Speech Perception In Children Research Articles

The Effects of Manufacturer's Prefit and Real-Ear Fitting on the Predicted Speech Perception of Children with Severe to Profound Hearing Loss.

The use of probe microphone measures in hearing aid verification is often neglected or not fully used by practitioners. Some practitioners rely on simulated gain and output provided by manufacturer's fitting software to verify hearing aids. This study aims to evaluate the effectiveness of manufacturer's prefit procedure in matching the prescribed real-ear targets. It also aims to study its correlated impact on the predicted speech perception in children with severe and profound hearing loss. This cross-sectional experiment was carried out by measuring the output of hearing aids based on prefit versus real-ear at low-, moderate-, and high-input levels. The predicted speech perception for different hearing aid fittings was determined based on the Speech Intelligibility Index (SII). Sixteen children (28 ears) aged between 4 and 7 yr, with severe to profound sensorineural hearing loss took part in the study. Two different types of hearing aids (Phonak and Unitron) were programmed based on their respective manufacturers' Desired Sensation Levels (DSL) v5 Child procedure. The hearing aids were then verified using coupler-based measurements and individual real-ear-to-coupler differences. The prefit outputs were compared with the DSL v5 Child-prescribed outputs at low-, moderate-, and high-input levels. The hearing aids were then adjusted to closely match the prescribed output. The SIIs were calculated for the fittings before and after adjustment. Sixty four percent of fittings that were based on the prefit procedure achieved the optimal fit-to-targets, with less than 5-dB RMS deviations from the DSL v5 Child targets. After adjusting the hearing aids to attempt to meet the DSL v5 Child targets, 75% of the ears tested achieved the optimal fit-to-targets. On average, hearing aid outputs generated by the manufacturer's prefit procedure had good and reasonable agreement with the DSL v5 Child-prescribed outputs at low- and mid-frequencies. Nonetheless, at 4000 Hz, the hearing aid output mostly fell below the DSL v5 Child-prescribed outputs. This was still the case even after the hearing aid was adjusted to attempt to match with the targets. At low input level, some prefit outputs were found to be higher than the prescribed outputs. The deviations of prefit outputs from the prescribed outputs were dependent on the type of hearing aid and input levels. There was no significant difference between the SII calculated for fittings based on the prefit and adjusted fit. Prefit procedure tends to produce outputs that were below the DSL v5 Child-prescribed outputs, with the largest mean difference at 4000 Hz. Even though the hearing aid gains were adjusted to attempt to match with the targets, the outputs were still below the targets. The limitations of hearing aids to match the DSL v5 Child targets at high-frequency region have resulted in no improvement in the children's predicted speech perception.

Speech perception in children with reading disabilities: Phonetic perception is the problem

Reading disability (RD) is widely accepted as a key obstacle in the development of literacy. Studies show that 15–20% of grade-school students are RD. Many quit high-school and go to jail. We shall show that RD for 8–12 yrs is related to inadequate phonetic identification ability, rooted in pre-school language development. We used two tests (10 thousand responses/child): (1) A 3-interval forced choice procedure (Syllable Confusion Oddball Task: SCO). (2) A single CV/VC presentation task with oral response, to label CV/VC phones (Nonsense Syllable Confusion Matrix: NSCM). The experimental results showed that for the SCO task the 10 RD cohort had, on average 5 times the error compared to the 6 RC reading control (RC) cohort. The errors were highly idiosyncratic, analyzed by logit. (1) RDs have significant speech perception problems, despite normal pure-tone hearing and language ability. (2) When comparing the SCO and NSCM results, our findings are consistent with a reduced ability to label CV/VC sounds presented in random temporal order. This seems consistent with phone memory dysfunction. (3) These conclusions are at odds with previous studies finding no indication of phone identification impairment.

Bone Conduction Amplification in Children: Stimulation via a Percutaneous Abutment versus a Transcutaneous Softband.

Objectives:Research suggests that the speech perception of children using bone conduction amplification improves if the device is coupled to an implanted abutment rather than to a softband. The purpose of the present study was to determine if the benefit of direct stimulation via an abutment is limited to small improvements in speech perception or if similar or greater benefits occur for other auditory tasks important for learning and communication.Design:Fourteen children (7 to 15 years of age) with bilateral conductive and three children with unilateral conductive or sensorineural hearing loss were enrolled. Each child completed four tasks while using a bone conduction device coupled to an implanted abutment and with the device coupled to a softband. The two devices were worn at the same time and activated one at a time for testing. The children completed four tasks under each coupling condition: (a) a traditional word recognition task, (b) an auditory lexical decision task in which the children repeated aloud, and indicated the category of, real and nonsense words, (c) a nonsense-word detection task which required the children to identify nonsense words within short sentences, and (d) a rapid word learning task in which the children learned to associate nonsense words with novel images.Results:Regression analyses revealed that age, duration of device use, in-situ hearing thresholds, or device output did not account for a significant portion of the variability in performance for any of the four tasks. Repeated-measures analysis of variance revealed significant increases in word recognition with the abutment as well as significantly better performance for the lexical decision and word learning tasks. The data indicated that the children with the poorest performance with the softband tended to benefit most with the abutment. Also, the younger children showed improved performance for more tasks with the abutment than the older children. No difference between coupling conditions was observed for nonsense-word detection.Conclusions:The improved recognition of familiar words, categorization and repetition of nonsense words, and speed of word learning with the abutment suggests that direct stimulation provides a higher-quality signal than indirect stimulation through a softband. Because these processes are important for vocabulary acquisition and language development, children may experience long-term benefits of direct stimulation for academic, social, and vocational purposes in addition to immediate improvement in communication.

Sequential bilateral cochlear implant: results in children and adolescents

IntroductionThe use of the bilateral cochlear implants can promote the symmetrical development of the central auditory pathways, thus benefiting the development of auditory abilities and improving sound localization and the ability of auditory speech perception in situations of competitive noise. ObjectiveTo evaluate the ability of speech perception in children and adolescents using sequential bilateral cochlear implants, considering the association of these variables: age at surgery, time of device use and interval between surgeries. MethodsA total of 14 individuals between 10 and 16 years of age, who demonstrated surgical indication for the use of sequential bilateral cochlear implants as intervention in the auditory habilitation process, were assessed. The speech perception ability was assessed through sentence lists constructed in the Portuguese language, presented in two situations: in silence, with fixed intensity of 60dB SPL, and in competitive noise, with a signal-to-noise ratio of +15dB. The evaluation was performed under the following conditions: unilateral with the first activated cochlear implant, unilateral with the second activated cochlear implant and bilateral with both devices activated. ResultsThe results of the speech perception tests showed better performance in both silence and in noise for the bilateral cochlear implant condition when compared to the 1st cochlear implant and the 2nd cochlear implant alone. A worse result of speech perception was found using the 2nd cochlear implant alone. No statistically significant correlation was found between age at the surgical procedure, interval between surgeries and the time of use of the 2nd cochlear implant, and the auditory speech perception performance for all assessed conditions. The use of a hearing aid prior to the 2nd cochlear implant resulted in benefits for auditory speech perception with the 2nd cochlear implant, both in silence and in noise. ConclusionThe bilateral cochlear implant provided better speech perception in silence and in noise situations when compared to the unilateral cochlear implant, regardless of the interval between surgeries, age at the surgical procedure and the time of use of the 2nd cochlear implant. Speech perception with the 1st cochlear implant was significantly better than with the 2nd cochlear implant, both in silence and in noise. The use of the hearing aid prior to the 2nd cochlear implant influenced speech perception performance with the 2nd cochlear implant, both in silence and in noise.

Children's Speech Perception in Noise: Evidence for Dissociation From Language and Working Memory

We examined the association between speech perception in noise (SPIN), language abilities, and working memory (WM) capacity in school-age children. Existing studies supporting the Ease of Language Understanding (ELU) model suggest that WM capacity plays a significant role in adverse listening situations. Eighty-three children between the ages of 7 to 11 years participated. The sample represented a continuum of individual differences in attention, memory, and language abilities. All children had normal-range hearing and normal-range nonverbal IQ. Children completed the Bamford-Kowal-Bench Speech-in-Noise Test (BKB-SIN; Etymotic Research, 2005), a selective auditory attention task, and multiple measures of language and WM. Partial correlations (controlling for age) showed significant positive associations among attention, memory, and language measures. However, BKB-SIN did not correlate significantly with any of the other measures. Principal component analysis revealed a distinct WM factor and a distinct language factor. BKB-SIN loaded robustly as a distinct 3rd factor with minimal secondary loading from sentence recall and short-term memory. Nonverbal IQ loaded as a 4th factor. Results did not support an association between SPIN and WM capacity in children. However, in this study, a single SPIN measure was used. Future studies using multiple SPIN measures are warranted. Evidence from the current study supports the use of BKB-SIN as clinical measure of speech perception ability because it was not influenced by variation in children's language and memory abilities. More large-scale studies in school-age children are needed to replicate the proposed role played by WM in adverse listening situations.

Attention to speech and spoken language development in deaf children with cochlear implants: a 10-year longitudinal study.

Early auditory/language experience plays an important role in language development. In this study, we examined the effects of severe-to-profound hearing loss and subsequent cochlear implantation on the development of attention to speech in children with cochlear implants (CIs). In addition, we investigated the extent to which attention to speech may predict spoken language development in children with CIs. We tested children with CIs and compared them to chronologically age-matched peers with normal hearing (NH) on their attention to speech at four time points post implantation; specifically, less than 1 month, 3 to 6 months, 12 months, and 18 months post implantation. We also collected a variety of well-established speech perception and spoken language measures from the children with CIs in a 10-year longitudinal study. Children with CIs showed reduced attention to speech as compared to their peers with NH at less than 1 month post implantation, but a similar degree of attention to speech as their NH peers during later time points. In addition, attention to speech at 3 to 6 months post implantation predicts speech perception in children with CIs. These results inform language acquisition theories and bring insights into our understanding of early severe-to-profound hearing loss on infants' attention to speech skills. In addition, the findings have significant clinical implications for early intervention on hearing loss, which emphasizes the importance of developing strong listening skills. A video abstract of this article can be viewed at: https://www.youtube.com/watch?v=f7xiYo3Ua08&feature=youtu.be.

Music and Speech Perception in Children Using Sung Speech.

This study examined music and speech perception in normal-hearing children with some or no musical training. Thirty children (mean age = 11.3 years), 15 with and 15 without formal music training participated in the study. Music perception was measured using a melodic contour identification (MCI) task; stimuli were a piano sample or sung speech with a fixed timbre (same word for each note) or a mixed timbre (different words for each note). Speech perception was measured in quiet and in steady noise using a matrix-styled sentence recognition task; stimuli were naturally intonated speech or sung speech with a fixed pitch (same note for each word) or a mixed pitch (different notes for each word). Significant musician advantages were observed for MCI and speech in noise but not for speech in quiet. MCI performance was significantly poorer with the mixed timbre stimuli. Speech performance in noise was significantly poorer with the fixed or mixed pitch stimuli than with spoken speech. Across all subjects, age at testing and MCI performance were significantly correlated with speech performance in noise. MCI and speech performance in quiet was significantly poorer for children than for adults from a related study using the same stimuli and tasks; speech performance in noise was significantly poorer for young than for older children. Long-term music training appeared to benefit melodic pitch perception and speech understanding in noise in these pediatric listeners.

The effects of stimulus rate on ABR morphology and its relationship to P1 CAEP responses and auditory speech perception outcomes in children with auditory neuropathy spectrum disorder: evidence from case reports

Objective: Auditory neuropathy spectrum disorder (ANSD) affects approximately 5–15% of children with sensorineural hearing loss (SNHL). ANSD is characterized by the presence of otoacoustic emissions (OAE) and an absent or abnormal auditory brainstem response (ABR). The purpose of this study was to investigate the prognostic value of slow-rate ABR in predicting the auditory cortical development and auditory speech perception outcomes in case studies of children with ANSD.Design: ABR waveform characteristics were collected at slow stimulation rates (5.1 clicks/second) and a fast stimulation rates (>11–31.1 clicks/second, rates typically used in a clinical setting) in three case reports of children with ANSD. P1 CAEP responses and measures of auditory speech perception using the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were also collected in these children. Retrospective analysis was performed to evaluate the prognostic value of slow- versus fast-rate ABR in predicting P1 CAEP responses and auditory speech perception outcomes in these children.Study sample: Participants included case reports of three paediatric participants with a clinical diagnosis of ANSD.Results: Slow-rate ABR did not elicit significant improvements in waveform morphology compared to fast-rate ABR. P1 CAEP results were present in 2 out of 3 cases and were consistent with auditory speech perception outcomes.Conclusions: Even when ABR stimulation rates were slowed, ABR responses in these children with ANSD did not display any characteristic or replicable pattern, and ABR responses were not predictive of cortical auditory maturation or behavioural performance. In contrast, P1 CAEP responses provided valuable information regarding the maturational status of the auditory cortex and P1 CAEP responses were consistent with behavioural measures of auditory speech perception. Overall, results highlight the high prognostic value of P1 CAEP testing when used in conjunction with behavioural measures of auditory speech perception in children with ANSD.

Vocabulary Facilitates Speech Perception in Children With Hearing Aids.

We examined the effects of vocabulary, lexical characteristics (age of acquisition and phonotactic probability), and auditory access (aided audibility and daily hearing aid [HA] use) on speech perception skills in children with HAs. Participants included 24 children with HAs and 25 children with normal hearing (NH), ages 5-12 years. Groups were matched on age, expressive and receptive vocabulary, articulation, and nonverbal working memory. Participants repeated monosyllabic words and nonwords in noise. Stimuli varied on age of acquisition, lexical frequency, and phonotactic probability. Performance in each condition was measured by the signal-to-noise ratio at which the child could accurately repeat 50% of the stimuli. Children from both groups with larger vocabularies showed better performance than children with smaller vocabularies on nonwords and late-acquired words but not early-acquired words. Overall, children with HAs showed poorer performance than children with NH. Auditory access was not associated with speech perception for the children with HAs. Children with HAs show deficits in sensitivity to phonological structure but appear to take advantage of vocabulary skills to support speech perception in the same way as children with NH. Further investigation is needed to understand the causes of the gap that exists between the overall speech perception abilities of children with HAs and children with NH.

Factors influencing speech perception in noise for 5-year-old children using hearing aids or cochlear implants

Objective: We investigated the factors influencing speech perception in babble for 5-year-old children with hearing loss who were using hearing aids (HAs) or cochlear implants (CIs). Design: Speech reception thresholds (SRTs) for 50% correct identification were measured in two conditions – speech collocated with babble, and speech with spatially separated babble. The difference in SRTs between the two conditions give a measure of binaural unmasking, commonly known as spatial release from masking (SRM). Multiple linear regression analyses were conducted to examine the influence of a range of demographic factors on outcomes. Study sample: Participants were 252 children enrolled in the Longitudinal Outcomes of Children with Hearing Impairment (LOCHI) study. Results: Children using HAs or CIs required a better signal-to-noise ratio to achieve the same level of performance as their normal-hearing peers but demonstrated SRM of a similar magnitude. For children using HAs, speech perception was significantly influenced by cognitive and language abilities. For children using CIs, age at CI activation and language ability were significant predictors of speech perception outcomes. Conclusions: Speech perception in children with hearing loss can be enhanced by improving their language abilities. Early age at cochlear implantation was also associated with better outcomes.

Some Neurocognitive Correlates of Noise-Vocoded Speech Perception in Children With Normal Hearing: A Replication and Extension of ).

Noise-vocoded speech is a valuable research tool for testing experimental hypotheses about the effects of spectral degradation on speech recognition in adults with normal hearing (NH). However, very little research has utilized noise-vocoded speech with children with NH. Earlier studies with children with NH focused primarily on the amount of spectral information needed for speech recognition without assessing the contribution of neurocognitive processes to speech perception and spoken word recognition. In this study, we first replicated the seminal findings reported by ) who investigated effects of lexical density and word frequency on noise-vocoded speech perception in a small group of children with NH. We then extended the research to investigate relations between noise-vocoded speech recognition abilities and five neurocognitive measures: auditory attention (AA) and response set, talker discrimination, and verbal and nonverbal short-term working memory. Thirty-one children with NH between 5 and 13 years of age were assessed on their ability to perceive lexically controlled words in isolation and in sentences that were noise-vocoded to four spectral channels. Children were also administered vocabulary assessments (Peabody Picture Vocabulary test-4th Edition and Expressive Vocabulary test-2nd Edition) and measures of AA (NEPSY AA and response set and a talker discrimination task) and short-term memory (visual digit and symbol spans). Consistent with the findings reported in the original ) study, we found that children perceived noise-vocoded lexically easy words better than lexically hard words. Words in sentences were also recognized better than the same words presented in isolation. No significant correlations were observed between noise-vocoded speech recognition scores and the Peabody Picture Vocabulary test-4th Edition using language quotients to control for age effects. However, children who scored higher on the Expressive Vocabulary test-2nd Edition recognized lexically easy words better than lexically hard words in sentences. Older children perceived noise-vocoded speech better than younger children. Finally, we found that measures of AA and short-term memory capacity were significantly correlated with a child's ability to perceive noise-vocoded isolated words and sentences. First, we successfully replicated the major findings from the ) study. Because familiarity, phonological distinctiveness and lexical competition affect word recognition, these findings provide additional support for the proposal that several foundational elementary neurocognitive processes underlie the perception of spectrally degraded speech. Second, we found strong and significant correlations between performance on neurocognitive measures and children's ability to recognize words and sentences noise-vocoded to four spectral channels. These findings extend earlier research suggesting that perception of spectrally degraded speech reflects early peripheral auditory processes, as well as additional contributions of executive function, specifically, selective attention and short-term memory processes in spoken word recognition. The present findings suggest that AA and short-term memory support robust spoken word recognition in children with NH even under compromised and challenging listening conditions. These results are relevant to research carried out with listeners who have hearing loss, because they are routinely required to encode, process, and understand spectrally degraded acoustic signals.

Classroom acoustics and children's speech perception

Children must learn in classrooms that contain multiple sources of competing sounds. While there are national standards aimed at creating classroom environments that optimize speech intelligibility (e.g., ANSI/ASA 2010), these standards are voluntary and many unoccupied classrooms fail to meet the acceptable levels specified. Moreover, little attention has been given to measuring and understanding effects of competing speech on children’s performance in the classroom. Data will be presented that describe typical noise levels in the classroom. Results from experiments investigating the consequences of competing noise and speech on speech perception at different time points during childhood will be presented. Findings from experiments investigating potential benefits associated with manipulating acoustic cues thought to aid in separating target from background speech will also be discussed.

Cortical auditory evoked potentials in children who stutter

IntroductionIt has been hypothesized that impaired auditory processing influence the occurrence of stuttering. Also, it is suggested that speech perception in children who stutter differed from normal. Auditory processing should be investigated in children who stutter shortly after the onset of stuttering in order to evaluate the extent to which impaired auditory processing contributes to the development of stuttering. CAEPs provide the necessary temporal and spatial resolution to detect differences in auditory processing and the neural activity that is related or time-locked to the auditory stimulus. The primary goal of the present study was to determine the difference in latency and amplitude of P1-N2 complex between children who stutter and non-stuttering children in response to speech stimuli. Material & methodsThis case-control study was performed over 60children, 30were non-stuttering children (control group) and 30were children who stutter (study group) ranging in severity from Bloodstien I to Bloodstien IV in the age range of 8–18 years. ResultsCAEPs of children who stutter with stuttering severity Bloodstien IV showed significant prolonged latencies and reduced amplitudes when blocks and IPDs were the most predominant core behaviors. P1 and N1 were prolonged in concomitant behaviors. ConclusionIt could be speculated that speech processing was affected in children who stutter with stuttering severity Bloodstien IV at the level of early perceptual auditory cortex.

Speech perception in children with cochlear implants for continua varying in formant transition duration

Purpose: To examine the developmental course of labial and alveolar manner of articulation contrasts, and to determine how that course may be different for typically developing (TD) children with cochlear implants (CI).Method: Eight young adults, eight TD 5–8 year-old children, and seven 5–8 year-old children with CIs participated. Labial /ba/–/wa/ and alveolar /da/–/ja/ continua stimuli were presented, with each continuum consisting of nine synthetic stimuli varying in F2 and F3 transition duration. Participants were asked to label the stimuli as either a stop or glide, and responses were analysed for phonetic boundaries and slopes.Result: For the /ba/–/wa/ contrast, children with CIs required longer transition durations compared to TD children or adults to cross from one phoneme category to another. The children with CIs demonstrated less confidence in labelling the stimuli (i.e. less steep slopes) than the TD children or the adults. For the /da/–/ja/ contrast, the children with CIs showed less steep slope values than adults.Conclusion: These results suggest that there are differences in the way TD children and children with CIs develop and maintain phonetic categories, perhaps differences in phonetic representation or in linking acoustic and phonetic representations.

Hearing loss in children with otitis media with effusion: a systematic review

Objectives: Otitis media with effusion (OME) is the presence of non-purulent inflammation in the middle ear. Hearing impairment is frequently associated with OME. Pure tone audiometry and speech audiometry are two of the most primarily utilised auditory assessments and provide valuable behavioural and functional estimation on hearing loss. This paper was designed to review and analyse the effects of the presence of OME on children’s listening abilities. Design: A systematic and descriptive review. Study sample: Twelve articles reporting frequency-specific pure tone thresholds and/or speech perception measures in children with OME were identified using PubMed, Ovid, Web of Science, ProQuest and Google Scholar search platforms. Results: The hearing loss related to OME averages 18–35 dB HL. The air conduction configuration is roughly flat with a slight elevation at 2000 Hz and a nadir at 8000 Hz. Both speech-in-quiet and speech-in-noise perception have been found to be impaired. Conclusions: OME imposes a series of disadvantages on hearing sensitivity and speech perception in children. Further studies investigating the full range of frequency-specific pure tone thresholds, and that adopt standardised speech test materials are advocated to evaluate hearing related disabilities with greater comprehensiveness, comparability and enhanced consideration of their real life implications.

An event related potentials method for assessing audiovisual speech integration: Implications for children with autism spectrum disorders

Perceptual studies of children with autism spectrum disorders (ASD) strongly implicate deficits in processing of audiovisual (AV) speech. Previous research with AV stimuli has typically been conducted in the context of auditory noise or with mismatched auditory and visual (“McGurk”) stimuli. Although both types of stimuli are well-established methods for testing typically developing (TD) participants, they may create additional processing problems for children with ASD. To more precisely examine audiovisual (AV) speech perception in children with ASD, we developed a novel measure of AV processing that involves neither noise nor AV cross-category conflict. The speech stimuli include clear exemplars of the syllable /ba/ and a modified version of /ba/ in which the consonant is substantially weakened so that the syllable is heard as “/a/ ”. These are dubbed with a video of the speaker saying /ba/. Audiovisual integration should result in the visual information effectively “restoring” the weakened auditory “/a/ ” cues so that the stimulus is perceived as /ba/. Using event related potentials (ERP), we will present evidence from typically developing adults and preliminary results from children with ASD and TD to examine whether children with ASD are weaker in AV speech integration.

Auditory Speech Perception Development in Relation to Patient's Age with Cochlear Implant.

Introduction A cochlear implant in adolescent patients with pre-lingual deafness is still a debatable issue. Objective The objective of this study is to analyze and compare the development of auditory speech perception in children with pre-lingual auditory impairment submitted to cochlear implant, in different age groups in the first year after implantation. Method This is a retrospective study, documentary research, in which we analyzed 78 reports of children with severe bilateral sensorineural hearing loss, unilateral cochlear implant users of both sexes. They were divided into three groups: G1, 22 infants aged less than 42 months; G2, 28 infants aged between 43 to 83 months; and G3, 28 older than 84 months. We collected medical record data to characterize the patients, auditory thresholds with cochlear implants, assessment of speech perception, and auditory skills. Results There was no statistical difference in the association of the results among groups G1, G2, and G3 with sex, caregiver education level, city of residence, and speech perception level. There was a moderate correlation between age and hearing aid use time, age and cochlear implants use time. There was a strong correlation between age and the age cochlear implants was performed, hearing aid use time and age CI was performed. Conclusion There was no statistical difference in the speech perception in relation to the patient's age when cochlear implant was performed. There were statistically significant differences for the variables of auditory deprivation time between G3 - G1 and G2 - G1 and hearing aid use time between G3 - G2 and G3 - G1.

The relationship between cortical auditory evoked potentials (CAEPs) and speech perception in children with Nurotron® cochlear implants during four years of follow-up

ObjectiveThe purpose of the current study was to evaluate the relationship between the presence or absence of cortical auditory evoked potentials (CAEPs) to speech stimuli and the performance of speech perception in Chinese pediatric recipients of the Nurotron® cochlear implant (CI).We also wanted to determine how the CAEPs might be used as an indicator for predicting early speech perception and could provide objective evidence for clinical applications of CAEPs. Methods23 pediatric unilateral CI recipients participated in this study. 15 males 8 females, and their ages at implantation ranged from 13 to 68 months, with a mean age of 36 months. CAEPs and Mandarin Early Speech Perception (MESP) tests were used to evaluate the audibility and speech perception of these CI users. The tests were administered at the first, second, third, and fourth year after the CI surgery. ResultsAll the subjects demonstrated improvements in detection of speech sounds with CI. The percentages of participants who could detect all three stimuli were 26% (6/23) at first year, to 100% (23/23) at the fourth year post-implantation. The percentages of participants who passed the Category 6 of MESP were from 9% (2/23) at first year, to 91% (21/23) at the fourth year post-implantation. Significant correlations (p<0.05) were found between CAEP scores and MESP at the first, second, third year after the CI surgery. The multiple regression equation for prediction of MESP categories from CAEP scores and hearing ages was MESP=1.088+(0.504×CAEP score)+(0.964×hearing ages) (F=72.919, p<0.001, R2=0.621). ConclusionThe results of this study suggested that aided cortical assessment was a useful tool to evaluate the outcomes of cochlear implantation. Cortical outcomes had a significant positive relationship with the MESP, which predicted the early speech perception of CI recipients.

Effect of room acoustics on speech perception by children with hearing loss

To study speech perception of children with hearing loss in virtual acoustic environments (VAE), a pair of research hearing aids has been previously integrated in a real-time dynamic binaural reproduction system. The auralization included simulations of room acoustics using individualized head-related and hearing aid-related transfer functions (HRTF and HARTF). In this study, a release from masking paradigm by Cameron and Dillon (2007) was adopted in German to investigate speech intelligibility by children fitted with hearing aids under realistic classroom acoustics. When immersed in VAE, each child was asked to repeat sentences spoken by a target talker always located at 0° azimuth, while two distractor talkers were continuously telling unfamiliar Grimm stories. The speech reception threshold (SRT) at 50% intelligibility was measured adaptively by changing the target talker speech level. A total of eight conditions were tested with each child by changing spatial cues (target-distractor collocated versus ...

Auditory neuropathy in Brown-Vialetto-Van Laere syndrome due to riboflavin transporter RFVT2 deficiency.

Mutations in the genes encoding the riboflavin transporters RFVT2 and RFVT3 have been identified in Brown-Vialetto-Van Laere syndrome, a neurodegenerative disorder characterized by hearing loss and pontobulbar palsy. Treatment with riboflavin has been shown to benefit individuals with the phenotype of RFVT2 deficiency. Understanding the characteristics of hearing loss in riboflavin transporter deficiency would enable early diagnosis and therapy. We performed hearing assessments in seven children (from four families) with RFVT2 deficiency and reviewed results from previous assessments. Assessments were repeated after 12 months and 24 months of riboflavin therapy and after cochlear implantation in one individual. Hearing loss in these individuals was due to auditory neuropathy spectrum disorder (ANSD). Hearing loss was identified between 3 years and 8 years of age and progressed rapidly. Hearing aids were not beneficial. Riboflavin therapy resulted in improvement of hearing thresholds during the first year of treatment in those with recent-onset hearing loss. Cochlear implantation resulted in a significant improvement in speech perception in one individual. Riboflavin transporter deficiency should be considered in all children presenting with an auditory neuropathy. Speech perception in children with ANSD due to RFVT2 deficiency may be significantly improved by cochlear implantation.