OBJECTIVE: In order to extend the knowledge of auditory neuropathy spectrum disorder (ANSD), the clinical data of a group of infants and young children with this disorder was reported. METHOD: This retrospective study involved 84 cases (151 ears). The subjects aged from 2 months to 6 years old. Descriptive analysis was carried out on case history, audiological and imaging results and the outcome of cochlear implantation. RESULT: There were a variety of case history, including 11 of hyperbilirubinemia, 2 of family history of deafness, 3 of hypoxia, 1 of prematurity, 1 of nuclei basales pathology, 2 of congenital atelencephalia,and 13 of cochlear nerve deficiency (CND). The audiological results varied too. Click ABR ranged from no response at the maximum output level to Wave V thresholds as low as 70 dB nHL. Behavioral thresholds were mild in 1, moderate in 2 and profound in 20. The inner ear MRI were normal in 16 cases, CND in 12 and CND accompanied by periventricular leukomalacia in 1. 5 cases were implanted in the affected ear. The results in 3 were comparable with that of cochlear loss, 1 were less effective than that of cochlear loss, 1 became none user of the device. CONCLUSION ANSD is a heterogeneous group with a wide variety on case history, audiological and imaging results and the outcome of cochlear implant. Therefore, comprehensive clinical examinations are necessary so that individualized intervention program can be made.
Audiometry, Pure-Tone ; Auditory Threshold ; Child ; Child, Preschool ; Cochlear Implantation ; Female ; Hearing Loss, Central ; pathology ; physiopathology ; therapy ; Humans ; Infant ; Male ; Retrospective Studies

OBJECTIVE: To present the clinical data of a group of children with audiological profile of auditory neuropathy. METHOD: Forty-eight infants and young children who had severely abnormal click ABRs along with present CMs and/or DPOAEs were included in this retrospective study. Click ABRs, CMs, DPOAEs, tympanograms, behavioral thresholds and inner ear MRI were analysed. RESULT: Fourty children present with bilateral audiological profile of AN, 8 were unilateral. Most of the ears (68.2%) were of absent click ABRs at the maximum presentation level of 100 dB nHL. However, some of them had repeatable Wave V at very high presentation levels. All ears showed present CMs while only 41.7% showed preserved DPOAEs. Four children were diagnosed as CND by further investigation of inner ear MRI. CONCLUSION It is recommended that CM testing be carried out routinely in children with severely abnormal click ABRs so as not to miss out AN; Behavioral hearing can not be predicted by auditory evoked responses; if audiological profile of AN is detected, further MRI exploration of well-developed cochlear nerve is necessary.
Audiometry, Pure-Tone ; Child, Preschool ; Diagnostic Errors ; Evoked Potentials, Auditory ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Tests ; Humans ; Infant ; Male ; Nervous System Malformations ; diagnosis ; Retrospective Studies ; Vestibulocochlear Nerve Diseases ; diagnosis ; physiopathology

OBJECTIVES: The incidence of infertility is increasing, more than 30% of them having related abnormal tubal patency. Four-dimensional (4D) hysterosalpingo-contrast sonography (HyCoSy) overcomes the shortcomings of 3D HyCoSy in the diagnosis of tubal patency, showing high specificity and accuracy. In addition, 4D HyCoSy discards iodine allergy and X-ray radiation and possesses easy-operating, contributing to good acceptance in clinical practice. However, there is no research to explore the imaging standards related to the possibility of natural pregnancy after 4D HyCoSy. If a predictive model of postoperative natural pregnancy was established using the analysis of clinical data combined with imaging characteristics of 4D HyCoSy of patients with tubal factor infertility, clinical decision-making can be wisely guided in the future. This study aims to establish a predictive model of natural pregnancy after 4D HyCoSy based on clinical data and imaging characteristics of patients with tubal factor in fertility. METHODS: A retrospective study was conducted for patients who were diagnosed with tubal factor infertility in Hunan Guangxiu Hospital from February 2017 to May 2018. The patients ought to possess complete 4D HyCoSy imaging data and at least one-side-unobstructed fallopian tube. General clinical data and imaging data were collected. Pregnancy outcome was followed up till 3 months after 4D HyCoSy. According to pregnancy outcome, patients were divided into a pregnancy group and a non-pregnancy group. Binary logistic regression was used to analyze the correlation between various variables and natural pregnancy after 4D HyCoSy. The variables with significant difference (P<0.05) in single-factor logistic regression were included in the natural pregnancy probability prediction model. The classification accuracy was further verified with 10-fold cross-validation. RESULTS: A total of 1 085 patients with clinically suspected tubal factor infertility who met the requirements and followed the doctors' prescription were collected. Clinical characteristics (age and duration of infertility) and 4D HyCoSy imaging characteristics (thickness of endometrium from the 3rd to the 7th day after the end of menstruation, visualization of the left fallopian tube, the diffusion of contrast agent around the left ovary, and the diffusion of contrast agent around the right ovary) were independent predictors for natural pregnancy 3 months after 4D HyCoSy. A natural pregnancy probability prediction model was established with the area under the curve (AUC) verified by the 10-fold cross-validation all greater than 0.75, and the best AUC was 0.868. The Q value obtained by the prediction model was the probability of natural pregnancy, and the cutoff value was 0.5. When the Q value was greater than 0.5, it was recommended to attempt natural pregnancy for 3 months, and when the Q value was less than 0.5, in vitro fertilization was adviced. CONCLUSIONS A predictive model for the evaluating probability of natural pregnancy in women with tubal factor infertility after 4D HyCoSy is successfully established based on the analysis for clinical data and imaging characteristics. This model shows a great potential in assisting clinical decision making.
Humans ; Female ; Pregnancy ; Contrast Media ; Retrospective Studies