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Published on: January 29, 2014
Cortical Auditory Evoked Potential Testing in Children With Auditory Neuropathy Spectrum Disorder
Thierry Morlet1,2,3, Jenna Valania4, Cassidy Walter5
1Auditory Physiology and Psychoacoustics Research Laboratory, Nemours Children Hospital, Wilmington, DE.
Insights
Cortical auditory evoked potentials (CAEPs) objectively assess auditory neuropathy spectrum disorder (ANSD) in children. While CAEPs are often present, their prognostic value for hearing outcomes requires further investigation.
Area of Science:
- Neuroscience
- Audiology
- Pediatrics
Background:
- Auditory neuropathy spectrum disorder (ANSD) presents diagnostic challenges in children.
- Objective electrophysiological measures are crucial for evaluating ANSD.
- Cortical auditory evoked potentials (CAEPs) offer a non-invasive method for assessing auditory pathway function.
Purpose of the Study:
- To review the role of CAEPs in evaluating and managing children with ANSD.
- To assess the relationship between CAEP characteristics and clinical features in ANSD patients.
- To understand the implications of CAEP findings for auditory stimulation and management.
Main Methods:
- Review of CAEP recordings from 66 children (2 months to 12 years) with ANSD.
- Analysis of CAEP prevalence, morphology, latencies, and amplitudes.
- Correlation of CAEP data with clinical features and management strategies.
Main Results:
- CAEPs were present in 85.2% of ears; factors like prematurity or syndromes did not affect presence.
- Shorter CAEP latencies were observed in ears with cochlear nerve deficiency.
- Three distinct CAEP patterns were identified in patients with bilateral ANSD and present cochlear nerves.
Conclusions:
- CAEPs can indicate the presence or absence of central auditory stimulation in ANSD.
- Absent CAEPs suggest auditory deprivation, often leading to cochlear implantation.
- Present CAEPs signify some auditory stimulation, but prognostic implications remain uncertain.
Purpose:
In the present report, we reviewed the role of cortical auditory evoked potentials (CAEPs) as an objective measure during the evaluation and management process in children with auditory neuropathy spectrum disorder (ANSD).
Method:
We reviewed the results of CAEP recordings in 66 patients with ANSD aged between 2 months and 12 years and assessed the relationship between their characteristics (prevalence, morphology, latencies, and amplitudes) and various clinical features, including the mode of medical management.
Results:
Overall, the CAEPs were present in 85.2% of the ears tested. Factors such as prematurity, medical complexity, neuronal issues, or presence of syndromes did not have an effect on the presence or absence of CAEPs. CAEP latencies were significantly shorter in ears with cochlear nerve deficiency than in ears with a normal caliber nerve. Three different patterns of CAEP responses were observed in patients with bilateral ANSD and present cochlear nerves: (a) responses with normal morphology and presence of both P1-P2complex and N2 components, (b) responses with abnormal morphology and presence of the N2 component but undefined P1-P2complex peak, and (c) entirely absent responses. None of the patients with normal, mild, or moderate degree of hearing loss had a complete absence of CAEP responses. No significant differences were uncovered when comparing the latencies across unaided and aided children and children who later received cochlear implants.
Conclusions:
The CAEP protocol used in our ANSD program did inform about the presence or absence of central auditory stimulation. Absent responses typically fit into an overall picture of complete auditory deprivation and all of these children were ultimately offered cochlear implants after failing to develop oral language. Present responses, on the other hand, were acknowledged as a sign of some degree of auditory stimulation but always interpreted with caution given that prognostic implications remain unclear.
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