Auditory evoked potentials: predicting speech therapy outcomes in children with phonological disorders

OBJECTIVES:

This study investigated whether neurophysiologic responses (auditory evoked potentials) differ between typically developed children and children with phonological disorders and whether these responses are modified in children with phonological disorders after speech therapy.

METHODS:

The participants included 24 typically developing children (Control Group, mean age: eight years and ten months) and 23 children clinically diagnosed with phonological disorders (Study Group, mean age: eight years and eleven months). Additionally, 12 study group children were enrolled in speech therapy (Study Group 1), and 11 were not enrolled in speech therapy (Study Group 2). The subjects were submitted to the following procedures: conventional audiological, auditory brainstem response, auditory middle-latency response, and P300 assessments. All participants presented with normal hearing thresholds. The study group 1 subjects were reassessed after 12 speech therapy sessions, and the study group 2 subjects were reassessed 3 months after the initial assessment. Electrophysiological results were compared between the groups.

RESULTS:

Latency differences were observed between the groups (the control and study groups) regarding the auditory brainstem response and the P300 tests. Additionally, the P300 responses improved in the study group 1 children after speech therapy.

CONCLUSION:

The findings suggest that children with phonological disorders have impaired auditory brainstem and cortical region pathways that may benefit from speech therapy.     

The influence of (central) auditory processing disorder on the severity of speech-sound disorders in children

OBJECTIVE:To identify a cutoff value based on the Percentage of Consonants Correct-Revised index that could indicate the likelihood of a child with a speech-sound disorder also having a (central) auditory processing disorder.METHODS:Language, audiological and (central) auditory processing evaluations were administered. The participants were 27 subjects with speech-sound disorders aged 7 to 10 years and 11 months who were divided into two different groups according to their (central) auditory processing evaluation results.RESULTS:When a (central) auditory processing disorder was present in association with a speech disorder, the children tended to have lower scores on phonological assessments. A greater severity of speech disorder was related to a greater probability of the child having a (central) auditory processing disorder. The use of a cutoff value for the Percentage of Consonants Correct-Revised index successfully distinguished between children with and without a (central) auditory processing disorder.CONCLUSIONS: The severity of speech-sound disorder in children was influenced by the presence of (central) auditory processing disorder. The attempt to identify a cutoff value based on a severity index was successful.     

Learning impaired children exhibit timing deficits and training-related improvements in auditory cortical responses to speech in noise

The physiological mechanisms that contribute to abnormal encoding of speech in children with learning problems are yet to be well understood. Furthermore, speech perception problems appear to be particularly exacerbated by background noise in this population. This study compared speech-evoked cortical responses recorded in a noisy background to those recorded in quiet in normal children (NL) and children with learning problems (LP). Timing differences between responses recorded in quiet and in background noise were assessed by cross-correlating the responses with each other. Overall response magnitude was measured with root-mean-square (RMS) amplitude. Cross-correlation scores indicated that 23% of LP children exhibited cortical neural timing abnormalities such that their neurophysiological representation of speech sounds became distorted in the presence of background noise. The latency of the N2 response in noise was isolated as being the root of this distortion. RMS amplitudes in these children did not differ from NL children, indicating that this result was not due to a difference in response magnitude. LP children who participated in a commercial auditory training program and exhibited improved cortical timing also showed improvements in phonological perception. Consequently, auditory pathway timing deficits can be objectively observed in LP children, and auditory training can diminish these deficits.     

Electrophysiologic assessment of central auditory processing by auditory brainstem responses in children with autism spectrum disorders

In addition to aberrant features in the speech, children with Autism Spectrum Disorder (ASD) may present unusual responses to sensory stimuli, especially to auditory stimuli. We investigated the auditory ability of children with ASD by using Auditory Brainstem Responses (ABR) as they can directly judge both hearing status and the integrity of auditory brainstem pathways. One hundred twenty-one children (71: ASD; M 58/ F 13, mean age; 41.8 months, 50: control group; M 41/ F 9, mean age; 38 months) were included in the study. As compared with the values in the control group, the latency of wave V, wave I-V, and wave III-V inter-peak latencies were significantly prolonged (p<0.05) in the ASD group. The findings indicate that children with ASD have a dysfunction or immaturity of the central auditory nervous system. We suggest any children with prolonged III-V inter-peak latencies, especially high functioning children should be further evaluated for central auditory processing to set up a more appropriate treatment plan.     

儿童感音神经性聋听觉稳态诱发反应阈与听性脑干反应阈比较

目的研究听觉稳态诱发反应(ASSR)和听性脑干反应(ABR)阈与纯音听阈的差别和相关性。方法选择74例儿童感音神经性聋患者(118耳)分别进行ASSR、ABR和电测听检查,比较ASSR、ABR反应阈及纯音听阈,同时就ASSR、ABR反应阈与纯音听阈进行相关性分析。结果 ASSR和ABR反应阈与纯音听阈均有良好的相关性。ABR的反应阈与纯音听阈阈值接近,而ASSR反应阈与纯音听阈间差值较大。ASSR反应阈与纯音听阈间的相关性要优于ABR反应阈与纯音听阈间的相关性。结论 ASSR和ABR均为较好的评估行为听阈的客观测听方法。     

Non-linguistic auditory processing and working memory update in pre-school children who stutter: an electrophysiological study

Non-linguistic auditory processing and working memory update were examined with event-related potentials (ERPs) in 18 children who stutter (CWS) and 18 children who do not stutter (CWNS). Children heard frequent 1 kHz tones interspersed with rare 2 kHz tones. The two groups did not differ on any measure of the P1 and N1 components, strongly suggesting that early auditory processing of pure tones is unimpaired in CWS. However, as a group, only CWNS exhibited a P3 component to rare tones, suggesting that developmental stuttering may be associated with a less efficient attentional allocation and working memory update in response to auditory change.     

Lateralisation of auditory processing in Down syndrome: a study of T-complex peaks Ta and Tb

It has long been argued that abnormal cerebral lateralisation might underlie the language problems that characterise Down syndrome, but to date only behavioural evidence has been provided. We used the auditory event-related potentials Ta and Tb of the T-complex to investigate lateralised processing of speech (vowels) and non-speech (simple and complex tones) sounds in children with Down syndrome and age-matched typically developing children. We also explored associations with speech and language abilities. Although changes in the Ta and Tb in response to increases in stimulus complexity and 'speechness' were similar across group, the Tb peak was delayed in children with Down syndrome across conditions. In addition, marked differences in the patterns of lateralisation of Ta latency and Tb amplitude were observed in children with Down syndrome, in response to both speech and non-speech sounds. No associations were found between Ta and Tb characteristics and speech and language abilities in children with DS.     

Hypersensitivity to acoustic change in children with autism: electrophysiological evidence of left frontal cortex dysfunctioning

Exaggerated reactions to even small changes in the environment and abnormal behaviors in response to auditory stimuli are frequently observed in children with autism (CWA). Brain mechanisms involved in the automatic detection of auditory frequency change were studied using scalp potential and scalp current density (SCD) mapping of mismatch negativity (MMN) in 15 CWA matched with 15 healthy children. Compared with the response in controls, MMN recorded at the Fz site in CWA showed significantly shorter latency and was followed by a P3a wave. Mapping of potentials indicated significant intergroup differences. Moreover, SCD mapping demonstrated the dynamics of the different MMN generators: Although temporal component was evidenced bilaterally in both groups, it occurred earlier on the left hemisphere in CWA, preceded by an abnormal early left frontal component. The electrophysiological pattern reported here emphasized a left frontal cortex dysfunctioning that might also be implicated in cognitive and behavioral impairment characteristic, of this complex neurodevelopmental disorder.     

Neural sources involved in auditory target detection and novelty processing: an event-related fMRI study

We used event-related functional magnetic resonance imaging (erfMRI) techniques to examine the cerebral sites involved with target detection and novelty processing of auditory stimuli. Consistent with the results from a recent erfMRI study in the visual modality, target processing was associated with activation bilaterally in the anterior superior temporal gyrus, inferior and middle frontal gyrus, inferior and superior parietal lobules, anterior and posterior cingulate, thalamus, caudate, and the amygdala/hippocampal complex. Analyses of the novel stimuli revealed activation bilaterally in the inferior frontal gyrus, insula, inferior parietal lobule, and in the inferior, middle, and superior temporal gyri. These data suggest that the scalp recorded event-related potentials (e.g., N2 and P3) elicited during similar tasks reflect an ensemble of neural generators located in spatially remote cortical areas.     

The commissure of the inferior colliculus shapes frequency response areas in rat: an in vivo study using reversible blockade with microinjection of kynurenic acid

The commissure of the inferior colliculus (CoIC) interconnects corresponding frequency-band laminae in the two inferior colliculi (ICs). Although the CoIC has been studied neurophysiologically in vitro, the effect of the CoIC on the responses of IC neurons to physiological stimuli has not been addressed. In this study, we injected the glutamate receptor blocker kynurenic acid into one IC while recording the frequency response areas (FRAs) of neurons in the other, to test the hypothesis that frequency response properties of IC neurons are influenced by commissural inputs from the contralateral IC. Following blockade of the commissure, 10 of 12 neurons tested exhibited an increase or a decrease in their FRAs. In most neurons (9/12) the response area changed in the same direction, irrespective of whether the neuron was stimulated monaurally (at the ear contralateral to the recorded IC) or binaurally. In one neuron, blockade of the CoIC resulted in an expansion of the response area under binaural stimulation and a contraction under monaural stimulation. In the remaining two units, no effect was observed. Changes in response areas that exceeded the criterion ranged between 17 and 80% of control values with monaural stimulation, and 35 and 77% with binaural stimulation. Area changes could also be accompanied by changes in spike rate and monotonicity. From our observation that FRAs contract following commissure block, we infer that the commissure contains excitatory fibres. The expansion of response areas in other cases, however, suggests that the commissure also contains inhibitory fibres, or that its effects are mediated by disynaptic as well as monosynaptic circuits. The small sample size precludes a definitive conclusion as to which effect predominates. We conclude that inputs from the contralateral IC projecting via the CoIC influence the spectral selectivity and response gain of neurons in the IC.     

Mental and personality disorders in infertile women with polycystic ovary: a case-control study

BackgroundPolycystic Ovarian Syndrome (PCOS) is one of the most common causes of infertility in women.ObjectiveThe current study investigated mental and personality disorders in infertile women with and without PCOS.MethodsThis case-control study evaluated 400 infertile women who referred to the Infertility Center in Babol city (North of Iran). Participants were categorized into the case group (201 PCOS) and the control group (199 without PCOS). All of the participants completed the Millon Clinical Multi-axial Inventory-III (MCMI-III).ResultsThe mean scores for clinical personality patterns were significantly higher for six personality disorders (schizoid, avoidant, antisocial, depressive, sadistic, and negativistic) and for three classes of severe personality disorder patterns (schizotypal, borderline, and paranoid) in infertile women with PCOS than in women without PCOS. The mean scores for eight clinical disorders (somatoform, manic disorder, dysthymia, alcohol-dependence, drug-dependence, post-trauma stress disorder, major depression, and delusion disorder) were also higher in infertile women with PCOS than in women without PCOS.ConclusionThe scores of many mental and personality disorders are higher in infertile women with PCOS than in women without PCOS. Thus, clinicians should prioritize recognizing and treating psychological problems of infertile women with PCOS.     

Atopic disorders in CHARGE syndrome: A retrospective study and literature review

Background

Atopic disorders have been reported in CHARGE syndrome, but the prevalence and underlying mechanisms are not known.

Visuospatial attention and motor reaction in children: an electrophysiological study of the "Posner" paradigm

To assess the processing stages involved in attention shifting and response selection tasks in children, we recorded event-related potentials (ERPs) and performance measures during a variant of the Posner paradigm. Subjects responded to visual targets, either preceded by a spatial cue (valid = same side; invalid = opposite side) or presented uncued. Valid targets evoked high-amplitude P1 responses, single-peaked P3s, and the shortest reaction times (RTs). Invalidity cued stimuli evoked delayed RTs, resulting in part from incorrectly oriented attention (decreased P1) leading to delayed target processing (belated N2-P3). Invalid targets also evoked a positive slow wave attributed to prolonged response selection due to cue/target incompatibility. Uncued stimuli elicited the longest RTs, unexplained by deficits in target detection or response selection, which likely resulted from a deficit in motor preparation due to the lack of warning signal. This method may be applied in clinical settings to disentangle selective processing deficits in target detection, response selection, or motor preparation stages.     

Safety and effectiveness of central venous catheterization in patients with cancer: prospective observational study

This study investigated the safety and effectiveness of each type of central venous catheters (CVC) in patients with cancer. We prospectively enrolled patients with cancer who underwent catheterization involving a subclavian venous catheter (SVC), peripherally inserted central venous catheter (PICC), or chemo-port (CP) in our department. From March 2007 to March 2009, 116 patients underwent 179 episodes of catheterization. A SVC was inserted most frequently (46.4%). Fifty-four complications occurred (30.1%): infection in 23 cases, malpositioning or migration of the tip in 18 cases, thrombosis in eight cases, and bleeding in five cases. Malpositioning or migration of the tip occurred more frequently with a PICC (P<0.001); infection occurred more often with a tunneled catheter (P=0.028) and was observed more often in young patients (P=0.023). The catheter life span was longer for patients with solid cancer (P=0.002) than for those with hematologic cancer, with a CP (P<0.001) than a PICC or SVC, and for an indwelling catheter with image guidance (P=0.014) than a blind procedure. In conclusion, CP is an effective tool for long term use and the fixation of tip is important for the management of PICC.     

Genotypes and phenotypes in children with short stature: clinical indicators of SHOX haploinsufficiency

Background

Short stature affects approximately 2% of children, representing one of the more frequent disorders for which clinical attention is sought during childhood. Despite assumed genetic heterogeneity, mutations or deletions of the short stature homeobox‐containing gene (SHOX) are found quite frequently in subjects with short stature. Haploinsufficiency of the SHOX gene causes short stature with highly variable clinical severity, ranging from isolated short stature without dysmorphic features to Léri‐Weill syndrome, and with no functional copy of the SHOX gene, Langer syndrome.

Methods

To characterise the clinical and molecular spectrum of SHOX deficiency in childhood we assessed the association between genotype and phenotype in a large cohort of children of short stature from 14 countries.

Results

Screening of 1608 unrelated individuals with sporadic or familial short stature revealed SHOX mutations or deletions in 68 individuals (4.2%): complete deletions in 48 (70.6%), partial deletions in 4 (5.9%) and point mutations in 16 individuals (23.5%). Although mean height standard deviation score (SDS) was not different between participants of short stature with or without identified SHOX gene defects (–2.6 vs –2.6), detailed examination revealed that certain bone deformities and dysmorphic signs, such as short forearm and lower leg, cubitus valgus, Madelung deformity, high‐arched palate and muscular hypertrophy, differed markedly between participants with or without SHOX gene defects (p<0.001). Phenotypic data were also compared for 33 children with Turner syndrome in whom haploinsufficiency of SHOX is thought to be responsible for the height deficit.

Conclusion

A phenotype scoring system was developed that could assist in identifying the most appropriate subjects for SHOX testing. This study offers a detailed genotype‐phenotype analysis in a large cohort of children of short stature, and provides quantitative clinical guidelines for testing of the SHOX gene.     

Hyperactivity within an extensive cortical distribution associated with excessive sensitivity in error processing in unmedicated depression: A combined event-related potential and sLORETA study

Individuals with depression are excessively sensitive to negative feedback and therefore overly cautious. To explore the neural mechanisms of response monitoring which contributed to their impaired behavioral adjustment, we recruited 22 individuals with depressive disorder and 24 healthy controls. Component analysis of the error-related negativity (ERN) and correct-related negativity (CRN), and sLORETA analysis of the ERN and CRN were combined. The comparable error rate and longer reaction time (RT) in individuals with depression as compared to healthy controls suggested a trade-off between accuracy and speed. The amplitude of the ERN and CRN was significantly enhanced in depression. Further sLORETA localizations of the ERN and CRN showed a significantly stronger current density with an extensive distribution in the anterior cingulate cortex (ACC), medial frontal cortex (MFC), inferior parietal lobule (IPL) and superior temporal gyrus (STG) in individuals with depression than in healthy controls. Increased activities in the ACC and MFC indexed increased response monitoring during automatic error detection, while hyperactivity over IPL and STG might indicate high uncertainness after error responses in depression. The hyperactivity within an extensive cortical distribution might be the neural basis of the excessive sensitivity to errors and the conservative accuracy/speed strategy in depression.     

Proband only exome sequencing in 403 Indian children with neurodevelopmental disorders: Diagnostic yield,utility and challenges in a resource-limited setting

Whole exome sequencing is recommended as the first tier test for neurodevelopmental disorders (NDDs) with trio being an ideal option for the detection of de novo variants. Cost constraints have led to adoption of sequential testing i.e. proband-only whole exome followed by targeted testing of parents. The reported diagnostic yield for proband exome approach ranges between 31 and 53%. Typically, these study designs have aptly incorporated targeted parental segregation before concluding a genetic diagnosis to be confirmed. The reported estimates however do not accurately reflect the yield of proband only standalone whole -exome, a question commonly posed to the referring clinician in self pay medical systems like India. To assess the utility of standalone proband exome (without follow up targeted parental testing), we retrospectively evaluated 403 cases of neurodevelopmental disorders referred for proband-only whole exome sequencing at Neuberg Centre for Genomic Medicine (NCGM), Ahmedabad during the period of January 2019 and December 2021. A diagnosis was considered confirmed only upon the detection of Pathogenic/Likely Pathogenic variants in concordance with patient's phenotype as well as established inheritance pattern. Targeted parental/familial segregation analysis was recommended as a follow up test where applicable. The diagnostic yield of the proband-only standalone whole exome was 31.5%. Only 20 families submitted samples for follow up targeted testing, and a genetic diagnosis was confirmed in twelve cases increasing the yield to 34.5%. To understand factors leading to poor uptake of sequential parental testing, we focused on cases where an ultra-rare variant was detected in hitherto described de novo dominant neurodevelopmental disorder. A total of 40 novel variants in genes associated with de novo autosomal dominant disorders could not be reclassified as parental segregation was denied. Semi-structured telephonic interviews were conducted upon informed consent to comprehend reasons for denial. Major factors influencing decision making included lack of definitive cure in the detected disorders; especially when couples not planning further conception and financial constraints to fund further targeted testing. Our study thus depicts the utility and challenges of proband-only exome approach and highlights the need for larger studies to understand factors influencing decision making in sequential testing.     

A meta-analysis on the effectiveness of intervention in children with primary speech and language delays/disorders: Focusing on China and the United States

The purpose of this meta-analysis was to investigate the immediate and long-term effects of intervention for children with primary speech and language delays/disorders and to explore whether some characteristics of interventions, specifics of the study and research participants moderate the magnitude of the effectiveness of interventions. Using the random effect model, we pooled the effect size and conducted a publication bias evaluation, a moderating effect analysis in CMA 2.0. Results of a random effects model analysis demonstrated a moderate immediate effect (g = 0.70), whereas the long-term efficacy was small (g = 0.23). Additionally, type of measure, language of intervention, parental involvement, intervention content and study quality, as well as the duration of intervention, significantly moderated the effect size of intervention effectiveness.