Study on the feature of cortical auditory evoked potential under different auditory tasks in cochlear implant children / 中华耳鼻咽喉头颈外科杂志

Objective: To investigate the variation regularity and influencing factors of cortical auditory evoked potential (CAEP) evoked by pure tone, syllable and tone stimuli in cochlear implant (CI) children. Methods: Cortical auditory evoked potential (CAEP) responses were collected from 46 CI children in the sound field. Pure tones with frequencies of 1 kHz and 2 kHz were used as the standard and the deviant respectively in the pure tone stimulation condition. The Chinese Mandarin tokens/ba/-/pa/and/ba1/-/ba4/pairs were used as the stimuli respectively in the syllable and tone stimulation condition. The latency, amplitude and presence rate of P1 and mismatch negative(MMN) were obtained and the correlation between the difficulty of auditory task, the age of hearing month, the duration of severe-profound hearing loss, the wearing history of hearing aid before CI, the hearing threshold of the better ear before CI and the latency and amplitude of P1 and MMN were analyzed. All statistical analyses and figures were conducted using SPSS 25.0. Results: The P1 presence rate of pure tone, syllable and tone group was 100% (17/17), 100% (13/13) and 75.0% (12/16), respectively, with significant difference (χ²=8.214, P=0.016). There was significant difference between pure tone group and tone group (χ²=4.836, P=0.028), but no significant difference between pure tone group and syllable group, syllable group and tone group. The MMN presence rate of pure tone, syllable and tone group was 94.1% (16/17), 84.6% (11/13) and 62.5% (10/16), respectively, but no significant difference among the three groups with different auditory tasks(χ²=0.066, P=0.066). No significant difference was observed among the three groups of different auditory tasks in the latency and amplitude of P1 and MMN. Multiple linear regression analysis showed that the latency of P1 was positively correlated with the difficulty of auditory task and the hearing threshold of the better ear before CI, and negatively correlated with hearing age and the history of hearing aid before CI. The latency of MMN was positively correlated with the difficulty of auditory task, and negatively correlated with hearing age. Conclusions: The P1 presence rate of pure tone auditory task is significantly higher than that of tone auditory task. The difficulty of auditory task, hearing age, the history of hearing aid before CI, and the hearing threshold of the better ear before CI has significant effects on the P1 latency. The difficulty of auditory task and hearing age has significant effects on the MMN latency.

Correlation study on anti-Ro52 antibodies frequently co-occur with other myositis-specific and myositis-associated autoantibodies / 北京大学学报(医学版)

OBJECTIVE@#Anti-Ro52 antibodies are frequently co-occur with other myositis-specific and myositis-associated autoantibodies, we here to study this phenomenon in Chinese patients suspected with inflammatory myopathies.@*METHODS@#In the study, 1 509 patients clinically suspected with inflammatory myopathies were tested for 11 kinds of myositis-specific and myositis-associated autoantibodies (including: anti-Jo-1, PL-7, PL-12, EJ, OJ, Mi-2, SRP, Ku, PM-Scl 75, PM-Scl 100, and Ro52 antibo-dies) by line-blot immunoassay from 2010 to 2016 in Peking University First Hospital. This retrospective study was to analyze these results to reveal the characteristics of anti-Ro52 antibodies co-occuring with other myositis autoantibodies. The data were analyzed using SPSS 17.0 and Graph Pad PRISM for Chi-square test, independent t-test, Pearson's correlation analysis, and drawing statistical graphs. Significance level was set at P < 0.05.@*RESULTS@#The positive rate of anti-Ro52 antibodies was 18.3% (276/1 509 cases), which was the most frequently detected myositis antibodies in our center. 51.8% (143/276) of the patients with anti-Ro52 antibodies were combined with the other myositis antibodies, and the most common co-occurred antibodies were anti-SRP antibodies (18.8%, 52/276), and the second common co-occurred antibodies were anti-Jo-1 antibodies (13.0%, 36/276). Anti-Ro52 antibodies were the most common antibodies that co-occurred in other myositis antibodies positive patients except in anti-OJ antibodies positive group. The co-positive rate with anti-Ro52 antibodies was the lowest in anti-PM-Scl 75 positive group (30.4%, 31/102), and the highest in anti-EJ positive group (80.0%, 12/15). The positive rate of anti-Ro52 antibodies in anti-synthase antibodies (including anti-Jo-1, EJ, OJ, PL-7, and PL-12 antibodies) positive group was 57.3% (75/131), which was significantly higher than that in the other antibodies (including: anti-Mi-2, SRP, Ku, PM-Scl 75, and PM-Scl 100 antibodies) positive group with 35.2% (119/338) (χ2=18.916, P < 0.001). The intensity of anti-Jo-1, EJ, and SRP antibodies in the group of the patients that co-occurred with anti-Ro52 antibodies was significantly higher than that in the other group without anti-Ro52 antibodies respectively (P < 0.05). The intensity of anti-SRP antibodies was significantly correlated with that of anti-Ro52 antibodies (r=0.44, P=0.001).@*CONCLUSION@#Anti-Ro52 antibodies were commonly associated with other myositis-specific and myositis-associated autoantibodies, especially with anti-synthase antibodies, and the co-presence of anti-Ro52 antibodies may be correlated with the myositis antibody intensity.

Muscle Magnetic Resonance Imaging for the Differentiation of Multiple Acyl-CoA Dehydrogenase Deficiency and Immune-mediated Necrotizing Myopathy / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Clinically, it is difficult to differentiate multiple acyl-CoA dehydrogenase deficiency (MADD) from immune-mediated necrotizing myopathy (IMNM) because they display similar symptoms. This study aimed to determine whether muscle magnetic resonance imaging (MRI) could be used for differential diagnosis between MADD and IMNM.</p><p><b>METHODS</b>The study evaluated 25 MADD patients, confirmed by muscle biopsy and ETFDH gene testing, and 30 IMNM patients, confirmed by muscle biopsy. Muscles were assessed for edema and fatty replacement using thigh MRI (tMRI). Degrees and distribution patterns of fatty infiltration and edema in gluteus maximus and thigh muscles were compared.</p><p><b>RESULTS</b>Total fatty infiltration and edema scores (median, [Q1, Q3]) were 4.00 (1.00, 15.00) and 0 (0, 4.00) in MADD and 14.50 (8.00, 20.75) and 22.00 (16.75, 32.00) in IMNM, respectively, which were significantly more severe in IMNM than that in MADD (P = 0.000 and P = 0.004, respectively). Edema scores for gluteus maximus, long head of biceps femoris, and semimembranosus were significantly higher in IMNM than in MADD (all P = 0.000). Fatty infiltration scores for anterior and medial compartments were significantly more severe in IMNM than that in MADD (all P = 0.000).</p><p><b>CONCLUSION</b>Different patterns of muscle involvement on tMRI can contribute to differential diagnosis between MADD and IMNM when clinical suspicions alone are insufficient, thereby reducing the need for muscle biopsy.</p>