Grades of Hearing Loss Affect the Presence of Acoustically Evoked Short Latency Negative Responses in Children with Large Vestibular Aqueduct Syndrome.

Objectives: We aimed to investigate the relationship between grades of hearing loss and the presence of acoustically evoked short latency negative response (ASNR) in children with large vestibular aqueduct syndrome (LVAS), so as to enhance the reference value of ASNR for the diagnosis of LVAS in children. Methods: Two hundred sixteen ears from 108 patients (aged 4-90 months) diagnosed with bilateral LVAS, with slight to profound hearing loss, were enrolled in the present study from January 2012 to December 2018. All of the cases were diagnosed with LVAS according to high-resolution computed tomography (HRCT) or magnetic resonance imaging (MRI) scans of the inner ears. The auditory brain stem response (ABR) tests were performed on these subjects with click stimulus (ck-ABR), and the ASNRs were detected based on the method recommended by previous studies. The degree of hearing loss for each ear was classified by the estimated pure-tone average (PTA) thresholds, which were calculated according to the ck-ABR thresholds. Results: ASNRs were present in 40.7% (88/216) ears during ck-ABR tests. Both thresholds of ABR (Z = 2.977, p = 0.003) and estimated PTA (Z = 2.977, p = 0.003) were significantly higher in the ASNR absent group than in the ASNR present group. The frequency of not profound hearing impairment (<= 80 dB HL) was much higher in the ASNR present group (44/88; 50%) than in the ASNR absent group (40/128; 31.3%) (chi(2) = 7.714, p = 0.005). The results of the logistic regression model, adjusted by cases' age and gender, showed that compared with those ears with profound hearing impairment (> 80 dB HL), the not profound impaired ears were associated with a 2.48-fold increased odds of recording ASNR presence in the ck-ABR test [odds ratio (OR) = 2.48, 95% confidence interval (CI): 1.38-4.46, p = 0.003]. Conclusions: Grades of hearing loss affect the presence of ASNR in children with LVAS, and manifesting as cases with not profound hearing impairment showed increased odds of recording ASNR in the ck-ABR test. Furthermore, more studies should be performed imperatively to determine the diagnosis value of ASNR in children with LVAS.