Analysis of Speech Perception Outcomes Among Patients Receiving Cochlear Implants With Auditory Neuropathy Spectrum Disorder

Cochlear Implantation versus Hearing Amplification in Patients with Auditory Neuropathy Spectrum Disorder

Stimulation Rate Reduction and Auditory Development in Poorly Performing Cochlear Implant Users With Auditory Neuropathy

Relationship Between Electrically Evoked Auditory Brainstem Response and Auditory Performance After Cochlear Implant in Patients With Auditory Neuropathy Spectrum Disorder

Cochlear Implants vs Hearing Aids in Auditory Neuropathy

Although speech perception is the gold standard for measuring cochlear implant (CI) users' performance, speech perception tests often require extensive adaptation to obtain accurate results, particularly after large changes in maps. Spectral ripple tests, which measure spectral resolution, are an alternate measure that has been shown to correlate with speech perception. A modified spectral ripple test, the spectral-temporally modulated ripple test (SMRT) has recently been developed, and the objective of this study was to compare speech perception and performance on the SMRT for a heterogeneous population of unilateral CI users, bilateral CI users, and bimodal users. Twenty-five CI users (eight using unilateral CIs, nine using bilateral CIs, and eight using a CI and a hearing aid) were tested on the Arizona Biomedical Institute Sentence Test (AzBio) with a +8 dB signal to noise ratio, and on the SMRT. All participants were tested with their clinical programs. There was a significant correlation between SMRT and AzBio performance. After a practice block, an improvement of one ripple per octave for SMRT corresponded to an improvement of 12.1% for AzBio. Additionally, there was no significant difference in slope or intercept between any of the CI populations. The results indicate that performance on the SMRT correlates with speech recognition in noise when measured across unilateral, bilateral, and bimodal CI populations. These results suggest that SMRT scores are strongly associated with speech recognition in noise ability in experienced CI users. Further studies should focus on increasing both the size and diversity of the tested participants, and on determining whether the SMRT technique can be used for early predictions of long-term speech scores, or for evaluating differences among different stimulation strategies or parameter settings.

Auditory neuropathy spectrum disorder (ANSD) is a distinct type of SNHL that is characterized by the presence of otoacoustic emissions and/or cochlear microphonics. Cochlear implantation was initially not recommended for ANSD children, later studies showed variable outcomes among ANSD. CI is currently the intervention option of choice for many children with ANSD who are unable to obtain benefit from conventional amplification. To review experiences with some of the preoperative and postoperative findings in a child who was diagnosed with auditory neuropathy and provided with cochlear implant. To describe changes in auditory function, which enabled to have significant improvement in hearing and communication skills through auditory verbal therapy (AVT) and regular follow ups. Pre and postoperative, findings in cochlear implant recipient who was diagnosed with ANSD. Child received complete medical examinations, including related consultations in audiology, otorhinolaryngology, paediatrics, neurology, psychology, speech language pathology, and radiology. A 3-year-old-female have brought to the hospital with a C/o not responding to sounds, name call and unable to speak. Medical and Audiological evaluations were initiated. The hearing assessments of thechild included appropriate behavioural audiometric techniques, objective measures of middle ear function, acoustic reflex studies, transient evoked (TEOAE), distortion product (DPOAE) otoacoustic emissions and auditory brainstem responses (ABR). Implanted with (HiRes Ultra CI HiFocus SlimJ Electrode), and objective measures were recorded intraoperatively electrode impedances and neural response telemetry (NRT) to assess the outcomes technically. These intraoperative objective measures were used to help program the speech processor for the child. Postoperatively, child has had regular follow-up with otorhinolaryngologist to assure complete healing of the surgical incision, to assess their general medical conditions, and audiologist for switch-on (speech processor) followed by mapping. The hearing and communication skills have been assessed, also continued Auditory Verbal Therapy (AVT) on a regular basis. Postoperatively, objective measures were recorded in regular intervals and monitored with therapy outcomes. The child has shown significant improvement in sound detection, speech perception abilities, communication skills and shown evidence of progression of good NRT results, which were recorded and had no postoperative complications. Experience with cochlear implantation for child diagnosed with ANSD that effectively received and benefited from CI. A detailed and careful evaluations, audiological follow-ups and tailored rehabilitation plans, can be considered as a beneficial management approach for CI, especially who diagnosed with ANSD. The regular use of cochlear implant in this diagnosis can lead to a clear increase in speech comprehension, development and overall progress in quality of life. Success or lack of success with a CI appears to be somewhat dependent on the specific site of lesion (pre- or post-synaptic). Currently there are no clinical measures available to diagnose the specific site of lesion. Indeed, CI appears to be an effective rehabilitation modality for ANSD patients. This may be explained by the fact that the implanted electrode delivers synchronized electrical impulses directly to the auditory nerve, bypassing the presynaptic IHCs and its synapse involved in the unsynchronized firing of the auditory nerve described in ANSD. However, genetic studies that have proven to be essential in the knowledge of underlying mechanisms of ANSD represent a promising therapeutic approach in the management of ANSD.

Following a review of the demographic and clinical characteristics of all pediatric patients diagnosed with auditory neuropathy spectrum disorder (ANSD) by a pediatric health care system from 2005 to 2020, the present report highlights the type and timing of intervention and outcomes in the same 260 patients with ANSD. This was a retrospective study reviewing the demographic data, medical history, imaging studies, audiological and speech language data, type of audiological intervention (hearing aids or cochlear implants), and mode of communication in 260 pediatric patients diagnosed with ANSD over a 15-year period. A significant decrease over time in the age at hearing aid fitting was observed. While a similar reduction in the age at implantation occurred over time, cochlear implantation is still rarely performed by 12 months of age in most ANSD patients. Among bilateral ANSD patients fitted with hearing aids, the majority (89.2%) did not benefit from conventional amplification and most received cochlear implants. Some hearing aid benefit for speech and language development was observed in 5.8%, though communication difficulties were persistent and most used a combination of oral and sign language for communication. Only six patients (5%) received significant benefit from their hearing aids for speech and language development. This review of ANSD management over a 15-year period reveals that hearing aids are not a viable option to develop speech and language for most infants and children with ANSD. This finding confirms previous reports and suggest that while hearing aid trials are warranted, children must be tracked closely so as to avoid delays in decision making. Cochlear implantation constitutes the major (if not only) rehabilitative intervention that allows for speech perception in patients who do not benefit from conventional amplification.

Objective: The neural dys-synchrony associated with auditory neuropathy spectrum disorder (ANSD) causes a temporal impairment that could degrade spatial hearing, particularly sound localization accuracy (SLA) and spatial release from masking (SRM). Unilateral cochlear implantation has become an accepted treatment for ANSD but treatment options for the contralateral ear remain controversial. We report spatial hearing measures in a child with ANSD before and after receiving a second cochlear implant (CI). Study sample: An 11-year-7-month old boy with ANSD and expressive and receptive language delay received a second CI eight years after his first implant. Design: The SLA and SRM were measured four months before sequential bilateral CIs (with the contralateral ear plugged and unplugged), and after nine months using both CIs. Results: Testing done before the second CI, with the first CI alone, suggested that residual hearing in the contralateral ear contributed to sound localization accuracy, but not word recognition in quiet or noise. Nine-months after receiving a second CI, SLA improved by 12.76° and SRM increased to 3.8–4.2 dB relative to pre-operative performance. Results were compared to published outcomes for children with bilateral CIs. Conclusions: The addition of a second CI in this child with ANSD improved spatial hearing.

Benefit of Bilateral Cochlear Implantation on Phonological Processing Skills in Deaf Children.

Unilateral Versus Bilateral Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder (ANSD).

An increasing number of severe-profoundly deaf adult unilateral cochlear implant (CI) users receive bimodal stimulation; that is, they use a conventional acoustic hearing aid (HA) in their nonimplanted ear. The combination of electric and contralateral acoustic hearing provides additional benefits to hearing and also to general health-related quality of life compared with unilateral CI use. Bilateral CI is a treatment alternative to both unilateral CI and bimodal stimulation in some healthcare systems. The objective of this study was to conduct an economic evaluation of bimodal stimulation compared with other management options for adults with bilateral severe to profound deafness. The economic evaluation took the form of a cost-utility analysis and compared bimodal stimulation (CI+HA) to two treatment alternatives: unilateral and bilateral CI. The analysis used a public healthcare system perspective based on data from the United Kingdom and the United States. Costs and health benefits were identified for both alternatives and estimated across a patient's lifetime using Markov state transition models. Utilities were based on Health Utilities Index estimates, and health outcomes were expressed in Quality Adjusted Life Years (QALYs). The results were presented using the Incremental Cost-Effectiveness Ratio and the Net Monetary Benefit approach to determine the cost-effectiveness of bimodal stimulation. Probabilistic sensitivity analyses explored the degree of overall uncertainty using Monte Carlo simulation. Deterministic sensitivity analyses and analysis of covariance identified parameters to which the model was most sensitive; that is, whose values had a strong influence on the intervention that was determined to be most cost-effective. A value of information analysis was performed to determine the potential value to be gained from additional research on bimodal stimulation. The base case model showed that bimodal stimulation was the most cost-effective treatment option with a decision certainty of 72 and 67% in the United Kingdom and United States, respectively. Despite producing more QALYs than either unilateral CI or bimodal stimulation, bilateral CI was found not to be cost-effective because it was associated with excessive costs. Compared with unilateral CI, the increased costs of bimodal stimulation were outweighed by the gain in quality of life. Bimodal stimulation was found to cost an extra £174 per person in the United Kingdom ($937 in the US) and yielded an additional 0.114 QALYs compared with unilateral CI, resulting in an Incremental Cost-Effectiveness Ratio of £1521 per QALY gained in the United Kingdom ($8192/QALY in the United States). The most influential variable was the utility gained from the simultaneous use of both devices (CI+HA) compared with Unilateral CI. The value of further research was £4,383,922 at £20,000/QALY ($86,955,460 at $50,000/QALY in the United States). This study provides evidence of the most cost-effective treatment alternative for adults with bilateral severe to profound deafness from publicly funded healthcare perspectives of the United Kingdom and United States. Bimodal stimulation was found to be more cost-effective than unilateral and bilateral CI across a wide range of willingness-to-pay thresholds. If there is scope for future research, conducting interventional designs to obtain utilities for bimodal stimulation compared with unilateral CI would reduce decision uncertainty considerably.

Current evidence supports the benefits of cochlear implants (CIs) in children with hearing loss, including those with auditory neuropathy spectrum disorder (ANSD). However, there is limited evidence regarding factors that hold predictive value for intervention outcomes. This retrospective case-control study consisted of 66 children with CIs, including 22 with ANSD and 44 with sensorineural hearing loss (SNHL) matched on sex, age, age at CI activation, and the length of follow-up with CIs (1:2 ratio). The case and control groups were compared in the results of five open-set speech perception tests, and a Forward Linear Regression Model was used to identify factors that can predict the post-CI outcomes. There was no significant difference in average scores between the two groups across five outcome measures, ranging from 88.40% to 95.65%. The correlation matrix revealed that younger ages at hearing aid fitting and CI activation positively influenced improvements in speech perception test scores. Furthermore, among the variables incorporated in the regression model, the duration of follow-up with CIs, age at CI activation, and the utilization of two CIs demonstrated prognostic significance for improved post-CI speech perception outcomes. Children with ANSD can achieve similar open-set speech perception outcomes as children with SNHL. A longer CI follow-up, a lower age at CI activation, and the use of two CIs are predictive for optimal CI outcome.

This study asks whether the LittlEARs Auditory Questionnaire (LEAQ), a caregiver measure, can differentiate between the early auditory development of children with bilateral cochlear implants (CIs), bilateral hearing aids (HAs), and children with Auditory Neuropathy Spectrum Disorder (ANSD) who wear CIs or HAs. The LEAQ is sensitive to impaired auditory development but has not previously been used to distinguish developmental changes between groups of children using different hearing technologies or with different types of hearing loss. We collected retrospective longitudinal LEAQ results from 43 children with HAs, 43 with CIs, and 18 with ANSD. The children with ANSD wore hearing technology. They were a similar age to the children without ANSD (23 months; SD = 15), while the CI group (14 months; SD = 8) was younger than the HA group (24 months; SD = 18) [F(2,98.48) = 3.4; p = 0.04]. The CI group often participated in their first LEAQ pretreatment. Participants completed between one and seven LEAQs. Scores ranged between zero and 35 (mean = 18.36). We conducted a linear mixed-effects analysis, which included age or time since device fitting, hearing type (HA, CI, or ANSD), and presence of a comorbidity as fixed effects. A secondary analysis assessed effects of device audibility, measured by the Speech Intelligibility Index or Articulation Index, and consistency of device use obtained from device datalogs. Children with CIs progressed faster than their peers with HAs or ANSD [χ2(8) = 24.51; p = 0.002]. However, within a subsample that included consistency of device use (β7 = -0.20 ± 0.38, t = -0.52; β8 = 0.93 ± 0.82, t = 1.13) and audibility (β6 = -0.70 ± 1.45, t = -1.87; β7 = 0.87 ± 0.89, t = 0.98), study group did not significantly influence rate of improvement on the LEAQ. In addition, children with developmental delays in all three study groups demonstrated significantly slower LEAQ score improvement [χ2(6) = 23.60; p < 0.001] and a trend toward decreased consistency of device use [F(1) = 3.31; p = 0.07]. As we expected, children in the CI and HA groups were more likely to achieve auditory skills indicated in early rather than later LEAQ questions. There was less variability in the responses of the ANSD group [CI: interquartile range (IQR) = 9; HA: IQR = 8; ANSD: IQR = 1]. There was no connection between LEAQ growth and speech perception outcomes in a subsample [r(6) = 0.42; p = 0.30]. The LEAQ is a useful tool for monitoring initial auditory development in very young children and can inform early treatment decisions.

Objectives:Although it has been established that bilateral cochlear implants (CIs) offer additional speech perception and localization benefits to many children with severe to profound hearing loss, whether these improved perceptual abilities facilitate significantly better language development has not yet been clearly established. The aims of this study were to compare language abilities of children having unilateral and bilateral CIs to quantify the rate of any improvement in language attributable to bilateral CIs and to document other predictors of language development in children with CIs.Design:The receptive vocabulary and language development of 91 children was assessed when they were aged either 5 or 8 years old by using the Peabody Picture Vocabulary Test (fourth edition), and either the Preschool Language Scales (fourth edition) or the Clinical Evaluation of Language Fundamentals (fourth edition), respectively. Cognitive ability, parent involvement in children’s intervention or education programs, and family reading habits were also evaluated. Language outcomes were examined by using linear regression analyses. The influence of elements of parenting style, child characteristics, and family background as predictors of outcomes were examined.Results:Children using bilateral CIs achieved significantly better vocabulary outcomes and significantly higher scores on the Core and Expressive Language subscales of the Clinical Evaluation of Language Fundamentals (fourth edition) than did comparable children with unilateral CIs. Scores on the Preschool Language Scales (fourth edition) did not differ significantly between children with unilateral and bilateral CIs. Bilateral CI use was found to predict significantly faster rates of vocabulary and language development than unilateral CI use; the magnitude of this effect was moderated by child age at activation of the bilateral CI. In terms of parenting style, high levels of parental involvement, low amounts of screen time, and more time spent by adults reading to children facilitated significantly better vocabulary and language outcomes. In terms of child characteristics, higher cognitive ability and female sex were predictive of significantly better language outcomes. When family background factors were examined, having tertiary-educated primary caregivers and a family history of hearing loss were significantly predictive of better outcomes. Birth order was also found to have a significant negative effect on both vocabulary and language outcomes, with each older sibling predicting a 5 to 10% decrease in scores.Conclusions:Children with bilateral CIs achieved significantly better vocabulary outcomes, and 8-year-old children with bilateral CIs had significantly better language outcomes than did children with unilateral CIs. These improvements were moderated by children’s ages at both first and second CIs. The outcomes were also significantly predicted by a number of factors related to parenting, child characteristics, and family background. Fifty-one percent of the variance in vocabulary outcomes and between 59 to 69% of the variance in language outcomes was predicted by the regression models.

BackgroundThere are many methods for assessing hearing performance after cochlear implantation. Standard evaluations often encompass objective hearing tests only, while patients’ subjective experiences gain importance in today’s healthcare. The aim of the current study was to analyze the correlation between subjective (self-reported questionnaires) and objective (speech perception and localization) hearing test results in adult cochlear implant (CI) users. Secondary, the correlation between subjective and objective hearing tests was compared between bilateral and unilateral CI patients.MethodsData for this study were prospectively collected as part of a multicentre randomized controlled trial. Thirty-eight postlingually deafened adult patients were randomly allocated to receive either unilateral (n = 19) or bilateral (n = 19) cochlear implantation. We used data gathered after one year of follow-up. We studied the correlation between objectively measured speech perception and localization skills on the one hand and related domains of the Speech, Spatial and Qualities of Hearing Scale (SSQ) and Nijmegen Cochlear Implant Questionnaire (NCIQ) on the other hand. We also compared these correlations between unilateral and bilateral CI users.ResultsWe found significant weak to moderate negative correlations between the subjective test results (speech domain of the SSQ and the advanced speech perception domain of the NCIQ) and the related objective speech perception in noise test results (r = −0.33 to −0.48). A significant moderate correlation was found between the subjective test results (spatial domain of the SSQ) and the related objective localization test results (r = 0.59). The correlations in the group of bilateral CI patients (r = −0.28 to −0.54) did not differ significantly from the correlations in the group of unilateral CI patients (r = 0.15 to −0.40).ConclusionsCurrent objective tests do not fully reflect subjective everyday listening situations. This study elucidates the importance and necessity of questionnaires in the evaluation of cochlear implantation. Therefore, it is advised to evaluate both objective and subjective tests in CI patients on a regular basis.Trial registrationThis trial was registered on March 11, 2009 in the Dutch Trial Register. Trial registration number: NTR1722.