These guidelines are designed to assist the pediatrician in caring for the child in whom a diagnosis of Down syndrome has been confirmed by chromosome analysis. Although a pediatrician's initial contact with the child is usually during infancy, occasionally the pregnant woman who has been given a prenatal diagnosis of Down syndrome will be referred for review of the condition and the genetic counseling provided. Therefore, this report offers guidance for this situation as well.

Health Supervision for Children With Down Syndrome

Steady-state evoked potentials can be recorded from the human scalp in response to auditory stimuli presented at rates between 1 and 200 Hz or by periodic modulations of the amplitude and/or frequency of a continuous tone. Responses can be objectively detected using frequency-based analyses. In waking subjects, the responses are particularly prominent at rates near 40 Hz. Responses evoked by more rapidly presented stimuli are less affected by changes in arousal and can be evoked by multiple simultaneous stimuli without significant loss of amplitude. Response amplitude increases as the depth of modulation or the intensity increases. The phase delay of the response increases as the intensity or the carrier frequency decreases. Auditory steady-state responses are generated throughout the auditory nervous system, with cortical regions contributing more than brainstem generators to responses at lower modulation frequencies. These responses are useful for objectively evaluating auditory thresholds, assessing suprathreshold hearing, and monitoring the state of arousal during anesthesia.

Human auditory steady-state responses.

Auditory selective attention is an important mechanism for top-down selection of the vast amount of auditory information our perceptual system is exposed to. In the present study, the impact of attention on auditory steady-state responses is investigated. This issue is still a matter of debate and recent findings point to a complex pattern of attentional effects on the auditory steady state response (aSSR). The present study aimed at shedding light on the involvement of ipsilateral and contralateral activations to the attended sound taking into account hemispheric differences and a possible dependency on modulation frequency. In aid of this, a dichotic listening experiment was designed using amplitude-modulated tones that were presented to the left and right ear simultaneously. Participants had to detect target tones in a cued ear while their brain activity was assessed using MEG. Thereby, a modulation of the aSSR by attention could be revealed, interestingly restricted to the left hemisphere and 20 Hz responses: contralateral activations were enhanced while ipsilateral activations turned out to be reduced. Thus, our findings support and extend recent findings, showing that auditory attention can influence the aSSR, but only under specific circumstances and in a complex pattern regarding the different effects for ipsilateral and contralateral activations.

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Top-down modulation of the auditory steady-state response in a task-switch paradigm.

Conclusion: The results suggest that auditory neuropathy is more common in the infant population than previously suspected. The effects of neuropathy on auditory function appear to be idiosyncratic, producing significant variations in both the detection and discrimination of auditory signals. As such, the management of children with this disorder must allow for individual differences.

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Clinical Findings for a Group of Infants and Young Children with Auditory Neuropathy

Nasal mucociliary clearance as a factor in nasal drug delivery

The authors investigated the 40-Hz steady-state responses (SSR) in 32 full-term newborns and in 10 normal children (5-8 years old), using 500-Hz tone bursts. The 40-Hz SSR threshold is located at about 50 and 30 dB nHL in newborns and older children, respectively. The latencies of both P1 and N1 waves decreased significantly with age, while the amplitudes increased. No significant latency and amplitude intersex differences have been observed. Moreover, with age, the 40-Hz SSR became more stable, their test-retest replicability improved, and P1-N1 wave occurrence increased. The authors finally discuss the possible underlying mechanisms of these findings and conclude that the 40-Hz SSR are difficult to obtain and are scarcely reliable in defining the low-frequency threshold in newborns. The stability and reliability of the responses increase with age, and the electrophysiological and behavioral thresholds to low-frequency stimuli tend to overlap.

40-Hz Steady-State Responses in Newborns and in Children

Pulmonary function of 31 heavy smokers with laryngeal cancer was evaluated before and during the 1st year after total (n = 21) and conservative (n = 10) laryngectomy. 2 of them died because of recurrences, 1 for bronchopulmonary complications. Long-lasting hoarseness was the only presenting symptom in all patients. Preoperative lung function data and mucociliary clearance were consistent with a coexisting chronic obstructive lung disease in most subjects and was probably due to smoking. No differences were observed comparing pre- and postoperative data in the 10 conservative laryngectomy patients. On the contrary, the total-laryngectomy patients showed a progressive impairment of bronchial obstruction and bacteriological infection of the trachea during the 1st year after the operation. An impressive increase in mucociliary clearance rates has been observed 2 months after total laryngectomy during the postoperative hypersecretory phase. The obtained data allow us to hypothesize that when clinical conditions of laryngectomized patients in whom local or distant recurrences have been excluded deteriorate, this is related to a progressive bronchial obstruction at any level of the bronchial tree due to descending bacterial infection of the airways. To our knowledge this is the only work demonstrating that total laryngectomized patients need a complete pre- and postoperative evaluation of lung function, airway dynamics, mucociliary function and tracheal bacteriology for long-term prognosis and treatment.

Laryngeal cancer: long-term follow-up of respiratory functions after laryngectomy

Auditory brain-stem (ABRs) and middle latency auditory responses (MLRs) in the prognosis of severely head-injured patients.

Forty heavy smokers, all males aged between 40 and 70 and affected by laryngeal cancer underwent mucociliary clearance evaluation the day before total laryngectomy, 60 days after and, in 6 of them, 5 years later. Specimens of nasal and tracheal mucosa were obtained during laryngectomy and other subsequent operations. Before total laryngectomy, no significant changes in nasal mucociliary clearance were observed in smokers and controls, and the nasal ciliary carpet was fairly well preserved. Bronchial mucociliary clearance was impaired in all patients, owing to the coexistent chronic obstructive bronchitis. 60 days after the operation, nasal mucociliary clearance was significantly improved when compared with the preoperative data and controls, owing to the increase in the endonasal temperature and humidity, and to the reduction of the nasal blood flow and disappearance of the nasal cycle, which follow tracheostomy. Surface morphologic studies show a change in the squamous epithelium of the anterior third of the nasal fossa into a columnar ciliated one. During the first 3 months after the operation, tracheobronchial mucociliary clearance increased of 50% vis-a-vis the preoperative data. During this period a clinically evident bronchial hypersecretion was observed. The reduction in nasal and tracheobronchial mucociliary clearance function which became evident 6 years after the operation, is probably due to secondary chronic infections.

Mucociliary Clearance and Mucosal Surface Characteristics before and after Total Laryngectomy

86 male subjects, aged between 60 and 86 years (M = 69.5), underwent pure-tone audiometry, impedance tests and brainstem response audiometry (BRA). Subjects have been classified into four age-related groups: 1) 34 subjects, aged between 60 and 65; 2) 22, aged between 66 and 70; 3) 22 aged between 71 and 75; 4) 8, aged between 76 and 86. They have also been classified into four groups on the basis of their mean auditory threshold at 0.5, 1, 2, 4 and 8 kHz: 1) 14 subjects with mean auditory threshold less than or equal to 30 dB HL; 2) 16, between 31 and 40; 3) 24, between 41 and 50; 4) 32, with mean auditory threshold greater than or equal to 51 dB HL. 19 normally hearing adults, aged between 28 and 42, were chosen as controls. Statistically significant correlations have been found between age and mean auditory threshold (P less than or equal to 0.001), between wave latency V and age (P less than or equal to 0.001), between wave latency V and the mean pure-tone auditory threshold at 0.5, 1, 2, 4 and 8 kHz (P less than or equal to 0.001) and between the V-I interval and age (P less than or equal to 0.001). The discrepancy between the mean auditory threshold and the ABR waveform, the overall amplitude's reduction of ABR waves and the progressive lengthening of V-I interval values, seem to indicate that age-related changes involve not only the end organ but also the brainstem auditory structures.

G. Paludetti

Papers

40-Hz Steady-State Responses in Newborns and in Children

Laryngeal cancer: long-term follow-up of respiratory functions after laryngectomy

Auditory brain-stem (ABRs) and middle latency auditory responses (MLRs) in the prognosis of severely head-injured patients.

Mucociliary Clearance and Mucosal Surface Characteristics before and after Total Laryngectomy

Auditory brainstem responses (ABR) in the aged.