1.A Novel Mutation in a 9-Month-Old Male Infant with Epileptic Spasms.
Wan-Ting LIU ; Qian CHEN ; Zhi-Jie GAO ; Xin-Na JI ; Ke-Ming XU ; Yan-Yan CAO
Chinese Medical Journal 2018;131(24):2991-2992
2.White Matter Deficits Underlying the Impaired Consciousness Level in Patients with Disorders of Consciousness.
Xuehai WU ; Jiaying ZHANG ; Zaixu CUI ; Weijun TANG ; Chunhong SHAO ; Jin HU ; Jianhong ZHU ; Yao ZHAO ; Lu LU ; Gang CHEN ; Georg NORTHOFF ; Gaolang GONG ; Ying MAO ; Yong HE
Neuroscience Bulletin 2018;34(4):668-678
In this study, we aimed to (1) identify white matter (WM) deficits underlying the consciousness level in patients with disorders of consciousness (DOCs) using diffusion tensor imaging (DTI), and (2) evaluate the relationship between DTI metrics and clinical measures of the consciousness level in DOC patients. With a cohort of 8 comatose, 8 unresponsive wakefulness syndrome/vegetative state, and 14 minimally conscious state patients and 25 patient controls, we performed group comparisons of the DTI metrics in 48 core WM regions of interest (ROIs), and examined the clinical relevance using correlation analysis. We identified multiple abnormal WM ROIs in DOC patients compared with normal controls, and the DTI metrics in these ROIs were significantly correlated with clinical measures of the consciousness level. Therefore, our findings suggested that multiple WM tracts are involved in the impaired consciousness levels in DOC patients and demonstrated the clinical relevance of DTI for DOC patients.
Adult
;
Brain Stem
;
diagnostic imaging
;
Consciousness
;
physiology
;
Consciousness Disorders
;
diagnostic imaging
;
pathology
;
Diffusion Tensor Imaging
;
methods
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Female
;
Humans
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Image Processing, Computer-Assisted
;
methods
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Male
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Middle Aged
;
White Matter
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pathology
;
physiopathology
3.Clinical Study on 136 Children with Sudden Sensorineural Hearing Loss.
Feng-Jiao LI ; Da-Yong WANG ; Hong-Yang WANG ; Li WANG ; Feng-Bo YANG ; Lan LAN ; Jing GUAN ; Zi-Fang YIN ; Ulf ROSENHALL ; Lan YU ; Sten HELLSTROM ; Xi-Jun XUE ; Mao-Li DUAN ; Qiu-Ju WANG
Chinese Medical Journal 2016;129(8):946-952
BACKGROUNDThe prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL.
METHODSOne hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors.
RESULTSAmong the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies.
CONCLUSIONSCSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators, including the level of WBC, platelet, homocysteine, ALP, positive CMV IgG antibody, fibrinogen, and some immunologic indicators, are closely related to CSSNHL.
Adolescent ; Child ; Child, Preschool ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Loss, Sensorineural ; blood ; etiology ; physiopathology ; Humans ; Male ; Otoacoustic Emissions, Spontaneous ; Retrospective Studies
4.Audiological and Otological Status of 45 Infants with Cleft Palate.
Dian-yin SHI ; Lian ZHOU ; Ying-ying SHANG
Acta Academiae Medicinae Sinicae 2016;38(2):136-139
OBJECTIVETo study the audiological and otological status of cleft palate infants with the application of distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), tympanometry with 1000 Hz and 226 Hz probe tones.
METHODSTotally 45 cleft palate infants aged 8-24 months were included in the study. Most of them were examined for DPOAE, ABR and two frequency tympanometry.
RESULTSMost infants failed the three tests,among whom 6.7% ears passed DPOAE and 33.3% of ears had normal ABR hearing threshold. In addition, 8.9% of ears turned out normal in the 1000 Hz probe-tone tympanometry, and 13.3% were type A in the 226 Hz probe-tone tympanometry. Finally, 1000 Hz tympanometry had more agreement with DPOAE and latency of ABR wave I than 226 Hz tympanometry.
CONCLUSIONMost cleft palate infants have audiological and otological problems, which should be evaluated in a more comprehensive manner.
Acoustic Impedance Tests ; Cleft Palate ; physiopathology ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Infant ; Infant, Newborn ; Neonatal Screening ; Otoacoustic Emissions, Spontaneous
5.Effects of electrical stimulation at acupoints in the distribution area of auricular vagus nerve combined with sound masking method on auditory brainstem response and neurotransmitters of inferior colliculus in rats of tinnitus.
Songbai YANG ; Zhigang MEI ; Lingjing TAN ; Wenhan MA ; Dingqi ZHANG ; Zhaojun WANG ; Tiantian LI ; Kunyan HUANG ; Sanjin CAI
Chinese Acupuncture & Moxibustion 2016;36(5):517-522
OBJECTIVETo explore the effects of electrical stimulation at acupoints in the distribution area of auricular vagus nerve combined with sound masking on auditory brainstem response (ABR) and contents of neurotransmitters of γ-aminobutyric acid (γ-GABA), 5-hydroxytryptamine (5-HT) and acetyl choline (Ach) in inferior colliculus of tinnitus rats.
METHODSTwenty-four male adult SD rats were randomized into a control group, a model group, a 7-d treatment group and a 15-d treatment group. Except the control group, rats in the remaining groups were treated with intraperitoneal injection of 10% salicylate sodium at a dose of 350 mg/kg to establish tinnitus model. Rats in the control group were treated with injection of 0.9% NaCl. Rats in the 7-d treatment group and 15-d treatment group were treated with electrical stimulation at "Shenmen (TF₄)" and "Yidan (CO₁₁)" in the distribution area of auricular vagus nerve combined with sound masking, once a day, for 7 days and 15 days. The SigGenRP software of TDT system was applied to provide voice for single ear and collect the signal, and the voice threshold of ABR was tested. The levels of γ-GABA, 5-HT and Ach in inferior colliculus of rats were detected by enzyme linked immunosorbent assay (ELISA) and compared.
RESULTSCompared with the model group, the threshold values of ABR in 12 kHz and 16 kHz voice stimulation in the 7-d treatment group were significantly lower all P < 0.05); the threshold values of ABR from 4 kHz to 28 kHz voice stimulation in the 15-d treatment group were signally reduced (P < 0.05, P < 0.01), which was more significant than those in the 7-d treatment group. The level of γ-GABA in the model group was significantly lower than that in the control group (P < 0.05), and that in the 15-d treatment group was apparently higher than that in the model group (P < 0.05). The level of 5-HT in the model group was markedly higher than that in the control group (P < 0.05), and that in the 7-d treatment group was lower than that in the model group (P < 0.05), while that in the 15-d treatment group was apparently higher than that in the model group (P < 0.05). The level of Ach in the model group was obviously; lower than that in the control group (P < 0.05), and that in the 7-d treatment group was higher than that in the model group (P < 0.05).
CONCLUSIONElectrical stimulation at auricular vagus nerve combined with sound masking) could regulate the threshold of ABR, especially in the 15-d treatment group. This may be ascribed to modulating the levels of neurotransmitter of γ-GABA, 5-HT and Ach in inferior colliculus.
Acupuncture Points ; Animals ; Brain Stem ; physiopathology ; Electric Stimulation ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Inferior Colliculi ; physiopathology ; Male ; Neurotransmitter Agents ; metabolism ; Rats ; Rats, Sprague-Dawley ; Serotonin ; metabolism ; Tinnitus ; physiopathology ; therapy ; Vagus Nerve ; physiopathology ; gamma-Aminobutyric Acid ; metabolism
6.Clinical observation on medulla oblongata palsy after brainstem infarction treated with electroacupuncture at eight-neck-occiput points.
Chinese Acupuncture & Moxibustion 2014;34(6):539-542
OBJECTIVETo compare the differences in the efficacy on speech and swallowing dysfunction of medulla oblongata palsy (MOP) after brainstem infarction between electroacupuncture at eight-neck-occiput points and routine acupoints.
METHODSSeventy-two patients were randomized into a neck-occiput points group and a meridian points group, 36 cases in each one. In the neck-occiput points group, the eight-neck-occiput points (Neck 1-4 points, Occiput 1-4 points) were selected. In the meridian points group, Lianquan (CV 23), Futu (LI 18), Tongli (HT 5), Hegu (LI 4) and the others were selected. Electroacupuncture was used in the two groups, dense-dispersion wave, retaining for 30 min. The treatment was given once a day, 5 treatments a week. Totally, 4 weeks were required. The symptom scores of speech and swallowing dysfunction were observed before and after treatment in the two groups. The efficacy was compared between the two groups.
RESULTSThe scores of speech and swallowing dysfunction were improved significantly after treatment in the two groups (P < 0.05, P < 0.01). The results in the neck-occiput points group were better than those in the meridian points group (both P < 0.01). The curative rate of speech dysfunction was 30.6% (11/36) and that of swallowing dysfunction was 22.2% (8/ 36) in the neck-occiput points group, which were better than 11.1% (4/36) and 5.6% (2/36) in the meridian points group, respectively. The differences were significant in comparison of the two groups (P < 0.01, P < 0.05).
CONCLUSIONElectroacupuncture at eight-neck-occiput points achieves a better efficacy on speech and swallowing dysfunction of MOP after brainstem infarction as compared with the routine acupoints. This therapy is characterized as more accurate point localization and safer operation.
Acupuncture Points ; Adult ; Aged ; Brain Stem Infarctions ; complications ; Bulbar Palsy, Progressive ; etiology ; physiopathology ; therapy ; Deglutition ; Electroacupuncture ; Female ; Humans ; Male ; Medulla Oblongata ; physiopathology ; Middle Aged
7.Change of auditory brainstem response in hearing deprivation rat model induced by bilateral cochlears ablation.
Haixia HAN ; Ou XU ; Heyin HUANG ; Yuxing WANG ; Hong LU
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2014;28(7):465-467
OBJECTIVE:
To observe the change of auditory brainstem response (ABR) in hearing deprivation rat model induced by bilateral cochlears ablation at different time points.
METHOD:
Forty SD rats were randomly divided into four experiment groups including 2-week group, 4-week group, 6-week group, 8-week group and four control groups with 5 rats (n = 10) in each group. Then bilateral cochlears ablation was applied to experiment groups. The threshold value of ABR was measured at different time and latent period of each wave was compared.
RESULT:
The threshold of ABR in experiment group was elevated significantly. The latent period of each wave was pro longed significantly (P < 0.01). In experiment group, the threshold value of ABR in 2-week and 4-week group was significantly greater than that in 6-week group and 8-week group (P < 0.01).
CONCLUSION
Bilateral cochlears ablation surgery could elevate the threshold of ABR and latent period of each wave prolonged. The effect of hearing deprivation became apparent after surgery for 4 weeks.
Animals
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Auditory Threshold
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Cochlea
;
physiopathology
;
surgery
;
Disease Models, Animal
;
Evoked Potentials, Auditory, Brain Stem
;
physiology
;
Hearing Loss
;
Male
;
Rats
;
Rats, Sprague-Dawley
8.Amplitude-integrated electroencephalographic changes in neonates with acute bilirubin encephalopathy.
Fang LUO ; Hui-jia LIN ; Yu BAO ; Zheng CHEN ; Xiao-lu MA ; Li-ping SHI ; Li-zhong DU
Chinese Journal of Pediatrics 2013;51(3):221-226
OBJECTIVETo characterize amplitude-integrated electroencephalo graphic (aEEG) traces in neonates with acute bilirubin encephalopathy (ABE), explore the value of aEEG in early diagnosis and prediction of neurological outcome of ABE.
METHODaEEG records of 10 cases with ABE (Oct 2009-Nov 2011) were reviewed to identify neonates with a diagnosis of ABE. Clinical data were collected. The aEEG traces were classified according to background activity (normal, moderate, or severely abnormal), presence of seizures and sleep-wake cycling (SWC). Brainstem auditory evoked potential (BAEP) and magnetic resonance imaging (MRI) were studied. The neuromotor development of survivors with ABE was assessed by using the Infant Neurological International Battery (INFANIB).
RESULTThe characteristics of aEEG tracings in these infants with ABE were shown continuous normal voltage (CNV, n = 5), discontinuous voltage (DNV, n = 4), discontinuous voltage with burst-suppression (BS)BS+ (n = 1); mature SWC (n = 2), immature SWC (n = 5), no SWC (n = 3); 8 infants (80%) had electrical seizures: single seizure (n = 2); repetitive seizures (n = 2), and status epilepticus (SE) (n = 4). Among the 10 infants with ABE, no infants had normal aEEG, 3 had mildly abnormal aEEG, and 7 had severely abnormal aEEG. Eight infants accepted BAEP test, 2 were mildly abnormal and 6 were severely abnormal. Six infants accepted MRI, 1 was normal and 5 were abnormal. By chi-square analysis and Spearman rank correlation analysis, the results of aEEG classification were correlated with the phase of ABE and the severity of BAEP. These infants were followed up for more than 6 months (range 6 months to 1 year). In 3 infants with mildly abnormal aEEG, 2 were normal and 1 was transit in infanib score at 6 months of age. Of 7 infants with severely abnormal aEEG, 1 died, 3 were abnormal (2 Spastic dyskinesia and 1 hypotonia), 2 were transit in infanib score at 6 months old. 1 lost to follow-up.
CONCLUSIONAmplitude-integrated electroencephalography can provide important information of the status of cerebral function in neonates with ABE and help to predict its neurological outcome.
Brain ; physiology ; Early Diagnosis ; Electroencephalography ; Evoked Potentials, Auditory, Brain Stem ; Female ; Humans ; Hyperbilirubinemia ; complications ; Infant, Newborn ; Infant, Premature ; Kernicterus ; diagnosis ; physiopathology ; Magnetic Resonance Imaging ; Male ; Predictive Value of Tests ; Seizures ; diagnosis ; etiology ; physiopathology ; Severity of Illness Index ; Sleep ; physiology
9.Changes in MLS-BAEP in newborn piglets with hypoxic-ischemic brain damage during selective moderate head cooling therapy.
Ji-Mei WANG ; Wen-Hao ZHOU ; Guo-Qiang CHENG ; Lai-Shuang WANG ; Ze-Dong JIANG ; Xiao-Mei SHAO
Chinese Journal of Contemporary Pediatrics 2013;15(6):484-489
OBJECTIVETo study the effect of selective moderate head cooling therapy on maximum length sequences brainstem auditory evoked potential (MLS-BAEP) in newborn piglets with hypoxic-ischemic brain damage.
METHODSSixteen newborn piglets aged 5-7 day old were randomly divided into three groups: normothermic control (n=4), HI (n=6) and mild hypothermia-treated (n=6). HI was induced through temporary occlusion of both carotid arteries, followed by mechanical ventilation with low concentration of oxygen (FiO2=0.06) for 30 minutes. Mild hypothermia was induced by equipment via circulating water. MLS-BAER was recorded before HI and at 12 hours, 24 hours, 36 hours, 48 hours, 60 hours, 72 hours, 4 days, 7 days, 10 days, 13 days and 15 days after HI.
RESULTSCompared with the normothermic control group, all latencies and intervals tended to increase significantly at 72 hours in the HI group and reached peak values on day 7. From day 10, all latencies and intervals tended to decrease, but apart from wave I latency, still differed significantly from those of the normothermic control group. MLS-BAER variables did not reach normal values until day 15. Ⅲ latency, Ⅰ-Ⅲ interval and Ⅰ-Ⅴ interval were significantly reduced in the hypothermia-treated group between 60 and 7 days after HI compared with the HI group (P<0.05). V latency and Ⅲ-Ⅴ interval in the hypothermia-treated group were also reduced compared with the HI group between 72 hours and 7 days after HI (P<0.05).
CONCLUSIONSBoth peripheral and central auditory systems are disturbed by HI, which shows as a significant increase in MLS-BAER variables (all latencies and intervals) in newborn piglets. Involvement in central brainstem auditory system reaches a peak on day 7 after injury. MLS-BAER variables still cannot reach to normal values until day 15. Selective moderate head cooling therapy can significantly reduce brainstem damage induced by HI.
Animals ; Animals, Newborn ; Evoked Potentials, Auditory, Brain Stem ; Hypothermia, Induced ; Hypoxia, Brain ; physiopathology ; therapy ; Swine
10.Relationship of B/A ratio and acidosis with abnormal brainstem auditory evoked potentials in neonates with severe hyperbilirubinemia.
Yan ZHUANG ; Gui-Nan LI ; Yong ZHOU ; Yue-Yuan HU ; Jun LI ; Cai-Xia ZHAN
Chinese Journal of Contemporary Pediatrics 2013;15(5):332-334
OBJECTIVETo investigate the relationship of bilirubin/albumin (B/A) ratio and acidosis with abnormal brainstem auditory evoked potentials (BAEPs) in neonates with severe hyperbilirubinemia and its clinical significance.
METHODSA total of 967 neonates with severe hyperbilirubinemia between November 2008 and October 2009 were enrolled in the study. They were divided into two groups according to their BAEPs: normal BAEP group (n=799) and abnormal BAEP group (n=168). Univariate analysis and age-stratified Chi-square test were used to determine the relationship of B/A ratio and acidosis with BAEP.
RESULTSThe univariate analysis showed that the abnormal BAEP group had significantly lower pH and base excess values and a significantly higher B/A ratio compared with the normal BAEP group (P<0.05). The age-stratified Chi-square test showed that neonates with acidosis or with a B/A ratio greater than 1.0 had a significantly higher incidence of abnormal BAEPs than those without acidosis or with a B/A ratio less than 1.0 in any age (days) group of neonates with severe hyperbilirubinemia (P<0.05).
CONCLUSIONSHigh B/A ratio and acidosis are the risk factors for abnormal BAEPs in neonates with severe hyperbilirubinemia, which is the case for those in any age group. In order to reduce the incidence of hearing loss in any age group of neonates with severe hyperbilirubinemia, we should correct the acidosis and lower the B/A ratio as soon as possible.
Acidosis ; physiopathology ; Bilirubin ; blood ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Hyperbilirubinemia ; blood ; physiopathology ; Infant, Newborn ; Serum Albumin ; analysis

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