Changes in Listening Effort Through Pupil Response After Atresioplasty in Children With Congenital Aural Atresia.

OBJECTIVES: This study aimed to determine whether the improvement of hearing by surgical treatment alleviates cognitive demands through pupil response in patients with unilateral congenital aural atresia (CAA). DESIGN: A prospective study was performed on patients with unilateral CAA who were scheduled to undergo primary atresioplasty between November 2017 and May 2020. Pure-tone audiometry, auditory digit span test, Korean Speech Perception in Noise test, pupil measurement during speech tests, and questionnaires (Sound-Spatial-Qualities of Hearing Scale; subjective listening effort rating) were performed before and 6 months after surgery. RESULTS: Of 30 consecutive patients who initially enrolled, only 18 patients (12 males and 6 females) were included in the analysis. When the improvement of the air-bone gap and interaural difference of air conduction within 30 dB was defined as a successful hearing outcome, successful hearing improvement was achieved in 50% of the 18 patients. In pupil measurement, the success group had a significantly smaller mean pupil dilation response than the nonsuccess group at 0 and -3 dB signal to noise ratio (SNR) (all p < 0.01). In addition, significant differences were identified between the two groups for peak dilation and peak latency at all noise levels (all p < 0.01). When analyzing the change in pupil response before and after surgery, the difference in relative mean pupil dilation in the success group was significantly greater than that in the nonsuccess group at -3 dB SNR ( p = 0.02). In addition, the success group showed a significantly greater change in peak latency than the nonsuccess group at the -3 dB SNR ( p < 0.01). The difference in peak dilation tended to be greater in the success group than in the nonsuccess group, but the difference was not statistically significant. CONCLUSIONS: Patients with unilateral CAA who achieved surgically improved hearing had a smaller pupil dilation response than those who did not. These results suggest that successful hearing outcomes after surgery in patients with unilateral CAA may reduce the cognitive effort required to understand speech under difficult listening conditions.

Embarazo Gemelar con Discrepancia Morfologíca. Complejo Malformación y Obstrucción Uretral: diagnóstico prenatal / Twin Pregnancy with Morphologic Discrepancy. Complex Malformation and Urethral Obstruction: prenatal diagnosis

Presentamos un caso de embarazo gemelar, monocorial-bianmiotico con discrepancias morfológicas, diagnóstico de malformación y obstrucción uretral prenatal y diagnóstico postnatal de Síndrome de Prune-Belly. Feto A: Dilatación macroscópica del sistema colector con vejiga grande con paredes finas, hidroureter bilateral , dilatación de la uretra, parenquima renal aumentado de tamaño. Feto B: sin alteraciones morfológicas. Postnatal se observó en feto A abdomen flácido y pastoso, ano imperforado, megavejiga e insuficiencia renal

We present a case of twin pregnancy, monocorial-bi-mixtic with morphological discrepancies, diagnosis of malformation and prenatal urethral obstruction and postnatal diagnosis of Prune-Belly syndrome. Fetus A: Macroscopic dilation of the collecting system with large bladder with fine walls, bilateral hydroureter, dilation of the urethra, renal parenchymal increased in size. Fetus B: without morphological alterations. Postnatal was observed in fetus at flaccid and pasty abdomen, imperforated anus, megavejiga and renal failure.

Prognosis of Congenital Anomalies in Conceptions Following In Vitro Fertilization: A Multicenter Retrospective Cohort Study in China.

Background: Conceptions following in vitro fertilization (IVF) or intracytoplasmic sperm injection (ICSI) have an increased risk of congenital anomalies. Few studies have explored the prognosis of fetuses with congenital anomalies. This study aimed to investigate the prevalence and prognosis of congenital anomalies in IVF/ICSI pregnancies, and to analyze the influencing factors contributing to poor prognosis. Methods: In this multicenter retrospective cohort study, we followed 405,473 embryo transfer cycles at 15 reproductive centers between January 2010 and December 2019 and enrolled 2,006 intrauterine pregnancies with congenital anomalies. The relatively positive prognosis group with one or more live births and neonatal survival for more than 7 days was compared with the poor prognosis group with poorer outcomes. Results: Among the 168,270 ongoing intrauterine pregnancy cycles, the prevalence of congenital anomalies was 1.19%, wherein the malformation rates of cycles with late abortion and delivery were 2.37% (716/30,202) and 0.93% (1,290/138,068), respectively. Among all IVF/ICSI cycles with congenital anomalies, the relatively positive prognosis rate was 61.39%. Moreover, the fertilization failure rate (2 pro-nuclei rate < 25%) in the poor prognosis group was significantly higher than that in the relatively positive prognosis group (10.89% vs. 5.09%, p < 0.001). Multivariate logistic regression analysis revealed no significant differences in the relatively positive prognosis rate among the various IVF/ICSI protocols. The relatively positive prognosis rate of fertilization failure cycles was 0.180 times that of normal fertilization cycles. Conclusion: Poor fertilization rates during IVF/ICSI treatments are more likely to have poor prognosis in fetuses or neonates with congenital anomalies, and obstetric management should be strengthened in pregnant women, with which pregnant women should be recommended to strengthen obstetric management.

The Ring-and-Sling complex - Does it "Ring" true?

BACKGROUND: The "Ring-and-Sling" complex (RSC) comprises congenital tracheal stenosis and an abnormal origin/course of the left pulmonary artery. Based on clinical and imaging studies, the luminal narrowing is assumed to be as a result of rings cartilage (forming an "O"). AIMS: This is a postmortem based study of tracheal histology in infants after an autopsy encounter of a case of RSC. SUBJECT AND METHODS: RSC was identified in an infant at autopsy. The tracheal histomorphology revealed the presence of cartilaginous plates (instead of rings) and fibro-elastotic proliferation at the site of trachealis muscle. These changes prompted a study on variations in the histology of the trachea (with no known anomaly) in 35 autopsied neonates and infants. The transverse sections of the trachea were taken at one or more levels (Level 1 - at the level of the thyroid, Level 2 - midway between the thyroid and the carina, and Level 3 - just above the carina. STATISTICAL ANALYSIS: Epi-info software (v1.4.3, CD, US). RESULTS: On histology, 83 sections showed the trachealis muscle on the posterior aspect. A single semicircular cartilage was identified in only 17 of the 83 sections studied (20.5%, 6 in level 1, 9 in level 2 and 2 in level 3). In the remaining 66 sections (79.5%), the cartilage was disposed as multiple plates, ranging in number from 2 to 10. No significant association was found between semicircular cartilage rings and age, sex, gestational age, and level of section (P < 0.05). However, 14 cases with sectioning at all three levels were taken into account; all levels showed more cartilaginous plates compared to single rings, which were more common at level 1 (P > 0.05). CONCLUSIONS: The "ring" in RSC and normal infantile tracheas show cartilage plates with intermittent semicircular cartilage rings. These findings may have surgical implications for tracheal anomalies and bode favorable surgical outcomes.

Ultrasound findings and detection of fetal abnormalities before 11 weeks of gestation.

OBJECTIVE: To determine the proportion of major fetal structural abnormalities that can be detected before 11 gestational weeks. METHODS: We conducted a retrospective study of individual patient files at a tertiary provider of obstetric and gynecological ultrasound in Melbourne, Australia. All women who had a pre-cell-free DNA ultrasound with a crown-rump length of less than 45 mm and had one or more ultrasounds at a later gestation were included in the analysis. The primary outcome was the incidence of a fetal structural abnormality. RESULTS: A total of 3333 cases were included in the final analysis. Overall, 316 fetuses (9.5%) had a structural abnormality detected at any point throughout gestation, of which 86 were major structural abnormalities (2.6%). Sixteen fetal abnormalities were detected before 11 weeks of gestation, including 15 major abnormalities (17.4% of the major anomalies). All major fetal abnormalities detected before 11 gestational weeks were confirmed at later ultrasound examinations or the pregnancy did not continue (in four cases due to termination of pregnancy and in one case spontaneous miscarriage before first trimester morphology ultrasound). CONCLUSION: Detection of fetal abnormalities is possible before 11 weeks of gestation. Early suspicion is more likely in cases of major structural abnormalities.

Effectiveness of Bone Conduction Hearing Aids in Young Children with Congenital Aural Atresia and Microtia.

BACKGROUND The aim of this study was to assess the effectiveness of bone conduction hearing aids in children under 2 years old who have congenital microtia and atresia. MATERIAL AND METHODS This prospective study involved 42 children under 2 years old with congenital microtia and atresia who were divided into 2 groups: 21 with unilateral defect and 21 with bilateral defect. All children were provided with bone conduction hearing aids on a softband. Air and bone auditory thresholds were assessed by auditory brainstem responses (ABRs). The LittlEARS questionnaire was used to evaluate auditory development at baseline and after 6 months of hearing aids use. Behavioral observation audiometry (BOA) was used to assess auditory thresholds and compare aided and unaided hearing. RESULTS After 6 months of hearing aid use, the total score of the LittlEARS questionnaire in children with unilateral defect was 24±5.60, while children with bilateral defect achieved a result of 26.29±6.17. Hearing thresholds in both groups with bone conduction hearing aids improved significantly and approached the normal level. CONCLUSIONS Our results confirm that bone conduction hearing aids provide an effective method of auditory rehabilitation for children with conductive and mixed hearing loss caused by microtia and atresia. Using bone conduction hearing aids in such children is crucial for proper hearing, speech, and language development.

Transiency of postoperative cervical kyphosis seen after surgical correction of sagittal malalignment in adult spinal deformity patients.

OBJECTIVE: In this study, we evaluated factors affecting changes in cervical lordosis after deformity correction and during follow-up period in adult spinal deformity (ASD) patients with severe sagittal imbalance. METHODS: Seventy-nine patients, with an average age of 71.6 years, who underwent long-segment fixation from T10 to S1 with sacropelvic fixation were included. We performed a comparative analysis of the radiographic parameters after surgery (Post) and at the last follow-up (Last). We calculated the Pearson's correlation coefficient and performed multilinear regression analysis to predict independent parameters for Post and Last cervical lordosis (CL), T1 slope (T1S), and thoracic kyphosis (TK). RESULTS: Hyperlordotic changes of -23.3° in CL before surgery was reduced to -7° after surgery, and Last CL had increased to -15.3°. T1S was reduced from 27° before surgery to 14.4° after surgery and had increased to 18.8° at the last follow-up. Through multilinear regression analysis, we found that Post CL and T1S were more significantly affected by the amount of LL correction (p = .045 and .049). The effect of Last T1S was significantly associated with the Last CL; the effect of Last TK, with the Last T1S; and the effect of Post PI-LL, with the Last TK (p < .05). CONCLUSION: The postoperative kyphotic change in CL in ASD patients with preoperative cervical hyperlordosis is not permanent and is affected by drastic LL correction and SVA restoration. To achieve spinopelvic harmony proportional to the difference in LL relative to PI, TK becomes modified over time to increase T1S and CL, in an effort to achieve optimal spine curvature.

[Congenital high airway obstruction syndrome (CHAOS): a case report]. / Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d'un cas.

Congenital upper airway obstruction syndrome is a rare malformation that can be fatal to a newborn baby. It is defined as a complete or almost complete obstruction of the upper airways. CHAOS rate is unknown. We here report the case of a 24-year-old primiparous patient with no medical or surgical history. First-trimester ultrasound didn't show any abnormality. During the second trimester, ultrasonography showed extensive subcutaneous edema, hypertrophied lung with hyperechoic appearance, hypoplastic heart and large-volume ascites associated with severe oligoamnios. After having excluded the most common causes, including isoimmunization (indirect negative Coombs), infections (negative serologies) kariotiping was not performed because the patient refused it. The results of ultrasonography suggested fetal hydrops secondary to CHAOS syndrome, because of pathognomonic signs of pulmonary hypertrophy with inverted or convex diaphragm. MRI was requested; it suggested CHAOS syndrome associated with other malformations: laryngeal atresia, microphthalmia with hypertelorism and deviation of the nasal septum with lack of visualization of the thymic tissue, no clear identification of the bladder, absence of right kidney and hypoplastic left kidney. The outcome of pregnancy was marked by premature labour at the 24th week of amenorrhea. The baby had a birth weight of 1475g, polimalformations such as sexual ambiguity, distended abdomen and polydactyly . The newborn died 3 min after birth. Karyotyping was performed which showed 46XX.

Common pathogenesis for sirenomelia, OEIS complex, limb-body wall defect, and other malformations of caudal structures.

Decades of clinical, pathological, and epidemiological study and the recent application of advanced microarray and gene sequencing technologies have led to an understanding of the causes and pathogenesis of most recognized patterns of malformation. Still, there remain a number of patterns of malformation whose pathogenesis has not been established. Six such patterns of malformation are sirenomelia, VACTERL association, OEIS complex, limb-body wall defect (LBWD), urorectal septum malformation (URSM) sequence, and MURCS association, all of which predominantly affect caudal structures. On the basis of the overlap of the component malformations, the co-occurrence in individual fetuses, and the findings on fetal examination, a common pathogenesis is proposed for these patterns of malformation. The presence of a single artery in the umbilical cord provides a visible clue to the pathogenesis of all cases of sirenomelia and 30%-50% of cases of VACTERL association, OEIS complex, URSM sequence, and LBWD. The single artery is formed by a coalescence of arteries that supply the yolk sac, arises from the descending aorta high in the abdominal cavity, and redirects blood flow from the developing caudal structures of the embryo to the placenta. This phenomenon during embryogenesis is termed vitelline vascular steal.

Acoustic Reflexes in Aural Atresia Patients: Evidence of an Intact Efferent System?

OBJECTIVE: To record crossed acoustic reflex thresholds (xART's) postoperatively from patients after surgical repair of unilateral congenital aural atresia (CAA). To seek explanations for when xARTs can and cannot be recorded. We hope to understand the implications for this central auditory reflex despite early afferent deprivation. METHODS: Patients who underwent surgery to correct unilateral CAA at a tertiary academic medical were prospectively enrolled to evaluate for the presence of xART. Preoperative ARTs in the normal (non-atretic) ear, and postoperative ipsilateral ARTs (stimulus in the normal ear) and contralateral ARTs (stimulus in the newly reconstructed atretic ear; record in the normal ear) were measured at 500, 1000, and 2000 Hz. RESULTS: Four of 11 patients with normal ipsilateral reflex thresholds preoperatively demonstrated crossed acoustic reflexes postoperatively (stimulus in reconstructed ear; record from normal ear). Four other patients demonstrated normal ipsilateral thresholds preoperatively but did not have crossed reflexes postoperatively. No reflexes (pre- or postoperatively) could be recorded in 3 patients. Crossed reflex threshold is significantly correlated with the postoperative audiometric threshold. There was no correlation between ipsilateral and contralateral reflex thresholds. CONCLUSION: Crossed acoustic reflexes can be recorded from some but not all postoperative atresia patients, and the thresholds for those reflexes correlate with the postoperative pure tone threshold. The presence of acoustic reflexes implies an intact CN VIII-to-opposite CN VII central reflex arc despite early unilateral sound deprivation.

Modified Vecchietti vaginoplasty using self-made single-port laparoscopy in Mayer-Rokitansky-Küster-Hauser syndrome.

OBJECTIVE: To introduce the minimally invasive Vecchietti procedure based on single-port laparoscopy with self-made surgical instruments for the surgical management of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH). DESIGN: Surgical video article. The approval of the Institutional Review Board and written consent from the patient were obtained. SETTING: University hospital. PATIENT(S): A 22-year-old woman presented with primary amenorrhea and inability to participate in penetrative sexual intercourse. A gynecological examination revealed a phenotypically normal vulva and absence of the vagina. A normal 46,XX karyotype was expressed. Magnetic resonance imaging and ultrasonic imaging of the abdomen and pelvis indicated that the ovaries were normal in size and there was vaginal agenesis with rudimentary uterus. No other congenital malformations were present. INTERVENTION(S): The modified Vecchietti procedure involved a transvaginal operation and laparoscopic surgery. The epidural catheter was stretched and made into two wires that were tucked into the core of the Veress needle and then folded into four strings for perineal puncture. A transverse incision of about 3 cm was made in the center of the navel, layer by layer into the abdomen, and placed in a self-made single port (made of a small wound protector and an 8-inch glove), introducing the laparoscope. Under laparoscopic surveillance, the Veress needle was inserted through the vesicorectal space guided by the index finger, which was placed in the rectum. The epidural catheter was pumped from the core into the abdominal cavity under laparoscopy. Subsequently, cystoscopy was performed to ensure that no bladder perforation occurred during the needle insertion. The ball-shaped acrylic device and the two rubber stoppers were attached to the epidural catheter of the vulva. An epidural puncture needle with wire perforated the peritoneum through McBurney's point and the opposite McBurney's point, pulling the epidural catheter out of the abdominal cavity. The epidural catheter was curled around the gauze rolls until the ball-shaped device could be accommodated into the newly created cavity at a sufficient depth. After the surgery, the top of neovagina was lifted about 1 cm every day by tightening gauze rolls to increase the traction, until a neovagina 9 cm long was achieved. MAIN OUTCOME MEASURE(S): The clinical and anatomical data such as the operative time, intraoperative bleeding, duration of hospitalization, and measurement of the final length of the newly created canal 30 days after surgery and 3 months after surgery. RESULT(S): The operative time was 30 minutes, and the intraoperative blood loss was 10 mL. The duration of hospitalization was 10 days. Before discharge, the vaginal depth was 9 cm at 5 days after the surgery, and the self-made traction system was removed. A plastic mold was then inserted using povidone-iodine. The vaginal dilator had to be worn day and night. The patient was advised to sit at the corner of a hard bed or chair from time to time to enhance the dilated effect of the vaginal mold. Three months after the surgery, it could be worn each night until regular sexual intercourse was initiated. The canal length 30 days and 3 months after the surgery was nearly 9 cm. CONCLUSION(S): The modified Vecchietti vaginoplasty is a simple, safe, cost-effective, and minimally invasive procedure, offering an anatomic and functional neovagina for MRKH patients.

Active middle ear implant (vibrant soundbridge) in children with unilateral congenital aural atresia.

BACKGROUND: Detailed studies have not been conducted on sound localization and speech perception in noise in patients with unilateral congenital aural atresia (UCAA). AIMS/OBJECTIVES: To evaluate the benefits of the use of the Vibrant Soundbridge (VSB) for UCAA by performing audiometric and sound localization tests. MATERIALS AND METHODS: Four children with UCAA underwent VSB (VORP 503) implantation from 2018 to 2019. Speech perception tests in noise were conducted using the Japanese monosyllable test. The spatial configuration for speech testing consisted of speech presented from the front and noise presented into the normal ear (S0N90). The sound localization test was conducted using nine loudspeakers equally distributed in a semicircle. RESULTS: The children's speech perception in noise 6 months after VSB activation was significantly better than before activation (p < .05). Additionally, the children's sound localization ability after VSB activation was significantly better than before VSB activation. CONCLUSION AND SIGNIFICANCE: The benefits of VSB use in children with UCAA were revealed using audiometric and sound localization tests.

Prevalence and associated factors of birth defects among newborns in sub-Saharan African countries: a systematic review and meta-analysis.

INTRODUCTION: Birth defects are the most serious causes of infant mortality and disability in sub-Saharan African countries with variable magnitude. Hence, this study was aimed to determine the pooled prevalence of birth defects and its associated risk factors among newborn infants in sub-Saharan African countries. METHODS: A total of 43 eligible studies were identified through literature search from Medline (PubMed), EMBASE, HINARI, Google scholar, Science Direct, Cochrane Library and other sources. Extracted data were analyzed using STATA 15.0 statistical software. A random effect meta-analysis model was used. RESULTS: Twenty-five studies in 9 countries showed that the pooled prevalence of birth defects was 20.40 per 1,000 births (95% CI: 17.04, 23.77). In the sub-group analysis, the highest prevalence was observed in southern Africa region with a prevalence of 43 per 1000 (95% CI: 14.89, 71.10). The most prevalent types of birth defects were musculo-skeletal system defects with a pooled prevalence of 3.90 per 1000 (95% CI: 3.11, 4.70) while the least was Down syndrome 0.62 per 1000 (95% CI: 0.40, 0.84). Lack of folic acid supplementation (95% CI: 1.95, 7.88), presence of chronic disease (95% CI: 2.00, 6.07) and intake of drugs (95% CI: 3.88, 14.66) during pregnancy were significantly associated with the birth defects. CONCLUSION: The prevalence of birth defects is relatively high with high degree of regional variabilities. The most common types of birth defects were musculoskeletal defects. Lack of folic acid supplementation, presence of chronic disease and intake of drugs during pregnancy were significantly associated with birth defects.

Cancer Risk by Attained Age among Children with Birth Defects in Arkansas.

BACKGROUND: Few studies have evaluated associations between birth defects and risk of pediatric cancers by age of attainment. Therefore, we assessed the risk of cancer among children with and without birth defects by age at attainment. METHODS: We examined cancer risk in children ≤14 years with and without birth defects born between 1996 and 2011 by linking data from the Arkansas Reproductive Health Monitoring System, Arkansas Central Cancer Registry, and birth certificates. Age of attainment for cancer was calculated as person-years from birth to cancer diagnosis, death, or end of study period, whichever occurred first. Using Cox proportional hazards models, we evaluated associations by attained age groups (<1, 1-4, 5-9, and 10-14 years) between: (1) groups of birth defects (any, chromosomal, and non-chromosomal) and any cancer; (2) non-chromosomal birth defects by organ system and any cancer; and (3) non-chromosomal birth defects and subtypes of cancer. RESULTS: In the cohort of 629,086 children, 23,341 (3.7%) children had birth defects and 1,037 (0.2%) children had cancer. For children with non-chromosomal birth defects, specifically cardiovascular and genitourinary, highest risk of any cancer was observed in first year of life (Hazard Ratio [HR] 18.5; 95% confidence interval [CI] 10.1-33.8). For children with chromosomal birth defects, increased cancer risk was observed among those 1-4 years-old (HR 20.0; 95% CI 8.3-48.4). CONCLUSION: Overall, cancer risk among children with birth defects was highest among those <5 years-old. Our findings, consistent with previous studies, may inform surveillance strategies for children with birth defects.

Large duplication in LMBR1 gene in a large Chinese pedigree with triphalangeal thumb polysyndactyly syndrome.

Polydactyly and syndactyly are digital abnormalities in limb-associated birth defects usually caused by genetic disorders. In this study, a five-generation Chinese pedigree was found with triphalangeal thumb polysyndactyly syndrome (TPTPS), showing an autosomal dominant pattern of inheritance. We utilized linkage analysis and whole genome sequencing (WGS) for the genetic diagnosis of this pedigree. Linkage analysis was performed using a genome-wide single nucleotide polymorphism (SNP) chip and three genomic regions were identified in chromosomes 2, 6, and 7 with significant linkage signals. WGS discovered a copy number variation (CNV) mutation caused by a large duplication region at the tail of chromosome 7 located in exons 1-5 of the LMBR1 gene, including the zone of polarizing activity regulatory sequence (ZRS), with a length of approximately 180 kb. A real-time polymerase chain reaction (PCR) assay confirmed the duplication. The findings of our study supported the notion that large duplications including the ZRS caused TPTPS. Our study showed that linkage analysis in combination with WGS could successfully identify the disease locus and causative mutation in TPTPS, which could help elucidate the molecular mechanisms and genotype-phenotype correlations in polydactyly.

Genetic diagnoses and associated anomalies in fetuses prenatally diagnosed with esophageal atresia.

Esophageal atresia (EA) is a congenital anomaly occurring in 2.3 per 10,000 live births. Due to advances in prenatal imaging, EA is more readily diagnosed, but data on the associated genetic diagnoses, other anomalies, and postnatal outcome for fetuses diagnosed prenatally with EA are scarce. We collected data from two academic medical centers (n = 61). Our data included fetuses with suspected EA on prenatal imaging that was confirmed postnatally and had at least one genetic test. In our cohort of 61 cases, 29 (49%) were born prematurely and 19% of those born alive died in the first 9 years of life. The most commonly associated birth defects were cardiac anomalies (67%) and spine anomalies (50%). A diagnosis was made in 61% of the cases; the most common diagnoses were vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula with esophageal atresia, radial or renal dysplasia, and limb anomalies association (43%, although 12% met only 2 of the criteria), trisomy 21 (5%), and CHARGE syndrome (5%). Our findings suggest that most fetuses with prenatally diagnosed EA have one or more additional major anomaly that warrants a more comprehensive clinical genetics evaluation. Fetuses diagnosed prenatally appear to represent a cohort with a worse outcome.

Long-term Outcomes of Clip Coupler Implantation in Patients with Unilateral Congenital Aural Atresia.

OBJECTIVE: To evaluate the long-term safety and efficacy of the Clip coupler attached to the stapes head in patients with unilateral congenital aural atresia (CAA). METHODS: This single-center retrospective study included 16 Mandarin-speaking patients who had unilateral microtia accompanied by CAA. All patients were divided into two groups: the short-term follow-up group (n = 9) and the long-term follow-up group (n = 7). The floating mass transducer of the Vibrant Soundbridge (VSB) was positioned in the stapes head by the Clip coupler. The safety of the VSB was investigated by comparing preoperative and postoperative bone-conduction (BC) thresholds as well as by complications. The effectiveness was evaluated by functional gain (FG), word recognition score (WRS), speech reception threshold (SRT) and signal-to-noise ratio (SNR). RESULTS: Pre- and post-operative BC thresholds were no different in all patients. And no complications developed. VSB-aided thresholds in the free-field had improved significantly in both short- and long-term follow-up groups. The improvements of WRS were observed in two groups. The monosyllabic VSB-aided WRS in the long-term follow-up group was significantly higher than that in the short-term follow-up group. When speech was from the impaired ear and noise presented to the side of normal ear (SVSBNCL), lower SNRs were found in two groups after VSB implantation. However, there was no statistical difference in aided SNR between the two groups at SVSBNCL status. CONCLUSIONS: Our results show that the FMT connected to the stapes head is a secure and useful device for patients with unilateral CHL/MHL, not only in terms of improved hearing thresholds, but also improved speech intelligibility in quiet and noisy environments.

Morgagni hernia and accessory liver lobe torsion in an adult.

Accessory liver lobe (ALL) is a rare congenital anomaly of the liver and is related to the focal excessive development of liver tissue. Accessory liver lobe torsion (ALLT) is a rare condition that can present with acute abdominal pain. Delay in diagnosis can lead to life-threatening complications such as sepsis and systemic inflammatory response syndrome. Imaging methods, especially computed tomography (CT), are of great importance for prompt diagnosis of acute abdominal pain causes, and CT images should be carefully examined. Herein we present a case of ALLT that was occurred due to Morgagni hernia in a 54-year-old male patient who presented with acute abdominal pain. Contrast-enhanced thoracoabdominal computed tomography (CT) was performed and CT demonstrated the Morgagni hernia and herniation of the accessory liver lobe into the hernia sac. CT showed decreased contrast-enhancement in the accessory liver lobe, compatible with ischemia. We also aimed to emphasize the clinical and CT imaging findings of ALLT.

[Iatrogenic urethral coitus]. / Iatrogene urethrale coïtus.

This case discussion on urethral coitus in Mayer-Rokitansky-Küster-Hauser syndrome shows that this rare syndrome can have major physical and psychological consequences. Further consideration shows that a lack of common sense and a lack of action concerning sexual history-taking and careful vulvar examination is the reason that an iatrogenic sexual problem arose in this case. This was not the result of the initial denial of a rare diagnosis. Both attention to these aspects during training and supervision are necessary, since other disorders and medical treatments can also have a negative impact on sexuality.

Sexual function and quality of life after the creation of a neovagina in women with Mayer-Rokitansky-Küster-Hauser syndrome: comparison of vaginal dilation and surgical procedures.

OBJECTIVE: To compare sexual function and outcomes of quality of life of patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome after vaginal dilation and surgical procedures. STUDY DESIGN: Cross-sectional study from January 2019 to June 2019. SETTING: Tertiary teaching hospital. PATIENT(S): Women with MRKH syndrome treated with vaginal dilation (n = 88) or surgical procedures (n = 45). INTERVENTION: WeChat-based questionnaires were distributed to every group member in our MRKH support group. MAIN OUTCOME MEASURE(S): Sexual functional were assessed by means of the Female Sexual Function Index (FSFI). Outcomes of quality of life were assessed by means of the 12-item World Health Organization Disability Assessment Schedule 2 (WHODAS2). Vaginal length was defined as the maximum depth of the placement of the vaginal mold. RESULT(S): The FSFI scores were similar between the dilation (24.49 ± 4.51) and surgery (23.79 ± 3.57) groups. Except for the higher orgasm score in the dilation group (9.96 ± 3.60 vs. 8.20 ± 2.67), the other dimensions of the FSFI were not significantly different between the groups. No significant differences were found in the WHODAS2 scores between the dilation group (median 8.33 [interquartile range 4.17-15.62]) and the surgery group (6.25 [2.08-14.58]). However, the vaginal length was significantly shorter in the dilation group (6.5 ± 2.04 cm) than in the surgery group (8.1 ± 1.59 cm). CONCLUSION(S): Although the vaginal length was shorter in the dilation therapy group than in the surgical therapy group, sexual function and quality of life were similar between these two groups. Vaginal dilation should be proposed as the first-line therapy for MRKH patients.