Objective Application of auditory brainstem response (ABR) in the study on the relationship of neonates with hypoxic-ischemic encephalopathy (HIE) and the children with hearing loss and auxiliary determine the prognosis of encephalopathy. Methods We prospectively selected neonates diagnosed as HIE in the department of neonatology of the Chengdu Women and Children Central Hospital from January, 2006 to June, 2008. Neonatal ABR was tested and the prognosis of neonates were observed through 3-year followed up in order to analyze the relationship between HIE severity and the severity of hearing handicap and the relationship between the severity of hearing handicap and prognosis. Statistical analysis was performed using SPSS 18.0. χ2 test was used to compare the rate between groups. Results 40 cases involving 80 ears were included, of which 33 cases accomplished the 3-year follow-up for prognosis. The results showed that, 86.3% HIE neonates had hearing handicap (mainly mild hearing loss, 40.0%). Medium-severe HIE groups had more serious hearing handicap than Mild HIE group with a statistical significance (continuity correction χ2=7.383, P=0.007). ABR results showed that, mild HIE is mainly manifested as I wave PL prolonged or poorly differentiated, accounting for 78.1%; medium - severe HIE are mainly manifested as III and V wave PL prolonged central segment abnormalities, accounting for 95.8%; the hearing threshold no more than 60 dB group had better prognosis than the hearing threshold more than 60 dB group prognosis (Fisher exact probability P=0.001). Conclusion ABR reflects that HIE severity and was positively related to the severity of hearing handicap. The more serious hearing loss in neonates is, the worse prognosis the neonates have. ABR can be used to assist the assessment of the prognosis of neonatal HIE.
【摘要】 目的 评价聚四氟乙烯人工听骨在慢性中耳炎听力重建中的应用效果。 方法 回顾性分析2008年8月〖CD3/5〗2009年12月慢性中耳炎手术中接受聚四氟乙烯部分/全人工听骨赝复物(Partial/Total ossicular replacement prostheses,PORP/TORP,美国美敦力公司)行听骨链重建的50例患者50只耳的临床资料及随访结果。比较听力学资料完整的39例39只耳手术前及手术后3～6个月语言频率(05、1.0、2.0 kHz)气导平均听力及气骨导差(airbonecap,ABG)。 结果 随访3～16个月,50只耳手术后1～3个月均干耳,手术后1个月鼓膜愈合良好。9只耳手术中人工听骨表面未置入耳屏软骨,其中1只耳手术后3个月人工听骨脱出,1只耳手术后2个月鼓膜内陷,人工听骨外端盘面外突。39只耳手术前气导听力为3667～9500 dB,平均为(5970±1433) dB;骨导听力为330～5170 dB,平均为(2278±1161) dB;手术后气导听力平均为(4660±1703) dB,与手术前比较,差异有统计学意义(Plt;001)。手术前ABG为10～60 dB,平均为(3700±1212) dB。手术后ABG平均为(2620±1156) dB,与手术前比较,差异有统计学意义(Plt;001)。手术后气导听力提高1000～3000 dB者(手术后听力提高有效)共24只耳,有效率为615%(24/39)。 结论 聚四氟乙烯人工听骨是慢性中耳炎体重建听骨的理想材料,手术后取得良好的听力结果,远期疗效需进一步观察。【Abstract】 Objective To evaluate the effects of hearing reconstruction in chronic otitis meadia with prothesis. Methods Retrospective reviews were performed in 50 patients(50 ears)who had undergone Teflon Partial/Total ossicular replacement prostheses (PORP/TORP) implants from August 2008 to December 2009. Audiometric measurements (the average hearing threshold at 05, 10, 20 kHz) were taken in 39 patients (39 ears) preoperatively and postoperatively. Comparisons of preoperative and postoperative mean air condition and air bone gap (ABG) were performed. Results All patients were followed up for three to 16 months, and the surgery resulted in a dry ear and a healing drum without perforation in all 50 patients. Of the nine patients without tragus cartilage interposed between the drum and the prosthesis, one patient occured prothesis extrusion, and one patient had retraction of the eardrum. The mean air conduction of the 39 patients before and after surgery were (5970±1433) dB and (4660±1703) dB. The mean air bone gap (ABG) was (3700±1212) dB before operation, while it was elevated to (2620±1156) dB when followed up (Plt;001). The surgical treatments were found to be successful (postoperative mean air condition improved 10.0030.00 dB) in 24 ears (615%). The effectiveness was 615%. Conclution Teflon could be an ideal material for ossicular reconstruction in chronic otitis media, and the hearing improvement is obtained after operation, but the problem of extrusion has not been solved at present, and the longterm effects should be surveyed.
ObjectiveTo investigate the effect of distortion product otoacoustic emission (DPOAE) in hearing assessment for children with mumps, by comparing the results of DPOAE and auditory brainstem response (ABR) threshold value examination. MethodsA total of 116 children (232 ears) with mumps and 50 healthy children (100 ears) without mumps received DPOAE and ABR threshold value examination between March 2010 and October 2012. The results of these two examinations were compared in the first place. Then, The passing rate of DPOAE and the normal rate of ABR were compared between the two groups. ResultsThe passing rate in the mumps group was significantly lower than that in the control group[94.83% (220/232), 100.00% (232/232); P<0.05]. The pure tone test of 6 children (12 ears) in the mumps group who did not pass the DPOAE screening test showed that they had slight or moderate hearing loss. The ABR hearing thresholds of all children were normal. No significant difference was detected in Ⅲ wave latency, Ⅰ-Ⅲ and Ⅲ-Ⅴ intervals based on 75 dB nHL (P>0.05). However, there was a significant difference in the latency of I wave, V wave and interval between Ⅰ and Ⅴ between the two groups (P<0.05). The normal rate of ABR was significantly higher than the passing rate of DPOAE in the mumps group (P<0.05). ConclusionThe hearing is normal in all mumps children. However, mumps virus infection can affect the function of the eighth nerve and some auditory nuclei in the brainstem. Although DPOAE can be a useful method for hearing assessment in the mumps children, other hearing tests including ABR should also be considered.
ObjectiveTo summarize the research progress in clinic, development, and genetics of the Klippel-Feil syndrome and its primary ear deformity. MethodsThe related 1iterature at home and abroad concerning the Klippel-Feil syndrome with ear malformation was reviewed, analyzed, and summarized. ResultsThe clinical manifestation and classification of Klippel-Feil syndrome are complicated. As one of the most important accompany malformations, ear deformity mainly leads to hearing impairment and abnormal appearance. However, it is still unclear exactly how the ear deformity forms in Klippel-Feil syndrome, and there is little deep study on the internal connection between the ear deformity and other malformations. The premise for the treatment of Klippel-Feil syndrome includes accurate diagnoses and comprehensive disease assessment, and multidisciplinary collaboration will be the important direction of clinical practice in the future. ConclusionEar malformation is one of the most important congenital dysplasias in the Klippel-Feil syndrome. Its etiology should be based on research in the development and genetic mechanism. And its diagnosis and treatment should be followed by multidisciplinary collaboration. It is important to pay attention to identifying with ear malformation in other syndromes as well.
ObjectiveTo establish a hereditary deafness genetic screening cohort and conduct prospective follow-up to evaluate the effectiveness of the Nantong newborn genetic deafness screening program. MethodsA study based on traditional screening of newborn hearing was conducted from January 2016 to June 2021. Newborns in six hospitals in Nantong were screened for 15 hotspot mutation loci in four common deafness genes. Cohort follow-up was conducted. ResultsA total of 40 403 newborns were included, with a carrier rate of 39.5 per 1 000 for the four common deafness genes. In total, 168 children with hearing loss (HL) were identified at screening and follow-up, of which 56.5% (95 cases) had severe or very severe HL. The detection rate of HL was significantly higher with combined screening than with traditional screening (3.0‰ vs. 3.9‰, P<0.001). All four carriers of pathogenic mutations with normal hearing developed late-onset HL within 2 years of age. At the end of follow-up, six of the polygenic heterozygous mutation carriers had congenital HL and five had late-onset HL. Carriers of polygenic heterozygous mutations were more common as compared to other carrier mutation populations (2.1% vs. 68.8%, P<0.001). In addition, 525 carriers of the SLC26A4 mutation and 118 carriers of the MT-RNR1 mutation were identified and their parents were counselled during the combined screening, and no children with HL was identified during the follow-up period. ConclusionGenetic screening for deafness improves the detection of HL at birth. It is recommended that carriers of pathogenic mutations with normal hearing at birth be followed up every 3 to 6 months until the age of 2 years. Carriers of polygenic heterozygous mutations should undergo extended screening for deafness genes and have their hearing monitored more intensively for early detection of late-onset or progressive HL.