学龄前儿童听力筛查

" 儿童中度至极重度(>40dBHL)听力损失(包括感音神经性听力损失和传导性听力损失)的发病率为1～6/1000[1,2,3],其中10%的儿童是极重度的感音神经性听力损失。儿童的听力损失在多大程度上影响其言语、语言、心理、学习和社交等方面发展,取决于听力损失发生的时间,持续的时间和严重程度。如果听力损失能够早期确诊,那么由于听力损失导致的相关问题就能够预防和改善。对儿童进行大规模听力筛查是基于二级预防的概念开展的[4]。认识到早期诊断和早期干预儿童先天和获得性听力障碍的重要性,很多国家都在开展听力筛查项目[5]。为了避免新生儿先…     

Hearing function in children with speech retardation

Hearing function was studied in 140 children aged between 2 and 5 years with speech retardation and perinatal pathology for formulation of further treatment policy and rehabilitation. Impedance audiometry, SEAAE, game audiometry identified hearing loss of the first, second, third, forth degree in 6 (4%), 10 (7%), 24 (17%), 31 (21%) children respectively. Deafness was registered in 17 (12%) children, 52 (37%) examinees were audiologically normal.     

Hearing assessment with young children.

This study evaluates the efficacy and reliability of auditory stimulus-response control training and assessment procedures with young children (nine to 25 months). The problems encountered in training stimulus-response control are discussed. Results indicate that reliable interest data can be obtained from young children using stimulus-response control procedures.     

儿童人工耳蜗植入后的听觉言语康复

植入人工耳蜗为听力障碍儿童感知声音、重建听力奠定了良好基础，但是要使儿童在人工耳蜗植入后获得听觉言语康复必须经过长期的听觉培建，语言学习和语音异常矫治。     

Variability and sequential order of consonant normalization in children with speech delay.

A source of discrepancy among attempts to establish a rank order in the development of English consonants has been the inability to cope concurrently with principled generalization and individual variability. This problem may be surmounted by survival analytic techniques. From the conversational speech of 24 children with speech delay (SD) recorded over 2 years, a Kaplan-Meier (KM) survival analysis provided a rank order of acquisition for all probabilities above .75. The rank ordering by chronological age uncovered an alignment by place and manner of articulation comparable to, but not identical with, the predictions of Jakobson (1941, 1968). When the derived normalization probabilities for the speech delayed children were lagged according to the strong delay hypothesis (Shriberg, Gruber, & Kwiatkowski, 1994), they were shown to converge with previous normative studies and the age of speech-sound determinations (Prather, Hedrick, & Kern, 1975; Sander, 1972). The convergence of results is interpreted both as an argument for generalizability and as support for the strong delay hypothesis.     

Probability estimates and paths to consonant normalization in children with speech delay.

Probable ages of normalization were calculated for 24 children with speech delay, using Kaplan-Meier analysis and a threshold score of 85% on the Percent of Consonants Correct, Percent of Consonants Correct-Adjusted, and Percent of Consonants Correct-Revised metrics. Simple formulas are provided that permit calculation of the likelihood that individual children with speech delay will normalize by a given age. The sex of a child was found to have no significant influence on age of normalization. Analysis revealed two different paths to normalization. In Path A, errors of deletion, substitution, and omission declined as correct productions increased. In Path B, common clinical distortions increased as deletions and substitutions decreased. The ages of more and less rapid phonological gain correspond to and partially explain the findings of Shriberg, Gruber, and Kwiatkowski (1994), who studied more severely involved children. Children who follow Path B are those who retain residual errors in their speech.     

Prevalence of speech delay in 6-year-old children and comorbidity with language impairment.

We estimate the prevalence of speech delay (L.D. Shriberg, D. Austin, B. A. Lewis, J. L. McSweeny, & D. L. Wilson, 1997b) in the United States on the basis of findings from a demographically representative population subsample of 1,328 monolingual English-speaking 6-year-old children. All children's speech and language had been previously assessed in the "Epidemiology of Specific Language Impairment" project (see J. B. Tomblin et al., 1997), which screened 7,218 children in stratified cluster samples within 3 population centers in the upper Midwest. To assess articulation, the Word Articulation subtest of the Test of Language Development-2: Primary (Newcomer & Hammill, 1988) was administered to each of the 1,328 children, and conversational speech samples were obtained for a subsample of 303 (23%) children. The 6 primary findings are as follows: (a) The prevalence of speech delay in 6-year-old children was 3.8%; (b) speech delay was approximately 1.5 times more prevalent in boys (4.5%) than girls (3.1%); (c) cross-tabulations by sex, residential strata, and racial/cultural backgrounds yielded prevalence rates for speech delay ranging from 0% to approximately 9%; (d) comorbidity of speech delay and language impairment was 1.3%, 0.51% with Specific Language Impairment (SLI); (e) approximately 11-15% of children with persisting speech delay had SLI; and (f) approximately 5-8% of children with persisting SLI had speech delay. Discussion includes implications of findings for speech-language phenotyping in genetics studies.     

The continuity of babble and speech in children with specific expressive language delay.

A natural language sample of babble and words was obtained for 37 two-year-olds with severe specific expressive language delay. Variables derived from this sample were used to predict individual differences in expressive language scores 5 months later. The rate of word use was positively related to language outcome, whereas rate of vowel babble was negatively related to outcome. Together, these two variables accounted for 41% of the variance in language outcome test scores. The addition of one nonlinguistic variable, a measure of behavior problems, allowed the prediction equation to account for over 50% of the variance in expressive language outcome. The single strongest correlate of language outcome was the proportion of consonantal to vowel babble. The degree of social responsiveness of babble and the length of babble were not related to later language scores. These findings indicate that for children with specific expressive language delay, vowel babble competes with expressive language, consonantal babble facilitates expressive language, and the length and social responsiveness of babble are independent of expressive language. The continuity between babble and speech is multidimensional and multidirectional.     

Longitudinal changes in articulation rate and phonetic phrase length in children with speech delay.

This study examined long-term changes in articulation rate (the pace at which speech segments are produced) and phonetic phrase length in the conversational speech of two groups of children with speech delay (SD) of unknown origin. Initial testing for both groups occurred at preschool age, with follow-up testing conducted for the Early Follow-Up Group (n = 17) at age 9 years and for the Late Follow-Up Group (n = 36) at age 12-16 years. At follow-up testing both groups produced significantly faster articulation rates (measured in both syllables per second and phones per second) and significantly longer phonetic phrases (measured in both syllables and phones) than at initial testing. Articulation rates at both test times were also judged to be similar to published values from typically developing children of similar ages when measured in syllables per second. However, findings for rate in phones per second suggested that at least at initial testing the children were articulating speech at a slower rate than their typically developing peers. This latter finding, however, may have been an artifact of the high frequency of errors--such as cluster reduction and final consonant deletion--observed in the initial samples. It would appear, therefore, that children with SD of unknown origin may start out with slower than normal articulation rates but eventually catch up to their typically developing peers.     

Rehabilitation and assessment of aural-oral speech development in children with cochlear implants

Updated models of cochlear implants provide good speech audibility and thus complete rehabilitation of children who have lost hearing after learning speech. All the children who lost hearing before learning speech can hear sounds of normal loudness and orient in sound media by means of cochlear implant. However, they need long-term audio-vocal rehabilitation the results of which depend on the age of the child's operation and hi(her) individual traits. Cochlear implants in children aged under 3 years are most perspective. Russian language methodology including 7 tests and 2 questionnaires is described. It is intended for assessment of audio-vocal development in children with cochlear implants and results of audio-vocal rehabilitation as well as of effectiveness of using cochlear implant in children over 2 years of age. Establishment of centers for cochlear implantation and introduction of cochlear implantation state program are recommended.     

Acoustic rhinometry in pre-school children

Acoustic rhinometry was performed in 35 normal nose-breathing children between 3 and 6 years. The average cross-sectional areas at the nasal valve, at the anterior end of the turbinates, and in the nasopharynx were 0.34±0.06 cm², 0.35±0.08 cm² and 1.37±0.48 cm² respectively. The average minimal cross-sectional area was 0.29±0.06 cm². The minimal cross-sectional area was located at the nasal valve in 14 and at the anterior end of nasal tubinates in 21 of the 35 children. As would be expected, the cross-sectional areas at different sites of the nasal cavity increased with increasing age of the children. But, whereas the minimal cross-sectional area increased by 0.024 cm² per year, the nasopharyngeal cross-sectional area increased by 0.20 cm² per year. No significant differences were found between boys and girls. Measurements of the posterior nasal and nasopharyngeal cross-sectional areas were unreliable, whenever the minimal cross-sectional area was less than 0.2 cm². Furthermore, assessment of the nasopharynx may be difficult because of involuntary movements of the soft palate.     

试论言语功能评估的标准及方法

本文旨在讨论言语功能评估标准及方法，阐述言语功能评估的一个基本理念：夯实基础，循序渐进，螺旋上升，即言语评估、测量与矫治是一个动态的过程。言语矫治以言语评估和测量为起点，在言语矫治中或训练一个阶段后，应再次进行言语评估和测量，从而监控言语矫治方案的有效性，调整言语矫治方案或提出更高的言语矫治目标。     

Hearing and speech assessment of cleft palate patients after palatal closure. Long-term results

OBJECTIVE: The goal of this study was the development of a clinical methodology to assess speech and hearing impairment 5 years after the primary surgical repair of the cleft and, further, to determine the relative importance and long-term consequences of each cleft type and age to the velopharyngeal and eustachian tube function in patients who did not undergo pharyngeal flap surgery following primary palatoplasty. MATERIALS AND METHODS: We evaluated with a certain assessment protocol hearing and speech abilities of 42 patients between 5 and 15 years of age: 9 with CP (cleft of the soft and hard palate), 19 with unilateral cleft lip and palate (UCLP), 14 with bilateral cleft lip and palate (BCLP), surgically treated by a team of surgeons using two different surgical techniques between 18 and 24 months of age. ACCORDING TO OUR RESULTS WE WERE ABLE TO EVALUATE: (a) the impact of hearing impairment to the development of speech in these patients. (b) The relation of hypernasality with compansatory articulation. (c) The influence of cleft type, by means of extent of the cleft palatal musculature, to speech integrity. RESULTS: Sixty-nine percent of our patients presented with mild and moderate hearing loss. Hypernasality was observed in 40.5%, compensatory articulation in 28.5% of our patients. CONCLUSIONS: Our findings indicated: (a) a simultaneous appearance of speech and hearing impairment at the same age for each cleft type post-surgically in our patients; (b) that the muscular and vomer complex rather than the anatomic extent of the cleft is a significant factor for speech outcome after surgical repair; and (c) that hypernasality is exacerbated by compensatory articulation.     

Hearing disorders in children with Down's syndrome

Among 4947 children in an outpatients unit for hearing disorders 102 children with Down's syndrome were seen and checked for hearing disorders. 57 had hearing deficiencies, 50 (88 %) conductive hearing loss, 4 (7 %) combined and 3 (5 %) a sensory neural hearing loss. Compared to other publications the number of very young children was very high. 32 patients under two years of age had a hearing disorder. The results underline the necessity of early diagnosis and follow up also in children with normal reactions during the first presentation. Early diagnosis enables early treatment, conservative, surgical or fitting with hearing aids, especially important in the rehabilitation of these children. Hearing aids may be given temporarily imploring communication during the development of the child.     

Hearing loss in children with mitochondrial disorders

Objective

At least 1–5 children per 1000 suffer from congenital hearing loss, and 50% of these cases can be attributed to genetic causes. It has been estimated that 1% of pre-lingual hearing loss is due to mutations in mitochondrial DNA. Previous literature reports audiometric data for few patients, usually less than 20 per study. The goal of this study was to characterize the hearing loss associated with mitochondrial mutations and determine whether previously characterized patterns of hearing loss in these patients (progressive, sensorineural, high frequency losses) are found in our population as well.

Methods

An IRB-approved retrospective chart review of the electronic medical records in the Nemours/Alfred I. dupont Hospital for Children system from January 2004 to October 2009 (a five-year period) was undertaken using ICD-9 codes 277.87 (mitochondrial disorder) and 359.89BA (mitochondrial myopathy). These 149 records were then evaluated for audiologic data, resulting in 26 charts with both a mitochondrial disorder and hearing evaluation.

Results

Of 26 patients with known mitochondrial disorders and audiometric documentation, 15 (58%) had hearing loss, and 11 patients had normal hearing (42%). Ten patients had sensorineural hearing loss (38%), two patients had conductive hearing loss (7.7%), one patient had a mixed hearing loss (3.8%), and two patients had an as yet undefined hearing loss (ABR had not yet been performed at the time of this study) (7.7%).

Conclusion

In comparison with previous studies, generally including less than 20 patients, this is one of the largest collections of audiometric data on children with mitochondrial disorders. Unlike prior studies describing a progressive, sensorineural loss across all frequencies or mainly affecting high frequencies, the hearing loss in our patients was more variable including low frequency losses, mid-frequency losses, and conductive losses and was often not progressive or even improved. Our overall 38% rate of sensorineural hearing loss correlates well with previous case series; this study clearly justifies the use of routine audiometric screening in children with mitochondrial disorders, including use of ABR and OAEs as ASND can be seen in this population, as well as repeat testing over time to evaluate for progression.