A clinical epidemiological study on congenital ear malformation (CEM).

BACKGROUND: There is no report about the definition, classification and clinical epidemiological study of congenital ear malformation (CEM).Aims/Objectives: To investigate the definition, clinical classification and distribution of a large number of CEM cases, along with the clinical and epidemiological characteristics associated with congenital malformation of the middle and outer ear (CMMOE). MATERIAL AND METHODS: A total of 3231 cases (4714 ears) with CEM and related malformations were retrospectively analyzed, including 2,658 cases (4,064 ears) CEM and 573 cases (650 ears) preauricular fistulas and accessory ears, specifically related malformations to CEM. RESULTS: Among the 2,658 cases (4,064 ears) CEM, 64.9% cases were male and 35.1% were female. 52.9% cases with bilateral, 29.1% with right and 18.0% with left CEM. A subgroup of 1,050 cases (1,331 ears) was identified as CMMOE out of 1,090 cases (1,379 ears) microtia. In the CMMOE subgroup, 74.0% cases were male, 46.3% on the right side, 26.8% showed bilateral involvement. Out of the CMMOE with available images, there were 947 cases (1,105 ears). Additionally, related malformations including preauricular fistulas and accessory ears, were solely occured in 573 cases (650 ears), except for their occurrence simultaneously with CEM. The classification and distribution of CEM are as follows:1. 2658 cases of CEM (1) 69.9% of them were confined to the ear, including: ① the outer ear 2.6%, the middle ear 17.0% and the inner ear 32.4%, ② two parts simultaneous malformations of the outer ear, the middle ear and the inner ear 17.2%, ③ three parts simultaneous malformations of the outer ear, the middle ear and the inner ear 0.6%, (2) Multiple malformations including in CEM 29.1%; (3) syndromes related to CEM 1.0%.2. 1050 cases of CMMOE (1) 36.3% of them were confined to the middle and outer ears; (2) Multiple malformations associated with CMMOE 61.4%, including hemifacial microsomia 50.5%, accessory ears 9.8%, preauricular fistulas 9.2%, heart malformation 7.1%, and kidney malformation 2.0%; and (3) syndromes related to CMMOE 2.3%, (4) Complications of the ear 16.9%, mainly including otitis media 7.9%, outer ear canal cholesteatoma 6.3%, retroauricular abscess 1.1%, and nasal and pharyngeal complications 18.6%. The proportions of microcia grades I-Ⅴ based on the images of 1105 ears CMMOE were 5.2%, 10.5%, 74.1%, 6.9%, and 3.3%, respectively. Among the 1331 ears CMMOE, the incidence rates of outer ear canal atresia, stenosis, normal, and ossicles malformation were 86.5%, 9.9%, 3.6%, and 98.3%, respectively.Conclusion and Significance: Approximately 69.9% of CEM cases were confined to the ear itself, with the highest proportion being inner ear malformation alone. Multiple malformations were predominantly associated with CMMOE. Syndromes related to CEM were relatively rare. Among cases of microtia, 96.3% were classified as CMMOE. These cases were predominantly observed in males, right ear and exhibited characteristics such as grade III microtia, atresia of the outer ear canal, ossicles malformation, and multiple malformations. The multiple malformations frequently included hemifacial microsomia, accessory ear, preauricular fistula, as well as heart and kidney deformities. CMMOE often coexisted with ear infections, cholesteatoma, and complications in the nasal and pharyngeal regions.

Relationship between congenital malformation of the outer ear and hearing.

Background: There is no report on the relationship between congenital malformation of the outer ear and hearing, which makes it possible to predict the hearing level just based on microtia grades.Aims/Objectives: To investigate the correlation between two types of congenital malformation of the outer ear [microtia and Outer Ear Canal Malformation (OECM)] and hearing, as well as the interrelationship among all three variables.Material and methods: A total of 535 cases (598 ears) of congenital malformation of the middle and outer ear (CMMOE) with hearing data, out of which 319 cases (349 ears) microtia with available images and graded by I-V, 449 cases (482 ears) OECM graded by atresia, stenosis and normal, and 87 cases (87 ears) OEC atresia graded I-IV, 301 cases (301 ears) with materials of microtia, OECM and hearing at the same time were carried out correlation analysis. The Average Air-Conduction Threshold of pure tone (AACT) at 0.5-4 KHz was calculated corresponding to the ears with different malformation grades. The differences in AACT among different malformation grades, the correlation between malformation severity and AACT, as well as the relationship among microtia, OECM and AACT were analyzed. The one-way analysis of variance (ANOVA) was employed to compare the differences in AACT, Kendall's tau-b rank correlation coefficient test was used for correlation analysis. A statistical significance level of p < 0 .05 was applied.Results: Among the 349 ears with microtia, the corresponding AACT values for grades I to V were 61.6, 63.0, 69.9, 75.4, and 75.0 (dB HL), respectively. Comparing grade III to grades II or IV, both p < 0 .05. However, p > 0 .05 between grade I and II or between grade IV and V. The correlation coefficient between microtia grades and AACT r = 0.219, p < 0.05. Among the 482 ears of OECM, the distribution was as follows: 73.6% atresia, 19.1% stenosis, and 7.3% normal, the corresponding AACT values were 64.1, 61.7, and 52.5 (dB HL), respectively. Comparing normal to stenosis or atresia, both p < 0.05, while between atresia and stenosis p > 0.05. The correlation between OECM and AACT was r = 0.104, p < 0.05. The AACT values corresponding to grades I to IV of OEC atresia in the 87 ears were 59.9, 65.1, 71.1, and 64.1 (dB HL), respectively. Comparing these grades, all p > 0.05. The correlation between the degree of atresia and AACT r = 0.23, p < 0 .05. The correlation coefficients for 301 ears microtia to OECM, microtia to AACT, OECM to AACT were r = 0.339, r = 0.163 and r = 0.128 respectively, with all p < 0 .05.Conclusion and significance: There are positive correlations among the degree of microtia, degree of OECM, and AACT values for each other, and so between the degree of OEC atresia and AACT, suggesting that as the severity of microtia or OECM increased, the AACT also tended to be higher, which make it possible to predict the hearing level and the degree of OECM based on microtia grades in clinical practice. Additionally, there are significant differences in AACT values in microtia grade III to grades II or IV, OEC normal to stenosis or atresia, while no differences in microtia grade I to II and grade IV to V, OEC stenosis to atresia, and among the grades I-IV of the OEC atresia.

[Effects of mouth opening breathing for different reasons on maxillofacial development in children].

Objective:To explore the effects of mouth opening breathing for different reasons on children's maxillofacial development. Methods:One hundred and fifty-one children were selected as the research objects of this experiment. They were divided into 49 cases of adenoid hypertrophy group（group A）, 52 cases of tonsillar hypertrophy group（group B） and 50 cases of adenoid with tonsillar hypertrophy group（Group C）. Healthy children in the same period were selected as the control group, a total of 45 cases. The reflex nasopharyngeal measurement parameters, facial development indexes and cephalometric parameters of group A, group B, group C and control group were analyzed, and the incidence of Angle ClassⅡand Angle Class Ⅲ in group A, group B and group C were studied. Results:Compared with the control group, the reflex nasopharyngeal measurement parameters in group A, group B and group C was significantly different（P<0.05）, and the cephalometric parameters changed with variation in groups（P<0.05）. The incidence of Angle Class Ⅱ facial pattern in group A and group C was higher, but the incidence of Angle Class Ⅲ facial pattern in group B and group C was higher（P<0.05）. Conclusion:Adenoid hypertrophy leads to mandibular retraction; tonsil hypertrophy leads to anterior mandibular arch; adenoid hypertrophy and tonsil hypertrophy are easy to lead to clockwise rotation of the mandible. In clinical practice, to avoid children's uncoordinated maxillofacial development, we should correct the maxillofacial situation of children as soon as possible.

Influence of the Coarse Grain Structure of a Titanium Alloy Ti-4Al-3V Formed by Wire-Feed Electron Beam Additive Manufacturing on Strain Inhomogeneities and Fracture.

In this work, based on the multilevel approach, the features of the structure and properties of titanium alloy, formed during high-performance additive manufacturing by wire-feed electron beam technology, were studied. Methods of non-destructive X-ray control and tomography, along with optical and scanning electron microscopy, were used to study the structure at different scale levels of the sample material. The mechanical properties of the material under stress were revealed via the simultaneous observation of the peculiarities of deformation development, using a Vic 3D laser scanning unit. Using microstructural and macrostructural data, as well as fractography, the interrelations of structure and material properties caused by the technological features of the printing process and the composition of used welding wire were revealed.

Efficacy of microsurgery in combined with topical-PDT in treating recurrent respiratory papillomatosis: compare JORRP with AORRP.

Background: Recurrent respiratory papillomatosis (RRP) remains a challenging and frustrating disease to treat.Objective: To explore the efficacy of microsurgery in combined with Topical-PDT in treating recurrent respiratory papillomatosis.Materials and methods: Fifty patients with RRP were treated with microsurgery in combined with Topical-PDT. Medical document of each patient was retrospectively reviewed. Detailed clinical information, metrics of clinical course, and current results were evaluated.Results: Juvenile onset RRP (JORRP) might experience a more aggressive course than AORRP (adult onset RRP) with higher Derkay score (p < .01) and higher operation frequency per year (p < .01). Microsurgical excision combined with Topical-PDT every 25 days achieved "remission" of disease in 78% of patients, "clearance" of disease in 52%, and "Cured" in two patients. Each patient who achieved "remission" of disease, performed 6.82 ± 3.39 operations, and continued 8.93 ± 7.03 months of treatment duration. No statistically differences were found in these two aspects between JORRP and AORRP. A negative correlation between tracheotomy and the efficacy of microsurgery in combined with Topical-PDT was found (p = .025, Pearson's r = -0.3).Conclusions and significance: Microsurgery in combined with Topical-PDT might be a powerful method to treat RRP. Tracheotomy is a negative factor for this therapy.

Anatomical Study of the Chinese Nasal Keystone Region.

BACKGROUND: The keystone region is an important anatomical structure to consider in rhinoseptoplasty, because there are few published data regarding the keystone region in Chinese noses. OBJECTIVES: The authors sought to describe the anatomy of the Chinese nasal keystone region and provide useful knowledge for rhinoseptoplasty. METHODS: Sixteen cadaveric heads (11 males, 5 females) were dissected, measured, and then compared with previously published data. RESULTS: Both the nasal bone caudal margin and nasal bone-upper lateral cartilage overlapping area displayed 5 distinct shapes, and the upper lateral cartilage showed 4 distinct shapes. The nasal bone-upper lateral cartilage overlap was 6.47 mm ± 2.50 in the midline, 3.53 mm ± 2.23 on the left, and 3.81 mm ± 2.56 on the right. The length of the whole quadrangular cartilage was 25.63 mm ± 4.27; 25% ± 8% was overlapped by the nasal bone. The height of the caudal quadrangular cartilage was 18.14 mm ± 3.44, and the width of the nasal bone-upper lateral cartilage articulation was 23.56 mm ± 8.30. The upper lateral cartilage-quadrangular cartilage complex stayed in position on the maxillary crest and nasal spine, even after being dissected from the nasal bone, perpendicular plate of the ethmoid bone, and vomer. Bilateral perichondrial-periosteal mucosa detached from the nasal septum. CONCLUSIONS: We report various anatomical and structural features of the Chinese keystone region, which will be valuable for rhinoseptoplasty planning in Chinese patients.

Nicotine exposure impairs germ cell development in human fetal ovaries cultured in vitro.

In the present paper, we found that human fetal ovaries (at ~16 weeks) express the transcripts for several subunits of the nicotinic acetylcholine receptor (nAChR). Exposure to the drug in vitro resulted in the marked increase of apoptosis in the ovaries in a time and dose-dependent manner. Evidence that adverse nicotine effects are potentially due to an increased level of reactive oxygen species (ROS) and consequent DNA damage, both in the ovarian somatic cells and germ cells, are reported. After 4 days of culture, exposure to 1 mM and 10 mM nicotine caused a 50% and 75% decrease, respectively, in the number of oogonia/oocytes present in the fetal ovaries. These results represent the first indication that nicotine may directly cause apoptosis in cells of the fetal human ovary and may lead to a reduction of the ovarian reserve oocytes and consequent precocious menopause in mothers smoking during pregnancy.

Protective effects of melatonin against nicotine-induced disorder of mouse early folliculogenesis.

In this paper, we show that neonatal mice injected for five consecutive days with nicotine display impaired germ cell cyst breakdown and primordial follicle assembly resulting in decreased ovarian reserve lasting until sex maturation age. The effects of nicotine on the pups ovaries were associated with decreased expression of oocyte specific genes such as Nobox, Lhx8, Figlα and Sohlh2. Moreover, the ovaries of pups injected with nicotine showed increased level of cell oxidative stress and autophagic markers (upregulation of AMPKα-1, increased ratio LC3-II/LC3-I, downregulation of AKT and mTOR). Noteworthy, all these effects were counteracted by the administration of the hormone melatonin in 1 µM. In vitro culture of 0 dpp ovaries for 5 days in the presence of 10 µM nicotine reproduced its effect on germ cell cyst breakdown and primordial follicle assembly, furthermore it also revealing about 20% reduction of somatic cells proliferation, and these effects was prevented when melatonin was added to the medium. Taken together these results show that nicotine exposure can adversely affect the establishment of the ovarian reserve in the mouse likely by locally inducing cellular stress altering the primordial follicle assembly and that melatonin, however, is able to counteract such effects.

Tension-relaxing method-A simplified revision of the endoscopic septoplasty technique: For both aesthetic appearance and functionality.

OBJECTIVE: This article describes a simplified endonasal approach compared with traditional techniques for the correction of crooked noses by using endoscopic tension-relaxing septoplasty in the absence of nasal splints, with attempts to improve both the aesthetic appearance and functionality. STUDY DESIGN: A retrospective study was conducted at our institution with all 26 patients who underwent tension-relaxing rhinoseptoplasty by endoscope between November 2008 and January 2013. METHODS: Patients who were concerned about their aesthetic appearance and nasal obstruction were subjected to anterior rhinoscopy, endoscopic examination of the nasal cavity, and computed tomography for the evaluation of correlations among deformity of the nasal structures and nasal airway. The tension-relaxing method was used in the endoscopic rhinoseptoplasty by an endonasal approach. We introduced this technique in the surgery for patients with a C- or an I-shaped crooked nose. Subjective (visual analog scale) and objective (quantitative electronic meter measurement) assessments were used to evaluate aesthetic appearance. Validated Nasal Obstruction Symptom Evaluation scale and active anterior rhinomanometry were used to assess nasal obstruction. RESULTS: All the patients indicated cosmetic satisfaction and reduced nasal obstruction. In cases with I-shaped and C-shaped crooked nose deformities, pre- and postoperative angle values (mean ± standard deviation) were 13.35 ± 3.36° versus 1.85 ± 1.66° (n = 15) and 153.69 ± 6.48° versus 176.64 ± 2.32° (n = 11), respectively. Postoperative correction rates were statistically significant (p < 0.001) in both groups. Results from active anterior rhinomanometry indicated significant improvement in objective nasal obstruction from a mean baseline value of 0.56 ± 0.07 Pa/cm(3)/s (range, 0.43- 0.69 Pa/cm(3)/s), to a 12-month value of 0.26 ± 0.02 Pa/cm(3)/s (range, 0.23-0.29 Pa/cm(3)/s) (p < 0.001). The mean rhinoseptoplasty duration time was 19.00 ± 3.53 minutes. The nose deformities were significantly improved, with no recurrences of septal deviation or crooked nose, nor complications of septal perforation and nasal infection 12 months after the operation. CONCLUSION: This simple technique is feasible and minimally invasive, and may be particularly beneficial to patients with a deviated septum who seek to improve both their aesthetic appearance and nasal functionality. However, this method is not appropriate for those with a crooked nose caused by nasal bone deformity, lateral cartilages, and severe septal deformity.

Facial palsy in Melkersson-Rosenthal syndrome and Bell's palsy: familial history and recurrence tendency.

OBJECTIVE: The aim of this study was to compare genetic predilection and recurrence tendency between facial palsy in Melkersson-Rosenthal syndrome (MRS) and Bell's palsy METHODS: We carried out an investigation on patients with facial palsy in MRS and those with Bell's palsy who visited the outpatient department in our hospital between February 2009 and February 2013. They were asked about familial history and whether it was the first episode, with the results recorded and compared. RESULTS: There were 16 patients with facial palsy in MRS and 860 patients with Bell's palsy involved in the study. Familial history was positive in 5 of 16 patients (31.3%) with facial palsy in MRS and 56 of 860 patients (6.5%) with Bell's palsy (P < .01). Twelve of 16 cases (75%) with facial palsy in MRS and 88 of 860 cases (10.2%) with Bell's palsy had a history of facial palsy in the past (P < .01). CONCLUSION: Compared to Bell's palsy, facial palsy in MRS has an obvious genetic predilection and recurrence tendency.

Coblation plus photodynamic therapy (PDT) for the treatment of juvenile onset laryngeal papillomatosis: case reports.

BACKGROUND: In treating juvenile-onset laryngeal papillomatosis, the most difficult aspect is preventing recurrence. After a single treatment, recurrence can begin after as soon as 20 days and the recurrent rate can be higher than 90%. The causes of recurrence include the presence of mucosal cells infected with papilloma virus, which are undetectable with the naked eyes, and surgery-induced infection. Photodynamic therapy (PDT) could effectively solve this problem. Virus-infected cells have a very high metabolic energy for capturing and internalizing the photosensitizer, which, after light stimulation, subsequently induces active oxygen species inside the nucleus, which kill infected cells. The second generation of photosensitizer agents (PA) are locally applied to avoid the intravenous systemic damage caused by first-generation PAs, and this method is widely used for the treatment of genital warts to very good effect. METHODS: We used the photodynamic method to treat laryngeal papillomatosis in children and obtained significant efficacy. We followed three juvenile subjects with recurrent laryngeal papillomatosis through a course of treatment (each course includes three PDT sessions), with a follow-up after 6 months. RESULTS: The characteristic procedures involve exposing the larynx with a laryngoscope and using low-temperature plasma technology to visualize the tumor resection, as the effects of plasma technology can reduce postoperative laryngeal edema and reduce intraoperative metastasis. PDT was performed during the first surgery, 20 days after and 30 days after surgery. At the 6-month follow-ups, there was no recurrence. CONCLUSION: This was the world's first successful reported case of the use of PDT treatment for juvenile laryngeal papillomatosis.

Clinical effect analysis of microscopic surgery for epiglottis cysts with coblation.

This study aims to explore the effects and advantages of coblation combined with microscopy to treat epiglottis cysts. Ninety patients with epiglottis cysts were randomly assigned to three groups: the first group: marsupialisation + electric coagulation group, n = 30; the second group: marsupialisation + coblation, n = 30; and the third group: marsupialisation + coblation + microsurgery, n = 30. To compare the cure rate, intraoperative bleeding volume, postoperative pain, operation time and postoperative complications were investigated among these three groups. The comparison among three procedures showed a significant difference for intraoperative bleeding volume, operation time and postoperative pain (P < 0.05), whereas no significant difference was observed for cure rate (P > 0.05). These three procedures are effective in treating epiglottis cysts. Microscopic surgery with coblation has the advantages of less bleeding, short procedure duration, less pain and few complications. Thus, microscopic surgery is worthy of clinical application.

Experimental studies on the inhibition of adenovirus-ING4-OSM therapy on nasopharyngeal carcinoma proliferation in vitro and in vivo.

In the present study, the effects of the co-transfer of the tumor growth inhibitor 4 gene (ING4) together with the Oncostatin M (OSM) were investigated on tumor regression and subsequent tumor recurrence. We constructed a recombinant adenovirus carrying ING4 and OSM, which could induce high-level expression of these three genes in NPC CNE-1 cells. Ad-ING4, Ad-OSM and Ad-ING4-OSM infection all inhibited the growth of CNE-1 cells in vitro, while the Ad-ING4-OSM exerted the strongest inhibitory effect. In CNE-1 xenograft tumor models mice, an intratumoral injection of Ad-ING4, Ad-OSM and Ad-ING4-OSM resulted in a reduced tumor burden, compared to normal saline controls. Therefore, we suggested that the introduction of adenovirus-mediated ING4 and OSM genes could synergistically decrease the recurrence or metastases and develop a control of NPC tumors, which advocate a promising therapeutic future in NPC treatment.

Antiproliferative potential of Artemisia capillaris polysaccharide against human nasopharyngeal carcinoma cells.

One water-soluble polysaccharide fraction (WACP) was purified from the Artemisia capillaris by DEAE-cellulose anion-exchange and Sephacryl S-400 gel-permeation chromatography. Based on gas chromatography (GC) analysis, WACP was an arabinogalactan (AG), consisting of arabinose and galactose in the ratio of 4:2. Its molecular weight was about 5.8×10(4) Da. The present study demonstrated the anticancer potential of WACP on human nasopharyngeal carcinoma CNE-2 cells and elucidated its possible mechanisms. MTT and flow cytometric analysis identified that WACP had a potent anti-proliferation activity on CNE-2 cells by inducing apoptosis, which was parallel with the morphological changes of CNE-2 cells under inverted microscope. In addition, WACP triggered the apoptosis via the mitochondrial apoptotic pathway, which included the loss of mitochondrial membrane potential, release of cytochrome c and activation of caspase-3/9. Taken together, these results suggest that WACP has anticancer potential in the treatment of human nasopharyngeal carcinoma.

[Correlation between allergic rhinitis and childhood obstructive sleep apnea-hypopnea syndrome].

OBJECTIVE: To investigate the correlation between allergic rhinitis and obstructive sleep apnea-hypopnea (OSAHS) syndrome in children. METHOD: (1) According to medical history, physical signs, skin-prick test, serum sIgE, endoscopic examination and polysomnography, the incidence of allergic rhinitis was confirmed in 574 cases of childhood obstructive sleeping apnea-hypopnea syndrome in our hospital between July in 2008 to June in 2010. (2) Effects of anti-allergic drugs were observed on 78 children with OSAHS and allergic rhinitis meanwhile. RESULT: (1) 258 cases of allergic rhinitis were confirmed in 574 cases of OSAHS, accounting for 44.9% of the OSAHS cases and 50.4% of all cases of allergic rhinitis during the same period. Most of them were perennial allergic rhinitis (223 cases, 86.4%), and 72.5% of them were aroused by fungal allergen. Compared with other allergen, statistically significant difference was found (P < 0.05). (2) After receiving anti-allergic drugs regularly for 3 weeks, 40 cases suffering from mild and moderate OSAHS and allergic rhinitis, 3 cases out of 38 cases suffering from serious OSAHS and allergic rhinitis showed satisfactory results, while other cases had little improvement. CONCLUSION: Allergic rhinitis is closely related to childhood OSAHS, and perennial allergic rhinitis dominates. The most common allergen is fungal allergen, the second is house and flour dust mites. So for patients of mild and moderate OSAHS with allergic rhinitis, regular anti-allergic drugs can lighten OSAHS effectively and may make patients avoid surgery. Severe OSAHS cases can receive surgical intervention if prior anti allergic therapy fails. Anti allergic therapy should be adopted routinely after tonsillectomy and adenoidectomy in case of hypopnea due to hypertrophy of inferior turbinate or tubal torus in allergic rhinitis.