The child with lip, maxillary, palatal cleft

Clefting of lip, alveolus and palate may occur in multiple variations. It causes aesthetic and functional detractions. Soft palate clefts may result in hearing-, speech- and swallowing-disorders. Therefore the otolaryngologist is a very important member in the interdisciplinary team directory. A cleft-palate child belongs to an interdisciplinary consulting hour in special hospitals, where different medical specialties are involved. Most important for a sufficient medical rehabilitation are maxillofacial surgery, otolaryngology, paediatrics, plastic surgery, speech therapy, psychology and human genetics. Also many other specialties may be involved. The cleft demands a complete follow up from the child's birth until it is grown up. Hearing disorders are caused by eustachian tube disfunction. There is a high prevalence of hearing loss and middle ear diseases in cleft palate patients. Hearing losses due to middle ear effusions in the very young child. Without therapy up to 50% of the cleft palate population will develop chronic middle ear diseases with and without cholesteatomas. Early and consequent therapy with myringotomy and insertion of a tympanostomy ventilation tube is necessary and helps to avoid chronic hearing problems. There is no general accepted system of speech disorders in cleft palate patients because of the difference in shaping of the cleft and rehabilitation development. Essential for speech rehabilitation are an intact velopharyngeal system and a keen sense of hearing. Both of it is disturbed in cleft palate children. Speech disorders are treated by speech therapists with prior consultation of the interdisciplinary team. The author presents a system of primary, secondary and tertiary speech disorders in cleft palate children. Primary speech disorders are caused by faulty velopharyngeal valving, offering in hypernasality, weak plosives, fricatives and affricates. Secondary speech disorders are substitute mechanisms for plosives, nasal and pharyngeal sounds. Tertiary speech disorders are hyper- and hypofunctional dysphonias following primary and secondary speech dysfunctions.     

Small abnormality of the middle ear--a genetically-induced defect?

Conductive and sensorineural hearing losses are of genetic origin in 20% to 60% of cases. In general, genetic abnormalities are more often expressed as a sensorineural hearing loss than as a conductive hearing loss. At present several genes for sensorineural hearing loss have been isolated. The most common genetically transmitted forms for isolated conductive hearing losses are otosclerosis and small malformations of the ossicles. To date no genes responsible for these deformations have been isolated. We present a family with four siblings having conductive hearing losses caused by ossification of the stapedial tendon. This finding is suggestive of an autosomal recessive inheritance. The early diagnosis of an hereditary conductive hearing loss contains the possibility for permitting normal development of speech.     

Consent: between legality and legitimacy or between "formal" and "informed" consent

OBJECTIVE: The purpose of this study was to examine the conversational skills of preschool and school-age children with cleft lip and palate. DESIGN: The children were audio- and videotaped during interactions with an unfamiliar adult. In addition, standardized measures of speech and language were administered, and ratings of resonance were obtained. Comparisons were made between the children with cleft lip and palate and their same-age peers on measures of conversational participation and a standardized test of pragmatic skills. PARTICIPANTS: Participants were 20 children with unilateral cleft lip and palate (10 preschoolers and 10 school-age children) recruited from the Craniofacial Team at Rainbow Babies and Children's Hospital, Cleveland (OH) and 20 noncleft peers matched for gender, age, and socioeconomic status. MAIN OUTCOME MEASURES: Separate comparisons were made for the preschool children with cleft lip and palate and their noncleft peers, and the school-age children with cleft lip and palate and their noncleft peers on eight measures of conversational assertiveness/responsiveness and the standardized tests of pragmatics. Next, each child with cleft lip and palate was classified for level of conversational participation. RESULTS: Paired t tests revealed no significant differences between the preschool and school-age children with cleft lip and palate and their noncleft peers in level of conversational participation. However, individual child comparisons revealed less assertive profiles of conversational participation for 50% of the preschool and 20% of the school-age children with cleft lip and palate. CONCLUSIONS: Children with cleft lip and palate may show a less assertive style of conversational participation, at least during the preschool years. Therefore, craniofacial team evaluations should include examination of conversational competency, particularly for children who are demonstrating difficulty with other aspects of speech, language, or social development.     

Speech characteristics associated with the Furlow palatoplasty as compared with other surgical techniques

Reported here are the results of a retrospective study of the speech outcome for 63 cleft subjects who had Furlow repairs compared with 20 subjects who had other procedures. The two groups of children were similar in cleft type, sex, and race. The same two surgeons repaired the palates in both groups, and the same two speech pathologists with high reliability examined the children at least 5 years postoperatively using the Pittsburgh Weighted Values for Speech Symptoms Associated with VPI (velopharyngeal incompetence). Subjects who had had Furlow repairs were superior on measures of hypernasality, articulation, and total speech scores; and fewer pharyngeal flaps were required by Furlow subjects. These findings suggest the need for randomized, double-blind investigations comparing outcome of the Furlow procedure with the intravelar veloplasty, the V-Y pushback, and other specified procedures.     

Hearing, speech, language, and vestibular disorders in the fetal alcohol syndrome: a literature review

Fetal alcohol syndrome (FAS) is characterized in part by mental impairment, as well as craniofacial and ocular anomalies. These conditions are traditionally associated with childhood hearing disorders, because they all have a common embryonic origin in malformations of the first and second branchial arches, and have similar critical periods of vulnerability to toxic insult. A review of human and animal research indicates that there are four types of hearing disorders associated with FAS. These are: (1) a developmental delay in auditory maturation, (2) sensorineural hearing loss, (3) intermittent conductive hearing loss due to recurrent serous otitis media, and (4) central hearing loss. The auditory and vestibular systems share the same peripheral apparatuses (the inner ear and eighth cranial nerve) and are embryologically and structurally similar. Consequently, vestibular disorders in FAS children might be expected. The evidence for vestibular dysfunction in FAS is ambiguous, however. Like other syndromes associated with craniofacial anomalies, hearing disorders, and mental impairment, FAS is also characterized by a high prevalence of speech and language pathology. Hearing disorders are a form of sensory deprivation. If present during early childhood, they can result in permanent hearing, language, and mental impairment. Early identification and intervention to treat hearing, language, and speech disorders could therefore result in improved outcome for the FAS child. Specific recommendations are made for intervention and future research.     

Experimental studies on the infectivity of Anguillicola crassus third-stage larvae (Nematoda) from paratenic hosts

Speech, language, and communication disorders are prominent reasons for referrals to a child development center. From 1984 to 1988, 1,090 preschool children were referred to our child development center, which serves the Tel Aviv metropolitan area. Of all referrals, 432 (41%) were primarily due to speech, language, and communication problems. After exclusion of those with IQ < 50 and those with non-language-related disabilities, 323 children remained. The children were classified into different subtypes of developmental language disorders and autistic spectrum disorders. The main developmental language disorder subtypes were combined expressive-receptive (49%) and expressive (44%). Central processing deficits were less common, occurring in 20 (7%) of the children. Parents of children with developmental language disorders had educational levels similar to those of parents of children referred to the child development center for other causes. However, parents of children with infantile autism had higher educational levels than parents of children with developmental language disorder or parents of children referred for other causes (P < .001). Our results reflect the distribution of language and related problems in an unselected population of preschool children referred to a child development center.     

The assembly of Ni2+-actin: some peculiarities

Cochlear implants have been very successful in restoring partial hearing to profoundly deaf people. Many individuals with implants are now able to communicate and understand speech without lip-reading, and some are able to talk over the phone. Children with implants can develop spoken-language skills and attend normal schools (i.e., schools with normal-hearing children). The greatest benefits with cochlear implantation have occurred in patients who (1) acquired speech and language before their hearing loss, and (2) have shorter duration of deafness. Gradual, but steady, improvements in speech production and speech perception have also occurred in prelingually deafened adults or children.     

Subgrouping children with familial phonologic disorders

Familial aggregation of speech and language disorders was examined as a basis of subgrouping children with phonologic disorders. Fifty-nine children with phonologic disorders were subgrouped according to whether or not other nuclear family members reported a history of speech/language disorders. Thirty-four subjects (58%) reported at least one other nuclear family member affected and 25 subjects (42%) reported no other nuclear family members affected. Groups were compared on measures of articulation, phonology, language, and oral motor skills to determine if the familial phonologic subgroup presented a unique profile of speech and language deficits. Significant group differences were not observed. However, children with positive nuclear family histories tended to perform more poorly than children without histories on all tasks, although not reaching significance. Although all parents were considered to have achieved normal adult articulation, parents of children with positive family histories also tended to perform more poorly than parents of children with negative histories. Results suggested that poorer oral motor coordination and productive phonology may distinguish individuals with familial phonologic disorders from individuals with phonologic disorders of unknown origin.     

Infants' early ability to segment the conversational speech signal predicts later language development: A retrospective analysis.

Two studies examined relationships between infants' early speech processing performance and later language and cognitive outcomes. Study 1 found that performance on speech segmentation tasks before 12 months of age related to expressive vocabulary at 24 months. However, performance on other tasks was not related to 2-year vocabulary. Study 2 assessed linguistic and cognitive skills at 4-6 years of age for children who had participated in segmentation studies as infants. Children who had been able to segment words from fluent speech scored higher on language measures, but not general IQ, as preschoolers. Results suggest that speech segmentation ability is an important prerequisite for successful language development, and they offer potential for developing measures to detect language impairment at an earlier age. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Communication between deaf children and their hearing mothers: the role of language, gesture, and vocalizations

In the present longitudinal study, 20 deaf and 20 hearing children were observed during free play with their hearing mothers when the children were 22 months and 3 years of age. Compared to hearing children, deaf children were severely language delayed, with deaf 3-year-olds using less language (speech or sign) than hearing 22-month-olds. Deaf children communicated primarily through nonlinguistic vocalizations, with increasing use of gesture from 22 months to 3 years of age. Although mothers of deaf children used more visual communication than mothers of hearing children, they still primarily communicated through speech. In addition, deaf children did not visually attend to much of their mothers' communication. Therefore, deaf children received much less communication than hearing children. These results suggest that intervention efforts should be focused on increasing the quantity of perceived linguistic input by the child.     

A modification of play audiometry to assess speech discrimination ability in severe-profoundly deaf 2- to 4-year-old children

OBJECTIVE: The aim was to develop an assessment procedure that was independent of language and speech production ability, to test speech feature discrimination in severe-profoundly deaf children 2 to 4 yr of age. DESIGN: The procedure being trialed was adapted from existing procedures. The child was required to respond with a game-like motor response to a "change" in a speech stimulus that was being presented repeatedly through a speaker. The change occurred at randomly determined times, and false alarm responses were measured during the waiting periods (while the child waited for the change). Two- to four-yr-old normally hearing children and hearing-impaired children using hearing aids and a group of 4-yr-old hearing-impaired children using cochlear implants were assessed on the task. RESULTS: More than 82% of the 3- and 4-yr-old normally hearing and hearing-impaired children were able to complete the testing for the eight speech sound contrasts within three 20 minute sessions. Fifty percent of the 2-yr-old normally hearing and hearing-impaired children were able to condition and complete the task. All of the normally hearing children who completed the task successfully discriminated all speech sound contrasts. The performance of the hearing-impaired children using hearing aids was influenced by the degree of hearing loss and the type of speech contrast being tested. Similarly, the average performance of the children using cochlear implants was better for easier contrasts such as /ba/bi/ with contrasting vowel formant cues. CONCLUSIONS: This procedure has potential for use as a reliable clinical and research tool for assessing the development of auditory discrimination ability in 2- to 4-yr-old severe-profoundly deaf children.     

Deficits in auditory temporal and spectral resolution in language-impaired children

Between 3 and 6 per cent of children who are otherwise unimpaired have extreme difficulties producing and understanding spoken language. This disorder is typically labelled specific language impairment. Children diagnosed with specific language impairment often have accompanying reading difficulties (dyslexia), but not all children with reading difficulties have specific language impairment. Some researchers claim that language impairment arises from failures specific to language or cognitive processing. Others hold that language impairment results from a more elemental problem that makes affected children unable to hear the acoustic distinctions among successive brief sounds in speech. Here we report the results of psychophysical tests employing simple tones and noises showing that children with specific language impairment have severe auditory perceptual deficits for brief but not long tones in particular sound contexts. Our data support the view that language difficulties result from problems in auditory perception, and provide further information about the nature of these perceptual problems that should contribute to improving the diagnosis and treatment of language impairment and related disorders.     

Establishing the etiology of childhood hearing loss

The cause of hearing loss in children is often difficult to identify. We evaluated a cohort of 114 children (47 boys, 67 girls) referred with newly diagnosed hearing loss (non-otitis media) to identify factors predictive of etiology and type of hearing loss. Clinical (history and physical examination), laboratory, and radiographic data were collected. One hundred children (87.7%) had sensorineural hearing loss, and 14 (12.3%) had conductive or mixed hearing loss. The cause of hearing loss was identified in 54 children (48%). Patients with isolated aural atresia (n = 7) or with a known diagnosis of congenital cytomegalovirus infection (n = 21) were excluded from further data analysis. We conducted statistical analysis to identify factors predictive of the cause and type of hearing loss. Clinical factors that aided in identifying a cause included abnormal physical examination findings (p = 0.001) and craniofacial anomalies (p = 0.006). Computed tomography of the temporal bones was the only diagnostic test predictive of cause (p < 0.001). Factors predictive of the type of hearing loss detected (sensorineural vs. conductive or mixed) were abnormal physical examination findings (p = 0.01) and craniofacial anomalies (p = 0.004). An exhaustive laboratory or radiographic workup did not prove beneficial in identifying the etiology of hearing loss in our series.     

Mild hearing loss in children with communication disorders

The prevalence of MHL in 202, 1 to 6 year-old children with communication disorders who visited our clinic in 1991 was investigated. 1) 31% of the subjects had MHL bilaterally. The prevalence of MHL was 44% at age 1 year, 20% at age 2 years, 36% at age 3 years, 24% at age 4 years, 39% at age 5 years, 33% at age 6 years. 2) 88% of children with MHL had OME, 10% had mild sensorineural hearing loss, and 2% had cerminous plug. 3) The prevalence of MHL in children with mental retardation and autistic disorders was 9%, that with stuttering was 9%, that of OME accompanied by moderate and severe hearing disorders was 6%. 4) The primary causes in 191, except for those with stuttering, were as follows; the prevalence of MHL was 30%, that of mental retardation and autistic disorders was 24%, and that of articulation disorders was 28%. 5) On the other hand, the prevalence of MHL in children with retarded language development and articulation disorders was 30%, which was significantly higher than that of the other communication disorders. Accordingly, the results of this study suggest that MHL in early childhood greatly influences communication disorders.     

Communication of oral deaf and normally hearing children at 36 months of age

Eighteen orally educated deaf and 18 normally hearing 36-month-old children were observed in a play session with their mother. Communicative behavior of the child was coded for modality and communicative function. Although the oral deaf children used a normal range of functions, both the quantity and proportions differed from normally hearing children. Whereas the normally hearing 3-year-olds used speech almost exclusively, the deaf children exhibited about equal use of speech, vocalizations, and gestures. Spoken language scores of the deaf children at 5 years of age were best predicted by (a) more frequent use of speech at age 36 months, (b) more frequent use of the Statement function, and (c) relatively infrequent use of the Directive function. It is suggested that some communicative functions are more informative or heuristic than others, and that the early use of these functions is most likely to predict later language competence.     

Oro-nasal fistula development and velopharyngeal insufficiency following primary cleft palate surgery—an audit of 148 children born between 1985 and 1997

We present an audit of primary cleft palate surgery in our unit including rates of two important post-operative complications.Multidisciplinary audit clinics ran from March 1998 to April 2002 to follow up all local patients with a cleft lip or palate who had undergone primary palatal surgery in our unit. One hundred and forty eight patients were studied. Patient ages at follow-up ranged from 3 years and 10 months to 17 years and 4 months. Two surgeons performed the primary surgery. One hundred and twenty eight Wardill-Kilner and 20 Von Langenbeck repairs were performed.We found a 4.7% rate of oro-nasal fistula development requiring surgical closure, and a 26.4% rate of velopharyngeal insufficiency (VPI) requiring subsequent pharyngoplasty. We noted that the type of cleft involved affected the rate of VPI, 16% of patients with unilateral cleft lip and palate versus 29.2% of patients with a solitary cleft palate requiring secondary surgery.Outcome of surgery was determined by a ‘Cleft Audit Protocol for Speech’ (CAPS) speech therapy assessment at follow-up clinics. Only 14.9% of all patients assessed demonstrated any degree of hypernasality.Our results compare favourably with other recent studies including the Clinical Standards Advisory Group (CSAG) report into treatment of children with cleft lip and palate.     

Acquisition of linguistic and cognitive skills by children with cleft palate

This study compared the early cognitive and linguistic development of young children with cleft palate (N = 28) to that of noncleft children (N = 29). Measures included the Mental scale of the Bayley Scales of Infant Development, the Minnesota Child Development Inventory, Mean Length of Utterance, and words acquired by 24 months. Children with cleft palate, although well within the normal range, performed significantly below the children in the control group on the Mental Scale of the Bayley Scales of Infant Development, some subscales of the Minnesota Child Development Inventory, and words acquired by 24 months. Differences observed in the cognitive development of children with and without cleft palate were verbal as opposed to nonverbal (i.e., linguistic in nature) and were related to hearing status at 12 months and velopharyngeal adequacy.     

Variability in deaf children's spelling: The effect of language experience.

French-speaking hearing and deaf children, ranging in age from 6 years 10 months to 14 years 7 months were required to spell words including phoneme-to-grapheme correspondences that were either statistically dominant or nondominant. Of interest was whether the nature of linguistic experience (cued speech vs. sign language) and the precocity of such experience (early vs. late exposure) determines accuracy in the use of phoneme-to-grapheme knowledge. Cued speech is a system delivering phonemically augmented speechreading through the visual modality. Hearing and deaf children exposed to cued speech early at home relied on accurate phoneme-to-grapheme correspondences, whereas children exposed to cued speech later and at school only, and children exposed to sign language, did not. A critical factor in the development of the phonological route for spelling seems to be early and intensive exposure to a system making all phonological distinctions easily perceivable. (PsycINFO Database Record (c) 2010 APA, all rights reserved)