Speech Perception and Cortical Event Related Potentials in Children with Auditory Neuropathy

Abstract

In approximately 50% of children with auditory neuropathy, the provision of amplification results in significant open-set speech perception improvements. The results confirm the previously published reports that speech perception ability cannot be reliably estimated from the behavioral audiogram in children with AN. Obligatory ERP test results may offer a means of predicting perceptual skills in newly diagnosed youngsters as the presence of ERPs (with age-appropriate latency and morphology) was correlated with significant open set speech perception abilities and amplification benefit. The absence of the ERP in contrast, indicated profound hearing disability evidenced by profound hearing loss and/or extremely poor speech perception.

The development of the N1 and N2 components in auditory oddball paradigms: a systematic review with narrative analysis and suggested normative values

“…The objective measurement of central auditory processing and integrity of final auditory pathway should not depend on just one component but on the presence of several ERPs (complex), reflecting progressive central sound processing and representation Näätänen et al 2007;Nikjeh et al 2009) and also preservation of neuronal synchrony encoding temporal information (Rance et al 2002;Tomé et al 2012). …”

Does parental experience of the diagnosis and intervention process differ for children with auditory neuropathy?

“…Unlike SNHL, threshold estimation is not possible with Auditory Brainstem Response (ABR) or Auditory Steady State Response (ASSR) [1,5,15]. As a results developmentally appropriate behavioral measures, which typically may only be possible after the age of 6 months must be used to determine hearing thresholds [17].…”

“…Cortical evoked potentials may become useful in cases where behavioural methods are inconclusive [1,22,23]. To date however cortical auditory evoked potentials have mostly been employed in laboratory settings, rather than in clinical settings and are not consistently included within a test battery for assessing auditory function [24] Using CAEP potentials in a clinical setting, may however provide hearing threshold estimates at earlier ages allowing for appropriate early intervention strategies at a younger age.…”

“…The term auditory neuropathy or auditory dys-synchrony is used to describe a form of hearing impairment in which cochlear outer hair cell function is evident, but afferent neural transmission is disordered [1,2,3]. The term 'auditory neuropathy spectrum disorder' (ANSD) was adopted more recently as a way of describing its heterogeneous and multifaceted nature [4].…”

“…By clinical definition, patients with this disorder have normal otoacoustic emissions (OAE's) and/or cochlear microphonic (CM) potentials, but exhibit an absent or severely abnormal auditory brainstem response (ABR) beyond the expected for the degree of hearing loss [1,2,3,5,6]. Although the diagnosis is clear with an absent ABR, no clear guideline exists on what exactly defines an abnormal ABR in cases of ANSD but it includes abnormal wave morphology with ABR thresholds significantly elevated above the expected degree of hearing loss [6].…”