P192 Thyroid screening practices in the first year of life in children with down syndrome

Introduction: It is assumed that the critical period for diagnosis of hearing disorders is the baby's first three months of life and that appropriate course and implementation of treatment and/or rehabilitation should begin before a child is six months old. However various kinds of problems may occur during auditory screening of a child may exceed this interval. Aim: The aim of this study was an evaluation of auditory screening results for children over 12 months old with congenital hearing loss. Material and methods: Results from 250 children were analyzed retrospectively. The study group consisted of children between one and three months old observed between 2015-2016. For the purpose of this analysis we divided the patients into four groups: children with Down Syndrome, children with nervous system disorders , children with cleft palate or both cleft palate and lip and children with congenital CMV. To discuss performed diagnostics and treatment two groups of children were esteblished: • with implementation of appropriate course of treatment • without instituted treatment Results: 250 children were examined in Level III NICUs in the years 2015 – 2016. The highest proportion of children with the implemented course of proceedings, was in groups with children with congenital CMV (71.1%). The highest proportion of children substituted with a hearing prosthesis was observed in the group of children with Down Syndrome. The lowest proportion of children with the implemented course of proceedings, was in groups with children with cleft palate or both cleft palate and lip (41.6%). Conclusions: • Early implementation of treatment and/or rehabilitation in children with hearing disorders is crucial to prevent depression of speech and psychological development. I t is important in children with cleft palate or both cleft palate and lip even if surgical correction is discussed. • The highest proportion of children with the implemented course of proceedings, was in groups with children with congenital CMV. This children should be observed despite of right results of hearing tests. • In children with nervous system disorders 1 year period of observation is too short to exclude problems with hearing.

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Stability of ABR Wave V Threshold in Early Hearing Diagnostics in Children from Selected Groups at Risk of Congenital Hearing Loss

Otolaryngologia Polska ◽

10.5604/01.3001.0014.3322 ◽

2020 ◽

Vol 74 (6) ◽

pp. 1-5

Author(s):

Sebastian Kocoń ◽

Konrad Skórkiewicz ◽

Paweł Stręk ◽

Remigiusz Ziarno ◽

Jacek Składzień ◽

...

Keyword(s):

At Risk ◽

Nervous System ◽

Hearing Loss ◽

Down Syndrome ◽

Cleft Palate ◽

Cleft Lip ◽

Risk Groups ◽

First Year ◽

Children With Down Syndrome ◽

First Year Of Life

Introduction: Upon hearing that the “little” patient has trouble with hearing, we are mostly interested in the level of his hearing threshold. When the child is in the first year of life, results can only be achieved by means of ABR test. Subsequent control tests, especially in children from the hearing loss risk groups selected in this study, show that the obtained outcomes are subject to fluctuations. Their fluctuating nature is manifested by the instability of wave V threshold in subsequent diagnostic periods. Such variability often delays the implementation of the appropriate proceeding. Knowledge of the dissimilarity of behavior of the wave V threshold occurring in individual groups at risk of hearing loss allows for the correct interpretation of the obtained results, and thus, effective therapeutic measures. Aim: The aim of the paper is to analyze the stability of wave V threshold during the first year of life in children from selected risk groups for congenital hearing disorders. Material and methods: From the patient population of 2,114 individuals examined in 2015–2016 at a reference center participating in the Universal Neonatal Hearing Screening Program in 2015–2016, the results of 250 children were subjected to retrospective analysis. Furthermore, 4 groups of little patients were formed (children with Down syndrome; children with other diseases or damage to the nervous system; children with cleft palate or cleft lip and cleft palate; children with congenital cytomegaly) in whom diagnostic practice revealed variable results of the wave V threshold. We analyzed the results of tests obtained during the first year of the child’s life divided into 4 diagnostic periods. Results: The highest percentage of instability in the established threshold of wave V between individual diagnostic periods occurred in the group of children with cleft palate or cleft lip and cleft palate. In the group of children with Down syndrome, it was observed that the instability of the ABR test results decreased over time. In the group of children with other diseases or damage of the nervous system, the highest percentage of the lack of stable ABR wave V thresholds was observed between the 1st and 2nd as well the 1st and 4th diagnostic periods. On the other hand, in the group of children with congenital CMV, there was a relatively low percentage of instability of results. Conclusions: (1) Although the ABR test is a diagnostic standard, in particular groups of patients the study is burdened with high variability of measurement results in subsequent diagnostic periods. Such a group of patients are children with cleft palate or cleft lip and cleft palate; therefore, it must receive particular attention in treatment planning; (2) in selected groups at risk of hearing loss, due to the high percentage of children with hearing impairment (70%), the validity of performing newborn hearing screening tests was confirmed.

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Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life?

European Journal of Cardio-Thoracic Surgery ◽

10.1016/j.ejcts.2004.11.027 ◽

2005 ◽

Vol 27 (3) ◽

pp. 405-409 ◽

Cited By ~ 27

Author(s):

M MASUDA ◽

H KADO ◽

Y TANOUE ◽

K FUKAE ◽

T ONZUKA ◽

...

Keyword(s):

Down Syndrome ◽

Septal Defect ◽

Atrioventricular Septal Defect ◽

Long Term Results ◽

First Year ◽

Complete Atrioventricular Septal Defect ◽

First Year Of Life ◽

Complete Atrioventricular

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D.03 Down syndrome: clinical and EEG correlates during development

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2016.76 ◽

2016 ◽

Vol 43 (S2) ◽

pp. S13-S13

Author(s):

S Nauth ◽

AK Bamaga ◽

MA Cortez

Keyword(s):

Down Syndrome ◽

Cognitive Abilities ◽

Cognitive Outcomes ◽

First Year ◽

Pharmacological Treatments ◽

Chi Square ◽

Significant Difference ◽

Chi Square Analysis ◽

First Year Of Life ◽

Electroencephalogram Eeg

Background: Down syndrome (DS) is the primary genetic cause of mental retardation and seizures are present in an estimated 5-13% of cases. One-third of seizures in DS are infantile spasms (IS). Hypsarrythmia (HS) is the cardinal electroencephalogram (EEG) feature of IS and has been found to affect cognition; however, its effect on DS patients is inconclusively reported. This study assesses the correlation of HS with cognitive outcomes in DS using the largest sample size to date. Methods: Retrospective study of medical records of children with DS [0-18yrs] at SickKids Hospital in Toronto, from 1990-2013. Seizure history, EEG findings, comorbities, and pharmacological treatments were identified. Developmental outcomes were also assessed from physician comments on motor, verbal and cognitive abilities. The cognitive outcomes of DS patients with and without HS were compared. Results: 70 [male=40] patients with DS and seizures were included. Among 31 (44.2%) patients with DS and IS, 27 had HS. Chi-square analysis showed a significant difference [P=0.007] in prevalence of severe developmental delay in patients with IS and HS versus all other seizure types. Conclusions: The developmental outcome of patients with Down syndrome appears to worsen when IS and HS had occurred in the first year of life.

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The Development of Swallowing Respiratory Coordination

Perspectives on Swallowing and Swallowing Disorders (Dysphagia) ◽

10.1044/sasd18.1.19 ◽

2009 ◽

Vol 18 (1) ◽

pp. 19-24

Author(s):

Maggie-Lee Huckabee

Keyword(s):

Cortical Activation ◽

First Year ◽

Healthy Children ◽

Nasal Airflow ◽

Single Session ◽

Primary Finding ◽

Patterns Of Behavior ◽

Cortical Modulation ◽

First Year Of Life ◽

Swallowing Apnea

Abstract Research exists that evaluates the mechanics of swallowing respiratory coordination in healthy children and adults as well and individuals with swallowing impairment. The research program summarized in this article represents a systematic examination of swallowing respiratory coordination across the lifespan as a means of behaviorally investigating mechanisms of cortical modulation. Using time-locked recordings of submental surface electromyography, nasal airflow, and thyroid acoustics, three conditions of swallowing were evaluated in 20 adults in a single session and 10 infants in 10 sessions across the first year of life. The three swallowing conditions were selected to represent a continuum of volitional through nonvolitional swallowing control on the basis of a decreasing level of cortical activation. Our primary finding is that, across the lifespan, brainstem control strongly dictates the duration of swallowing apnea and is heavily involved in organizing the integration of swallowing and respiration, even in very early infancy. However, there is evidence that cortical modulation increases across the first 12 months of life to approximate more adult-like patterns of behavior. This modulation influences primarily conditions of volitional swallowing; sleep and naïve swallows appear to not be easily adapted by cortical regulation. Thus, it is attention, not arousal that engages cortical mechanisms.

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