Outcomes after cochlear implant provision in children with cochlear nerve hypoplasia or aplasia

Ehrmann-Müller, Désirée; Kühn, Heike; Matthies, Cordula; Hagen, Rudolf; Shehata‐Dieler, Wafaa

doi:10.1016/j.ijporl.2018.06.038

Cited by 16 publications

(8 citation statements)

References 54 publications

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“…In participants with measurable ECAP responses, the percentage of electrodes with ECAP, ECAP thresholds, maximum amplitude, and slopes of the ECAP I/O function varied greatly among individual children, highlighting the variability of cochlear nerve function in individuals with cochlear nerve aplasia. Such variability may contribute to the various outcomes of cochlear implantation observed in children with cochlear nerve aplasia (Birman et al, 2016 ; Ehrmann-Muller et al, 2018 ; Yousef et al, 2021 ). Furthermore, in our patients, the slope of the ECAP I/O function and the maximum ECAP amplitude tended to gradually decrease, while the ECAP threshold tended to gradually increase, from basal to apical electrodes.…”

Section: Discussionmentioning

confidence: 99%

“…However, children with cochlear nerve deficiency (CND) have poor outcomes after cochlear implantation. Previous studies reported that the benefits of cochlear implants (CIs) in patients with CND were worse than in other children with SNHL who had normal-sized cochlear nerves and varied greatly among individual children (Ehrmann-Muller et al, 2018 ; Arumugam et al, 2020 ). Although only a few patients can achieve simple open-set speech perception skills, most patients only exhibit improvements in sound awareness, and a few patients may experience no benefits following implantation (Kang et al, 2010 ; Kutz et al, 2011 ; Young et al, 2012 ; Vincenti et al, 2014 ).…”

Section: Introductionmentioning

confidence: 99%

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Value of Preoperative Imaging Results in Predicting Cochlear Nerve Function in Children Diagnosed With Cochlear Nerve Aplasia Based on Imaging Results

et al. 2022

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This study aimed to assess the function of the cochlear nerve using electrically evoked compound action potentials (ECAPs) for children with cochlear implants who were diagnosed with cochlear nerve aplasia and to analyze the correlation between preimplantation imaging results and ECAP responses. Thirty-five children diagnosed with cochlear nerve aplasia based on magnetic resonance imaging (MRI) were included. Preimplantation MRI and high-resolution computed tomography (HRCT) images were reconstructed, and the width of the bone cochlear nerve canal (BCNC), the diameter of the vestibulocochlear nerve (VCN), and the diameter of the facial nerve (FN) were measured. ECAP input/output (I/O) functions were measured at three electrode locations along the electrode array for each participant. The relationship between ECAP responses (including ECAP threshold, ECAP maximum amplitude, and slope of ECAP I/O function) and sizes of the BCNC and VCN was analyzed using Pearson's correlation coefficients. Our analysis revealed that ECAP responses varied greatly among individual participants. Overall, ECAP thresholds gradually increased, while maximum amplitudes and ECAP I/O function slopes gradually decreased, as the electrode location moved from the basal to the apical direction in the cochlea. ECAP responses exhibited no significant correlations with BCNC width or VCN diameter. The ratio of the VCN to FN diameters was significantly correlated with the slope of the ECAP I/O function and the maximum amplitude. BCNC width could not predict the function of the cochlear nerve. Compared with the absolute size of the VCN, the size of the VCN relative to the FN may represent an indicator for predicting the functional status of the cochlear nerve in children diagnosed with cochlear nerve aplasia based on imaging results.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Value of Preoperative Imaging Results in Predicting Cochlear Nerve Function in Children Diagnosed With Cochlear Nerve Aplasia Based on Imaging Results

et al. 2022

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“…Only one study mentioned, that ASSR testing was inconclusive in patients with cochlear nerve aplasia without giving further information [20]. Also, Ehrmann-Müller et al referred to measurable ASSR thresholds in children with CNH and no ABR threshold as a basis for cochlear implantation and investigated the outcome without discussing the discrepancy of ABR and ASSR thresholds [21].…”

Section: Discussionmentioning

confidence: 99%

Comparison of ABR and ASSR using narrow-band-chirp-stimuli in children with cochlear malformation and/or cochlear nerve hypoplasia suffering from severe/profound hearing loss

Eder

Polterauer

Semmelbauer

et al. 2021

Eur Arch Otorhinolaryngol

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Objectives In 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. Methods Two 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. Results ABR 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. Conclusion ABR 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.

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“…Se debe igualmente ofrecer un dispositivo auditivo, como por ejemplo un audífono con presentación contralateral de la señal (ejemplo CROS) o un procesador de sonido de conducción ósea 7 . Dependiendo de la disponibilidad, se puede considerar realizar un PEATC con estímulo eléctrico (grabación de ventana redonda o promontorio) para niños pequeños cuando no se logra una respuesta luego de un PEATC obtenido mediante estimulación acústica convencional, debido a que la implantación coclear se ha intentado en niños con aplasia o hipoplasia del nervio coclear, y ha dado algunos resultados prometedores 8 .…”

Section: Discussionunclassified

Estenosis congénita aislada del conducto auditivo interno como causa de hipoacusia neurosensorial unilateral y paresia del nervio facial

2019

Rev. Otorrinolaringol. Cir. Cabeza Cuello

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Outcomes after cochlear implant provision in children with cochlear nerve hypoplasia or aplasia

Cited by 16 publications

References 54 publications

Value of Preoperative Imaging Results in Predicting Cochlear Nerve Function in Children Diagnosed With Cochlear Nerve Aplasia Based on Imaging Results

Value of Preoperative Imaging Results in Predicting Cochlear Nerve Function in Children Diagnosed With Cochlear Nerve Aplasia Based on Imaging Results

Comparison of ABR and ASSR using narrow-band-chirp-stimuli in children with cochlear malformation and/or cochlear nerve hypoplasia suffering from severe/profound hearing loss

Estenosis congénita aislada del conducto auditivo interno como causa de hipoacusia neurosensorial unilateral y paresia del nervio facial

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