Clinical role of electrocochleography in children with auditory neuropathy spectrum disorder

Abstract

OBJECTIVES To assess electrocochleography (ECochG) to tones as an instrument to account for CI speech perception outcomes in children with auditory neuropathy spectrum disorder (ANSD). MATERIALS & METHODS Children (<18 years) receiving CIs for ANSD (n=30) and non-ANSD (n=74) etiologies of hearing loss were evaluated with ECochG using tone bursts (0.25–4 kHz). The total response (TR) is the sum of spectral peaks of responses across frequencies. The compound action potential (CAP) and the auditory nerve neurophonic (ANN) in ECochG waveforms were used to estimate nerve activity and calculate nerve score. Performance on open-set monosyllabic word tests was the outcome measure. Standard statistical methods were applied. RESULTS On average, TR was larger in ANSD than in non-ANSD subjects. Most ANSD (73.3%) and non-ANSD (87.8%) subjects achieved open-set speech perception; TR accounted for 33% and 20% of variability in the outcomes, respectively. In the ANSD group, the PTA accounted for 69.3% of the variability, but there was no relationship with outcomes in the non-ANSD group. In both populations, nerve score was sensitive in identifying subjects at risk for not acquiring open-set speech perception, while the CAP and the ANN were more specific. CONCLUSION In both subject groups, the TRs correlated with outcomes but these measures were notably larger in the ANSD group. There was also strong correlation between PTA and speech perception outcome in ANSD group. In both subject populations, weaker evidence of neural activity was related to failure to achieve open-set speech perception.

Auditory and Speech Outcomes of Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder: A Systematic Review and Meta-Analysis

“…Two studies reported the data of open-set speech perception. 34,39 Most of the children in both groups achieved openset speech perception. According to the data collected, there was no significant difference between the 2 groups in open-set speech perception (RR = 0.85, 95% CI: 0.69, 1.05; P = .142).…”

“…Nine studies reported the data of speech recognition score. 9,19,[34][35][36][37][38]41,44 The pooled estimate showed that the speech recognition score was not significantly different between ANSD and SNHL groups (SMD = 0.01, 95% CI: −0.45, 0.47; P = .959) (Figure 2). The heterogeneity test was significant (I 2 = 84.4%, P = .001).…”

“…The main characteristics of included studies are summarized in Table 1. Three of the included studies used a prospective cohort design, 9,34,35 5 with retrospective cohort design, [36][37][38][39][40] and 7 with retrospective comparative design. 19,[41][42][43][44][45][46] Among the included studies, 5 were carried out in the USA, 2 in China, 2 in Australia, 2 in Korea, 1 in Canada, 1 in Germany, 1 in the UK, and 1 in Egypt.…”

Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports

“…[1][2][3][4]. The affected subjects present important speech discrimination difficulties, particularly in background noise, that are out of proportion compared to their pure-tone detection thresholds [5]. Impairments in the coding of temporal features of acoustic stimuli seem to be the main underlying mechanism responsible for these difficulties.…”

Electrophysiology and genetic testing in the precision medicine of congenital deafness: A review

“…(2014) showed how the composite measure known as “total response” (ECochG-TR) can predict 40% of post-CI speech variation in adult CI users. Similarly, Fontenot et al. (2017) measured ECochG-TRs and phonetically balanced kindergarten (PBK) word scores in 30 ANSD children and 74 non-ANSD children.…”