Abstract
ObjectivesIn pediatric audiology, objective techniques for hearing threshold estimation in infants and children with profound or severe hearing loss play a key role. Auditory brainstem responses (ABR) and auditory steady-state responses (ASSR) are available for frequency-dependent hearing threshold estimations and both techniques show strong correlations but sometimes with considerable differences. The aim of the study was to compare hearing threshold estimations in children with and without cochlear and cochlear nerve malformations.MethodsTwo groups with profound or severe hearing loss were retrospectively compared. In 20 ears (15 children) with malformation of the inner ear and/or cochlear nerve hypoplasia and a control group of 20 ears (11 children) without malformation, ABR were measured with the Interacoustics Eclipse EP25 ABR system® (Denmark) with narrow-band CE-chirps® at 500, 1000, 2000 and 4000 Hz and compared to ASSR at the same center frequencies under similar conditions.ResultsABR and ASSR correlated significantly in both groups (r = 0.413 in malformation group, r = 0.82 in control group). The malformation group showed a significantly lower percentage of “equal” hearing threshold estimations than the control group. In detail, patients with isolated cochlear malformation did not differ significantly from the control group, whereas patients with cochlear nerve hypoplasia showed significantly greater differences.ConclusionABR and ASSR should be used jointly in the diagnostic approach in children with suspected profound or severe hearing loss. A great difference in hearing threshold estimation between these techniques could hint at the involvement of cochlear nerve or cochlear nerve hypoplasia itself.
Highlights
The therapeutic strategy in infants and children with severeto-profound hearing loss is based on hearing threshold estimations by frequency-specific auditory brainstem responses (ABR) in combination with behavioral measures and other diagnostic tools
We retrospectively evaluated data from 20 ears of 15 children (7 females, 8 males) with an age from 9 months to 7 years with cochlear malformation or/and hearing nerve hypoplasia proven by magnetic resonance imaging (MRI) and/or computer tomography (CT) and profound or severe hearing loss that was obtained between 07/2014 and 01/2018
In a Bland-Altmann Plot, it becomes obvious, that measurements by auditory steady-state responses (ASSR) in general estimate better hearing thresholds than by ABR, on the other hand, the difference between ABR and ASSR hearing threshold estimations is lower in ears without malformation (Fig. 2, right) with a mean difference of 5.3 dB compared to ears with cochlear malformation (CM) and/ or cochlear nerve hypoplasia (CNH) (Fig. 2, left) with a mean difference of 15.5 dB, the range of standard deviation is similar
Summary
The therapeutic strategy in infants and children with severeto-profound hearing loss is based on hearing threshold estimations by frequency-specific auditory brainstem responses (ABR) in combination with behavioral measures and other diagnostic tools. Auditory steady-state responses (ASSR) offer frequency-dependent hearing threshold estimation and came into play [1, 2]. Both methods are widely discussed, compared to each other, and referenced towards. ASSR could not establish as single “gold-standard” for hearing threshold estimation in infants and children with severe-toprofound hearing loss to date
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