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Application of Auditory Brainstem Response in the Hearing Loss and Prognosis Research on Neonatal Hypoxic Ischemic Encephalopathy

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.

The Bithermal Test and Pure Tone Test for Patients with Idiopathic Benign Paroxysmal Positional Vertigo

【摘要】　目的　了解原发性良性阵发性位置性眩晕（benign paroxysmal positional vertigo，BPPV）的双温试验及纯音测听结果临床特点。　方法　2009年6月—2010年6月诊断为原发性BPPV患者54例，于手法复位前行双温试验及纯音听阈测试，分析原发性BPPV患者的双温试验、纯音测听结果的临床特点。　结果　40例患耳无半规管轻瘫，其病程为（4.25±2.75）周，14例患耳半规管轻瘫，其病程为（9.21±5.85）周，两组间平均病程差异具有统计学意义（t=4.235，Plt;0.05）；39例后半规管BPPV中11例患耳半规管轻瘫，15例水平半规管BPPV中3例患耳半规管轻瘫，两组患耳半规管轻瘫发生率无统计学意义（χ2=2.679，Pgt;0.05）；39例后半规管BPPV中16例患耳听力下降，15例水平半规管BPPV中7例患耳听力下降，两组患耳纯音测听异常率无明显统计学差异（χ2=0.141，Pgt;0.05）。　结论　原发性后半规管BPPV与水平半规管BPPV半规管轻瘫及纯音听阈异常发生率相近，病程长者更易出现半规管轻瘫。【Abstract】　Objective　To investigate the clinical features of results of bithermal test and pure tone test for patients with idiopathic benign paroxysmal positional vertigo (BPPV), and discuss strategies for prevention. 　Methods　A total of 54 patients diagnosed to have BPPV during June 2009 to June 2010 underwent bithermal caloric test and pure tone test before particle repositioning maneuver. Then, we analyzed the clinical features of the test results. 　Results　The course of 40 patients with canal paresis (CP) was (4.25±2.75) weeks, which had a significant difference from the course of 14 patients with CP in the abnormal side, which was (9.21±5.85) weeks (t=4.235, Plt;0.05). Among the 39 patients with posterior semicircular canal benign paroxysmal positional vertigo (PSC-BPPV), 11 cases of canal paresis were found in the abnormal ear, and of the 15 patients with horizontal semicircular canal benign paroxysmal positional vertigo (HSC-BPPV), 3 cases of canal paresis were found. There was no statistical difference in the rate of canal paresis between the two groups of patients mentioned above (χ2=2.679,Pgt;0.05). Sixteen out of the 39 PSC-BPPV patients and seven out of the 15 HSC-BPPV patients had hearing loss (HL) in the abnormal ear with no statistical difference between the two groups of patients in the incidence of hearing loss (χ2=0.141,Pgt;0.05). 　Conclusion　The incidence of CP and HL in the abnormal ear of patients with HSC-BPPV and PSC-BPPV is similar, and patients with long course of disease are more likely to have CP.

Analysis on the Hearing Test Results for Patients with Chronic Suppurative Otitis Media

【摘要】　目的　了解慢性化脓性中耳炎患者的听力学检查特点。　方法　回顾性分析2005年8月—2008年12月间就诊的382例确诊为慢性化脓性中耳炎患者的听力学检查资料。　结果　慢性化脓性中耳炎中男性和单耳患者居多，任何年龄均可发病，以中度传导性听力损失为主，鼓室图形态各异，耳道容积变大。　结论　慢性化脓性中耳炎是常见的耳科疾病，需及时治疗，听力学检查对于指导治疗方案和评估预后意义重大。【Abstract】　Objective　To learn the audiological characteristics of chronic suppurative otitis media patients.　Methods　We retrospectively analyzed the audiologic data of 382 patients diagnosed with chronic suppurative otitis media treated in our hospital from August 2005 to December 2008.　Results　The number of male chronic suppurative otitis media patients was more than female patients, and single ear infections are more than infections of both ears. The onset of the disease could be at any age. Most of the patients had a moderate conductive hearing loss. All kinds of tympanogram were found,and ear canal volume of the patients was enlarged.　Conclusion　Chronic suppurative otitis media is a common disease of the ear, which needs timely treatment, and audiological tests are important in guiding treatment plans and assessing prognosis.

拉米夫定致听力障碍的不良反应一例

【摘要】　目的　报道1例拉米夫定引起听力障碍的不良反应。　方法　对不良反应发生的过程及原因进行阐述及分析。　结果　患者听力障碍的不良反应系由拉米夫定引起的。　结论　拉米夫定可引起听力障碍的不良反应，临床应用中应引起重视。

Effects of Hearing Reconstruction by Teflon Prothesis in Chronic Otitis Media

【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(05、1.0、2.0 kHz)气导平均听力及气骨导差(airbonecap,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3667～9500 dB,平均为(5970±1433) dB;骨导听力为330～5170 dB,平均为(2278±1161) dB;手术后气导听力平均为(4660±1703) dB,与手术前比较,差异有统计学意义(Plt;001)。手术前ABG为10～60 dB,平均为(3700±1212) dB。手术后ABG平均为(2620±1156) dB,与手术前比较,差异有统计学意义(Plt;001)。手术后气导听力提高1000～3000 dB者(手术后听力提高有效)共24只耳,有效率为615%(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 05, 10, 20 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 (5970±1433) dB and (4660±1703) dB. The mean air bone gap (ABG) was (3700±1212) dB before operation, while it was elevated to (2620±1156) dB when followed up (Plt;001). The surgical treatments were found to be successful (postoperative mean air condition improved 10.0030.00 dB) in 24 ears (615%). The effectiveness was 615%. 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 longterm effects should be surveyed.

Application of Distortion Product Otoacoustic Emission in Hearing Assessment for Children with Mumps

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.

RESEARCH PROGRESS OF KLIPPEL-FEIL SYNDROME WITH EAR MALFORMATION

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.

Newborn hereditary deafness genetic screening in Nantong city: a prospective cohort study

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.

猪链球菌脑膜炎患者行人工耳蜗植入术一例

以听力下降为首发症状的鹦鹉热衣原体肺炎一例并文献复习

目的 阐述以听力下降为首发症状的鹦鹉热衣原体肺炎的临床特点、诊治要点及可能机制。方法 报道以听力下降为首发症状的鹦鹉热衣原体肺炎1例。以“鹦鹉热衣原体”和“听力”为检索词，检索中国知网、万方数据库、维普数据库；以“psittacosis”或“Chlamydia psittaci”和“hearing”为检索词，检索PubMed数据库，检索时限均为2022年3月15日之前。结果 本病例以听力下降为首发症状，3 d后出现高热、咳嗽，胸部CT提示左肺炎症，病情进展快，迅速出现肝功能受损，Ⅰ型呼吸衰竭，行肺泡灌洗液宏基因组二代测序检测，检出鹦鹉热衣原体序列，调整为盐酸米诺环素联合莫西沙星抗感染，听力下降及肺部感染均显著好转。检索数据库，未发现国内相似病例，国外2篇相似病例报道。结论 听力下降可以为鹦鹉热衣原体感染的首发症状及肺外表现，具体机制尚不清楚，及时治疗后可痊愈，临床预后较好。微生物宏基因组二代测序技术有利于明确鹦鹉热衣原体肺炎的诊断。