先天性小耳畸形耳廓再造及 BAHA 安装一期手术的研究

目的:研究通过多孔高密度聚乙烯(MEDPOR)支架植入,一期完成全耳廓再造+骨锚式助听器BAHA安装,术后观察耳廓外形变化,听力恢复程度,了解一期联合手术的初期临床疗效。方法:对前来就诊的先天性小耳畸形患者,术前进行外形,听力,影像学评估,对于Jahrsdoerfer评分小于及等于5分,行多孔高密度聚乙烯(MEDPOR)支架植入,一期耳廓再造术+骨锚式助听器(BAHA)植入术。术后两周起,定期观察患者耳廓改善及听力恢复情况,了解手术的治疗效果。结果:临床病例3例,术后两周起开始随访,再造耳廓外形逼真,无支架外露;所有患者听力均获得较大程度改善。结论:该方法具有疗程短、并发症少、临床效果佳等优点。该方法切实可行,可用于临床推广应用。     

先天性小耳畸形危险因素研究进展

先天性小耳畸形即小耳症,主要指耳廓、外耳道的畸形、听力损害等,少数患者合并外耳道闭锁、中耳及面部畸形。尽管先天性小耳畸形的发病机制尚未完全明确,但已有研究证实其发生可能与环境和遗传因素有关。因此,对于相关流行病学调查的相关因素与风险因素的研究与规避,并以此指导开展针对性防治工作显得尤为重要。本文将从流行病学特征及相关危险因素两个方面对于病因学进行探讨与总结。     

Goosecoid基因突变与先天性小耳畸形的关系

目的:对收集的43例先天性小耳畸形患者进行遗传因素分析,对Goosecoid(GSC)基因测序,探讨GSC基因与先天性小耳畸形的关系。方法:采集43例小耳畸形患者的外周血提取基因组DNA,对GSC基因的三个外显子分别设计引物,经PCR扩增纯化后直接测序。结果:6例患者在第二外显子的第197bp处发生了C→T的同义突变,编码氨基酸仍为酪氨酸;2例患者在第三外显子的第125bp处发生了A→G的错义突变,编码氨基酸由谷氨酸变成谷氨酰胺。结论:在先天性小耳畸形的患者中发现了GSC基因的突变。     

EarWell耳矫治器对新生儿耳廓畸形的效果及其预后不良影响因素分析

摘要 目的：探讨EarWell耳矫治器对新生儿耳廓畸形的效果及其预后不良影响因素。方法：选取我院2019年3月到2022年3月收治的60例（共78耳）耳廓畸形新生儿作为研究对象,依照患儿年龄进行分组,将年龄≤7 d的16例（21耳）患儿分为A组,8-14 d的21例（26耳）分为B组,15-28 d的23（31耳）分为C组,对所有患儿采取EarWell耳矫治器治疗,对比不同组别患儿新生儿耳廓畸形的治疗总有效率,并发症和矫治时间。通过复查随访评价患儿预后情况,将48例（60耳）预后良好的患儿分为预后良好组,将12例（18耳）预后不良的患儿分为预后不良组,对比两组患儿临床相关资料。最后,采用logistic回归分析分析EarWell耳矫治器对新生儿耳廓畸形治疗预后不良的影响因素。结果：三组患儿治疗总有效率差异显著,A组（100.00 %）高于B组（88.46 %）与C组（64.52 %）（P＜0.05）;A组患儿并发症发生率为14.29 %,B组为15.38 %,C组为19.35 %,组间对比有差异（P＞0.05）;预后良好组与预后不良组患儿性别、畸形部位对比无差异（P＞0.05）,预后良好组与预后不良组患儿年龄、耳廓畸形Max分型、外耳道闭锁分级以及容貌耳长差值对比差异显著（P＜0.05）;logistic回归分析结果显示,年龄、耳廓畸形Max分型为新生儿耳廓畸形治疗预后不良的独立影响因素（P＜0.05）。结论：EarWell耳矫治器对于新生儿耳廓畸形矫治效果显著,并发症发生率较低,且年龄越小矫治效果越好。年龄、耳廓畸形Max分型为耳廓畸形新生儿的预后不良的独立影响因素,临床上针对此类患儿需采取一定预防措施,预防预后不良现象的发生。     

自体肋软骨移植耳廓再造术的护理

耳廓是由皮肤和软骨等构成的位于头颅两侧较薄的形态复杂的三维立体器官［１］。先天性小耳畸形系胚胎时期第一、二鳃弓发育异常所造成［２］,患耳无正常的耳廓形态,常伴有外耳道闭锁,耳屏缺如,严重影响外观,由于耳廓形态十分复杂,全耳再造术是整形再造术中最复杂最...     

睫状神经营养因子对听觉损伤的保护作用

本研究以耳廓反射、听觉脑干诱发电位、耳蜗生物电和耳蜗铺片组织学检测为指标,观察重组人睫状神经营养因子对豚鼠庆大霉素耳毒性的防治作用。实验结果表明,睫状神经营养因子能减轻庆大霉素对耳蜗及听神经的损害,具有保护听觉功能的作用。     

先天性耳前瘘管二次手术68 例治疗体会

目的:探讨先天性耳前瘘管术后复发原因,以及二次手术临床治疗的效果。方法:回顾性分析我院自2006年1月至2010年12月的共诊断收治的68例复发先天性耳前瘘管病例,观察其二次手术效果。结果:68例先天性耳前瘘管复发患者中,66例Ⅰ期愈合,治愈率97.06%。结论:再次手术是治疗先天性耳前瘘管术后复发的有效途径,完全切除残余的瘘管及上皮组织是防止再复发的关键。     

64排螺旋CT三维透明化VR重建在内耳畸形诊断中的应用

目的:研究三维透明化VR重建在内耳畸形中的表现,为先天性内耳疾病提供准确的影像诊断和临床治疗信息.方法:回顾32耳内耳畸形的64排HRCT容积数据,行三维透明化重建处理,按内耳畸形分类总结三维透明化重建方法及影像表现.结果:32耳的三维透明化VR重建图像结合透明化MPR重组图像均能很好揭示内耳畸形病变部位及程度,其中VR图像可以直观、立体地显示畸形的空间形态结构,透明化MPR重组图像可很好显示病变细节.本组先天内耳发育畸形有以下几种:耳蜗未发育(2耳);共同腔畸形(4耳);不完全分隔Ⅰ型(2耳,两例患者对侧耳均为共同腔畸形);不完全分隔Ⅱ型(即Mondini型)(16耳,多合并前庭、半规管及前庭导水管畸形);单纯前庭-半规管畸形2耳;单纯前庭导水管扩大(6耳).结论:三维透明化个性重建能准确评价内耳先天性疾病的类型和程度,为临床治疗提供重要的参考依据.     

耳内镜与显微镜下Ⅰ型鼓室成形术治疗慢性化脓性中耳炎的疗效比较及术后短期内听力恢复效果的影响因素分析

摘要 目的：比较耳内镜与显微镜下Ⅰ型鼓室成形术治疗慢性化脓性中耳炎的疗效,并分析术后短期内听力恢复效果的影响因素。方法：选取2019年3月~2022年2月期间我院收治的158例慢性化脓性中耳炎患者,均接受Ⅰ型鼓室成形术治疗,根据手术方式不同分为耳内镜组（81例）和显微镜组（77例）,比较两组临床疗效及术后6个月的听力恢复不良发生率。收集相关资料,采用多因素Logistic回归分析术后短期内听力恢复效果的影响因素。结果：两组鼓膜穿孔发生例数组间对比无统计学差异（P>0.05）。耳内镜组的手术时间、住院时间短于显微镜组,术中出血量、医疗费用、干耳时间>1个月例数、耳廓麻木发生例数少于显微镜组（P<0.05）。耳内镜组、显微镜组术后6个月的听力恢复不良发生率组间对比无统计学差异（P>0.05）。单因素分析结果显示,慢性化脓性中耳炎患者术后6个月听力恢复效果与鼓室黏膜、鼓膜张肌腱、咽鼓管情况、是否鼓室硬化、听小骨周围是否肉芽包裹、术前鼓室内是否有脓性分泌物有关（P<0.05）。多因素Logistic回归分析显示,鼓膜张肌腱缺损、咽鼓管不通、鼓室硬化、听小骨周围肉芽包裹、术前鼓室内有脓性分泌物是慢性化脓性中耳炎患者术后短期内听力恢复不良的危险因素（P<0.05）。结论：与显微镜下Ⅰ型鼓室成形术治疗慢性化脓性中耳炎相比,耳内镜下进行手术可缩短手术时间、住院时间,减少术中出血量和住院费用,降低并发症发生率。此外,患者术后短期内听力恢复效果受到鼓膜张肌腱、咽鼓管、鼓室硬化、听小骨周围肉芽包裹、术前鼓室内脓性分泌物等多种因素的影响。     

一个先天性耳瘘管家系的调查

先天性耳瘘管是较常见的耳部畸形,是一种胚胎发育异常所致的上皮性小盲管。耳瘘管多开口于耳轮脚前或耳轮上,一般无害,但有的可反复感染,需手术治疗。     

Framework growth after reconstruction for microtia: is it real and what are the implications?

Historically, fashioning an auricle for a patient born with microtia has been one of the most challenging endeavors in the repertoire of reconstructive surgeons. Despite many ideas advanced on types of materials for the auricular framework, the hands-down favorite and today's medium of choice is autogenous costal cartilage. A subject that remains up for discussion, however, is the question of growth potential in these cartilaginous frameworks. Popularization of the surgical technique for auricular reconstruction has led to much bandying about of opinions on this very important question of growth. Although previous reports allude to the probability of an increase in auricular size after reconstruction, this report is the first to document changes in auricular size with measurements taken directly from patients at the time of graft implantation and during subsequent long-term follow-up. The goals of this study are to define the behavior of the autogenous framework after reconstruction of the microtic auricle. This information serves to clarify the issue of proper framework sizing and to make evident the early age at which reconstruction can begin.The records of patients who underwent costal cartilage auricular reconstruction for grade III microtia between 1990 and 1996 were reviewed, and a cohort of 10 patients was chosen for inclusion based on availability for follow-up and lack of any interval modifications of their cartilaginous framework. The average age was 6.7 years, and the average time interval from initial reconstruction to follow-up was 3.2 years. Measurements of the auricular framework height and width were taken at implantation and at time of final follow-up, and measurements were recorded of the normal ears of patients with unilateral microtia. The mean auricular size was examined for significance of interval change using the two-sample Student's t tests, assuming unequal variances.The results revealed an average height increase of 5 mm (10.4 percent) in the study population. Auricular width changes averaged 2.75 mm (7.02 percent). Growth trends revealed a distinct tendency toward increasing auricular framework size over time, with slowing as patients neared adolescence. Comparison of the reconstructed auricle to the normal ear of each patient with unilateral microtia showed that the reconstructed ear paralleled the growth of the normal side, with no statistically significant differences in height or width at follow-up.This is the first report of auricular framework behavior based on patients having direct measurement of their framework initially and in long-term follow-up. This patient sample underscores a clear pattern of growth in the reconstructed auricles paralleling that of the normal ears. The implications of this finding are important in that the initial oversizing of the framework becomes unnecessary. Moreover, the decision as to age at initial reconstruction is not affected by anticipated growth rates.     

Differential expression of long noncoding RNAs in congenital microtia

ObjectiveTo analyse lncRNA expression profiles in microtia using bioinformatics analysis.MethodsWe examined lncRNA expression profiles in residual ear cartilage and normal ear cartilage from individual congenital microtia patients.ResultsThe gene chips used in this study included 30586 lncRNAs and 26109 mRNA probes. Intotal, 180 lncRNAs with differential expression weredetected in the residual ear cartilage compared with the normal cartilage, including 74 up-regulated and 106down-regulated lncRNAs. Signalling pathway analysis highlighted glyceride metabolism, osteoclast differentiation, andtumour growth. The results of qRT-PCR analysis were consistent with those of themicroarray.ConclusionDifferential expression of lncRNAs occurs in microtia. These lncRNAs and related signalling pathways may play an important role in the occurrence and development ofmicrotia.     

Correction of congenital microtia using the tissue expander

We attempted auricular reconstruction using Radovan-type inflatable silicone expanders in six children and one adult, with the complete hypoplastic, the conchal remnant, and constricted type of microtia. Ear frameworks, including the helix, anthelix, concha, and tragus, were prepared using autologous rib cartilage. Based on the surface area of the normal adult auricle, the silicone expander was tentatively shaped and sized into a rotated semiellipse and expanded with 70 cc saline. Auricular reconstruction on the framework was completed at the time of insertion in four of the seven patients, requiring no elevation of the ear. The reconstructed auricle was satisfactory in both color and texture and had nearly normal sensation. Mild complications were noted in three of the seven patients. However, no resorption of the inserted rib cartilage has been observed 14 months to 2 years and 5 months after the operation. Slight shrinkage of the expanded skin was noted in each patient.     

Microtia reconstruction: does the cartilage framework grow?

The use of free rib cartilage ear frameworks in unilateral microtia reconstruction has prompted much discussion about their potential for growth. The senior author has reconstructed ear frameworks in 132 microtia patients, most of whom were under 3 years of age when surgery was initiated. Of this group, 29 were assessed for ear growth through comparison of the lead-plate model of the original normal ear to the normal ear growth and the reconstructed ear framework after a period of at least 2 years. Similarly, 14 reconstructed ears were compared to 14 normal ears at least 2 years after reconstruction. The perimeters of tracings made from the original lead plates and of tracings of normal and reconstructed ears were determined by image analysis techniques. The results demonstrated no significant difference in growth between normal ears and reconstructed ear frameworks after an interval of at least 2.5 years. Therefore, the reconstructed ear is growing at a rate similar to that of the normal ear.     

Conchal transposition flap for postburn ear deformities

Severe thermal injuries to the external ear often lead to extensive loss of peripheral structures such as helix and lobule but frequently spare the more central parts of the ear, even though they may be grossly deformed by scar contracture. The use of spared conchal structures as a transposition flap in combination with remodeling of the residual auricle and release of surrounding scar when indicated has been a useful technique in the reconstruction of a frequently occurring type of postburn ear deformity. Twenty-four ears have been reconstructed in 18 patients over the past 5 years using a conchal transposition flap. The residual concha with its overlying skin can be transposed superiorly, based on a very narrow pedicle in the area of the crus helicis. The raw central area remaining is then resurfaced with a split-thickness skin graft. This technique maximally utilizes the unique remaining auricular elements and can provide a satisfactory reconstruction in selected patients without resorting to more complex and extensive procedures. There have been no significant complications in this series, and patient acceptance of the results has been excellent.     

Congenital upper auricular detachment

A rare case of unilateral congenital ear deformity has been presented. The deformity is characterized by detachment and posterior rotation of the right upper auricle in an otherwise grossly normal auricle. We believe that this deformity may be related to defective mesenchymal fusion or accretion between the auricular hillocks of the hyoid and mandibular arches. Satisfactory correction was achieved by auricular repositioning with two triangular flaps.     

Tissue engineering: revolution and challenge in auricular cartilage reconstruction

External ear reconstruction for congenital deformity such as microtia or following trauma remains one of the greatest challenges for reconstructive plastic surgeons. The problems faced in reconstructing the intricate ear framework are highly complex. A durable, inert material that is resistant to scar contracture is required. To date, no material, autologous or prosthetic, is available that perfectly mimics the shapely elastic cartilage found in the ear. Current procedure involves autologous costal cartilage that is sculpted to create a framework for the overlying soft tissues. However, this is associated with donor-site morbidity, and few surgeons worldwide are skilled in the techniques required to obtain excellent results. Various alloplastic materials have therefore been used as a framework. However, a degree of immunogenicity and infection and extrusion are inevitable, and results are often disappointing. Tissue-engineered cartilage is an alternative approach but, despite significant progress in this area, many problems remain. These need to be addressed before routine clinical application will become possible. The current tissue-engineered options are fragile and inflexible. The next generation of auricular cartilage engineering is promising, with smart materials to enhance cell growth and integration, and the application of stem cells in a clinical setting. More recently, the authors' team designed the world's first entirely synthetic trachea composed of a novel nanocomposite material seeded with the patient's own stem cells. This was successfully transplanted in a patient at the Karolinska Hospital in Sweden and may translate into a tissue-engineered auricle in the future.     

Management of the hairline using a local flap in total reconstruction for microtia

In cases of microtia with a low hairline, the manner in which hair is removed from the reconstructed auricle must be taken into consideration. This is one of the most common but difficult problems with reconstruction for microtia. The authors describe a new technique that uses a simple regional flap to resolve this problem. The hair-bearing skin in the estimated auricular region and its covering are removed using a local flap from the hairless mastoid region. This is done in the first stage of auricular reconstruction, the costal cartilage grafting is done in the second stage, and elevation of the auricle is done in the last stage. In 38 auricles of 36 patients who were treated from 1993 to 1995, eight auricles of eight patients were treated with this technique. In all cases, the hairless flap healed well, without vascular stasis or skin necrosis. In addition, no complications from using this technique occurred in the later stages of auricular reconstruction. With this technique, the skin of the flap provides a good texture and color match to the auricle. In addition, the skin of the flap has good elasticity for the cutaneous pocket for cartilage grafting. The harvested area of the flap can be hidden behind the reconstructed auricle. The authors initially wondered whether the marginal scar of the transposed flap's position in the auricle would be conspicuous. However, all of the scar became inconspicuous because it was positioned in the scaphoid fossa.