Cortical Auditory Evoked Potential Testing in Children With Auditory Neuropathy Spectrum Disorder.

PURPOSE: In the present report, we reviewed the role of cortical auditory evoked potentials (CAEPs) as an objective measure during the evaluation and management process in children with auditory neuropathy spectrum disorder (ANSD). METHOD: We reviewed the results of CAEP recordings in 66 patients with ANSD aged between 2 months and 12 years and assessed the relationship between their characteristics (prevalence, morphology, latencies, and amplitudes) and various clinical features, including the mode of medical management. RESULTS: Overall, the CAEPs were present in 85.2% of the ears tested. Factors such as prematurity, medical complexity, neuronal issues, or presence of syndromes did not have an effect on the presence or absence of CAEPs. CAEP latencies were significantly shorter in ears with cochlear nerve deficiency than in ears with a normal caliber nerve. Three different patterns of CAEP responses were observed in patients with bilateral ANSD and present cochlear nerves: (a) responses with normal morphology and presence of both P1-P2complex and N2 components, (b) responses with abnormal morphology and presence of the N2 component but undefined P1-P2complex peak, and (c) entirely absent responses. None of the patients with normal, mild, or moderate degree of hearing loss had a complete absence of CAEP responses. No significant differences were uncovered when comparing the latencies across unaided and aided children and children who later received cochlear implants. CONCLUSIONS: The CAEP protocol used in our ANSD program did inform about the presence or absence of central auditory stimulation. Absent responses typically fit into an overall picture of complete auditory deprivation and all of these children were ultimately offered cochlear implants after failing to develop oral language. Present responses, on the other hand, were acknowledged as a sign of some degree of auditory stimulation but always interpreted with caution given that prognostic implications remain unclear.

Examining the Relation Between Exercise and Word Learning in Preschool-Age Children.

PURPOSE: There is evidence suggesting that aerobic exercise immediately after vocabulary training can improve word recall in school-age children. This work examined whether the previously identified word-learning benefits associated with exercise can be extended to preschoolers. Additionally, we evaluated whether the effects of physical activity on vocabulary learning may be influenced by existing language skills that the child possesses. METHOD: Children ages 3-6 years completed the study (N = 42). Data were collected via a virtual testing session in which participants completed a word-learning task that included two experimental conditions (exercise and resting). In the resting measure, children were taught names of novel objects and then sat down and colored for 3 min before being tested on their ability to identify the trained words. The exercise condition was identical, except that participants engaged in 3 min of guided aerobic exercise before testing. Additionally, at the end of the visit, participants completed the Quick Interactive Language Screener (QUILS), which measured general language skills. RESULTS: Accuracy of word recognition was significantly higher after the exercise condition compared to the resting condition. Furthermore, this pattern of results was not related to children's existing language skills, as measured by the QUILS. CONCLUSIONS: This study is one of the first to closely examine the relation between physical activity and word-learning abilities in children as young as 3-6 years of age. Results align with previous findings stating that aerobic exercise can boost vocabulary learning and suggest that this is the case regardless of existing language skills.

Developmental Changes in the Perception of Vocal Loudness and Voice Quality in 3- to 6-Year-Old Children.

PURPOSE: The study's primary aim was to investigate developmental changes in the perception of vocal loudness and voice quality in children 3-6 years of age. A second aim was to evaluate a testing procedure-the intermodal preferential looking paradigm (IPLP)-for the study of voice perception in young children. METHOD: Participants were categorized in two age groups: 3- to 4-year-olds and 5- to 6-year-olds. Children were tested remotely via a Zoom appointment and completed two perceptual tasks: (a) voice discrimination and (b) voice identification. Each task consisted of two tests: a vocal loudness test and a voice quality test. RESULTS: Children in the 5- to 6-year-old group were significantly more accurate than children in the 3- to 4-year-old group in discriminating and identifying differences between voices for both loudness and voice quality. The IPLP, used in the identification task, was found to successfully detect differences between the age groups for overall accuracy and for most of the sublevels of vocal loudness and voice quality. CONCLUSIONS: Results suggest that children's ability to discriminate and identify differences in vocal loudness and voice quality improves with age. Findings also support the use of the IPLP as a useful tool to study voice perception in young children.

Pediatric Moyamoya Disease and Syndrome in Italy: A Multicenter Cohort.

Background: Moyamoya is a rare progressive cerebral arteriopathy, occurring as an isolated phenomenon (moyamoya disease, MMD) or associated with other conditions (moyamoya syndrome, MMS), responsible for 6-10% of all childhood strokes and transient ischemic attacks (TIAs). Methods: We conducted a retrospective multicenter study on pediatric-onset MMD/MMS in Italy in order to characterize disease presentation, course, management, neuroradiology, and outcome in a European country. Results: A total of 65 patients (34/65 women) with MMD (27/65) or MMS (38/65) were included. About 18% (12/65) of patients were asymptomatic and diagnosed incidentally during investigations performed for an underlying condition (incMMS), whereas 82% (53/65) of patients with MMD or MMS were diagnosed due to the presence of neurological symptoms (symptMMD/MMS). Of these latter, before diagnosis, 66% (43/65) of patients suffered from cerebrovascular events with or without other manifestations (ischemic stroke 42%, 27/65; TIA 32%, 21/65; and no hemorrhagic strokes), 18% (12/65) of them reported headache (in 4/12 headache was not associated with any other manifestation), and 26% (17/65) of them experienced multiple phenotypes (≥2 among: stroke/TIA/seizures/headache/others). Neuroradiology disclosed ≥1 ischemic lesion in 67% (39/58) of patients and posterior circulation involvement in 51% (30/58) of them. About 73% (47/64) of patients underwent surgery, and 69% (45/65) of them received aspirin, but after diagnosis, further stroke events occurred in 20% (12/61) of them, including operated patients (11%, 5/47). Between symptom onset and last follow-up, the overall patient/year incidence of stroke was 10.26% (IC 95% 7.58-13.88%). At last follow-up (median 4 years after diagnosis, range 0.5-15), 43% (26/61) of patients had motor deficits, 31% (19/61) of them had intellectual disability, 13% (8/61) of them had epilepsy, 11% (7/61) of them had behavioral problems, and 25% (13/52) of them had mRS > 2. The proportion of final mRS > 2 was significantly higher in patients with symptMMD/MMS than in patients with incMMS (p = 0.021). Onset age <4 years and stroke before diagnosis were significantly associated with increased risk of intellectual disability (p = 0.0010 and p = 0.0071, respectively) and mRS > 2 at follow-up (p = 0.0106 and p = 0.0009, respectively). Conclusions: Moyamoya is a severe condition that may affect young children and frequently cause cerebrovascular events throughout the disease course, but may also manifest with multiple and non-cerebrovascular clinical phenotypes including headache (isolated or associated with other manifestations), seizures, and movement disorder. Younger onset age and stroke before diagnosis may associate with increased risk of worse outcome (final mRS > 2).

Webcams, Songs, and Vocabulary Learning: A Comparison of In-Person and Remote Data Collection as a Way of Moving Forward With Child-Language Research.

This article evaluates a testing procedure for collecting eye-gaze data with toddlers and preschoolers during a word-learning task. We provide feasibility and precision data by comparing performance in an in-person version of the study (conducted under controlled conditions in the lab), with performance in a virtual version in which participants completed the testing procedure from home. Our data support the feasibility of collecting remote eye-gaze data with young children, and present it as a viable alternative for conducting developmental language research when in-person interactions with participants cannot take place. Additionally, we use this methodological approach to examine a topic that has gained popularity in recent years-the role of music and songs on vocabulary learning. We provide evidence suggesting that while songs may help increase attention during a particular task, greater attention does not lead to greater learning. In fact, preschoolers show improved word-learning performance for items that were trained in a spoken sentence compared to items that were trained in a song. This means that while songs may be beneficial for increasing child engagement, spoken sentences may be best for supporting deep level learning of language concepts.

Examining the Role of Physical Activity on Word Learning in School-Aged Children.

Purpose Previous studies show that there is increased brain activity after exercise, leading to improved word recall in adults. The aim of this study was to examine whether different types of exercise (i.e., aerobic vs. anaerobic) may also lead to improved performance during vocabulary learning in children. Method A total of 48 participants (24 in Experiment 1 and 24 in Experiment 2) between the ages of 6 and 12 years completed a word learning task. Training of words took place in a resting and in an exercise condition using a within-subject design. In the resting measure, children were taught names of novel objects and then colored for 3 min before being tested on their ability to recognize the words. In the exercise condition, the same steps were followed, but instead of coloring, children engaged in 3 min of either aerobic exercise (i.e., swimming in Experiment 1) or anaerobic exercise (i.e., a CrossFit-like workout in Experiment 2). Results In Experiment 1, accuracy of word recognition was significantly higher for words that were trained in the aerobic exercise compared to the resting condition. In Experiment 2, there was no significant difference in performance between the anaerobic exercise and resting conditions. Conclusions These findings suggest that previously identified benefits of exercise on language abilities in adults also extend to school-aged children. However, not all types of physical activity lead to this boost in performance, as only aerobic (but not anaerobic) exercise improved children's ability to acquire new word-object relations. Supplemental Material https://doi.org/10.23641/asha.14462187.

A comparison of monolingual and bilingual toddlers' word recognition in noise.

Aims and objectives: The purpose of this study was to examine whether differences in language exposure (i.e., being raised in a bilingual versus a monolingual environment) influence young children's ability to comprehend words when speech is heard in the presence of background noise. Methodology: Forty-four children (22 monolinguals and 22 bilinguals) between the ages of 29 and 31 months completed a preferential looking task where they saw picture-pairs of familiar objects (e.g., balloon and apple) on a screen and simultaneously heard sentences instructing them to locate one of the objects (e.g., look at the apple!). Speech was heard in quiet and in the presence of competing white noise. Data and analyses: Children's eye-movements were coded off-line to identify the proportion of time they fixated on the correct object on the screen and performance across groups was compared using a 2 × 3 mixed analysis of variance. Findings: Bilingual toddlers performed worse than monolinguals during the task. This group difference in performance was particularly clear when the listening condition contained background noise. Originality: There are clear differences in how infants and adults process speech in noise. To date, developmental work on this topic has mainly been carried out with monolingual infants. This study is one of the first to examine how background noise might influence word identification in young bilingual children who are just starting to acquire their languages. Significance: High noise levels are often reported in daycares and classrooms where bilingual children are present. Therefore, this work has important implications for learning and education practices with young bilinguals.

Toddlers' fast-mapping from noise-vocoded speech.

The ability to recognize speech that is degraded spectrally is a critical skill for successfully using a cochlear implant (CI). Previous research has shown that toddlers with normal hearing can successfully recognize noise-vocoded words as long as the signal contains at least eight spectral channels [Newman and Chatterjee. (2013). J. Acoust. Soc. Am. 133(1), 483-494; Newman, Chatterjee, Morini, and Remez. (2015). J. Acoust. Soc. Am. 138(3), EL311-EL317], although they have difficulty with signals that only contain four channels of information. Young children with CIs not only need to match a degraded speech signal to a stored representation (word recognition), but they also need to create new representations (word learning), a task that is likely to be more cognitively demanding. Normal-hearing toddlers aged 34 months were tested on their ability to initially learn (fast-map) new words in noise-vocoded stimuli. While children were successful at fast-mapping new words from 16-channel noise-vocoded stimuli, they failed to do so from 8-channel noise-vocoded speech. The level of degradation imposed by 8-channel vocoding appears sufficient to disrupt fast-mapping in young children. Recent results indicate that only CI patients with high spectral resolution can benefit from more than eight active electrodes. This suggests that for many children with CIs, reduced spectral resolution may limit their acquisition of novel words.

Monolingual and Bilingual Word Recognition and Word Learning in Background Noise.

The question of whether bilingualism leads to advantages or disadvantages in linguistic abilities has been debated for many years. It is unclear whether growing up with one versus two languages is related to variations in the ability to process speech in the presence of background noise. We present findings from a word recognition and a word learning task with monolingual and bilingual adults. Bilinguals appear to be less accurate than monolinguals at identifying familiar words in the presence of white noise. However, the bilingual "disadvantage" identified during word recognition is not present when listeners were asked to acquire novel word-object relations that were trained either in noise or in quiet. This work suggests that linguistic experience and the demands associated with the type of task both play a role in the ability for listeners to process speech in noise.

Dónde está la ball? Examining the effect of code switching on bilingual children's word recognition.

Hearing words in sentences facilitates word recognition in monolingual children. Many children grow up receiving input in multiple languages - including exposure to sentences that 'mix' the languages. We explored Spanish-English bilingual toddlers' (n = 24) ability to identify familiar words in three conditions: (i) single word (ball!); (ii) same-language sentence (Where's the ball?); or (iii) mixed-language sentence (Dónde está la ball?). Children successfully identified words across conditions; however, the advantage linked to hearing words in sentences was present only in the same-language condition. This work hence suggests that language mixing plays an important role on bilingual children's ability to recognize spoken words.

Effect of the relationship between target and masker sex on infants' recognition of speech.

When faced with multiple people speaking simultaneously, adult listeners use the sex of the talkers as a cue for separating competing streams of speech. As a result, adult listeners show better performance when a target and a background voice differ from one another in sex. Recent research suggests that infants under 1 year do not show this advantage. So when do infants begin to use talker-gender cues for stream segregation? These studies find that 16-month-olds do not show an advantage when the masker and target differ in sex. However, by 30 months, toddlers show the more adult-like pattern of performance.

Toddlers' comprehension of degraded signals: Noise-vocoded versus sine-wave analogs.

Recent findings suggest that development changes the ability to comprehend degraded speech. Preschool children showed greater difficulties perceiving noise-vocoded speech (a signal that integrates amplitude over broad frequency bands) than sine-wave speech (which maintains the spectral peaks without the spectrum envelope). In contrast, 27-month-old children in the present study could recognize speech with either type of degradation and performed slightly better with eight-channel vocoded speech than with sine-wave speech. This suggests that children's identification performance depends critically on the degree of degradation and that their success in recognizing unfamiliar speech encodings is encouraging overall.

Linguistically-based informational masking in preschool children.

Previous work has shown that young children exhibit more difficulty understanding speech in the presence of speech-like distractors than do adults, and are more susceptible to at least some form of informational masking (IM). Yet little is known about how/when the "susceptibility" to linguistically-based IM develops. The authors tested adults, school-age children (aged 8 yrs), and preschool-age children (aged 4 yrs) on sentence recognition in the presence of normal speech, "jumbled" speech, and reversed speech distractors. As has been found previously with adults [e.g., Summers and Molis (2004). J. Speech, Lang. Hear. Res. 47, 245-256], children in both age groups showed a release of masking when the distractor was uninterpretable (reversed speech). This suggests that children already demonstrate linguistically-based IM by the age of 4 yrs.

CTNND2 deletion and intellectual disability.

Neurodevelopmental disorders are a group of diseases characterized by either structural or functional alterations. The clinical spectrum can vary from isolated intellectual disability to more complex syndromes. Molecular karyotyping can explain 14%-18% of cases due to the presence of large pathogenic CNVs. Moreover, small CNVs involving single genes might result in a monogenic disease. In this article we report two cases of intragenic CTNND2 deletion, detected by molecular karyotyping, in patients with isolated intellectual disability.

Look at the gato! Code-switching in speech to toddlers.

We examined code-switching (CS) in the speech of twenty-four bilingual caregivers when speaking with their 18- to 24-month-old children. All parents CS at least once in a short play session, and some code-switched quite often (over 1/3 of utterances). This CS included both inter-sentential and intra-sentential switches, suggesting that at least some children are frequently exposed to mixed-language sentences. However, we found no evidence that this exposure to CS had any detrimental effect on children's word learning: children's overall vocabulary size did not relate to parental inter-sentential CS behavior, and was positively related to within-sentence CS. Parents often repeated words across their two languages, but this did not appear to increase the likelihood of children having translation equivalents in their vocabulary. In short, parents appear to CS fairly often to young children, even within sentences, but there is no evidence that this delays child lexical acquisition.

Additive effects of repetition and predictability during comprehension: evidence from event-related potentials.

Previous research has shown that neural responses to words during sentence comprehension are sensitive to both lexical repetition and a word's predictability in context. While previous research has often contrasted the effects of these variables (e.g. by looking at cases in which word repetition violates sentence-level constraints), little is known about how they work in tandem. In the current study we examine how recent exposure to a word and its predictability in context combine to impact lexical semantic processing. We devise a novel paradigm that combines reading comprehension with a recognition memory task, allowing for an orthogonal manipulation of a word's predictability and its repetition status. Using event-related brain potentials (ERPs), we show that word repetition and predictability have qualitatively similar and additive effects on the N400 amplitude. We propose that prior exposure to a word and predictability impact lexical semantic processing in an additive and independent fashion.

Infants' name recognition in on- and off-channel noise.

Previous work by Polka, Rvachew, and Molnar [Infancy 13(5), 421-439 (2008)] has reported that infants are poor at focusing their attention on a particular frequency range, and, as a result, are distracted by maskers that are outside of the target frequency range. The current study explores this effect of irrelevant distractors further and finds that 8-month-old infants are significantly less affected by maskers outside the frequency range (off-channel maskers) than by on-channel maskers. Thus while infants may display difficulty ignoring irrelevant distractors, they are able to do so to at least some degree, suggesting some ability to perceive speech from spectrally remote maskers, despite the demonstrated presence of greater informational masking at this age.