgery , Guangdong No. 2 Provincial People's Hospital affiliated Southern Medical University , Guangzhou , China. · d Department of Communicative Disorders and Sciences , Center for Hearing and Deafness, the State University of New York at Buffalo , Buffalo , NY , USA. ·Acta Otolaryngol · Pubmed #29741433.

ABSTRACT: BACKGROUND: Familial nonsyndromic hearing loss (NSHL) with incomplete partition type II (IP-II) is a very rare condition. AIMS/OBJECTIVES: To determine the audiological feature, inheritance patterns and genetic etiology of familial NSHL with IP-II in a Chinese family with eight family members. MATERIAL AND METHODS: Clinical data were collected from all eight family members, selected deafness genes were sequenced in proband and whole genome sequencing of seven family members was performed. RESULTS: The proband were a pair of male nonidentical twins (III:1, III:2). Three patients in this family, including the twins and their father (II:1), were diagnosed with bilateral NSHL with IP-II, and no mutation was found in the genes of SLC26A4, GJB2, GJB3, mitochondrial 12S rRNA, and MITF. Whole genome sequencing data indicated de novo mutations of the gene DSPP, c.3085A > G and c.3087C > T, which resulted in p.N1029D and co-segregated with deafness phenotype, were the underlying genetic etiology. CONCLUSION AND SIGNIFICANCE: Familial NSHL with IP-II is extremely rare. In this family, de novo DSPP gene mutations, were considered to be the most probable genetic etiology. And this is the first report to reveal DSPP gene mutations leading to familial NSHL with IP-II.

8 Article Loss of sestrin 2 potentiates the early onset of age-related sensory cell degeneration in the cochlea. 2017

Zhang, Celia / Sun, Wei / Li, Ji / Xiong, Binbin / Frye, Mitchell D / Ding, Dalian / Salvi, Richard / Kim, Mi-Jung / Someya, Shinichi / Hu, Bo Hua. ·Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. Electronic address: celiazha@buffalo.edu. · Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. Electronic address: weisun@buffalo.edu. · University of Mississippi Medical Center, Department of Physiology and Biophysics, University of Mississippi, 2500 North State Street, Jackson, MS 39216, USA. Electronic address: jli3@umc.edu. · Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. Electronic address: xiongbb2015@126.com. · Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. Electronic address: mfrye@buffalo.edu. · Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. Electronic address: dding@buffalo.edu. · Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. Electronic address: salvi@buffalo.edu. · Departments of Aging and Geriatric Research, College of Medicine, University of Florida, 1600 SW Archer Road, Gainesville, FL 32610, USA. Electronic address: mijungkim@ufl.edu. · Departments of Aging and Geriatric Research, College of Medicine, University of Florida, 1600 SW Archer Road, Gainesville, FL 32610, USA. Electronic address: someya@ufl.edu. · Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 137 Cary Hall, Buffalo, NY 14214, USA. Electronic address: bhu@buffalo.edu. ·Neuroscience · Pubmed #28818524.

ABSTRACT: Sestrin 2 (SESN2) is a stress-inducible protein that protects tissues from oxidative stress and delays the aging process. However, its role in maintaining the functional and structural integrity of the cochlea is largely unknown. Here, we report the expression of SESN2 protein in the sensory epithelium, particularly in hair cells. Using C57BL/6J mice, a mouse model of age-related cochlear degeneration, we observed a significant age-related reduction in SESN2 expression in cochlear tissues that was associated with early onset hearing loss and accelerated age-related sensory cell degeneration that progressed from the base toward the apex of the cochlea. Hair cell death occurred by caspase-8 mediated apoptosis. Compared to C57BL/6J control mice, Sesn2 KO mice displayed enhanced expression of proinflammatory genes and activation of basilar membrane macrophages, suggesting that loss of SESN2 function provokes the immune response. Together, these results suggest that Sesn2 plays an important role in cochlear homeostasis and immune responses to stress.

9 Article Molecular mechanisms underlying the protective effects of hydrogen-saturated saline on noise-induced hearing loss. 2017

Chen, Liwei / Han, Mingkun / Lu, Yan / Chen, Daishi / Sun, Xuejun / Yang, Shiming / Sun, Wei / Yu, Ning / Zhai, Suoqiang. ·a Department of Otolaryngology Head and Neck Surgery , Chinese PLA General Hospital , Beijing , China. · b Department of Otolaryngology Head and Neck Surgery , Affiliated Fuzhou First Hospital of Fujian Medical University , Fujian , China. · c Department of Neurosurgery Erlangen , Medical Faculty of the Friedrich Alexander University of Erlangen-Numberg , Erlangen , Germany. · d Department of Diving Medicine, Faculty of Naval Medicine , Second Military Medical University , Shanghai , China. · e Department of Communicative Disorders and Sciences , University at Buffalo The State University of New York , Buffalo , NY , USA. ·Acta Otolaryngol · Pubmed #28549396.

ABSTRACT: OBJECTIVES: This study aimed to explore the molecular mechanism of the protective effects of hydrogen-saturated saline on NIHL. METHODS: Guinea pigs were divided into three groups: hydrogen-saturated saline; normal saline; and control. For saline administration, the guinea pigs were given daily abdominal injections 3 d before and 1 h before noise exposure. ABR were tested to examine cochlear physiology changes. The changes of 8-hydroxy-desoxyguanosine (8-HOdG), interleukin-1 (IL-1), interleukin-6 (IL-6), interleukin-10 (IL-10), tumor necrosis factor-α (TNF-α), intercellular cell adhesion molecule-1 (ICAM-1) and high mobility group box-1 protein (HMGB1) in the cochlea were also examined. RESULTS: The results showed that pre-treatment with hydrogen-saturated saline could significantly attenuate noise-induced hearing loss. The concentration of 8-HOdG was also significantly decreased in the hydrogen-saturated saline group compared with the normal saline group. After noise exposure, the concentrations of IL-1, IL-6, TNF-α, and ICAM-1 in the cochlea of guinea pigs in the hydrogen-saturated saline group were dramatically reduced compared to those in the normal saline group. The concentrations of HMGB-1 and IL-10 in the hydrogen-saturated saline group were significantly higher than in those in the normal saline group immediately and at 7 d after noise exposure. CONCLUSIONS: This study revealed for the first time the protective effects of hydrogen-saturated saline on noise-induced hearing loss (NIHL) are related to both the anti-oxidative activity and anti-inflammatory activity.

10 Article Hyperexcitability of inferior colliculus and acoustic startle reflex with age-related hearing loss. 2017

Xiong, Binbin / Alkharabsheh, Ana'am / Manohar, Senthilvelan / Chen, Guang-Di / Yu, Ning / Zhao, Xiaoming / Salvi, Richard / Sun, Wei. ·Department of Otolaryngology, Zhuhai Hospital Affiliated with Jinan University, Zhuhai, Guangdong, 519000, PR China; Center for Hearing & Deafness, Department of Communicative Disorders and Science, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, United States. · Center for Hearing & Deafness, Department of Communicative Disorders and Science, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, United States. · Research Institute of Otolaryngology, General Hospital of PLA, 28 Fuxing Road, Beijing, 100853, PR China. · Department of Otolaryngology, Zhuhai Hospital Affiliated with Jinan University, Zhuhai, Guangdong, 519000, PR China. · Center for Hearing & Deafness, Department of Communicative Disorders and Science, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, United States. Electronic address: weisun@buffalo.edu. ·Hear Res · Pubmed #28431308.

ABSTRACT: Chronic tinnitus and hyperacusis often develop with age-related hearing loss presumably due to aberrant neural activity in the central auditory system (CAS) induced by cochlear pathologies. However, the full spectrum of physiological changes that occur in the CAS as a result age-related hearing loss are still poorly understood. To address this issue, neurophysiological measures were obtained from the cochlea and the inferior colliculus (IC) of 2, 6 and 12 month old C57BL/6J mice, a mouse model for early age-related hearing loss. Thresholds of the compound action potentials (CAP) in 6 and 12 month old mice were significantly higher than in 2 month old mice. The sound driven and spontaneous firing rates of IC neurons, recorded with 16 channel electrodes, revealed mean IC thresholds of 22.8 ± 6.5 dB (n = 167) at 2 months, 37.9 ± 6.2 dB (n = 132) at 6 months and 47.1 ± 15.3 dB (n = 151) at 12 months of age consistent with the rise in CAP thresholds. The characteristic frequencies (CF) of IC neurons ranged from 3 to 32 kHz in 2 month old mice; the upper CF ranged decreased to 26 kHz and 16 kHz in 6 and 12 month old mice respectively. The percentage of IC neurons with CFs between 8 and 12 kHz increased from 36.5% in 2 month old mice, to 48.8% and 76.2% in 6 and 12 month old mice, respectively, suggesting a downshift of IC CFs due to the high-frequency hearing loss. The average spontaneous firing rate (SFRs) of all recorded neurons in 2 month old mice was 3.2 ± 2.5 Hz (n = 167). For 6 and 12 month old mice, the SFRs of low CF neurons (<8 kHz) was maintained at 3-6 spikes/s; whereas SFRs of IC neurons with CFs > 8 kHz increased to 13.0 ± 15.4 (n = 68) Hz at 6 months of age and then declined to 4.8 ± 7.4 (n = 110) spikes/s at 12 months of age. In addition, sound-evoked activity at suprathreshold levels at 6 months of age was much higher than at 2 and 12 months of age. To evaluate the behavioral consequences of sound evoked hyperactivity in the IC, the amplitude of the acoustic startle reflex was measured at 4, 8 and 16 kHz using narrow band noise bursts. Acoustic startle reflex amplitudes in 6 and 12 month old mice (n = 4) were significantly larger than 2 month old mice (n = 4) at 4 and 8 kHz, but not 16 kHz. The enhanced reflex amplitudes suggest that high-intensity, low-frequency sounds are perceived as louder than normal in 6 and 12 month old mice compared to 2 month olds. The increased spontaneous activity, particularly at 6 months, may be related to tinnitus whereas the increase in sound-evoked activity and startle reflex amplitudes may be related to hyperacusis.

11 Article Changes in Properties of Auditory Nerve Synapses following Conductive Hearing Loss. 2017

Zhuang, Xiaowen / Sun, Wei / Xu-Friedman, Matthew A. ·Department of Biological Sciences, University at Buffalo, State University of New York, Buffalo, New York 14260, and. · Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, University at Buffalo, State University of New York, Buffalo, New York 14260. · Department of Biological Sciences, University at Buffalo, State University of New York, Buffalo, New York 14260, and mx@buffalo.edu. ·J Neurosci · Pubmed #28077712.

ABSTRACT: Auditory activity plays an important role in the development of the auditory system. Decreased activity can result from conductive hearing loss (CHL) associated with otitis media, which may lead to long-term perceptual deficits. The effects of CHL have been mainly studied at later stages of the auditory pathway, but early stages remain less examined. However, changes in early stages could be important because they would affect how information about sounds is conveyed to higher-order areas for further processing and localization. We examined the effects of CHL at auditory nerve synapses onto bushy cells in the mouse anteroventral cochlear nucleus following occlusion of the ear canal. These synapses, called endbulbs of Held, normally show strong depression in voltage-clamp recordings in brain slices. After 1 week of CHL, endbulbs showed even greater depression, reflecting higher release probability. We observed no differences in quantal size between control and occluded mice. We confirmed these observations using mean-variance analysis and the integration method, which also revealed that the number of release sites decreased after occlusion. Consistent with this, synaptic puncta immunopositive for VGLUT1 decreased in area after occlusion. The level of depression and number of release sites both showed recovery after returning to normal conditions. Finally, bushy cells fired fewer action potentials in response to evoked synaptic activity after occlusion, likely because of increased depression and decreased input resistance. These effects appear to reflect a homeostatic, adaptive response of auditory nerve synapses to reduced activity. These effects may have important implications for perceptual changes following CHL. SIGNIFICANCE STATEMENT: Normal hearing is important to everyday life, but abnormal auditory experience during development can lead to processing disorders. For example, otitis media reduces sound to the ear, which can cause long-lasting deficits in language skills and verbal production, but the location of the problem is unknown. Here, we show that occluding the ear causes synapses at the very first stage of the auditory pathway to modify their properties, by decreasing in size and increasing the likelihood of releasing neurotransmitter. This causes synapses to deplete faster, which reduces fidelity at central targets of the auditory nerve, which could affect perception. Temporary hearing loss could cause similar changes at later stages of the auditory pathway, which could contribute to disorders in behavior.

12 Article Early age noise exposure increases loudness perception - A novel animal model of hyperacusis. 2017

Alkharabsheh, Ana'am / Xiong, Fen / Xiong, Binbin / Manohar, Senthilvelan / Chen, Guangdi / Salvi, Richard / Sun, Wei. ·Center for Hearing and Deafness, State University of New York at Buffalo, 137 Cary Hall, 3435 Main Street, Buffalo, NY 14241, USA; Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 122 Cary Hall, 3435 Main Street, Buffalo, NY 14214, USA. · Center for Hearing and Deafness, State University of New York at Buffalo, 137 Cary Hall, 3435 Main Street, Buffalo, NY 14241, USA. · Center for Hearing and Deafness, State University of New York at Buffalo, 137 Cary Hall, 3435 Main Street, Buffalo, NY 14241, USA; Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 122 Cary Hall, 3435 Main Street, Buffalo, NY 14214, USA. Electronic address: weisun@buffalo.edu. ·Hear Res · Pubmed #27746216.

ABSTRACT: The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 h) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies. Loudness was evaluated using sound reaction time and loudness response functions in an operant conditioning-based behavioral task using narrow-band noise (40-110 dB SPL, centered at 2, 4 and 12 kHz). Interestingly, the sound reaction time of the noise group was significantly shorter than the control group at supra-threshold levels. The average reaction time was less than 100 ms in the noise group at 100 dB SPL, which was three times shorter than the control group. Our results indicate that early noise-induced hearing loss leads to a significant increase of loudness, a behavior indicative of hyperacusis. Our results are consistent with clinical reports suggesting that hearing loss at an early age is a significant risk factor for hyperacusis.

13 Article Apical hair cell degeneration causes the increase in the amplitude of summating potential. 2016

Wang, Dayong / Xiong, Binbin / Xiong, Fen / Chen, Guang-Di / Hu, Bo Hua / Sun, Wei. ·a Department of Otolaryngology , Chinese PLA General Hospital , Beijing , PR China. · b Center for Hearing & Deafness, Department of Communicative Disorders and Science , State University of New York at Buffalo , Buffalo , NY , USA. · c Department of Otolaryngology , Zhuhai Hospital Affiliated with Jinan University , Guangdong , PR China. ·Acta Otolaryngol · Pubmed #27424625.

ABSTRACT: CONCLUSION: This study indicates that the lesion of hair cells in the apical turn of the cochlea can cause the change in the summating potential (SP)/Compound potential (CAP) ratio. OBJECTIVES: Electrocochleography is a valuable clinic test for diagnosis of cochlear pathologies and the ratio of SP to CAP has been used to identify Meniere's disease. However, it remains controversial whether the increase of the SP/CAP ratio represents exclusively the endolymphatic hydrops. METHOD: This study measured the SP and CAP in mice that displayed outer hair cell (OHC) degeneration in the apical section of the cochlea as their age increased. RESULTS: As compared with the mice aged 8-10 months, the 24-month old mice displayed a significant increase in the amplitude of SP at 12-16 kHz. This result suggests that the degeneration of OHCs in the apical turn leads to the increase of the + SP at the middle frequencies. In contrast, the aging mice did not have a significant change in the CAP amplitude at super-threshold levels.

14 Article A de novo silencer causes elimination of MITF-M expression and profound hearing loss in pigs. 2016

Chen, Lei / Guo, Weiwei / Ren, Lili / Yang, Mingyao / Zhao, Yaofeng / Guo, Zongyi / Yi, Haijin / Li, Mingzhou / Hu, Yiqing / Long, Xi / Sun, Boyuan / Li, Jinxiu / Zhai, Suoqiang / Zhang, Tinghuan / Tian, Shilin / Meng, Qingyong / Yu, Ning / Zhu, Dan / Tang, Guoqing / Tang, Qianzi / Ren, Liming / Liu, Ke / Zhang, Shihua / Che, Tiandong / Yu, Zhengquan / Wu, Nan / Jing, Lan / Zhang, Ran / Cong, Tao / Chen, Siqing / Zhao, Yiqiang / Zhang, Yue / Bai, Xiaoqing / Guo, Ying / Zhao, Lidong / Zhang, Fengming / Zhao, Hui / Zhang, Liang / Hou, Zhaohui / Zhao, Jiugang / Li, Jianan / Zhang, Lijuan / Sun, Wei / Zou, Xiangang / Wang, Tao / Ge, Liangpeng / Liu, Zuohua / Hu, Xiaoxiang / Wang, Jingyong / Yang, Shiming / Li, Ning. ·State Key Laboratory for Agrobiotechnology, College of Biological Sciences, National Engineering Laboratory for Animal Breeding, China Agricultural University, Beijing, 100193, China. · Key Laboratory of Pig Industry Sciences (Ministry of Agriculture), Chongqing Academy of Animal Science, Chongqing, 402460, China. · Department of Otolaryngology, Head & Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, 100853, China. · Institute of Animal Genetics and Breeding, College of Animal Science and Technology, Sichuan Agricultural University, Ya'an, Sichuan, 625014, China. · Department of Communicative Disorders and Sciences, Center for Hearing and Deafness, State University of New York at Buffalo, Buffalo, New York, USA. · Key Laboratory of Pig Industry Sciences (Ministry of Agriculture), Chongqing Academy of Animal Science, Chongqing, 402460, China. kingyou@vip.sina.com. · Department of Otolaryngology, Head & Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, 100853, China. yangsm301@263.net. · State Key Laboratory for Agrobiotechnology, College of Biological Sciences, National Engineering Laboratory for Animal Breeding, China Agricultural University, Beijing, 100193, China. ninglcau@cau.edu.cn. ·BMC Biol · Pubmed #27349893.

ABSTRACT: BACKGROUND: Genesis of novel gene regulatory modules is largely responsible for morphological and functional evolution. De novo generation of novel cis-regulatory elements (CREs) is much rarer than genomic events that alter existing CREs such as transposition, promoter switching or co-option. Only one case of de novo generation has been reported to date, in fish and without involvement of phenotype alteration. Yet, this event likely occurs in other animals and helps drive genetic/phenotypic variation. RESULTS: Using a porcine model of spontaneous hearing loss not previously characterized we performed gene mapping and mutation screening to determine the genetic foundation of the phenotype. We identified a mutation in the non-regulatory region of the melanocyte-specific promoter of microphthalmia-associated transcription factor (MITF) gene that generated a novel silencer. The consequent elimination of expression of the MITF-M isoform led to early degeneration of the intermediate cells of the cochlear stria vascularis and profound hearing loss, as well as depigmentation, all of which resemble the typical phenotype of Waardenburg syndrome in humans. The mutation exclusively affected MITF-M and no other isoforms. The essential function of Mitf-m in hearing development was further validated using a knock-out mouse model. CONCLUSIONS: Elimination of the MITF-M isoform alone is sufficient to cause deafness and depigmentation. To our knowledge, this study provides the first evidence of a de novo CRE in mammals that produces a systemic functional effect.

15 Article Adenosine Triphosphate (ATP) Inhibits Voltage-Sensitive Potassium Currents in Isolated Hensen's Cells and Nifedipine Protects Against Noise-Induced Hearing Loss in Guinea Pigs. 2016

Ye, Rui / Liu, Jun / Jia, Zhiying / Wang, Hongyang / Wang, YongAn / Sun, Wei / Wu, Xuan / Zhao, Zhifei / Niu, Baolong / Li, Xingqi / Dai, Guanghai / Li, Jianxiong. ·Department of Radiation Oncology, Chinese PLA General Hospital, Beijing, China (mainland). · Department of Otolaryngology Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, China (mainland). · , Xinjiang Cancer Hospital, Urumqi, Xinjiang, China (mainland). · , Academy of Military Medical Sciences, Beijing, China (mainland). · Center for Hearing & Deafness, State University of New York (SUNY) at Buffalo, Buffalo, NY, American Samoa. · Department of Radiation Oncology, Hainan Branch of Chinese PLA General Hospital, Sanya, Hainan, China (mainland). · Department of Oncology, Chinese PLA General Hospital, Beijing, China (mainland). ·Med Sci Monit · Pubmed #27292522.

ABSTRACT: BACKGROUND There is increasing evidence that adenosine triphosphate (ATP), a well-known neurotransmitter and neuromodulator in the central nervous system, plays an important role as an extracellular chemical messenger in the cochlea. MATERIAL AND METHODS Using a whole-cell recording technique, we studied the effects of ATP on isolated Hensen's cells, which are supporting cells in the cochlea, to determine if they are involved in the transduction of ions with hair cells. RESULTS ATP (0.1-10 µM) reduced the potassium current (IK+) in the majority of the recorded Hensen's cells (21 out of 25 cells). An inward current was also induced by high concentrations of ATP (100 µM to 10 mM), which was reversibly blocked by 100 µM suramin (a purinergic antagonist) and blocked by nifedipine (an L-type calcium channel blocker). After the cochleas were perfused with artificial perilymph solutions containing nifedipine and exposed to noise, the amplitude increase in the compound action potential (CAP) threshold and the reduction in cochlear microphonics was lower than when they were exposed to noise alone. CONCLUSIONS Our results suggest that ATP can block IK+ channels at a low concentration and induce an inward Ca2+ current at high concentrations, which is reversed by purinergic receptors. Nifedipine may have a partially protective effect on noise-induced hearing loss (NIHL).

16 Article Temperature sensitive auditory neuropathy. 2016

Zhang, Qiujing / Lan, Lan / Shi, Wei / Yu, Lan / Xie, Lin-Yi / Xiong, Fen / Zhao, Cui / Li, Na / Yin, Zifang / Zong, Liang / Guan, Jing / Wang, Dayong / Sun, Wei / Wang, Qiuju. ·Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: zqj99@163.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: ll-301@263.net. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: sw0215@sohu.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: anna_lan@126.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: xielinyi1234@126.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: 870505713@qq.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: zhaocui619@126.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: lna301@126.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: Yinbug@163.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: cell-099@163.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: ggy3u@126.com. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: wangdy301@126.com. · Department of Communicative Disorders & Sciences, Center for Hearing and Deafness, University at Buffalo, The State University of New York, Buffalo, NY 14215, USA. Electronic address: weisun@buffalo.edu. · Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China. Electronic address: wqcr@263.net. ·Hear Res · Pubmed #26778470.

ABSTRACT: Temperature sensitive auditory neuropathy is a very rare and puzzling disorder. In the present study, we reported three unrelated 2 to 6 year-old children who were diagnosed as auditory neuropathy patients who complained of severe hearing loss when they had fever. Their hearing thresholds varied from the morning to the afternoon. Two of these patients' hearing improved with age, and one patient received positive results from cochlear implant. Genetic analysis revealed that these three patients had otoferlin (OTOF) homozygous or compound heterozygous mutations with the genotypes c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, or c.2382_2383delC/c.1621G>A, respectively. Our study suggests that these gene mutations may be the cause of temperature sensitive auditory neuropathy. The long term follow up results suggest that the hearing loss in this type of auditory neuropathy may recover with age.

17 Article SMAD4 Defect Causes Auditory Neuropathy Via Specialized Disruption of Cochlear Ribbon Synapses in Mice. 2016

Liu, Ke / Ji, Fei / Yang, Guan / Hou, Zhaohui / Sun, Jianhe / Wang, Xiaoyu / Guo, Weiwei / Sun, Wei / Yang, Weiyan / Yang, Xiao / Yang, Shiming. ·Department of Otolaryngology, Head and Neck Surgery, The Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China. · The State Key Laboratory of Proteomics, Genetics Laboratory of Development and Disease, Institute of Biotechnology, AMMS, Beijing, 100071, China. · Department of Communicative Disorders and Sciences, The State University of New York at Buffalo, Buffalo, NY, 14214, USA. · The State Key Laboratory of Proteomics, Genetics Laboratory of Development and Disease, Institute of Biotechnology, AMMS, Beijing, 100071, China. yangx@bmi.ac.cn. · Department of Otolaryngology, Head and Neck Surgery, The Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853, China. yangsm301@263.net. ·Mol Neurobiol · Pubmed #26491026.

ABSTRACT: More than 100 genes have been associated with deafness. However, SMAD4 is rarely considered a contributor to deafness in humans, except for its well-defined role in cell differentiation and regeneration. Here, we report that a SMAD4 defect in mice can cause auditory neuropathy, which was defined as a mysterious hearing and speech perception disorder in human for which the genetic background remains unclear. Our study showed that a SMAD4 defect induces failed formation of cochlear ribbon synapse during the earlier stage of auditory development in mice. Further investigation found that there are nearly normal morphology of outer hair cells (OHCs) and post-synapse spiral ganglion nerves (SGNs) in SMAD4 conditional knockout mice (cKO); however, a preserved distortion product of otoacoustic emission (DPOAE) and cochlear microphonic (CM) still can be evoked in cKO mice. Moreover, a partial restoration of hearing detected by electric auditory brainstem response (eABR) has been obtained in the cKO mice using electrode stimuli toward auditory nerves. Additionally, the ribbon synapses in retina are not affected by this SMAD4 defect. Thus, our findings suggest that this SMAD4 defect causes auditory neuropathy via specialized disruption of cochlear ribbon synapses.

18 Article Scopolamine attenuates auditory cortex response. 2015

Deng, Anchun / Liang, Xiaojun / Sun, Yuchen / Xiang, Yanghong / Yang, Junjie / Yan, Jingjing / Sun, Wei. ·a 1 Department of Otolaryngology Head and Neck Surgery, Xinqiao Hospital, Third Military Medical University , Chongqing, PR China. · b 2 Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo , Buffalo, NY, USA. ·Acta Otolaryngol · Pubmed #26144861.

ABSTRACT: CONCLUSION: Scopolamine, a tropane alkaloid drug that mainly acts as an antagonist of muscarinic acetylcholine receptors, was found to reduce the local field potentials (LFP) of auditory cortex (AC) evoked by tone and gap-offsets whose effects may compensate the cortical hyperexcitability related to tinnitus. OBJECTIVE: To study the effects of scopolamine on the AC and the inferior colliculus (IC) of awake rats in order to understand scopolamine's effect on tinnitus and gap detection. METHOD: Silent gaps (duration varied from 2-100 ms) embedded in otherwise continuous noise were used to elicit AC and IC response. Gap evoked AC and IC field potentials were recorded from awake rats before and after treatment of scopolamine (3 mg/kg, i.m.). RESULTS: Acute injection of scopolamine (3 mg/kg, i.m.) induced a significant reduction of the AC response, but not the IC response, to the offset of the gaps embedded in white noise. The results suggest that scopolamine may reduce AC neural synchrony.

19 Article Mutations in apoptosis-inducing factor cause X-linked recessive auditory neuropathy spectrum disorder. 2015

Zong, Liang / Guan, Jing / Ealy, Megan / Zhang, Qiujing / Wang, Dayong / Wang, Hongyang / Zhao, Yali / Shen, Zhirong / Campbell, Colleen A / Wang, Fengchao / Yang, Ju / Sun, Wei / Lan, Lan / Ding, Dalian / Xie, Linyi / Qi, Yue / Lou, Xin / Huang, Xusheng / Shi, Qiang / Chang, Suhua / Xiong, Wenping / Yin, Zifang / Yu, Ning / Zhao, Hui / Wang, Jun / Wang, Jing / Salvi, Richard J / Petit, Christine / Smith, Richard J H / Wang, Qiuju. ·Department of Otolaryngology-Head and Neck Surgery, Institute of Otolaryngology, PLA General Hospital, Beijing, China. · Molecular Otolaryngology and Renal Research Laboratories and the Department of Otolaryngology-Head and Neck Surgery, University of Iowa, Iowa City, Iowa, USA Department of Otolaryngology-Head & Neck Surgery, Stanford University School of Medicine, Stanford, California, USA. · Department of Otolaryngology-Head and Neck Surgery, Institute of Otolaryngology, PLA General Hospital, Beijing, China Beijing Institute of Otorhinolaryngology, Beijing Tongren Hospital, Capital Medical University, Beijing, China. · National Institute of Biological Sciences, Beijing, China. · Molecular Otolaryngology and Renal Research Laboratories and the Department of Otolaryngology-Head and Neck Surgery, University of Iowa, Iowa City, Iowa, USA. · Department of Communicative Disorders & Sciences, Center for Hearing and Deafness, University at Buffalo, Buffalo, New York, USA. · Department of Radiology, PLA General Hospital, Beijing, China. · Department of Neurology, PLA General Hospital, Beijing, China. · Key Laboratory of Mental Health, Institute of Psychology, Chinese Academy of Sciences, Beijing, China. · BGI-Shenzhen, Shenzhen, China. · Unité de Génétique et Physiologie de l'Audition, Institut Pasteur, Collège de France, Paris, France. ·J Med Genet · Pubmed #25986071.

ABSTRACT: BACKGROUND: Auditory neuropathy spectrum disorder (ANSD) is a form of hearing loss in which auditory signal transmission from the inner ear to the auditory nerve and brain stem is distorted, giving rise to speech perception difficulties beyond that expected for the observed degree of hearing loss. For many cases of ANSD, the underlying molecular pathology and the site of lesion remain unclear. The X-linked form of the condition, AUNX1, has been mapped to Xq23-q27.3, although the causative gene has yet to be identified. METHODS: We performed whole-exome sequencing on DNA samples from the AUNX1 family and another small phenotypically similar but unrelated ANSD family. RESULTS: We identified two missense mutations in AIFM1 in these families: c.1352G>A (p.R451Q) in the AUNX1 family and c.1030C>T (p.L344F) in the second ANSD family. Mutation screening in a large cohort of 3 additional unrelated families and 93 sporadic cases with ANSD identified 9 more missense mutations in AIFM1. Bioinformatics analysis and expression studies support this gene as being causative of ANSD. CONCLUSIONS: Variants in AIFM1 gene are a common cause of familial and sporadic ANSD and provide insight into the expanded spectrum of AIFM1-associated diseases. The finding of cochlear nerve hypoplasia in some patients was AIFM1-related ANSD implies that MRI may be of value in localising the site of lesion and suggests that cochlea implantation in these patients may have limited success.

20 Article Tinnitus and hyperacusis involve hyperactivity and enhanced connectivity in auditory-limbic-arousal-cerebellar network. 2015

Chen, Yu-Chen / Li, Xiaowei / Liu, Lijie / Wang, Jian / Lu, Chun-Qiang / Yang, Ming / Jiao, Yun / Zang, Feng-Chao / Radziwon, Kelly / Chen, Guang-Di / Sun, Wei / Krishnan Muthaiah, Vijaya Prakash / Salvi, Richard / Teng, Gao-Jun. ·Jiangsu Key Laboratory of Molecular Imaging and Functional Imaging, Department of Radiology, Zhongda Hospital, Medical School, Southeast University, Nanjing, China. · Department of Physiology, Southeast University, Nanjing, China. · Center for Hearing and Deafness, University at Buffalo, The State University of New York, Buffalo, United States. ·Elife · Pubmed #25962854.

ABSTRACT: Hearing loss often triggers an inescapable buzz (tinnitus) and causes everyday sounds to become intolerably loud (hyperacusis), but exactly where and how this occurs in the brain is unknown. To identify the neural substrate for these debilitating disorders, we induced both tinnitus and hyperacusis with an ototoxic drug (salicylate) and used behavioral, electrophysiological, and functional magnetic resonance imaging (fMRI) techniques to identify the tinnitus-hyperacusis network. Salicylate depressed the neural output of the cochlea, but vigorously amplified sound-evoked neural responses in the amygdala, medial geniculate, and auditory cortex. Resting-state fMRI revealed hyperactivity in an auditory network composed of inferior colliculus, medial geniculate, and auditory cortex with side branches to cerebellum, amygdala, and reticular formation. Functional connectivity revealed enhanced coupling within the auditory network and segments of the auditory network and cerebellum, reticular formation, amygdala, and hippocampus. A testable model accounting for distress, arousal, and gating of tinnitus and hyperacusis is proposed.

21 Article Loudness perception affected by high doses of salicylate--a behavioral model of hyperacusis. 2014

Zhang, Chao / Flowers, Elizabeth / Li, Jun-Xu / Wang, Qiuju / Sun, Wei. ·Department of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, PR China; Center for Hearing & Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, United States. · Center for Hearing & Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, United States. · Department of Pharmacology and Toxicology, the State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, United States. · Department of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, PR China. Electronic address: wqcr301@sina.com. · Center for Hearing & Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, United States. Electronic address: weisun@buffalo.edu. ·Behav Brain Res · Pubmed #24882611.

ABSTRACT: The major side-effects of high doses of salicylate include sensorial hearing loss and tinnitus. Although salicylate decreases cochlear output, it enhances the evoked potentials recorded from the central auditory system (CAS), suggesting an increase to sound sensitivity. However, the loudness change after salicylate administration has not yet been directly measured. In this study, we established an operant conditioning based behavioral task in rats and measured their loudness perception changes before and after high doses of salicylate injection (250 mg/kg, i.p.). We found that high doses of salicylate induced a significant increase to loudness response in 40% of the rats (out of 20 rats), suggesting a hyperacusis behavior. In another 40% of rats, a rapid increase of loudness response was detected, suggesting loudness recruitment. The reaction time of the rats was also measured during the loudness tests before and after salicylate exposure. The reaction time level functions are highly correlated to the loudness response functions. Our studies confirmed that increased sound sensitivity, which is commonly seen in patients with tinnitus and hyperacusis, can be induced by high doses of salicylate. This loudness change induced by salicylate may be related with hypersensitivity in the CAS.

22 Article Loudness perception affected by early age hearing loss. 2014

Sun, Wei / Fu, Qiang / Zhang, Chao / Manohar, Senthilvelan / Kumaraguru, Anand / Li, Ji. ·Center for Hearing & Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, USA. Electronic address: weisun@buffalo.edu. · Department of Pharmacology and Toxicology, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, USA; Department of Biochemistry and Molecular Biology, West China School of Preclinical and Forensic Medicine, Sichuan University, Chengdu 610041, PR China. · Center for Hearing & Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, USA; Department of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, PR China. · Center for Hearing & Deafness, Department of Communicative Disorders and Sciences, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, USA. · Department of Pharmacology and Toxicology, State University of New York at Buffalo, 3435 Main Street, Buffalo, NY 14214, USA. ·Hear Res · Pubmed #24747532.

ABSTRACT: Tinnitus and hyperacusis, commonly seen in adults, are also reported in children. Although clinical studies found children with tinnitus and hyperacusis often suffered from recurrent otitis media, there is no direct study on how temporary hearing loss in the early age affects the sound loudness perception. In this study, sound loudness changes in rats affected by perforation of the tympanic membranes (TM) have been studied using an operant conditioning based behavioral task. We detected significant increases of sound loudness and susceptibility to audiogenic seizures (AGS) in rats with bilateral TM damage at postnatal 16 days. As increase to sound sensitivity is commonly seen in hyperacusis and tinnitus patients, these results suggest that early age hearing loss is a high risk factor to induce tinnitus and hyperacusis in children. In the TM damaged rats, we also detected a reduced expression of GABA receptor δ and α6 subunits in the inferior colliculus (IC) compared to the controls. Treatment of vigabatrin (60 mg/kg/day, 7-14 days), an anti-seizure drug that inhibits the catabolism of GABA, not only blocked AGS, but also significantly attenuated the loudness response. Administration of vigabatrin following the early age TM damage could even prevent rats from developing AGS. These results suggest that TM damage at an early age may cause a permanent reduction of GABA tonic inhibition which is critical towards the maintenance of normal loudness processing of the IC. Increasing GABA concentration during the critical period may alleviate the impairment in the brain induced by early age hearing loss.

23 Article Round window closure affects cochlear responses to suprathreshold stimuli. 2013

Cai, Qunfeng / Whitcomb, Carolyn / Eggleston, Jessica / Sun, Wei / Salvi, Richard / Hu, Bo Hua. ·Center for Hearing and Deafness, State University of New York at Buffalo, Buffalo, New York. ·Laryngoscope · Pubmed #24114866.

ABSTRACT: OBJECTIVES/HYPOTHESIS: The round window acts as a vent for releasing inner ear pressure and facilitating basilar membrane vibration. Loss of this venting function affects cochlear function, which leads to hearing impairment. In an effort to identify functional changes that might be used in clinical diagnosis of round window atresia, the current investigation was designed to examine how the cochlea responds to suprathreshold stimuli following round window closure. STUDY DESIGN: Prospective, controlled, animal study. METHODS: A rat model of round window occlusion (RWO) was established. With this model, the thresholds of auditory brainstem responses (ABR) and the input/output (IO) functions of distortion product otoacoustic emissions (DPOAEs) and acoustic startle responses were examined. RESULTS: Round window closure caused a mild shift in the thresholds of the auditory brainstem response (13.5 ± 9.1 dB). It also reduced the amplitudes of the distortion product otoacoustic emissions and the slope of the input/output functions. This peripheral change was accompanied by a significant reduction in the amplitude, but not the threshold, of the acoustic startle reflex, a motor response to suprathreshold sounds. CONCLUSIONS: In addition to causing mild increase in the threshold of the auditory brainstem response, round window occlusion reduced the slopes of both distortion product otoacoustic emissions and startle reflex input/output functions. These changes differ from those observed for typical conductive or sensory hearing loss, and could be present in patients with round window atresia. However, future clinical observations in patients are needed to confirm these findings.

24 Article Noise exposure enhances auditory cortex responses related to hyperacusis behavior. 2012

Sun, Wei / Deng, Anchun / Jayaram, Aditi / Gibson, Brittany. ·Center for Hearing & Deafness, The State University of New York at Buffalo, NY 14214, USA. weisun@buffalo.edu ·Brain Res · Pubmed #22402030.

ABSTRACT: Hyperacusis, a marked intolerance to normal environmental sound, is a common symptom in patients with tinnitus, Williams syndrome, autism, and other neurologic diseases. It has been suggested that an imbalance of excitation and inhibition in the central auditory system (CAS) may play an important role in hyperacusis. Recent studies found that noise exposure, one of the most common causes of hearing loss and tinnitus, can increase the auditory cortex (AC) response, presumably by increasing the gain of the AC. However, it is not clear whether the increased cortical response will affect sound sensitivity and induce hyperacusis. In this experiment, we studied the effects of noise exposure (narrow band noise, 12 kHz, 120 dB SPL, 1 hour) on the physiological response of the inferior colliculus (IC) and the AC, and the behavioral sound reaction in conscious Sprague Dawley rats. Noise exposure induced a decrease of sound evoked potential in the IC. However, significant increases of AC response including sound evoked potentials and the spike firing rates of AC neurons were recorded right after the noise exposure. These results suggest that noise exposure induces hyperexcitability of AC presumably by increasing the post-synaptic response of AC neurons. The behavioral consequence of the noise exposure on sound perception was measured by the amplitude of the acoustic startle response before and after noise exposure in a separate group of rats. Although noise exposure caused a moderate hearing loss, the acoustic startle amplitude at the super-threshold level was significantly increased. These results suggest that noise exposure can cause exaggerated the sound reaction which may be related with the enhanced responsiveness of the AC neurons. This phenomenon may be related with noise induced hyperacusis.This article is part of a Special Issue entitled: Tinnitus Neuroscience.

25 Article Early age conductive hearing loss causes audiogenic seizure and hyperacusis behavior. 2011

Sun, Wei / Manohar, Senthilvelan / Jayaram, Aditi / Kumaraguru, Anand / Fu, Qiang / Li, Ji / Allman, Brian. ·Center for Hearing & Deafness, Department of Communicative Disorders and Sciences, The State University of New York at Buffalo, Buffalo, NY 14214, USA. weisun@buffalo.edu ·Hear Res · Pubmed #21872651.

ABSTRACT: Recent clinical reports found a high incidence of recurrent otitis media in children suffering hyperacusis, a marked intolerance to an otherwise ordinary environmental sound. However, it is unclear whether the conductive hearing loss caused by otitis media in early age will affect sound tolerance later in life. Thus, we have tested the effects of tympanic membrane (TM) damage at an early age on sound perception development in rats. Two weeks after the TM perforation, more than 80% of the rats showed audiogenic seizure (AGS) when exposed to loud sound (120 dB SPL white noise, < 1 min). The susceptibility of AGS lasted at least sixteen weeks after the TM damage, even the hearing loss recovered. The TM damaged rats also showed significantly enhanced acoustic startle responses compared to the rats without TM damage. These results suggest that early age conductive hearing loss may cause an impaired sound tolerance during development. In addition, the AGS can be suppressed by the treatment of vigabatrin, acute injections (250 mg/kg) or oral intakes (60 mg/kg/day for 7 days), an antiepileptic drug that inhibits the catabolism of GABA. c-Fos staining showed a strong staining in the inferior colliculus (IC) in the TM damaged rats, not in the control rats, after exposed to loud sound, indicating a hyper-excitability in the IC during AGS. These results indicate that early age conductive hearing loss can impair sound tolerance by reducing GABA inhibition in the IC, which may be related to hyperacusis seen in children with otitis media.