Large Vestibular Aqueduct Syndrome Research Articles

Update on diagnostic procedures in third window syndromes. German version

The diagnosis of third window syndromes often poses achallenge in clinical practice. This paper provides an up-to-date overview of diagnostic procedures in third window syndromes, with special emphasis on superior canal dehiscence syndrome(SCDS), large vestibular aqueduct syndrome(LVAS), andX-chromosomal malformation of the cochlea. Aliterature search was performed in PubMed up to December 2023. Furthermore, aselection of the authors' own cases is presented. Audiovestibular tests for the diagnosis of third window syndromes are most often reported for patients with SCDS in the literature. In this context, cut-off values with different sensitivities and specificities have been defined for different outcome parameters of vestibular evoked myogenic potentials. Current developments include the application of electrocochleography, broadband tympanometry, video head impulse testing, and vibration-induced nystagmus. Genetic analyses are increasingly applied in LVAS. The diagnosis of third window syndromes is always based on the synthesis of patients' symptoms, clinical signs, audiovestibular test results, and imaging.

Wideband acoustic immittance characteristics and machine learning-based diagnostic model for children with large vestibular aqueduct syndrome

Objective:This study was to investigate the wideband acoustic immittance（WAI） characteristics of children with large vestibular aqueduct syndrome（LVAS） and to construct a diagnostic model for LVAS based on WAI and machine learning（ML） techniques. Methods:We performed a retrospective analysis of the data from 38 children（76 ears） with LVAS and 44 children（88 ears） with normal hearing. The data included conventional audiological examination, temporal bone CT scan and WAI test. We performed statistical analysis and developed multivariate diagnostic models based on different ML techniques. Results:The two groups were balanced in terms of ear, gender, and age（P>0.05）. The wideband absorbance（WBA） of the LVAS group was significantly lower than that of the control group at 1 000-2 519 Hz, while the WBA of the LVAS group was significantly higher than that of the control group at 4 000-6 349 Hz（P<0.05）. WBA at 5 039 Hz under ambient pressure had a certain diagnostic value（AUC=0.767）. The multivariate diagnostic model had a high diagnostic value（AUC>0.8）, among which the KNN model performed the best（AUC=0.961）. Conclusion:The WAI characteristics of children with LVAS are significantly different from those of normal children. The diagnostic model based on WAI and ML techniques has high accuracy and reliability, and provides new ideas and methods for intelligent diagnosis of LVAS.

A Long-Term Follow-Up Study on the Auditory Performance and Speech Intelligibility of Mandarin-Speaking Prelingually Deaf Children With Isolated Large Vestibular Aqueduct Syndrome After Cochlear Implantation.

The purpose of this study was to evaluate the auditory performance and speech perception of 104 children with isolated large vestibular aqueduct syndrome (LVAS) and 523 children with no inner ear malformation (IEM) for 5 years after cochlear implantation, in order to explore whether isolated LVAS can affect the long-term hearing and speech rehabilitation of deaf children after cochlear implantation. A cohort study was established consisting of 627 children who underwent cochlear implantation at Beijing Tongren Hospital from 1999 to 2016. The children were examined at 0, 6, 12, 24, 36, 48, and 60 months after cochlear implantation to assess their auditory performance and speech perception using the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) questionnaires. The CAP scores of the isolated LVAS group increased significantly during the baseline to the 24th month, after which they gradually rose until reaching the plateau during the 24th to the 60th month. The CAP scores of the non-IEM group increased significantly during the baseline to the 36th month and then increased steadily. The SIR scores went up significantly during the baseline to the 48th month, and increased in a gradual manner in other stages of isolated LVAS evaluation. In comparison, The SIR scores of non-IEM group grew remarkably from the baseline to the 60th month. There were no significant differences in the CAP or SIR scores between isolated LVAS and non-IEM groups in each stage of evaluation, with the only exception being the CAP score at baseline month after cochlear implantation. The CAP and SIR questionnaires are helpful tools for quantifying the early hearing and speech skills of younger prelingually deafened cochlear implant recipients. This long-term follow-up study shows that the speech and hearing development of children in isolated LVAS and non-IEM groups follow similar patterns, and isolated LVAS does not affect the long-term rehabilitation of deaf children after cochlear implantation.

Characteristics of vestibular-evoked myogenic potentials in children with vestibular malformation and severe sensorineural hearing loss

BackgroundWith the increasing clinical focus on the safety of bilateral cochlear implantation (CI) and the potential risk of bilateral vestibular dysfunction, evaluating vestibular end-organ function in patients with vestibular malformations with accompanying abnormalities has been strongly recommended. ObjectivesTo identify the vestibular-evoked myogenic potential (VEMP) values among children with sensorineural hearing loss (SNHL) with vestibular malformation and assess the effectiveness of VEMP testing for inner ear malformations (IEM) diagnosis. MethodsThis study included 96 children (192 ears), including those with vestibular malformations (48 ears), large vestibular aqueduct syndrome (LVAS) (50 ears), and SNHL without IEM (94 ears; control group). All groups underwent ocular and cervical VEMP (oVEMP and cVEMP, respectively) testing. The response rates, VEMP parameters, and wave characteristics were compared. ResultsThe cVEMP response rates were 37.5 %, 64 %, and 58.51 % and the oVEMP response rates were 42.86 %, 78.95 %, and 77.27 % in the vestibular malformation, LVAS, and control groups, respectively, and significantly differed between groups (cVEMP: X2 = 18.228, P<0.001) (oVEMP: X2 = 7.528, P = 0.023). Significant inter-group differences were observed for the cVEMP and oVEMP latency and amplitude (P < 0.05). The LVAS group's waveform exhibited a prolonged latency and increased amplitude compared with that of the other groups. ConclusionPatients with SNHL were highly susceptible to otolith dysfunction, regardless of comorbid vestibular malformation presence. Measuring VEMPs is an effective and rapid evaluation technique for vestibular function and could provide a basis for vestibular rehabilitation training.

Analysis of 59 cases of large vestibular aqueduct syndrome SLC26A4gene mutation frequency and new mutation sites

Objective:To study the frequency of SLC26A4 gene mutation sites in children with enlarged vestibular aqueduct deafness in Yunnan, report the new mutation sites of SLC26A4 gene, further clarify the mutation spectrum of SLC26A4gene, and explore the association between biallelic and monoallelic mutations of SLC26A4 gene and CT phenotype of inner ear, so as to provide basis for clinical and genetic diagnosis of deafness. Methods:Review the results of temporal bone CT examination of 390 children after cochlear implantation in the Department of Otolaryngology, Kunming Children's Hospital from August 2016 to September 2021. Sanger sequencing of SLC26A4 gene was performed in 59 children with enlarged vestibular aqueduct. According to the genetic test results, the children who underwent temporal bone CT examination were divided into two groups: SLC26A4 biallelic mutation group（homozygous mutation and compound heterozygous mutation）, monoallelic mutation group, and the association with inner ear CT phenotype was analyzed, and the new sites were summarized and analyzed. Results:The c.919-2a>g mutation was the most common mutation in children with enlarged vestibular aqueduct with SLC26A4 gene mutation. Three new variants of SLC26A4 gene were found; CT examination combined with genetic testing found that a part of children with enlarged vestibular aqueduct was associated with SLC26A4 monoallelic mutation or no SLC26A4 gene mutation was detected. Further research is needed to investigate the involvement of other pathogenic factors in the pathogenesis of EVA.

Retracted: Prediction of Hearing Prognosis of Large Vestibular Aqueduct Syndrome Based on the PyTorch Deep Learning Model.

[This retracts the article DOI: 10.1155/2022/4814577.].

Follow-up of infants with mild-to-moderate sensorineural hearing loss over three years.

To observe and analyse the hearing outcome in infants with mild-to-moderate sensorineural hearing loss (SNHL) who failed universal newborn hearing screening (UNHS). This retrospective cohort analysis included infants with mild-to-moderate SNHL and with complete etiological diagnosis and followed up over three years. Out of 96 infants with mild-to-moderate SNHL 72 were stable (75%). Only one case was normal (1.04%), ten cases were improved (10.42%), and 13 were deteriorated (13.54%). The pathogenic mutation of GJB2 was the most common cause (50/96, 52.08%), and most of them were homozygous or complex heterozygous mutations of p.V37I (44/50, 88%). There were 11 cases (11.49%) with large vestibular aqueduct syndrome (LVAS) and nine cases (9.38%) with perinatal risk factors. Infants with GJB2 pathogenic mutation and those without certain etiology mostly had unchanged hearing levels, accounting for 84% (42/50) and 84.61% (22/26), respectively. Hearing deterioration in LVAS was associated with seven cases (63.64%). There was no difference in types of outcomes in perinatal risk factor infants, who were more likely to improve than the other groups, but there were three cases (33.3%) deteriorated to profound hearing loss. Comparison of outcomes of different etiologies showed statistically significant difference (Chi-square=28.673, p=0.000). Normal and improved hearing in infants with mild-to-moderate SNHL was rare before the age of three, unlike in many previous studies, and appropriate intervention is recommended. However, intervention should be adjusted according to the hearing outcomes because of the possibility of improvement or deterioration. The etiological diagnosis of infants with mild-to-moderate SNHL would be helpful for predicting the outcome and managing intervention.

Characteristics of large vestibular aqueduct syndrome in wideband acoustic immittance.

To describe the characteristics of large vestibular aqueduct syndrome (LVAS) in wideband acoustic immittance (WAI) and to explore whether inner ear deformity has an impact on WAI results. Subjects with typical LVAS (LVAS group) and control subjects with a normal anatomical structure of the inner ear (control group) were screened from pediatric patients with cochlear implants using thin-slice computed tomography (CT) images of the temporal bone. With inflammation of the auditory canal and middle ear excluded by routine ear examination and 226 Hz acoustic immittance, WAI data were acquired. Then, the maximum absorbance as the major observation indicator on the mean tympanogram was compared between the LVAS group and control group, and a descriptive comparison of the mean tympanogram and frequency-absorbance curve at peak pressure was performed between the two groups. The LVAS group included 21 cases (38 ears), and the control group included 27 cases (45 ears). All LVAS subjects met the Valvassori criteria, and the VA at the horizontal semicircular canal displayed flared expansion. On the mean tympanogram, the maximum absorbance in the LVAS group (0.542 ± 0.087) was significantly higher than that in the control group (0.455 ± 0.087) (p < 0.001). The tympanogram in the LVAS group showed an overall elevation, and the absorbance at all pressure sampling points was significantly higher than that in the control group (p < 0.001). The frequency-absorbance curve at peak pressure first increased and then decreased in both groups, and the LVAS group showed higher absorbance than the control group in the frequency range below 2,828 Hz. The absorbance at 343-1,124 Hz was significantly different between the two groups (p < 0.001), and 343-1,124 Hz was the major frequency range at which the maximum absorbance on the mean tympanogram increased in the LVAS group. Large vestibular aqueduct syndrome (LVAS) shows increased absorbance in low and medium frequency ranges in WAI. The maximum absorbance on the mean tympanogram can serve as a reliable evaluation indicator. Inner ear factors must be considered when middle ear lesions are analyzed by WAI.

Fluid-solid coupling model and biological features of large vestibular aqueduct syndrome.

Objective: Computed tomography (CT) images of the temporal bone of large vestibular aqueduct syndrome (LVAS) patients were used to establish 3D numerical models based on the structure of the inner ear, which are, in turn, used to construct inner ear fluid-solid coupling models. The physiological features and pathophysiology of LVAS were analyzed from a biomechanical perspective using finite element analysis. Methods: CT images of the temporal bone were collected from five children attending the Second Hospital of Dalian Medical University in 2022. The CT images were used to build 3D models of the inner ear containing the vestibular aqueduct (VA) by Mimics and Geomagic software, and round window membrane models and fluid-solid coupling models were built by ANSYS software to perform fluid-solid coupling analysis. Results: By applying different pressure loads, the deformation of the round window membranes occurred, and their trend was basically the same as that of the load. The deformation and stress of the round window membranes increased with the increase in load. Under the same load, the deformation and stress of the round window membranes increased with the expansion of the midpoint width of the VA. Conclusion: CT images of the temporal bone used clinically could establish a complete 3D numerical model of the inner ear containing VA. Fluctuations in cerebrospinal fluid pressure could affect inner ear pressure, and VA had a limiting effect on the pressure from cerebrospinal fluid. The larger the VA, the smaller the limiting effect on the pressure.

Hearing Loss Related to Gene Mutations in Distal Renal Tubular Acidosis

Introduction: Distal renal tubular acidosis (dRTA) is a disease that may develop either primarily or secondarily, resulting from urinary acidification defects in distal tubules. Hearing loss may accompany primary forms of dRTA. This study aims to determine the characteristics of hearing loss due to different gene mutations in patients with dRTA. Methods: Behavioral and electrophysiological audiological evaluations were performed after otolaryngology examination in 21 patients with clinically diagnosed dRTA. Radiological imaging of the inner ear (n = 9) was conducted and results of genetic analyses using next-generation sequencing method (n = 16) were included. Results: Twenty-one patients with dRTA from 20 unrelated families, aged between 8 months and 33 years (median = 12, interquartile range = 20), participated. All patients with ATP6V1B1 mutations (n = 9) had different degrees of hearing loss. There was one patient with hearing loss in patients with ATP6V0A4 mutations (n = 6). One patient with the WDR72 mutation had normal hearing. Large vestibular aqueduct syndrome (LVAS) was detected in 6 (67%) of 9 patients whose radiological evaluation results were available. Conclusions: LVAS is common in patients with dRTA and may influence the type and severity of hearing loss in these patients. The possibility of both congenital and late-onset and progressive hearing loss should be considered in dRTA patients. A regular audiological follow-up is essential for the early detection of a possible late-onset or progressive hearing loss in these patients.

Advances in the study of fluctuating sensorineural hearing loss

Fluctuating sensorineural hearing loss（FSNHL） is a special type of sensorineural hearing loss, which can be manifested in many clinical diseases. In this paper, some clinical diseases associated with FSNHL are summarized, such as Meniere's disease, large vestibular aqueduct syndrome, acute low frequency sensorineural hearing loss, delayed endolymphatic hydrops, autoimmune inner ear disease and syndromes leading to FSNHL. The pathogenesis, diagnosis and treatment of FSNHL were summarized in order to improve clinicians' understanding of FSNHL, reduce the probability of misdiagnosis of related diseases and improve the prognosis of patients.

Single-cell whole-genome sequencing, haplotype analysis in prenatal diagnosis of monogenic diseases.

Monogenic inherited diseases are common causes of congenital disabilities, leading to severe economic and mental burdens on affected families. In our previous study, we demonstrated the validity of cell-based noninvasive prenatal testing (cbNIPT) in prenatal diagnosis by single-cell targeted sequencing. The present research further explored the feasibility of single-cell whole-genome sequencing (WGS) and haplotype analysis of various monogenic diseases with cbNIPT. Four families were recruited: one with inherited deafness, one with hemophilia, one with large vestibular aqueduct syndrome (LVAS), and one with no disease. Circulating trophoblast cells (cTBs) were obtained from maternal blood and analyzed by single-cell 15X WGS. Haplotype analysis showed that CFC178 (deafness family), CFC616 (hemophilia family), and CFC111 (LVAS family) inherited haplotypes from paternal and/or maternal pathogenic loci. Amniotic fluid or fetal villi samples from the deafness and hemophilia families confirmed these results. WGS performed better than targeted sequencing in genome coverage, allele dropout (ADO), and false-positive (FP) ratios. Our findings suggest that cbNIPT by WGS and haplotype analysis have great potential for use in prenatally diagnosing various monogenic diseases.

Vestibular Loss in Children Affected by LVAS and IP2 Malformation and Operated with Cochlear Implant.

This is a single center cohort study regarding the prevalence of vestibular loss in hearing impaired children affected by large vestibular aqueduct syndrome (LVAS) with incomplete cochlear partition malformation type II (IP2), fitted with cochlear implant (CI). Twenty-seven children received CI operations at 0.4-13 years on one or both ears and tested for vestibular loss with head impulse test, video head impulse test, mini ice-water test and cervical VEMP. Vestibular loss was found in 19% of operated ears and in 13.9% of non-operated ears. The difference was not statistically significant and was not significantly modified by age at implantation, age at testing, sex, presence of SLC26A4 gene mutation or bilaterality. However, the presence of anatomic anomalies at the level of the vestibulum or semicircular canals was significantly associated with a higher incidence of vestibular loss in CI operated children but not in those non-operated. No other factors, such as the surgical access, the electrode type, the presence of Gusher perioperatively, or post-operative vertigo modified significantly the prevalence of vestibular loss. In conclusion, LVAS/IP2 appears to be the major determinant of vestibular loss in these children, with a less obvious impact of CI, excluding the cases with vestibulum/canal anomalies: this group might have a higher risk for vestibular loss after CI surgery.

Three-dimensional wideband absorbance immittance findings in young adults with large vestibular aqueduct syndrome.

To investigate the effect of large vestibular aqueduct syndrome (LVAS) on middle ear sound transmission using wideband absorbance immittance (WAI). WAI results from young adult LVAS patients and normal adults were compared. Averaged energy absorbance (EA) at ambient and peak pressure in the LVAS group showed differences to the normal group. Under ambient pressure, the average EA of the LVAS group was significantly higher than the normal group at frequencies 472-866 Hz and 6169-8000 Hz (p< .05) and lower at frequencies 1122-2520 Hz (p< .05). Under peak pressure, absorbance was increased at frequencies 515-728, 841, and 6169-8000 Hz (p< .05) and decreased at 1122-1374Hz and 1587-2448 Hz (p< .05). An investigation into the effect of external auditory canal pressure on EA across frequencies in the pressure-frequency domain, showed that EA differed significantly in the low-frequency region of 707and 1000 Hz from 0 to 200 daPa and 500 Hz at 50 daPa (p< .05). There was also a significant difference in EA between the two groups at 8000 Hz (p< .05) in the pressure range -200-300 daPa. WAI is a valuable tool to measure the effect of LVAS on middle ear sound transmission. LVAS has a significant effect on EA at low and mid frequencies under ambient pressure, while the frequencies affected are mainly at low frequencies when positive pressure is presented. Level 3a.

Research progress in accurate diagnosis of large vestibular aqueduct syndrome

大前庭导水管综合征（LVAS）是常见的隐性遗传性听力损失疾病。LVAS患者可表现为双耳对称或非对称性的、波动或进行性听力损失，部分为迟发性听力损失；具有低频气骨导差，高频感音神经性听力损失、出现声诱发短潜伏期负反应等临床特点；颞骨高分辨CT（HRCT）和内耳核磁共振（MRI）相结合的影像学检查是诊断的金标准；深入分析SLC26A4基因型，加强SLC26A4单等位基因突变者的遗传学研究，并重视EPHA2、FOXI1和KCNJ10基因等与SLC26A4的协同致病性。大前庭导水管综合征的影像学诊断、听力学诊断和基因诊断三个方面彼此补充且相互结合，可进一步实现其精准诊断。.

Advances in vestibular testing in patients with large vestibular aqueduct syndrome

大前庭水管综合征（large vestibular aqueduct syndrome，LVAS）是临床常见的遗传性听力障碍疾病。近年来，有关该病前庭障碍的报道逐渐增多，目前已知该病的前庭症状具有多样性。新型前庭功能检查技术，如前庭诱发肌源性电位、视频头脉冲试验的开展，使得临床上可以深入了解LVAS的前庭病理生理机制。如LVAS患者前庭诱发肌源性电位具有低阈值、高幅值的特点，这符合“内耳第三窗”疾病的特点；而且，其冷热试验/视频头脉冲试验结果的分离性与“内淋巴积水”疾病相一致。随着LVAS人工耳蜗植入手术的开展，与之相关的前庭症状及前庭功能改变也见诸报道。因此，回顾LVAS相关的前庭评估（症状与前庭功能检查），有助于探寻其病理生理机制，有助于在进行临床决策时，除听力外兼顾患者的前庭功能，从而提高患者生活质量。.

Prediction of Hearing Prognosis of Large Vestibular Aqueduct Syndrome Based on the PyTorch Deep Learning Model.

In order to compare magnetic resonance imaging (MRI) findings of patients with large vestibular aqueduct syndrome (LVAS) in the stable hearing loss (HL) group and the fluctuating HL group, this paper provides reference for clinicians' early intervention. From January 2001 to January 2016, patients with hearing impairment diagnosed as LVAS in infancy in the Department of Otorhinolaryngology, Head and Neck Surgery, Children's Hospital of Fudan University were collected and divided into the stable HL group (n = 29) and the fluctuating HL group (n = 30). MRI images at initial diagnosis were collected, and various deep learning neural network training models were established based on PyTorch to classify and predict the two series. Vgg16_bn, vgg19_bn, and ResNet18, convolutional neural networks (CNNs) with fewer layers, had favorable effects for model building, with accs of 0.9, 0.8, and 0.85, respectively. ResNet50, a CNN with multiple layers and an acc of 0.54, had relatively poor effects. The GoogLeNet-trained model performed best, with an acc of 0.98. We conclude that deep learning-based radiomics can assist doctors in accurately predicting LVAS patients to classify them into either fluctuating or stable HL types and adopt differentiated treatment methods.

Objective vestibular function changes in children following cochlear implantation.

BACKGROUND:To date, systematically objective evaluations of vestibular function in children with cochlear implantation (CI) have been conducted sparsely, especially in children with large vestibular aqueduct syndrome (LVAS).OBJECTIVE:Our goal was to investigate the function of all five vestibular end-organs pre- and post-cochlear implantation in children with LVAS and normal CT.METHODS:In this retrospective cohort study, 34 children (age 4–17 years) with bilateral profound sensorineural hearing loss (SNHL) undergoing unilateral CI were included. Participants included 18 (52.9%) children with LVAS. Objective modalities to evaluate vestibular function included the caloric test, cervical vestibular-evoked myogenic potentials (cVEMP), ocular vestibular-evoked myogenic potentials (oVEMP), and video head impulse test (vHIT). All measurements were performed before surgery and 9 months after surgery.RESULTS:Mean age at CI was 8.1±3.7 years. Caloric testing showed hypofunction in 38.2% of cases before implantation and in 50% after (p > 0.05). We found a significant increase of overall abnormality rate in cVEMP and oVEMP from pre- to post-CI (p < 0.05). In all three semicircular canals tested by vHIT, there were no statistically significant mean gain changes (p > 0.05). Higher deterioration rates in cVEMP (53.3%) and oVEMP (52.0%) after surgery were observed (p < 0.05). In children with LVAS, cVEMP revealed a higher deterioration rate than superior semicircular canal (SSC) and posterior semicircular canal (PSC) (p < 0.05). In children with normal CT, the deterioration rates in VEMPs were both higher than those in vHIT (p < 0.05).CONCLUSIONS:In general, the otolith organs were the most affected peripheral vestibular sensors in children after cochlear implantation. The variations in otolith function influenced by CI were different between children with LVAS and normal CT. We recommend the use of this vestibular function test battery for children with cochlear implantation.

Establishment of Reference Values for Early Auditory Preverbal Skills of Children with Cochlear Implants.

Auditory and verbal abilities of children with early cochlear implant (CI) surgery usually take some time to develop, and a reference index of early development for horizontal comparisons is urgently needed to guide the mapping process and adjust rehabilitation programs. Therefore, the aim of this study was to establish a reference value for early auditory preverbal skills development in children with CI and investigate the developmental curve and influencing factors for these children. The LittlEARS® Auditory Questionnaire (LEAQ) scores of 287 eligible Chinese participants were obtained at 1, 2, 3, 6, 9, 12, 18, 24, 28, and 36 months interval after CI activation. The median and standard deviation of the LEAQ score of each hearing stage for these children with different activation ages were calculated to establish the reference values. Quadratic regression was conducted to fit the expected developmental curve of the LEAQ score ( [0 to 20 months]). With a linear mixed-effects model, we found that the receptive auditory behavior increased rapidly after CI activation, while expressive language skills developed at a steady rate. We also found that the following factors all significantly influenced the LEAQ: the duration of CI use, the development quotient, age of implantation and activation, and the presence of large vestibular aqueduct syndrome or auditory neuropathy spectrum disorder. The reference values and the expected developmental curve for the LEAQ in children with CI established by the present study provides guidance to clinicians and parents as well as realistic expectations regarding language and speech outcomes.

Characteristics of electrically evoked auditory brainstem response in children with large vestibular aqueduct syndrome after cochlear implantation

Background The vestibular aqueduct (VA) width may affect the auditory conduction pathway of large vestibular aqueduct syndrome (LVAS) children. Objective Analyzing the electrically evoked auditory brainstem response (EABR) after cochlear implantation (CI) in severe to profound sensorineural hearing loss (SNHL) children with LVAS. Materials and methods Fifty-four children with SNHL who received CI were selected, including 21 children with LVAS and 33 children without inner ear malformations (IEMs). The VA width was measured in LVAS children. The post-operative EABRs were recorded in all children. Results For the LVAS group, the VA width was positively correlated with wave III (eIII) latency of EABR at the No. 2 electrode (E2), E5, E8, and E11, the VA width was positively correlated with wave V (eV) latency of EABR at E2, E5, E8, and E11. Only the eV latency of E2 showed a significant difference between the two groups. Conclusion and significance SNHL children with LVAS and no IEMs have similar conduction of the auditory pathway. In the LVAS group, the conduction of the auditory pathway becomes better with the decrease of VA width. Rational use of post-operative EABRs and the measurement of anatomical parameters can effectively assess the development of part auditory pathways in LVAS children, which may be helpful in predicting post-operative speech and hearing recovery.