Electrophysiologic and Behavioral Outcomes of Cochlear Implantation in Children With Auditory Nerve Hypoplasia

Valero, Jerome; Blasér, Susan; Papsin, Blake C.; James, Adrian L.; Gordon, Karen A.

doi:10.1097/aud.0b013e3182263460

Cited by 67 publications

(62 citation statements)

References 64 publications

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“…The detection of an electrically generated ABR has been suggested as a test for candidacy assessment in cochlear nerve hypoplasia [Nikolopoulos and Kiprouli, 2004]. However, our observations on the efficacy of cochlear implantation in children with cochlear nerve hypoplasia do not support cochlear implantation in such cases [Valero et al, 2012].…”

Section: Discussioncontrasting

confidence: 54%

Cochlear Nerve Aplasia with Detectable Olivocochlear Efferent Function: A Distinct Presentation of Auditory Neuropathy Spectrum Disorder

2018

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Background: Cochlear nerve aplasia (CNA) may present with features of auditory neuropathy spectrum disorder (ANSD), having detectable otoacoustic emissions (OAE) but profound hearing loss. We propose that some children with CNA have a distinct form of afferent ANSD in which efferent cochlear nerve function can be detected using contralateral suppression of OAE. Methods: Children were prospectively enrolled with MRI and auditory brainstem response evidence of unilateral CNA, a normal contralateral ear, and detectable OAE bilaterally. Distortion product OAE (DPOAE) levels were recorded in real time with default primary tone settings: frequency (f)₂ = 4.5 kHz and f₂/f₁ = 1.22 kHz, with level (L)₁ = 65 dB SPL and L₂ = 55 dB SPL. Recordings were made over 2 min with simultaneous application of an intermittent contralateral broadband noise (CBBN) stimulus at 60 dB SPL. Results: Three girls, aged 4.5, 7, and 8 years, participated. Suppression of DPOAE of 0.15–1.3 dB was detected in all 3 ears with CNA in response to CBBN stimulation. No response was detected in the normal ears. Conclusions: Children with unilateral ANSD can have normal efferent cochlear nerve function despite MRI evidence of ipsilateral CNA. The importance of these findings for newborn hearing screening and cochlear implantation is discussed.

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Section: Discussioncontrasting

confidence: 54%

Cochlear Nerve Aplasia with Detectable Olivocochlear Efferent Function: A Distinct Presentation of Auditory Neuropathy Spectrum Disorder

2018

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“…In order to be able to differentiate the beneficial information obtained from MRI and HRCT, Pagarkar et al [2011] suggested a combination of the diagnostic imaging tools with audiological tests for the evaluation of the integrity of the hearing nerve. Bilaterally deaf children with uni-or bilateral cochlear nerve aplasia/hypoplasia showed overall poorer results in comparison to their peers having other malformations, such as an enlarged vestibular aqueduct (EVA) [Buchman et al, 2011;Valero et al, 2012]. Therefore, the authors do not recommend CI surgery in SSD patients with proven CND in view of the anticipated poor outcomes which in turn may negatively impact bilateral hearing compared to the unaided condition.…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implantation in Children with Single-Sided Deafness: Does Aetiology and Duration of Deafness Matter?

et al. 2015

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For adult patients with single-sided deafness (SSD), treatment with a cochlear implant (CI) is well established as an acceptable and beneficial hearing rehabilitation method administered routinely in clinical practice. In contrast, for children with SSD, CI has been applied less often to date, with the rationale to decide either on a case-by-case basis or under the realm of clinical research. The aim of our clinical study was to evaluate the longitudinal benefits of CI for a group of children diagnosed with SSD and to compare their outcomes with respect to patient characteristics. Evaluating a pool of paediatric SSD patients presenting for possible CI surgery revealed that the primary aetiology of deafness was congenital cochlear nerve deficiency. A subgroup of children meeting the CI candidacy criteria for the affected ear (the majority with acquired hearing loss) were enrolled in the study. Preliminary group results suggest substantial improvements in speech comprehension in noise and in the ability to localise sound, which was demonstrated through objective and subjective assessments after CI treatment for the group, with results varying from patient to patient. Our study shows a trend towards superior outcomes for children with acquired hearing loss and a shorter duration of hearing loss compared to congenitally deafened children who had a longer duration of SSD. This indicates an interactive influence of the age at onset, aetiology and duration of deafness upon the restoration of binaural integration and the overall benefits of sound stimulation to two ears after CI treatment. Continued longitudinal investigation of these children and further studies in larger groups may provide more guidance on the optimal timing of treatment for paediatric patients with acquired and congenital SSD.

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“…19 In addition, it was previously reported that children with hypoplasia or aplasia of the CN showed poor auditory performance after CI. 20,21 We found that the size of the CN was correlated with post-CI AO sentence test scores. However, there was only a weak correlation between the AO (post-CI minus pre-CI) sentence test scores and the size of the CN, which was not statistically significant.…”

Section: Auditory Performance and Cn Sizementioning

confidence: 93%

Correlation of Cochlear Nerve Size and Auditory Performance After Cochlear Implantation in Postlingually Deaf Patients<alt-title>Cochlear Nerve Size and Auditory Performance</alt-title>

Kim¹

2013

JAMA Otolaryngol Head Neck Surg

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Electrophysiologic and Behavioral Outcomes of Cochlear Implantation in Children With Auditory Nerve Hypoplasia

Cited by 67 publications

References 64 publications

Cochlear Nerve Aplasia with Detectable Olivocochlear Efferent Function: A Distinct Presentation of Auditory Neuropathy Spectrum Disorder

Cochlear Nerve Aplasia with Detectable Olivocochlear Efferent Function: A Distinct Presentation of Auditory Neuropathy Spectrum Disorder

Cochlear Implantation in Children with Single-Sided Deafness: Does Aetiology and Duration of Deafness Matter?

Correlation of Cochlear Nerve Size and Auditory Performance After Cochlear Implantation in Postlingually Deaf Patients<alt-title>Cochlear Nerve Size and Auditory Performance</alt-title>

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