Neurodevelopmental Aspects and Cortical Auditory Maturation in Children with Cochlear Implants.

Background and objectives: The cochlear implant is not only meant to restore auditory function, but it also has a series of benefits on the psychomotor development and on the maturation of central auditory pathways. In this study, with the help of neuropsychological tests and cortical auditory potentials (CAEPs), we intend to identify a series of instruments that allow us to monitor children with a cochlear implant, and later on, to admit them into an individualized rehabilitation program. Materials and methods: This is a longitudinal study containing 17 subjects (6 boys and 11 girls) diagnosed with congenital sensorineural hearing loss. The average age for cochlear implantation in our cohort is 22 months old. Each child was tested before the cochlear implantation, tested again 3 months after the implant, and then 6 months after the implant. To test the general development, we used the Denver Developmental Screening Test (DDST II). CAEPs were recorded to assess the maturation of central auditory pathways. Results: The results showed there was progress in both general development and language development, with a significant statistical difference between the overall DQ (developmental quotient) and language DQ before the cochlear implantation and three and six months later, respectively. Similarly, CAEP measurements revealed a decrease of positive-going component (P1) latency after cochlear implantation. Conclusion: CAEPs and neuropsychological tests prove to be useful instruments for monitoring the progress in patients with cochlear implants during the rehabilitation process.

Cochlear implants: clinical and societal outcomes.

Over the past 30 years, hearing care clinicians have increasingly relied on cochlear implants to restore auditory sensitivity in selected patients with advanced sensorineural hearing loss. This article examines the impact of intervention with cochlear implantation in children and adults. The authors report a range of clinic-based results and patient-based outcomes reflected in the reported literature on cochlear implants. The authors describe the basic assessment of the physiologic response to auditory nerve stimulation; measures of receptive and productive benefit; and surveys of life effects as reflected measures of quality of life, educational attainment, and economic impact.

Long-term monaural auditory deprivation and bilateral cochlear implants.

Long-term binaural auditory deprivation is associated with poorer speech recognition outcomes after cochlear implantation, even for postlingual hearing loss. It is, however, unknown to what extent the outcomes of implantation are related to the peripheral changes occurring monaurally or to changes at a higher level in the auditory system related to binaural deafness. This retrospective study aimed to unravel peripheral and central contributions to cochlear implantation outcomes by comparing outcomes obtained in individual ears for adults with long-term monaural auditory deprivation (i.e. unilateral use of hearing aid) who received bilateral cochlear implants. Results showed that similar outcomes can be obtained with the implant placed in the auditory-deprived or in the aided ear. This suggests that the peripheral changes related to monaural auditory deprivation have little effect on outcomes of cochlear implantation.

Audiologic management of auditory neuropathy spectrum disorder in children: a systematic review of the literature.

PURPOSE: This review summarizes current evidence related to the audiologic management of children with auditory neuropathy spectrum disorder (ANSD). METHOD: A systematic search of the literature was conducted in 25 electronic databases (e.g., PubMed, CINAHL, and ERIC) using key words such as auditory neuropathy, auditory neuropathy spectrum disorder, auditory neuropathy/dyssynchrony, and hearing loss. Eighteen studies met the inclusion criteria by addressing 1 or more of 8 clinical questions. Studies were evaluated for methodological quality, and data regarding participant, intervention, and outcome variables are reported. RESULTS: Fifteen of the 18 studies addressed the use of cochlear implantation, and 4 addressed conventional acoustic amplification. All participants demonstrated improved auditory performance; however, all 18 studies were considered exploratory, and many had methodological limitations. CONCLUSION: The clinical evidence related to intervention for ANSD is at a very preliminary stage. Additional research is needed to address the efficacy of acoustic amplification and cochlear implantation in children with ANSD and the impact of this disorder on developmental outcomes.

Cochlear implantation in children with auditory neuropathy spectrum disorder.

OBJECTIVE: To report the patient's characteristics, preoperative audiological profiles, surgical outcomes, and postoperative performance for children with auditory neuropathy spectrum disorder (ANSD) who ultimately received cochlear implants (CIs). DESIGN: Prospective, longitudinal study of children with ANSD who received CIs after a stepwise management protocol that included electrophysiologic and medical assessment, documentation of behavioral audiometric thresholds and subsequent fitting of amplification according to Desired Sensation Level targets, auditory-based intervention with careful monitoring of skills development and communication milestones, and finally implantation when progress with the use of acoustic amplification was insufficient. RESULTS: Of 140 children with ANSD, 52 (37%) received CIs in their affected ears (mean duration of use of 41 mos). Many of these children were born prematurely (42%) and impacted by a variety of medical comorbidities. More than one third (38%) had abnormal findings on preoperative magnetic resonance imaging of the brain and inner ear, and 81% had a greater than severe (>70 dB HL) degree of hearing loss before implantation. Although 50% of the implanted children with ANSD demonstrated open-set speech perception abilities after implantation, nearly 30% of them with >6 months of implant experience were unable to participate in this type of testing because of their young age or developmental delays. No child with cochlear nerve deficiency (CND) in their implanted ear achieved open-set speech perception abilities. In a subgroup of children, good open-set speech perception skills were associated with robust responses elicited on electrical-evoked intracochlear compound action potential testing when this assessment was possible. CONCLUSIONS: This report shows that children with ANSD who receive CIs are a heterogeneous group with a wide variety of impairments. Although many of these children may ultimately benefit from implantation, some will not, presumably because of a lack of electrical-induced neural synchronization, the detrimental effects of their other associated conditions, or a combination of factors. When preoperative magnetic resonance imaging reveals central nervous system pathology, this portends a poor prognosis for the development of open-set speech perception, particularly when CND is evident. These results also show that electrical-evoked intracochlear compound action potential testing may help identify those children who will develop good open-set speech perception. Instead of recommending CI for all children with electrophysiologic evidence of ANSD, the stepwise management procedure described herein allows for the identification of children who may benefit from amplification, those who are appropriate candidates for cochlear implantation, and those who, because of bilateral CND, may not be appropriate candidates for either intervention.

Central auditory development: evidence from CAEP measurements in children fit with cochlear implants.

UNLABELLED: In normal-hearing children the latency of the P1 component of the cortical evoked response to sound varies as a function of age and, thus, can be used as a biomarker for maturation of central auditory pathways. We assessed P1 latency in 245 congenitally deaf children fit with cochlear implants following various periods of auditory deprivation. If children experience less than 3.5 years of auditory deprivation before implantation, P1 latencies fall into the range of normal following 3-6 months of electrical stimulation. Children who experience greater than 7 years of deprivation, however, generally do not develop normal P1 latencies even after years of stimulation. Moreover, the waveforms for these patients can be markedly abnormal. Cortical reorganization stimulated by deprivation is likely to be a significant factor in both variation in the latency and morphology of the cortical evoked response to sound for children fit with a cochlear implant and variation in the development of oral speech and language function. LEARNING OUTCOMES: The reader will be introduced to research using cortical evoked responses (CAEPs), positron emission tomography (PET) scans and in-depth recording from the auditory cortex of congenitally deaf cats that converges on the existence of a sensitive period for the development of central auditory pathways in children. The reader will also be provided with two case studies that illustrate the use of the P1 response as biomarker for development of central auditory pathways. Finally, suggestions for future research will be provided.

A sensitive period for the development of the central auditory system in children with cochlear implants: implications for age of implantation.

OBJECTIVE: The aim of the present experiment was to assess the consequences of cochlear implantation at different ages on the development of the human central auditory system. DESIGN: Our measure of the maturity of central auditory pathways was the latency of the P1 cortical auditory evoked potential. Because P1 latencies vary as a function of chronological age, they can be used to infer the maturational status of auditory pathways in congenitally deafened children who regain hearing after being fit with a cochlear implant. We examined the development of P1 response latencies in 104 congenitally deaf children who had been fit with cochlear implants at ages ranging from 1.3 yr to 17.5 yr and three congenitally deaf adults. The independent variable was the duration of deafness before cochlear implantation. The dependent variable was the latency of the P1 cortical auditory evoked potential. RESULTS: A comparison of P1 latencies in implanted children with those of age-matched normal-hearing peers revealed that implanted children with the longest period of auditory deprivation before implantation-7 or more yr-had abnormal cortical response latencies to speech. Implanted children with the shortest period of auditory deprivation-approximately 3.5 yr or less-evidenced age-appropriate latency responses within 6 mo after the onset of electrical stimulation. CONCLUSIONS: Our data suggest that in the absence of normal stimulation there is a sensitive period of about 3.5 yr during which the human central auditory system remains maximally plastic. Plasticity remains in some, but not all children until approximately age 7. After age 7, plasticity is greatly reduced. These data may be relevant to the issue of when best to place a cochlear implant in a congenitally deaf child.

Fetal central auditory system metabolic response to cochlear implant stimulation.

OBJECTIVES: The purpose of this study was to examine the effects of profound auditory deprivation and its treatment by cochlear implantation and stimulation on the metabolic activity of the central auditory system in fetal sheep. METHODS: Six ovine fetuses at 85% to 90% gestation were bilaterally deafened by kanamycin perfusion and unilaterally implanted with cochlear electrode arrays. Half of the implanted animals were stimulated with an extrauterine sound processor, and half were not. Four animals served as hearing controls. One week postoperatively, central nervous system metabolic activity was evaluated in ambient laboratory noise by quantitative autoradiography using (14)C-deoxyglucose. RESULTS: Kanamycin perfusion deafened all treated animals as verified by auditory brainstem response and scanning electron microscopy. Glucose utilization in the inferior colliculus was markedly lower in deafened and unstimulated animals relative to hearing controls. Glucose utilization in implanted-stimulated animals was similar to normal controls. CONCLUSIONS: Changes in central auditory system metabolic activity associated with congenital deafness may be minimized by prompt auditory habilitation.

SPET monitoring of auditory cortex activation by electric stimulation in a patient with auditory brainstem implant.

Auditory cortex activation following multifrequency acoustic stimulation has been evaluated by means of single photon emission tomography (SPET) in one patient before and after an auditory brainstem implant (ABI). No activation could be observed after acoustic stimulation before ABI. After ABI stimulation in the coronal and axial slices, the activation within the temporal cortex contralateral to the stimulated ear was twice (43.76%) that of normal controls (23.94 +/- 2.74%). This marked difference was not present in other selected cortical auditory areas (homolateral temporal, homolateral and contralateral parietal cortices). The temporal cortex was also examined with six consecutive sagittal slices from 18.75 mm up to 56.25 mm lateral to the midline. A very strong activation (51.20%) compared with that of normal controls (9.94 +/- 7.45%) was detected in the 25.26-mm sagittal slice of the temporal cortex contralateral to the stimulated side. The remaining sagittal slices showed an almost normal post-stimulatory activation. As the 25.26-mm sagittal slice corresponds to the medial part of the auditory temporal cortex, its activation suggests that electrode stimulation is concentrated on the region of the cochlear nucleus in which the neurons that transduce high frequencies are located. SPET can be considered useful, in combination with electric auditory-evoked potentials, to obtain information on ABI placement and function, effectiveness of acoustic stimulation, degree of cortical stimulation and tonotopic spatial distribution of auditory cortex activation.