Use It or Lose It? Lessons Learned from the Developing Brains of Children Who are Deaf and Use Cochlear Implants to Hear

Gordon, Karen A.; Wong, Daniel; Valero, Jerome; Jewell, Stephanie; Yoo, Patrick; Papsin, Blake C.

doi:10.1007/s10548-011-0181-2

Cited by 69 publications

(59 citation statements)

References 56 publications

(87 reference statements)

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“…Although expected cortical electrophysiologic responses were measured from the first implanted ear, responses from the second, laterimplanted ear remained abnormal. [114][115][116][117] The developing auditory brainstem, the first point of binaural integration in the ascending pathways, is already affected. Brainstem responses rapidly change over the first year of unilateral CI use in children with early-onset deafness.…”

Section: Evidence For An Aural Preference Syndromementioning

confidence: 99%

Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options

2015

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Deafness affects ∼2 in 1000 children and is one of the most common congenital impairments. Permanent hearing loss can be treated by fitting hearing aids. More severe to profound deafness is an indication for cochlear implantation. Although newborn hearing screening programs have increased the identification of asymmetric hearing loss, parents and caregivers of children with single-sided deafness are often hesitant to pursue therapy for the deaf ear. Delayed intervention has consequences for recovery of hearing. It has long been reported that asymmetric hearing loss/single-sided deafness compromises speech and language development and educational outcomes in children. Recent studies in animal models of deafness and in children consistently show evidence of an "aural preference syndrome" in which singlesided deafness in early childhood reorganizes the developing auditory pathways toward the hearing ear, with weaker central representation of the deaf ear. Delayed therapy consequently compromises benefit for the deaf ear, with slow rates of improvement measured over time. Therefore, asymmetric hearing needs early identification and intervention. Providing early effective stimulation in both ears through appropriate fitting of auditory prostheses, including hearing aids and cochlear implants, within a sensitive period in development has a cardinal role for securing the function of the impaired ear and for restoring binaural/spatial hearing. The impacts of asymmetric hearing loss on the developing auditory system and on spoken language development have often been underestimated. Thus, the traditional minimalist approach to clinical management aimed at 1 functional ear should be modified on the basis of current evidence.Deafness is one of the most common congenital impairments. 1,2 Newborn hearing screening programs, implemented in many countries, have decreased the age at diagnosis of hearing loss. When hearing loss occurs in only 1 ear, the screening result may be overlooked or dismissed as unimportant, particularly when hearing in the opposite ear is normal (unilateral or single-sided deafness). The consequence will be a failure to intervene until long after major developmental effects have set in, which causes significant negative clinical implications. Screening programs will also miss children who acquire deafness in 1 ear from infection, trauma, or worsening of preexisting hearing loss. 3-6 Acquired unilateral deafness can go unidentified until educational, social, or other impairments push families and caregivers to seek medical consult. Because the prevalence of permanent unilateral hearing loss in neonates is reported to vary from 0.45 to 2.7 in 1000 7,8 In this State-of-the-Art Review, we present evidence from basic and applied neuroscience, audiology, and otology that points to the existence of an impairment of the central representation of the poorer hearing ear if developmental asymmetric hearing is left untreated for years. First, we review the current state of the problem as viewed in the clin...

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Section: Evidence For An Aural Preference Syndromementioning

confidence: 99%

Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options

2015

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“…It is more likely that this can be explained by aberrant development of the auditory brainstem and, most of all, the auditory cortex. Studies in children using cochlear implants (Gordon et al, 2011) and animal models (Butler & Lomber, 2013) have shown that the subcortical nuclei of the auditory brainstem require auditory input for further maturation; a sophisticated tonotopy, for instance, does not develop (Butler & Lomber, 2013). Synaptic activity in the primary auditory cortex will deviate substantially from normal including less activation of the deeper cortical layers (Kral, 2007).…”

Section: Introductionmentioning

confidence: 99%

Fitting prelingually deafened adult cochlear implant users based on electrode discrimination performance

Debruyne

Francart

Janssen

et al. 2016

International Journal of Audiology

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Objective: This study investigated the hypotheses that (1) prelingually deafened CI users do not have perfect electrode discrimination ability and (2) the deactivation of non-discriminable electrodes can improve auditory performance. Design: Electrode discrimination difference limens were determined for all electrodes of the array. The subjects' basic map was subsequently compared to an experimental map, which contained only discriminable electrodes, with respect to speech understanding in quiet and in noise, listening effort, spectral ripple discrimination and subjective appreciation. Study Sample: Subjects were six prelingually deafened, late implanted adults using the Nucleus cochlear implant. Results: Electrode discrimination difference limens across all subjects and electrodes ranged from 0.5 to 7.125, with significantly larger limens for basal electrodes. No significant differences were found between the basic map and the experimental map on auditory tests. Subjective appreciation was found to be significantly poorer for the experimental map. Conclusions: Prelingually deafened CI users were unable to discriminate between all adjacent electrodes. There was no difference in auditory performance between the basic and experimental map. Potential factors contributing to the absence of improvement with the experimental map include the reduced number of maxima, incomplete adaptation to the new frequency allocation, and the mainly basal location of deactivated electrodes.

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“…Although CIs can stimulate developmental plasticity in the auditory brainstem even after many years of deafness in childhood, changes in the auditory cortex are limited. This might also be caused - at least in part - by the degree of reorganization which occurred during the period of deafness [45]. In accordance, a recent study using positron emission tomography showed differences in cerebral functions at several points after CI implantation between adults with pre- and postlingual deafness.…”

Section: Discussionmentioning

confidence: 76%

Speech Production Quality of Cochlear Implant Users with Respect to Duration and Onset of Hearing Loss

Ruff¹,

Nöth

Müller³

et al. 2017

ORL

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Purpose: To assess whether postlingual onset and shorter duration of deafness before cochlear implant (CI) provision predict higher speech intelligibility results of CI users. Methods: For an objective judgement of speech intelligibility, we used an automatic speech recognition system computing the word recognition rate (WR) of 50 adult CI users and 50 age-matched control individuals. All subjects were recorded reading a standardized text. Subjects were divided into three groups: pre- or perilingual deafness (A), both >2 years before implantation, postlingual deafness <2 years before implantation (B), or postlingual deafness >2 years before implantation (C). Results: CI users with short duration of postlingual deafness (B) had a significantly higher WR (median 74%) than CI users with long duration of postlingual deafness (C; 68%, p < 0.001) or pre-/perilingual onset (A; 56%, p < 0.001). Compared to their control groups only CI users with short duration of postlingual deafness reached similar WR, others showed significantly lower WR. Other factors such as hearing loss onset, duration of CI use, or duration of amplified hearing showed no consistent influence on speech quality. Conclusions: The speech production quality of adult CI users shows dependencies on the onset and duration of deafness. These features need to be considered while planning rehabilitation.

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Use It or Lose It? Lessons Learned from the Developing Brains of Children Who are Deaf and Use Cochlear Implants to Hear

Cited by 69 publications

References 56 publications

Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options

Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options

Fitting prelingually deafened adult cochlear implant users based on electrode discrimination performance

Speech Production Quality of Cochlear Implant Users with Respect to Duration and Onset of Hearing Loss

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