Is deafness a disease of poverty? The association between socio-economic deprivation and congenital hearing impairment

An association between congenital hearing impairment and deprivation has been suggested, but evidence is limited. We studied children born in Greater Glasgow, 1985-94, with bilateral congenital hearing impairment. The children were divided into seven deprivation categories using the Carstairs Deprivation Index. One hundred and twenty-four hearing-impaired children were born over the study period, an incidence of 1.18/1000 live births. There was a clear association between deprivation category and incidence, ranging from 0.47/1000 to 1.72/1000. An association with deprivation was seen for children with a family history and perinatal problems (such as prematurity and low birth weight). No association was found for other aetiological groups such as craniofacial syndromes or early postnatal infection. Deprivation had no effect on age of diagnosis or hearing aid provision. Deprivation is associated with congenital hearing impairment, due to more prematurity and low birth weight in deprived families, and the fact that families with many hearing-impaired members are economically disadvantaged. There should be a strong emphasis on the needs of the socio-economically disadvantaged when planning services for hearing-impaired children.     

Epidemiology of permanent childhood hearing impairment in the Tyrol, 1980-94.

In recent years the need for reliable data on permanent childhood hearing impairment (PCHI) has increased, owing to both scientific interest and practical aspects such as implementation of neonatal hearing screening. In order to obtain data about the epidemiology of PCHI in the Austrian state of Tyrol, the medical records of all hearing-impaired Tyrolean children born between 1980 and 1994 were researched. A total of 165 children with at least moderate hearing impairment in the better ear were registered, of whom 52.7% were found to be moderately hearing-impaired, 24.2% severely hearing-impaired and 23.0% profoundly hearing-impaired. Sensorineural hearing loss was observed in 87.3%, conductive hearing loss in 6.7% and mixed hearing loss in 6.1%. As for aetiology, non-syndromic hereditary hearing loss was diagnosed in 22% of the children and syndromic hearing loss in 8%. Congenital malformation of the ear was found in 3%. In 19% of the children, perinatal risk factors were seen, and in 10% a pre-, peri- or postnatal infection had occurred. Aetiology remained unknown in 36% of the children. The prevalence rate of newborn hearing impairment was 1.27/1000 newborns. The results are considered to fit well to the PCHI findings reported from other European regions.     

Auditory sensory memory and working memory processes in children with normal hearing and cochlear implants

There can be wide variation in the level of oral/aural language ability that prelingually hearing-impaired children develop after cochlear implantation. Automatic perceptual processing mechanisms have come under increasing scrutiny in attempts to explain this variation. Using mismatch negativity methods, this study explored associations between auditory sensory memory mechanisms and verbal working memory function in children with cochlear implants and a group of hearing controls of similar age. Whilst clear relationships were observed in the hearing children between mismatch activation and working memory measures, this association appeared to be disrupted in the implant children. These findings would fit with the proposal that early auditory deprivation and a degraded auditory signal can cause changes in the processes underpinning the development of oral/aural language skills in prelingually hearing-impaired children with cochlear implants and thus alter their developmental trajectory.     

Is visual perception of hearing-impaired children different from healthy children?

OBJECTIVE: The purpose of this study was to evaluate visual perception of hearing-impaired children, and to determine their insufficiency in rehabilitation programs. METHODS: Forty children with hearing impairment aged 8-10 years were evaluated, and were compared with age matched 40 healthy children. Children having 71 dB and over sensorineural auditory impairment in both ears were included in this study. Figure-ground perception, position in space, and design copying tests were used to evaluate the visual perception of the subjects (Ayres Southern California Sensorial Integration tests). RESULTS: The mean hearing impairment level was 95.5 +/- 13.86 dB for the right ear, and 92.25 +/- 14.3 dB for the left. There were no significant differences by mean of age, height, and body weight between the groups (P > 0.05). All of the test scores of the control group were significantly higher than those of hearing-impaired children (P < 0.05). However there was no significant difference in the completion time of the design copying test between the groups (P > 0.05). CONCLUSIONS: Motivation insufficiency and learning difficulty may be developed in hearing-impaired children depending on the communication problems. The result of this study may bring light into literature about the development of new assessment techniques, and proper rehabilitation programmes for hearing-impaired children or adults in different age groups.     

Genetics and molecular biology of deafness. Update.

This article discusses the latest research in the molecular biology and genetics of hearing impairment and its importance to otolaryngologists. Recent research has led to the discovery of many of the genes and gene products that are responsible for hereditary hearing impairment. State mandated screening of newborn infants for hearing loss ensures that a large number of hearing-impaired children will be detected at a very early age. Additionally, these children often will be referred to the otolaryngologist for evaluation of the hearing impairment. It is the otolaryngologist who must gather a detailed family history and perform a thorough physical examination to fully assess the cause of the hearing impairment. In taking the family history, it is important to note that the diagnosis of a hereditary hearing impairment often involves the evaluation of a large-sized family that has a history of hearing disorders. A history of an affected individual in a small family does not necessarily support a diagnosis of hearing impairment in later affected offspring because of the small sample size. Often, a hearing impairment that is part of a syndrome may not be detected because the physical findings associated with a syndrome are subtle in a young infant. For example, the white forelock seen in patients with Waardenburg's syndrome type I cannot be visualized in the infant who lacks hair. Additionally, some patients with syndromic hearing impairment do not present with physical findings, but rather they exhibit abnormal laboratory studies. Additional points to remember include the following: As infectious iatrogenic causes of hearing impairment decrease, the relative incidence of hereditary hearing impairment will increase. Hereditary hearing impairment can present as an isolated finding, or in association with a number of anomalies recognizable as a syndrome. The study of genetics and molecular biology has led to the identification of genes associated with hearing impairment and will allow for future screening and possible therapy for the hearing-impaired. The screening of newborns for hearing impairment using the techniques of molecular biologists and geneticists will result in early identification and appropriate intervention for those at risk for hereditary hearing impairment. An understanding of the syndromic and nonsyndromic causes of hereditary hearing impairment can help the otolaryngologist make a diagnosis and provide appropriate audiologic and educational management to the patient.     

Hörgeräte-Trageakzeptanz bei Kindern: eine längsschnittliche Analyse

There exist no systematic longitudinal studies concerning the acceptance of hearing aids in Germany. This study examines the acceptance of a hearing aid (defined by its daily/weekly use) in the management of children with persistent sensorineural hearing loss over a period of years. 35 children with monaural or binaural hearing loss were treated with a hearing aid. All children had at least a 25-dB averaged mid-frequency pure-tone hearing loss (500, 1000, 2000, 4000 Hz). The data consist of standardized parent ratings at 4 points in time over a period of nearly 30 months. Unilateral-impaired children wore their hearing aids less often than bilateral-impaired children. This effect was not significant at the beginning (P = 0.85) but increased over time. By the end the difference was significant (P = 0.004). Mild to moderate monaural hearing-impaired children accepted their hearing aids, whereas children with severe to profound hearing loss refused to wear them. Bilateral hearing-impaired children demonstrated, a priori, a better wearing acceptance that even improved with time. There was never a significant difference between boys and girls in their average wearing time. A significant correlation of age and wearing acceptance was also not observed at any time. Hearing aids are an effective treatment with high acceptance and compliance, especially by children with bilateral sensorineural hearing loss. The quality of acceptance of monaural hearing-impaired children needs to be studied further.     

The prevalence and causes of hearing impairment in Oman: a community-based cross-sectional study

A community-based nationwide survey for hearing loss was conducted in Oman in 1996-97. Audiometric tests and ear examinations were conducted for 12,400 persons in phase I. For children aged less than 4 years, subjective screening tests were used. In phase II, otologists examined the hearing-impaired subjects to determine the cause. The prevalence of bilateral hearing impairment was 55/1000 (95% CI 51.08-59.47). Gender difference was not significant. The rates were 325/1000 and 17/1000, respectively, in the > or = 60-year and < 10-year age groups. Presbyacusis and middle ear diseases, respectively, were the causes of 33% and 20% of bilateral hearing impairment. In 30% of the bilateral hearing-impaired subjects, the cause could not be determined. The prevalence of bilateral disabling hearing loss was 21/1000 (95% CI 18.07-23.29). Noise-induced trauma was responsible for only 1.4% of cases of disabling hearing loss. Establishing primary ear care, introducing hearing screening for neonates and schoolchildren, promoting safe preventive practices for ear care, strengthening secondary-level ear care services and introducing comprehensive rehabilitative initiatives for the hearing-disabled are recommended to reduce the hearing loss rates.     

Waardenburg综合征患者人工耳蜗植入术后听觉言语康复效果分析

目的评价Waardenburg综合征（Waardenburgsyndrome,ws）患者人工耳蜗植入术后的听觉言语康复效果。方法用纯音听阈、听觉行为分级标准（CategoriesofAuditoryPerformance,CAP）、言语可懂度分级标准（SpeechIntelligibilityRating,SIR）、儿童听觉言语能力家长评估问卷（Parents’EvaluationofAuraI／OralPerformanceofChildren,PEACH）,对行人工耳蜗植入术的4例WS1型患者、17例ws2型患者进行术后效果调查,并与非综合征且无明显病因的人工耳蜗植入患者比较,用SPSS13．0进行统计学分析。结果9例WS患者与9例对照组患者术后平均助听听阀（250Hz、500Hz、1000Hz、2000Hz、4000Hz）比较,差别无统计学意义（P〉0．05）。WS患者组CAP评分、SIR评分、安静环境下得分比、噪声环境下得分比、电话交流得分与对照组相比,差异均无统计学意义（P〉0．05）;WSl型组患者CAP评分、SIR评分、安静环境下得分比、噪声环境下得分比、电话交流得分与WS2型组患者相比,差异均无统计学意义（P〉0．05）,所有患者安静环境下得分比明显高于噪声环境下得分比（P〈O．05）。结论伴有重度或极重度感音神经性聋的Waardenburg综合征患者人工耳蜗植入术后的听觉言语能力与耳蜗形态正常的重度或极重度感音神经性聋患者无显著差别,安静环境下听觉言语能力好于噪声环境下听觉言语能力,且WS1型与WS2型患者人工耳蜗植入术后的听觉言语能力无显著差别。     

广东省听障儿童康复评估工具使用现状调查报告

目的 初步了解广东省听障儿童康复教师使用评估工具的情况.方法 自编问卷,对广东省听障儿童康复教师使用评估工具的情况进行调查.采用SPSS 13.0软件包对数据进行统计分析.结果 基层听障儿童康复教师评估手段单一,评估技能薄弱,评估与康复实践脱节,现任康复教师急需专业评估技能培训.结论 听障儿童康复教师的评估观念亟需更新,评估技能亟待提高.     

Hearing screening--aspects of epidemiology and identification of hearing impaired children

Mass screening of hearing in children is based on the concept of secondary prevention. In recognition of the importance of an early identification and intervention in children with congenital or early-acquired (i.e. neonatal period) hearing disability, numerous hearing screening programs have been introduced throughout the world. The devastating consequences of a congenital/early acquired hearing disability upon the speech, language, and social development of a child, and the estimated prevalence rates of at least 1-1.5/1000 live births of congenital permanent hearing impairment, represent an important health problem. The increase in the estimated prevalence of permanent hearing impairment in childhood, reaching at least 3.6-8.2% of live births at 5-9 years of age further emphasizes the importance of the problem. The delayed identification of children with congenital/early acquired hearing disability should result in the implementation of universal neonatal hearing screening, and the negative impact on the learning processes during school age from hearing impairment acquired throughout childhood seems to justify the introduction or maintenance of a hearing screening at school entrance. Implementation of efficient hearing screening programs throughout the neonatal period, infancy, or childhood should result in secondary prevention of this important health problem.     

Prevalence and age of identification of permanent childhood hearing impairment in Cyprus

Permanent childhood hearing impairment is a significant public health issue in the United Kingdom and elsewhere. It has effects on the hearing-impaired individual's linguistic, psychological, educational, and vocational development. Data on prevalence are an essential prerequisite for efficient service planning and development. This paper reports the first study to be undertaken in Cyprus to investigate the prevalence and the age at identification of permanent bilateral (congenital or acquired/late-onset) childhood hearing impairment of 50 dB HL or greater. The study was based upon a case ascertainment approach for children born between 1979 and 1996. Two methods were employed for the data collection: interrogation of case records and questionnaires administered to parents. The results for those years in which most cases are likely to have been identified showed an estimated prevalence of 1.19/1,000 live births (congenital) and 0.40/1,000 live births (acquired/late onset), with a mean identification age of 44.0 months for congenital cases. These findings are discussed with reference to the service development needs in Cyprus.     

3～5岁听障儿童与健听儿童鼻漏气的比较研究

目的：比较3～5岁听障儿童与健听儿童鼻漏气的差异,探讨其原因,为听障儿童康复训练提供参考依据。方法选取36名健听儿童和111名听障儿童,使用鼻息镜测试元音/a/,/i/,/u/的鼻漏气情况,并进行对比分析。结果年龄、性别与鼻漏气无显著性相关,听障儿童与健听儿童/a/的鼻漏气有极显著差异（P＜0.01）,/i/和/u/的鼻漏气有显著性差异（P＜0.05）。结论听障儿童/a/,/i/,/u/的鼻漏气现象显著多于健听儿童,其主要原因是听力障碍导致发音错误习得而造成的腭咽闭合功能不全。     

The prevalence and causes of hearing impairment in Oman: a community-based cross-sectional study

A community-based nationwide survey for hearing loss was conducted in Oman in 1996–97. Audiometric tests and ear examinations were conducted for 12 400 persons in phase I. For children aged less than 4 years, subjective screening tests were used. In phase II, otologists examined the hearing-impaired subjects to determine the cause. The prevalence of bilateral hearing impairment was 55/1000 (95% CI 51.08–59.47). Gender difference was not significant. The rates were 325/1000 and 17/1000, respectively, in the _60-year and _10-year age groups. Presbyacusis and middle ear diseases, respectively, were the causes of 33% and 20% of bilateral hearing impairment. In 30% of the bilateral hearing-impaired subjects, the cause could not be determined. The prevalence of bilateral disabling hearing loss was 21/1000 (95% CI 18.07–23.29). Noise-induced trauma was responsible for only 1.4% of cases of disabling hearing loss. Establishing primary ear care, introducing hearing screening for neonates and schoolchildren, promoting safe preventive practices for ear care, strengthening secondary-level ear care services and introducing comprehensive rehabilitative initiatives for the hearing-disabled are recommended to reduce the hearing loss rates.     

Nonsyndromic 35 delG mutation of the connexin 26 gene associated with deafness in syndromic children: two case reports

OBJECTIVES/HYPOTHESIS: Several genetic diseases, such as velocardiofacial syndrome Del(22q11) and Down syndrome, are associated with hearing impairment. STUDY DESIGN: Case reports. METHODS: The authors reported two cases of hearing-impaired children, one with Del (22q11) and one with Down syndrome, both with bilateral nonevolutive profound sensorineural deafness. Because of unusual features of their deafness and familial history, genetic evaluation was proposed. A homozygous 35delG mutation on the Connexin 26 gene was found in both children (DFNB1 phenotype). RESULTS: A review of the reported otological features of Del (22q11) and Down syndrome showed that sensorineural deafness is rare and seldom profound. The authors found no evidence for a genetic link between Del(22q11) or Down syndrome and 35delG mutation on the Connexin 26 gene. CONCLUSION: The case reports reveal a coincidental association between DFNB1 and a multiple congenital anomaly syndrome. The clinician must be aware of this type of association to manage genetic counseling, appropriate otological care, and suitable treatment.     

Der Wortschatzumfang bei jungen sensorineural schwerhörigen Kindern

The primary aim of the study was to measure the receptive and expressive vocabulary with respect to clinical parameters and selected socio-demographic variables and to assess whether the size of vocabulary could be predicted by certain variables in a consecutive series of children identified as having bilateral sensorineural hearing loss ("G?ttinger H?r-Sprachregister"). METHOD: All children aged between 2;6 to 6;3 years diagnosed in the Department of Phoniatrics/Pedaudiology of the University G?ttingen as having a permanent bilateral sensorineural hearing impairment ("G?ttinger H?r-Sprachregister") in a defined period (july 1995-september 2000) were examined with standardized receptive and expressive vocabulary tests as well as a nonverbal intelligence test (on average 5.5 months after diagnosis and 2.9 months after fitting with hearing aids). SAMPLE: 37 children (20 boys, 17 girls). Mean age at diagnosis: 48.5 (SD 15.9; min. 18, max. 75) months, mean age at hearing aid fitting: 51 months (SD 15.2). Multiple handicapped children, children with additional conductive hearing impairment and those with postnatal losses were excluded. The sample as a whole demonstrated lexical deficits of varying severity that were greater in the expressive than in the receptive vocabulary, depending on the clinical parameter. Children with congenital hearing impairment, with severe-to-profound hearing impairment (> 70 dB) and bilingual children demonstrated on average the smallest receptive and expressive vocabulary. An early fitting with hearing aids did not correspond with a better lexical development when a severe-to-profound hearing impairment existed. Severity of a hearing impairment and nonverbal intelligence significantly predicted the average size of the receptive vocabulary. A moderate hearing impairment, high non-verbal intelligence, and gender (female) emerged as the strongest significant predictors of the expressive vocabulary. The vocabulary test result of a bilateral sensorineurally hearing-impaired child may be an indicator for early cognitive training.     

Longitudinal study of hearing-disabled children. A follow-up investigation

A group of 138 hearing-impaired children, 76 boys and 62 girls, at a median age of 13 years, range 7-17 years, born during the period 1970-80, were examined in order to ascertain the incidence of progressive hearing loss, and if presence of progressive hearing loss relates it to specific causes of hearing impairment. After a 5-year interval from the median age of 8-13 years, progression of the hearing loss was demonstrated in 2-6% of the children (depending on the criterion used). No relationship could be established between progressive hearing loss and specific causes of hearing impairment.     

Improvement in early detection of congenital hearing impairment due to universal newborn hearing screening

OBJECTIVE: The aim of this study was to determine whether universal newborn hearing screening (UNHS) is effective in increasing the number of children whose hearing impairment is detected early, i.e. within the first 6 months of life. It also investigated whether UNHS contributes most to the early detection of moderately and severely hearing-impaired newborns, as suggested by a recently published report. METHODS: The study consisted of a retrospective analysis of the data of all children born in Tyrol between 1980 and 1999 and having an at least moderate permanent hearing loss in the better ear. RESULTS: The findings are that since UNHS was introduced in some newborn nurseries in 1995, a substantially higher number of hearing-impaired children has been detected early. For the whole sample, the increase of the early detection rate is 39.9%, with a 95% confidence interval of 33.2-46.8% (P<0.0001). For moderate hearing loss the increase is 49.2 with a 95% confidence intervall of 39.6-58.8% (P=0.000). CONCLUSIONS: On the whole, our findings lend support to the view that UNHS is effective in early detection of congenital hearing impairment. We conclude that UNHS provides the greatest benefit for moderately hearing-impaired children who, otherwise, would have been detected last.     

Diagnostic implications of acoustic cry features

Cry samples from 40 young children with severe to profound hearing loss and a control group of 24 normally-hearing youngsters were measured as to fundamental frequency, relative intensity and duration. The measurements were analyzed for differences between the hearing-impaired and normally-hearing groups, between the hearing-impaired and normally-hearing children at 6 month age ranges, and among children grouped according to degree of hearing impairment.

The youngsters with significant hearing impairment cried at higher fundamental frequencies than normally-hearing counterparts, while demonstrating similar intensity dynamics and duration characteristics. This held true at each 6 month age range.

A practical clinical application in the early diagnosis of hearing loss is suggested.     

Language development in hearing-impaired children. Establishment of a reference material for a 'Language test for hearing-impaired children', LATHIC

OBJECTIVE: In Sweden, there has previously been no normalised test material for the evaluation of language development in individual hearing-impaired children, and for the assessment of various methods of auditory habilitation. The purpose of the present study was to compose, apply and evaluate a test for language development in hearing-impaired children, and to establish the first set of reference values related to age, sex, type and degree of hearing impairment. METHODS: A test consisting of nine subtests was assembled and developed for, and subsequently applied to, hearing-impaired children in the age range 4-6 years. The inclusion criteria were a pure tone average of 80 dBHL or less and oral language (Swedish) as the first language. Two hundred and eleven hearing-impaired children and 87 normal hearing control children were tested. RESULTS: The results show that: (1) children with hearing impairment-also unilateral-have a delayed language development; (2) the delay is greater in children with larger losses and tends to decrease with increasing age; (3) 6-year-olds with hearing loss greater than 60 dB have not reached the level of the control group; (4) no difference between right- or left sided deafness with respect to language development was observed; (5) a reference material, applicable during clinical assessment, was established for the most common types of hearing impairment. CONCLUSIONS: The test designed gave graded measures of important aspects of language development in hearing-impaired children. The results merit further application of the test material.     

An assessment of cognitive abilities in hearing and hearing-impaired preschool children

There have been many investigations of cognitive development in older hearing-impaired children, but few with preschool hearing-impaired children. The performance of 40 hearing and 40 hearing-impaired children of preschool age (2 1/2 to 5 1/2 years) was compared on five nonverbal cognitive tasks and three subtests from the Perceptual Performance subscale of the McCarthy Scales of Children's Abilities (1972). For this set of tasks there was a significant effect of age consistent with a developmental change. A one-way MANCOVA on all dependent measures with age as the covariate revealed no significant differences between the hearing-impaired and hearing children. The results suggest that despite a deficiency in language abilities, the cognitive development of young hearing-impaired children is comparable to that of hearing children of the same age.