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| 1 | De novo mutation in ATP6V1B2 impairs lysosome acidification and causes dominant deafness-onychodystrophy syndrome显示文摘 | Yongyi Yuan Jianguo Zhang Qing Chang Jin Zeng Feng Xin Jianjun Wang Qingyan Zhu Jing Wu Jingqiao Lu Weiwei Guo Xukun Yan Hui Jiang Binfei Zhou Qi Li Xue Gao Huijun Yuan Shiming Yang Dongyi Han Zixu Mao Ping Chen Xi Lin Pu Dai | 2014 | Cell Research2014，24，11： | 13 |
| 2 | Identification of a novel mutation in POU3F4 for prenatal diagnosis in a Chinese family with X-linked nonsyndromic hearing loss显示文摘We present the clinical and genetic findings for a Chinese family with X-linked non-syndromic hearing loss in which the affected males showed congenital profound sensorineural hearing impairment. In two affected brothers, the computer tomography of temporal bone showed bilateral dilation of the internal auditory canal with fistulous communication between the lateral canal and the basal cochlear turn, which is consistent with the typical DFNX2 phenotype. A missense mutation (c.647G→A) in the POU3F4 gene caused a substitu- tion from glycine to glutamic acid at position 216 (p.G216E), and this mutation was found to consistently cosegregate with the deafness phenotype in the family. The mutation resulted in the loss of function of the POU3F4 by decreasing the affinity between the protein and DNA, as shown in silico by the structural analysis. Prenatal diagnosis of pregnant proband of this family revealed the c.647G→A muta- tion in DNA extracted from the amniotic fluid surrounding the fetus. The appropriate use of genetic testing and prenatal diagnosis plays a key role in reducing the recurrence of genetic defects in high-risk families. | Jianzhong Li Jing Cheng Yanping Lu Yu Lu Airing Chen YiSun Dongyang Kang Xin Zhang Pu Dai Dongyi Han Huijun Yuan | 2010 | Journal of Genetics and Genomics2010，37，12： | 9 |
| 3 | Mitochondrial DNA A1555G mutation screening using a testing kit method and its significance in preventing aminoglycoside-related hearing loss显示文摘To report a new screening method for mitochondrial DNA 1555A→G mutation and the results of genotype analysis in 19 maternal inherited deafness pedigrees. Method Five hundred and forty-six non-syndromic neuro-sensory hearing loss patients were tested for 1555A→G mutation using a new compact testing kit, which allows clear distinction between wild type and 1555 A→G mutated mtDNAs. Results Nineteen subjects among the 546 patients (3.48%) were found to carry mtDNA A1555G mutation. The results were confirmed by sequencing in an ABI 3100 Avant sequencer. Conclusions Maternal inherited deafness families are a frequently seen in outpatient group. The detection of mtDNA 1555 A→G mutation with a low cost, ready to use detection kit is needed and suitable in China for large scale screening and preventive testing before usage of aminoglycoside antibiotics. | LIU Xin,1 DAI Pu,1* HUANG Deliang,1 YUAN Huijun,1 LI Weiming,1 YU Fei,1 ZHANG Xin,1 KANG Dongyang,1 CAO Juyang,1 YANG Weiyan,1 HAN Dongyi,1 JIN Zhengce2, GUAN Minxin3 1. Department of Otolaryngology, Chinese PLA General Hospital, Beijing, China2. Weihai Aomaier Gene Technological CO.,LTD.,Weihai,Shandong 264200, China.3. Division and Program in Human Genetics and Center for Hearing and Deafness Research, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA | 2006 | Journal of Otology2006，1，1： | 7 |
| 4 | Development of a script of phonemically balanced monosyllable lists of Mandarin-Chinese显示文摘Objectives To develop a set of monosyllable audiometry test material that can be used in speech recognition testing in Mandarin Chinese. Materials and Methods In an effort to improve reliability and efficiency in clinical practice, a set of 30 test lists of 25 monosyllable test items each was designed with consideration of the following: length of the list, phonemical balance (PB), word utility frequency, coverage of common words, and equivalency among lists. The 30 lists contained 750 monosyllabic words and were constructed on the basis of Chinese phoneme distribution probability summarized in the Manual of Acoustic. The occurrence incidence of the 22 consonants, 36 vowels, and 4 tones of the Chinese Mandarin characters in the 30 test lists were calculated. To achieve PB, 489 monosyllables were selected from the 2500 most commonly used Chinese characters and the 4000 most commonly used Chinese phrases to compile the 30 monosyllable test lists using a computer algorithm with manual adjustment. Results Thirty phonemical balanced word lists of common Mandarin Chinese monosyllabic words were compiled. Each list consisted of 25 monosyllables. A total of 489 phonemes (consonants, vowels and tones) were included in the set. Conclusions This set of lists can potentially be used as the basic lists for future development of Mandarin PB monosyllable speech test materials. | JI Fei, CHEN Aiting, ZHAO Yang, XI Xin, HAN Dongyi Departments of Otorhinolaryngology-Head and Neck Surgery, Institute of Otorhinolaryngology, General Hospital of People’s Liberation Army, Beijing 100853, China | 2010 | Journal of Otology2010，5，1： | 4 |
| 5 | Hearing Preservation in Acoustic Neuroma Surgery显示文摘Objective To report the authors’ experiences in hearing preservation during acoustic neuroma (AN) resection procedures. Methods Two cases of AN removal via retrosigmoid approach were reviewed. Hearing preservation was attempted in the aid of endoscopic technique along with continuous monitoring of the compound action potential (CAP) and auditory brainstem response(ABR) during the surgery. Results The tumor in Case 1 was 1.5 cm in diameter. The average pure-tone hearing threshold was 30 dB HL and ABR was normal. Waves I, III and V of ABR were present following tumor removal. At 7th month follow-up, audiometric thresholds and ABR inter-peak intervals had recovered to pre-operative levels, with normal facial nerve function. The patient in Case 2 had bilateral AN. The tumors measured 4.0 cm(left) and 5.0 cm (right) on MRI scans. The AN on the right side was removed first, followed by removal of the left AN four months later. Intraoperative CAP monitoring was employed during removal of the left AN. While efforts to preserve the cochlear nerve were not successful, CAPs were still present after tumor removal. Conclusions Intraoperatively recorded CAPs are not reliable in predicting postoperative hearing outcomes. In contrast, ABRs are an indicator of function of the peripheral auditory pathway. Presence of waves I, III and V following tumor removal may represent preservation of useful hearing. | HAN Dongyi, YU Limei, YANG Shiming, YU LimingDepartment of Otolaryngology-Head and Neck Surgery, PLA General Hospital, Beijing 100853 | 2006 | Journal of Otology2006，1，1： | 4 |
| 6 | Extremely low penetrance of deafness associated with the mitochondrial 12S rRNA mutation in 16 Chinese families: Implication for early detection and prevention of deafness显示文摘 | Pu Dai Xin Liu Dongyi Han Yaping Qian Deliang Huang Huijun Yuan Weiming Li Fei Yu Ruining Zhang Hongyan Lin Yong He Youjun Yu Quanzhu Sun Huaiyi Qin Ronghua Li Xin Zhang Dongyang Kang Juyang Cao Wie-Yen Young Min-Xin Guan | 2005 | Biochemical and Biophysical Research Communications2005，，1： | 2 |
| 7 | Synthesis and characterizationofcompositesofpolyanilineand polyurethanemolified epoxy显示文摘 | Chiou Yang WenChin Han DongYi | 2006 | Polymer International2006，55，11： | 1 |
| 8 | Clinical and molecular analysis of a four-generation Chinese family with aminoglycoside-induced and nonsyndromic hearing loss associated with the mitochondrial 12S rRNA C1494T mutation显示文摘 | Qiuju Wang Qing-Zhong Li Dongyi Han Yali Zhao Lidong Zhao Yaping Qian Hu Yuan Ronghua Li Suoqiang Zhai Wie-Yen Young Min-Xin Guan | 2005 | Biochemical and Biophysical Research Communications2005，，2： | 1 |
| 9 | Mitochondrial haplotype and phenotype of 13 Chinese families may suggest multi-original evolution of mitochondrial C1494T mutation显示文摘 | Yuhua Zhu Qi Li Zhengyi Chen Yao Kun Lijia Liu Xin Liu Huijun Yuan Suoqiang Zhai Dongyi Han Pu Dai | 2009 | Mitochondrion2009，，6： | 1 |
| 10 | ADVANCES IN SURGICAL TREATMENT OF ACOUSTIC NEUROMA | HAN Dongyi CAI Chaochan | 2012 | Journal of Otology2012，7，2： | 1 |
| 11 | Biocompatible and Biodegradable Functional Polysaccharides for Flexible Humidity Sensors显示文摘Using wearable devices to monitor respiration rate is essential for reducing the risk of death or permanent injury in patients.Improving the performance and safety of these devices and reducing their environmental footprint could advance the currently used health monitoring technologies.Here,we report high-performance,flexible bioprotonic devices made entirely of biodegradable biomaterials.This smart sensor satisfies all the requirements for monitoring human breathing states,including noncontact characteristic and the ability to discriminate humidity stimuli with ultrahigh sensitivity,rapid response time,and excellent cycling stability.In addition,the device can completely decompose after its service life,which reduces the risk to the human body.The cytotoxicity test demonstrates that the device shows good biocompatibility based on the viability of human skin fibroblast-HSAS1 cells and human umbilical vein endothelial(HUVECs),illustrating the safety of the sensor upon integration with the human skin. | Lili Wang Zheng Lou Kang Wang Shufang Zhao Pengchao Yu Wei Wei Dongyi Wang Wei Han Kai Jiang Guozhen Shen | 2020 | Research2020，，1： | 1 |
| 12 | Functional Mutation of SMAC / DIABLO , Encoding a Mitochondrial Proapoptotic Protein, Causes Human Progressive Hearing Loss DFNA64显示文摘 | Jing Cheng Yuhua Zhu Sudan He Yanping Lu Jing Chen Bing Han Marco Petrillo Kazimierz O. Wrzeszczynski Shiming Yang Pu Dai Suoqiang Zhai Dongyi Han Michael Q. Zhang Wei Li Xuezhong Liu Huawei Li Zheng-Yi Chen Huijun Yuan | 2011 | The American Journal of Human Genetics2011，，1： | 1 |
| 13 | Clue to a New Deafness Gene:A Large Chinese Nonsyndromic Hearing Loss Family Linked to DFNA4显示文摘Hereditary hearing loss is one of the most common neurosensory defects in humans.Approximately 70%of cases are nonsyndromic and could be inherited in autosomal dominant, autosomal recessive,mitochondrial,X-linked,and Y-linked manners(Wang et al.,2004;Alford,2011).The autosomal dominant type,comprising 15%-20%of nonsyndromic hearing loss,is monogenic and genetically heterogeneous.Since the first dominant deafness | Liang Zong Chunye Lu Yali Zhao Qian Li Dongyi Han Weiyan Yang Yan Shen Qingyin Zheng Qiuju Wang | 2012 | Journal of Genetics and Genomics2012，39，12： | 0 |
| 14 | Crucial role of iron plaque on thallium uptake by rice plant显示文摘Iron plaque is a Fe-containing oxide film produced by the oxidation of Fe(II)in the rice root system under the combined action of oxygen infiltration and other microorganisms.Owing to its special surface structure and physio-chemical properties,the iron plaque has a strong absorption capacity for a variety of heavy metal ions.This study aimed to first investigate the effects of Fe species on the geochemical fractionation of Tl in typical paddy soil systems affected by industrial activities,followed by pot culture experiments to probe the effects of Fe species on the uptake and translocation of Tl in rice plants.The results of field work preliminarily showed that iron at different valences affected the conversion of the Tl geochemical fraction in the soil.Oxidizable Tl exerted significant positive correlation relationships with Fe2+and negative correlation relationships with Fe3+,while reducible Tl only displayed a positive correlation with Fe3+.Further analysis by pot culture experiments revealed that the contents of Fe were significantly positively correlated with Tl contents in Fe plaque(R2=0.529).In contrast,the water-soluble Tl contents in the soil were significantly negatively correlated with the contents of Fe(R2=–0.90,p<0.05).It suggests that the iron plaque promoted the absorption and fixation of Tl on the root surface of rice plants,causing Tl to accumulate in the iron plaque.Besides,the Tl content in the Fe plaque on the root surface of rice plants was greater than that in the above-ground tissues,which indicates that most Fe plaque exerts a certain degree of inhibition on Tl migration into the above-ground tissues of rice plants.All these findings indicate that Fe film is also an important carrier of Tl transfer in the soil–rice plant system,which provides new scientific support for the remediation of typical Tl-contaminated rice fields. | Xiaoyin Zhang Wenhuan Yuan Juan Liu Haoran Li Han Cai Haiyao Hu Dongyi Ren Yuhua Zhang Yuxiang Shen Jin Wang | 2023 | Waste Disposal and Sustainable Energy2023，5，1： | 0 |
| 15 | UTILITY OF VIBRANT SOUNDBRIDGE IN PATIENTS WITH CONGENITAL MIDDLE AND OUTER EAR DEFORMITIES | ZOU Yihui LI Jianan CHEN Aiting DAI Bu HAN Dongyi LIU Huizhan YANG Shiming | 2012 | Journal of Otology2012，7，2： | 0 |
| 16 | Basic flbroblast growth factor protects auditory neurons and hair cells from noise exposure and glutamate neurotoxicity显示文摘The purpose of the present study was to determine protectivie effects of basic fibroblast growth factor (bFGF) on cochlear neurons and hair cells in vitro and in vivo. In experiment I, cultured spiral ganglion neurons (SGNs) prepared from P3 mice were exposed to 20mM glutamate for 2 hours before the culture medium was replaced with fresh medium containing 0, 25, 50, and 100 ng/ml bFGF, respectively. Fourteen days later, all cultures were fixed with 4% paraformaldehyde, and stained with 1% toluidine blue. The number of surviving SGNs were counted and the length of SGNs neurites were measured. Exposure to 20 mM glutamate for 24 hours resulted in an inhibition on neurite outgrowth of SGNs and elevated cell death. Treatment of the cultures with bFGF led to promotion of neurite outgrowth and elevated number of surviving SGNs. Effects of bFGF were dose dependent with the highest potency at 100 ng/ml. In experiment Ⅱ, in vivo studies were carried out with guinea pigs in which bFGF or artificial perilymph was perfused into the cochlea to assess possible protective effects of bFGF on cochlear hair cells and compound action potentials(CAP). The CAPs were measured before, immediatly and 48 hours after exposure to noise. Significant differences in CAP were observed (p<0. 05 ) among the bFGF perfused group, control group(t =3. 896 ) and artificial perilymph perfused group (t =2. 520) at 48 hours after noise exposure, Cochleae were removed and hair cell Loss was analyzed in surface preparations prepared from all experimental animals. Acoustic trauma caused loss of 651 and 687 inner hair cells in the control and artificial perilymph perfused group, respectively. In sharp contrast, only 31 inner hair cells were lost in the bFGF perfused ears. Similarly, more outer hair cells died in the control and perilymph perfuesed group (41830 and 41968, respectively) than in the group treated with bFGF (34258). Our results demonstrate that bFGF protected SGNs against glutmate neurotoxicity in vitro. In addition, treatment with bFGF also protected hair cells from acoustic trauma. | 翟所强 王大君 王嘉陵 Han Dongyi YANG Weiyan | 2003 | 中华耳科学杂志2003，1，1： | 0 |
| 17 | Autosomal recessive hereditary auditory neuropathy显示文摘Objectives: Auditory neuropathy (AN) is a sensorineural hearing disorder characterized by absent or abnormal auditory brainstem responses (ABRs) and normal cochlear outer hair cell function as measured by otoacoustic emissions (OAEs). Many risk factors are thought to be involved in its etiology and pathophysiology. Three Chinese pedigrees with familial AN are presented herein to demonstrate involvement of genetic factors in AN etiology. Methods: Probands of the above - mentioned pedigrees, who had been diagnosed with AN, were evaluated and followed up in the Department of Otolaryngology Head and Neck Surgery, China PLA General Hospital. Their family members were studied and the pedigree diagrams were established. History of illness, physical examination,pure tone audiometry, acoustic reflex, ABRs and transient evoked and distortion- product otoacoustic emissions (TEOAEs and DPOAEs) were obtained from members of these families. DPOAE changes under the influence of contralateral sound stimuli were observed by presenting a set of continuous white noise to the non - recording ear to exam the function of auditory efferent system. Some subjects received vestibular caloric test, computed tomography (CT)scan of the temporal bone and electrocardiography (ECG) to exclude other possible neuropathy disorders. Results: In most affected subjects, hearing loss of various degrees and speech discrimination difficulties started at 10 to16 years of age. Their audiological evaluation showed absence of acoustic reflex and ABRs. As expected in AN, these subjects exhibited near normal cochlear outer hair cell function as shown in TEOAE & DPOAE recordings. Pure- tone audiometry revealed hearing loss ranging from mild to severe in these patients. Autosomal recessive inheritance patterns were observed in the three families. In Pedigree Ⅰ and Ⅱ, two affected brothers were found respectively, while in pedigree Ⅲ, 2 sisters were affected. All the patients were otherwise normal without evidence of peripheral neuropathy at the time of this writing. Conclusions: In this study, patients with feature of non- syndromic hereditary auditory neuropathy were identified in three Chinese families.Pedigree analysis indicates autosomal recessive inheritances in the pedigrees. The observed inheritance and clinical audiologic findings are different from those previously described for non-syndromic low-frequency sensorineural hearing loss. This information should facilitate future molecular candidate genes screening for understanding the mechanism of AN. | 王秋菊 顾瑞 曹菊阳 Yu Liming GUO WEIWEI YU Ning ZHOU Na Han Dongyi YANG Weiyan | 2003 | 中华耳科学杂志2003，1，1： | 0 |
| 18 | Prevalence of type 2 diabetes among rheumatoid arthritis patients:a large retrospective study显示文摘To the Editor:Rheumatoid arthritis(RA)is a chronic inflammatory disease characterized by an excess of cardiovascular disease(CVD)risk,estimated to be at least 50%greater than the general population.[1]CVD has been recognized as the main cause of mortality in established RA patients,but recent data confirm this trend also in earlier stages of the disease.[2]Several factors have been evoked as determinants of this additional risk,but the most consolidated theory attributes this phenomenon to the interplay between chronic high-grade inflammation and elevated prevalence of“classical”cardiovascular risk factors,including diabetes. | Zhenge Han Qi Zhou Hairong Han Weizhen Qiao Zhonghong Qie Dongyi He | 2022 | Chinese Medical Journal2022，135，20： | 0 |
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