[Analysis of first-time hearing screening tests among singlets and twin neonates in neonatal intensive care unit].

Objective:To investigate the failure in the hearing screening test among twin neonates in neonatal intensive care unit （NICU） and to further clarify the etiology of neonatal hearing impairment, thus to provide insights into prevention and early intervention. Methods:Automated auditory brainstem response（AABR）, distortion product otoacoustic emission（DPOAE） and acoustic immittance were performed on 1452 neonates（including 130 twins） admitted in NICU from January 2015 to June 2018 and the risk factors including premature birth, hyperbilirubinemia, neonatal respiratory distress syndrome, etc. were analyzed retrospectively by univariate chi-square test and multivariate logistic regression analysis. Results:The incidence of C-section, premature birth, hyperbilirubinemia, low birth weight, very low birth weight, in-vitro fertilization, pregnancy-induced hypertension syndrome and formula or mixed feeding among twin neonates were significantly higher than those of singleton neonates （P<0.05）. The pass rates of the first-time AABR, DPOAE and acoustic immittance were significantly lower than singleton neonates. The proportion of twin neonates who failed the initial screening but recovered in the following test was as high as 72.86%. AABR pass rate was correlated with congenital heart disease, neonatal respiratory distress syndrome, C-section and （very） low birth weight. The pass rate of DPOAE was correlated with low birth weight and C-section. The pass rate of acoustic immittance was correlated with preterm birth, C-section, low birth weight, gestational diabetes and gestational hypertension. The pass rate of diagnostic ABR was associated with gestational diabetes. And the pass rate of diagnostic DPOAE was associated with maternal age ≥40 years old. Conclusion:The first-time hearing screening pass rate of twin neonates in NICU is lower than that of neonatal singleton. Most twin neonates who fail in the first screening test will recover. Preterm birth, neonatal respiratory distress syndrome, （very） low birth weight, congenital heart disease, gestational diabetes, pregnancy-induced hypertension syndrome, maternal age ≥ 40 years old and C-section are associated with the first-time failure in hearing screening tests among twin neonates, thus entailing close follow-up.

[Cochlear implantation in a patient with Noonan syndrome caused by a variant in PTPN11: a case report].

Noonan syndrome is a multisystem disease with widespread heterogeneity regarding the genetic and clinical characteristics, which can be accompanied by distinctive facial dysmorphism, congenital heart defects, short stature, cryptorchidism, lymphatic malformations, bleeding disorders and skeletal malformations. Some patients have hearing impairment. Noonan syndrome is a rare cause of sensorineural hearing loss. The study describes a Noonan syndrome patient with profound bilateral hearing loss. He received a cochlear implantation successfully. The patient had clinical characteristics of Noonan syndrome, and the diagnosis was confirmed by the detection of pathogenic variants in PTPN11 by whole exome sequencing. According to the authors' knowledge, this is the first report regarding cochlear implantation in a Noonan syndrome patient in China.

Risk Factors for Failure in First-Time Hearing Screening Tests among High-Risk Neonates in Neonatal Intensive Care Unit.

OBJECTIVE: The aim of the study was to investigate into the risk factors for failure in the first-time screening test among high-risk neonates in neonatal intensive care unit (NICU) in order to further clarify the etiology of neonatal hearing impairment, thus providing insights into early prevention and intervention. METHODS: We performed automated auditory brainstem response (AABR), distortion product otoacoustic emission (DPOAE), and acoustic immittance (AI) on 2,194 high-risk neonates admitted into the NICU of Shanghai Children's Medical Center from January 2015 to December 2019, and the risk factors, including premature birth, hyperbilirubinemia, and infant respiratory distress syndrome, were analyzed retrospectively by the univariate χ2 test and multivariate stepwise logistic regression analysis. RESULTS: The pass rates of AABR, DPOAE, and AI were 70.21, 78.44, and 93.12%, respectively, in 2,194 cases of high-risk neonates screened, which are significantly lower than those of healthy controls. The most common diagnoses included artificial feeding, preterm birth, C-section, low birth weight (LBW), neonatal hyperbilirubinemia (NHB), neonatal respiratory distress syndrome (NRDS), congenital heart disease (CHD), gestational diabetes mellitus, pregnancy-induced hypertension syndrome, advanced maternal age (AMA), twins, and in vitro fertilization. Stepwise logistic regression analysis indicated that the AABR pass rate was negatively correlated with LBW (p = 0.002), NHB (p < 0.001), NRDS (p = 0.007), artificial or mixed feeding (p = 0.018), and CHD (p = 0.005). The pass rate of DPOAE was negatively correlated with artificial or mixed feeding (p = 0.041), NHB (p < 0.001), LBW (p = 0.007), very LBW (VLBW) (p = 0.008), and C-section (p < 0.001). The pass rate of AI was negatively correlated with revised AMA (≥40 year) (p < 0.001), NHB (p = 0.043), C-section (p = 0.005), and artificial/mixed feeding (p = 0.036). CONCLUSION: The hearing screening pass rates of high-risk neonates in the NICU were lower than those of normal neonates, among which the rate of AABR was significantly lower than that of DPOAE. NRDS, NHB, LBW, revised AMA, CHD, C-section, and artificial feeding are potential risk factors of hearing impairment. The combination of different hearing screening tests is necessary for accurate diagnosis of congenital hearing disorders.

Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report.

BACKGROUND: Diffuse pulmonary lymphangiomatosis (DPL) mainly affects the lung and pleura. There are very few pathological reports of lung damage accompanied by diffuse involvement of the extrapulmonary lymph nodes and surrounding soft tissue. The clinicopathological significance of coexistence of pulmonary and extrapulmonary lesions is unknown. METHODS: Here, we report a 16-year-old male patient. The pathological specimens of the supraclavicular lymph node and soft tissue together with the lung biopsy were analyzed by pathological observation and immunohistochemical staining. Literatures were reviewed and clinical and imaging findings were discussed. RESULTS: The patient presented with coughing and expectoration for 1 year and intermittent hemoptysis for 4 months. Ultrasound revealed swollen lymph nodes in bilateral neck, left armpit, and pubic symphysis. Chest CT scan showed diffuse grid and linear shadows, bilateral pleural thickening, and nodule formation. Multiple enlarged lymph nodes were mainly investigated in bilateral hilar, mediastinal, para-aortic, lesser curvature, and retroperitoneal. Supraclavicular lymph node biopsy confirmed the lymphatic hyperplasia and expansion in the capsule and surrounding soft tissue. The thoracoscopic examination found bloody chylothorax on the left chest. And lung biopsy showed the lymphatic vessel hyperplasia and expansion on the pleura and adjacent lung tissue. Immunohistochemical stains showed that the lymphatic endothelial cells were positive for D2-40 and CD31. Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue was diagnosed based on the aforementioned histological findings. CONCLUSION: Lymphangiomatosis of superficial lymph node mainly involves the capsule of lymph nodes and its surrounding soft tissue. The information obtained from the lymph node biopsy can prompt and assist the diagnosis of DPL.