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Biomedical subjects

A Parving

Publications and source records attributed to A Parving.

At least 19 recordsLinked to original sources

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.

Child

Clinical otosclerosis, prevalence estimates and spontaneous progress.

This retrospective study was performed in order to estimate the prevalence of clinical otosclerosis as a function of age and gender, characterize the hearing level in otosclerosis and describe the spontaneous progress of the disease as a function of age. Clinically based samples were obtained from an audiological department, including 556 subjects: n = 166 (30%) males and n = 390 (70%) females with a median age of 75 years (range 22-95 years) at the time of examination. A subdivision of the sample into 3 age-bands, 20-39 years (n = 39), 40-59 years (n = 78) and > or = 60 years (n = 439), was performed. The overall prevalence estimate of clinical otosclerosis in the area in question was 1.41/1,000 [95% confidence interval (CI) = 1.3-1.5/1,000] with an estimate of 0.9/1,000 (95% CI = 0.8-1.0/1,000) in males and 1.85/1,000 (95% CI = 1.7-2.1/1,000) in females, with an increase in the prevalence as a function of age from 0.22/1,000 (95% CI = 0.15-0.29/1,000) to 3.53/1,000 (95% CI = 3.2-3.86/1,000) in the elderly. The estimates should be considered underestimates, as not all clinical otosclerosis in the area was included. No significant differences in the better and worse ear hearing levels averaged across 0.5-4 kHz (BEHL/WEHL 0.5-4 kHz) were found as a function of gender, and in general the impairment in the BEHL 0.5-4 kHz was fairly moderate until the age of 60 years. In ears previously subjected to surgery a significantly better hearing level of median 63 dB (range 24-119 dB) was found than in the no-surgery ears, with a median hearing level of 71 dB (range 5-120 dB). An estimate of the progress showed an increment in the hearing level in the elderly > or = 60 years of 30 dB over 30 years in non-operated ears, fairly similar to the 40 dB progress in operated ears. It was concluded that a significantly higher prevalence of clinical otosclerosis is present in females than in males, that the overall hearing level in otosclerosis is fairly moderate until the age of 60 years, and that previously operated ears have significantly better hearing than non-operated ears.

Age Factors

Prevalence of hereditary hearing impairment in adults.

This contribution, part of an EU-Concerted Action on the genetics of hearing impairment (H.E.A.R.), describes the preliminary estimated prevalence of hereditary hearing impairment based on retrospective data from a clinical series. Of 27,692 subjects examined in the period 1987-91, we sampled 1265 suffering from unilateral or bilateral hereditary hearing impairment, which is roughly 5% of those examined (n = 384 (31%) male; n = 881 (69%) female). Median age of the subjects is 70 years (range 22-98). Subdividing them into 10-year birth cohorts and applying the local annual population statistics, the prevalence of an overall age-related hereditary hearing impairment was roughly estimated to be 3.2/1000, reflecting prevalences as a function of age from 0.8 to 9.4/1000--prevalence in females being significantly more than in males (4.1/1000 and 2.1/1000, respectively). Overall, a moderate hearing impairment of median 51 dB in the better hearing ear was found, averaged across 0.5-4 kHz, this being fairly constant up to the age of 60, when a significant reduction in hearing sensitivity developed. No significant differences are present as a function of gender, except for the birth cohorts 1910-19 and 1920-29. The most frequent type of hereditary hearing impairment in this sample is otosclerosis, comprising 2% of the total clinical series with a rough population prevalence estimate of 1.4/1000. It is concluded that the established database may be of importance in the aggregation of very rare diseases, and for providing the inspiration for future prospective population studies, resulting in knowledge on the epidemiology of hereditary hearing impairment in adults.

Adult

The need for universal neonatal hearing screening--some aspects of epidemiology and identification.

The devastating consequences of a congenital/early-acquired hearing disability on 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 are important health problems. Universal neonatal hearing screening programs have provided the opportunity to detect neonates with permanent congenital hearing loss, and thus initiate auditory rehabilitation before the age of 3 mo. Universal neonatal hearing screening represents secondary prevention of hearing impairment/deafness, and the world-wide documented delayed identification of children with congenital/early-acquired hearing impairment will no doubt be improved with the implementation of universal neonatal hearing screening programs.

Age of Onset

Prevalence of hearing impairment and subjective hearing problems in a rural Danish population aged 31-50 years.

The objective of the present study was to describe the prevalence of subjective hearing problems and hearing impairment and to evaluate the relation between subjective hearing problems and audiometric thresholds, in a random sample of subjects aged 31-50 years. The study is cross-sectional and based on data from questionnaires (N = 1397) and pure tone audiometry (N = 905) in the Ebeltoft Health Promotion Project in Denmark. Hearing problems were significantly more prevalent in males than females. Males had significantly poorer average audiometric thresholds (0.5, 1, 2 and 4 kHz) in the better hearing ear (BE) and worse hearing ear (WE) than females. Average audiometric thesholds were poorer in the 41-50-year-old age group compared with the 31-40-year-old age group, the difference being most marked in males. Overall prevalence of hearing impairment (at least two threshold levels >25 dB HL) was 7.4% (95% confidence interval: 5.7-9.1%) in BE and 15.9% (13.5-18.3%) in WE. Prevalence of impairment > or = 25, > or = 35 and > or = 45 dB HL (average across 0.5-4 kHz): (BE) 3.4% (2.2-4.6%), 1.0% (0.3-1.6%) and 0.2% (0.0-0.5%); (WE) 11.6% (9.5-13.7%), 3.6% (2.4-4.9%) and 1.7% (0.8-2.5%). An interesting sex difference was noted as females were aware of hearing problems at an earlier stage of impairment than males. In conclusion, hearing impairment is fairly prevalent in this young rural population, less prevalent, however, than in the British National Study of Hearing. It is proposed that general practitioners should take a more active part in primary prevention, early detection of hearing impairment and early referral for rehabilitation.

Adult

Procedure for clinical testing of hearing aids.

In 1996, the Danish audiological clinics and hearing aid manufacturers jointly developed a clinical procedure for testing novel hearing aids in a clinical setting. This endeavour cane about because it was being acknowledged that clinical testing of hearing aids was likely to become more prominent as a result of the more advanced signal processing schemes enabled by digital technology in particular. It was understood that future hearing aids would encompass a large variety of diverse features, and that a rigid and detailed test protocol would therefore be inappropriate. The procedure was therefore devised as a framework within which a test protocol can be developed, and constitutes the framework of good scientific and clinical practice.

Hearing Aids

Audiometry in general practice: validation of a pragmatic pure-tone audiometry method.

The aim of this study was to validate the results of diagnostic pure-tone audiometry performed in a typical practice setting by comparing with test results obtained in a standardized audiological setting in accordance with the ISO standards. In a single-blinded crossover design, 119 persons were tested (0.25-8 kHz) in both settings. The mean deviations as a function of frequency were in the order of less than 2 dB (0.5-4 kHz) and otherwise up to 4 dB; the practice setting representing the poorer thresholds. The validity of the practice audiometry at three criteria of hearing impairment (0.5-4 kHz) was characterized by sensitivity (82-100%), specificity (95-99%); positive predictive values (75-90%) and negative predictive values (98-100%) focusing on the better ear. It is concluded that pure-tone audiometry of appropriate validity can be performed in general practice and that it is useful in selecting patients with no need of further audiological examination. Guidelines are needed.

Adult

Two frequent missense mutations in Pendred syndrome.

Pendred syndrome is an autosomal recessive disorder characterized by early childhood deafness and goiter. A century after its recognition as a syndrome by Vaughan Pendred, the disease gene ( PDS ) was mapped to chromosome 7q22-q31.1 and, recently, found to encode a putative sulfate transporter. We performed mutation analysis of the PDS gene in patients from 14 Pendred families originating from seven countries and identified all mutations. The mutations include three single base deletions, one splice site mutation and 10 missense mutations. One missense mutation (L236P) was found in a homozygous state in two consanguineous families and in a heterozygous state in five additional non-consanguineous families. Another missense mutation (T416P) was found in a homozygous state in one family and in a heterozygous state in four families. Pendred patients in three non-consanguineous families were shown to be compound heterozygotes for L236P and T416P. In total, one or both of these mutations were found in nine of the 14 families analyzed. The identification of two frequent PDS mutations will facilitate the molecular diagnosis of Pendred syndrome.

Adolescent

[Hearing in a geriatric perspective].

To assess whether hearing rehabilitation of older people can be improved by co-operation between the audiology and geriatric departments and the home service, 139 old and frail audiological patients were allocated to three groups with three different fitting procedures: 1) conventional fitting including verification of acoustical gain in the patient's ear; 2) home-fitting by hearing therapists, and 3) home-fitting by a specially trained geriatric nursing assistant, the home help also being present. Outcome was assessed by the ordinary questionnaire mailed to hearing aids users three to four months after fitting and by a geriatric evaluation procedure. The response rate in the conventionally fitted group was highly unsatisfactory (36%) and too small for further data-analysis. In the educational group a tendency was found towards better manipulation skills and significantly higher hearing aid use. However, the response rate was lower than in the geriatric group (71% compared to 81%), and no knowledge of hearing aid use was registered in the home service by this procedure. In the geriatric group a correlation was found between practical ability and use and satisfaction with the hearing aid. However, two thirds of the group were dependent on lasting help for the handling of the aid. Most patients in this group were already known by the hospital and home service, and the individual home help showed an interest in learning about hearing aid use. Home fitting by a joint audiological and geriatric effort in collaboration with the home help has proven feasible and valuable to both patient and home help. Extended co-operation is recommended between the health care and the social sector concerning hearing aid use.

Aged

The gene for Pendred syndrome is located between D7S501 and D7S692 in a 1.7-cM region on chromosome 7q.

Pendred syndrome is an autosomal recessive disorder characterized by goiter and congenital deafness. The primary defect is not yet known, although the gene causing Pendred syndrome has been localized very recently on chromosome 7q, a region that also contains a gene responsible for nonsyndromal hearing loss (DFNB4). We confirmed linkage to this chromosome 7 region in five Pendred families originating from different ethnic groups, with a highest cumulative lod score of 8.26 for marker D7S501. In combination with previous reports, our results define a candidate region for the Pendred gene of 1.7 cM flanked by markers D7S501 and D7S692.

Chromosome Mapping

The prevalence of Usher syndrome and other retinal dystrophy-hearing impairment associations.

The study was undertaken to procure population-based prevalence data on the various types of Usher syndrome and other retinal dystrophy-hearing impairment associations. The medical files on 646 patients with a panretinal pigmentary dystrophy aged 20-49 years derived from the Danish Retinitis Pigmentosa (RP) register were scrutinised. The data were supplemented by a prior investigation on hearing ability in a part of the study population. After exclusion of patients with possibly extrinsic causes of hearing impairments, 118 patients, including 89 cases of Usher syndrome were allocated to one of five clinically defined groups. We calculated the following prevalence rates: Usher syndrome type I: 1.5/100,000, Usher syndrome type II: 2.2/100,000, and Usher syndrome type III: 0.1/100,000 corresponding to a 2:3 ratio between Usher syndrome type I and II. The overall prevalence rate of Usher syndrome was estimated to 5/100,000 in the Danish population, devoid of genetic isolates. The material comprised 11 cases with retinal dystrophy, hearing impairment, and additional syndromic features. Finally, 18 subjects with various retinal dystrophy-hearing impairment associations without syndromic features were identified, corresponding to a prevalence rate of 0.8/100,000. This group had a significant overrepresentation of X-linked RP, including two persons harboring a mutation in the retinitis pigmentosa GTP-ase regulator (RPGR) gene.

Abnormalities, Multiple

Profound permanent hearing impairment in childhood: causative factors in two European countries.

A collaboration between Denmark and Wales was established in order to evaluate any differences between countries in factors causing permanent childhood hearing impairment (PCHI). Two identical 6-year birth-cohorts from 1975-1980 suffering from a permanent hearing loss > or = 90 dB HL in the better hearing ear, averaged across 0.5-4 kHz (BEHL), were included in the analysis. The overall estimated prevalence of children with this loss was 0.41/live births in Wales (n = 59). In Denmark the overall prevalence was 0.45/1,000 live births (n = 34), there being no significant differences in prevalence between the two countries. Both cohorts were subjected to a multidisciplinary investigation protocol. No significant differences were found between countries in the proportion of hearing impairment caused by the various factors, when based on uniform criteria and identical birth-cohorts. The proportion of inherited hearing loss is 47-50% in the two countries. Within inheritance the proportion of non-syndromal/syndromal inheritance differs slightly, as non-syndromal inheritance comprises 50% in Wales, and 76% in Denmark. The reported proportion of 16-34% of unknown factors causing profound PCHI emphasises the need for appropriate protocols for aetiological evaluation.

Child

Hearing instruments and health technology--an evaluation.

This contribution compares a currently worn hearing instrument (i.e. R-HI) with a programmable multiband full-dynamic range compression behind-the-ear HI (i.e. T-HI), based on subject preference for HI, and, in addition, compares the preference for a linear/ non-linear amplification-mode (i.e. L-mode/C-mode). Finally, it evaluates some health technology aspects related to the devices. N = 34 experienced HI-users at a median age of 72 years (range 22-84) completed the trial, n = 5 being monaurally, and n = 29 being binaurally fitted. The trial comprises an unblinded comparative study of the R-HI and T-HI, in either C-mode or L-mode amplification, and a double-blind crossover study evaluating the C-mode versus the L-mode amplification. The results of the unblinded study showed that 94% (n = 32/34) preferred the T-HI, whereas two subjects preferred their current HI. The double-blind crossover study concerning the preference for the C-mode and L-mode, respectively, in the T-HI, showed that 72% (n = 23/32) had a significant preference for the non-linear amplification, whereas 28% (n = 9/32) preferred the linear amplification. Provided that the price of the T-HI is kept unchanged, a health technology evaluation may imply an estimate of 30 mill. DKR added costs on a national basis, which should be taken into account within the national hearing services.

Adult

The Valby Project: a survey of hearing in the elderly > or = 80 years of age not provided with hearing aids.

This contribution forms part of an epidemiological study of hearing in the elderly > or = 80 years of age, living in an urban area. The study concentrates on the prevalence of self-reported hearing problems in a sample of subjects not provided with hearing aids (HAs), hearing level, and knowledge of and attitudes to HAs. Among the total population of 2915 > or = 80 years of age, an age- and sex-matched sample of 565 subjects was invited to attend an extensive audiological evaluation, including a questionnaire, giving information on knowledge about and attitudes to HAs. Forty-one percent (n = 231) attended, and dividing the attenders into 5 or 10 year age bands showed that the better and worse ear hearing levels, averaged across 0.5-4 kHz (BEHL/ WEHL0.5-4 kHz), deteriorate significantly with increasing age, and that no differences as a function of gender could be found. The prevalence of self-reported hearing problems was 44% (n = 101), and among these 30% (n = 30) indicated the potential need for a HA. A significantly poorer BEHL0.5-4 kHz was found in these subjects compared to those without hearing problems and/or need of a HA. In the total sample, 63% reported that a HA can ameliorate a hearing problem; however, 62% indicated that the HA should be invisible, and 28% that a HA 'makes you old'.

Age Factors

Hearing in the elderly > or = 80 years of age. Prevalence of problems and sensitivity.

This article is part of an epidemiological study on hearing in an urban population > or = 80 years of age, and concentrates on the prevalence of hearing problems and hearing sensitivity as a function of age and gender. Prior to the study, 2915 residents aged 80+ from the Valby area in Copenhagen were selected for investigation and subdivided into two groups. The first group, comprising n = 859 subjects previously provided with HA, and the second group, resulting from an invitation mailed to n = 565 subjects, were matched according to the age and gender distribution of the population, i.e. 24% males and 76% females. Among these, only 41% at a median age of 84 years, range 80-96, accepted the invitation, being significantly younger than the non-attenders. The estimated prevalence of self, reported hearing problems ranged between 33 and 66%, increasing with increasing age. Significant differences were found in the hearing sensitivity as a function of gender, i.e. the hearing in the low-frequency area < 1 kHz is better, whereas the hearing sensitivity at > 2 kHz is significantly worse in males compared with females. The speech recognition score (SRS) was significantly higher in females compared with males, and a comparison between ears supports the finding that the right ear speech recognition score is better than the left. The study demonstrates the difficulties in obtaining reliable epidemiological data on the hearing in the elderly > or = 80 years, which represents an obstacle for the planning of appropriate hearing health services directed towards this age group, and collaborative studies are suggested in order to accumulate more knowledge.

Aged