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Dafydd Stephens

Publications and source records attributed to Dafydd Stephens.

18 recordsLinked to original sources

Distortion product otoacoustic emissions in patients with King-Kopetzky syndrome.

In the present study, mild impairment of cochlear function in patients with King-Kopetzky syndrome was investigated using DPOAEs. A significant decrease in DPOAE levels in both ears was found in patients with King-Kopetzky syndrome compared with the controls after considering the thresholds as a co-variable. It is noteworthy that the global mean levels of DPOAEs were still significantly greater in controls than in patients with matched thresholds. Further frequency analyses showed a significant decrease in DPOAE levels over the mid- and high-frequency range in patients with better hearing thresholds when compared with those in the control group. Moreover, significantly smaller DPOAEs were found in the ears of patients with King-Kopetzky syndrome and without SOAEs, than in such ears of control subjects after considering the thresholds as a co-variable. However, when SOAEs were present there was no difference. Decreases in DPOAE level appear to represent evidence of minor cochlear pathology, and provide a pathological basis for the difficulty of hearing speech in the presence of background noise, which characterizes King-Kopetzky syndrome.

Acoustic Stimulation↗

The impact of a family history of hearing problems on those with hearing difficulties themselves: an exploratory study.

The objective of the study was to investigate the effects of a family history of hearing impairment on those people with the hearing impairment themselves. The subjects were 102 consecutive patients with a family history of hearing impairment, seen in an audiological rehabilitation clinic. Each was given an open-ended questionnaire asking whether such a family history had any effects on them and, if so, to list any effects. Results were qualitatively analysed. 57 subjects indicated effects. Of those listed, 57% were positive, 19% negative, and 24% neutral. The most common positive effects concerned realising the importance of hearing aids and early help-seeking. Common negative effects were concern for the future of themselves and their children. Neutral effects were a reported ignorance or denial of a family history. The results emphasize the importance of asking patients about any family history when planning their rehabilitative programme.

Adult↗

The viability of speech-in-noise audiometric screening using domestic audio equipment.

Speech-in-noise audiometry has potential application as a low-cost, self-screening test for sensorineural hearing loss. To realize this potential, the influence of variations in audio equipment and listening environment need assessment. The present study assessed: 1) the frequency response and distortion produced by a wide range of commercially available audio equipment; 2) the effects of such variations upon test results with normally hearing subjects using a simple, open-set, word-identification test; 3) the effect of distortion on the speech reception threshold using digitally applied distortion; and 4) the reliability of the test in listening environments with different levels of reverberation. In addition, preliminary tests were conducted with elderly listeners. The results indicate that variations in equipment have negligible effects on speech-in-noise audiometry. The only factor that substantially elevated normally hearing listeners' thresholds was high levels of room reverberation when using loudspeaker presentation. Variations in equipment and environment thus present no significant obstacle to the development of a self-administered audiometric screening test based on speech in noise.

Acoustic Stimulation↗

The epidemiology of hearing problems: how should we investigate it?

Within published reports, the prevalence of reported hearing difficulties is quite variable. In the present study, we have considered factors that could influence the apparent prevalence of hearing difficulties in the Welsh population, surveyed in a number of different studies. The first important factor to emerge was whether the questions were asked in the context of a general health approach (together with questions about other aspects of health), in which case the prevalence was usually less than 15%. When the same questions were posed in conjunction with a number of other questions on auditory function the prevalence was 20% or greater. Secondly, when a household survey approach is used, with one person being asked to respond on behalf of all members of the household, the prevalence among those reporting is markedly higher than among the other members of the household whose problems they are reporting.

Adult↗

Transient evoked otoacoustic emissions in patients with middle ear disorders.

The aim of this study is to investigate the middle ear dynamic characteristics and their influence on TEOAEs in patients with middle ear disorders. The middle ear dynamic characteristics and TEOAE findings were investigated in 89 patients with middle ear disorders using the sweep frequency impedance (SFI) meter and the ILO88 system, respectively. These patients were divided into six subcategories: tympanic membrane aberrations, otitis media with effusion, chronic otitis media, tympanic membrane perforation, otosclerosis, and ossicular chain dislocation. Details of the TEOAE frequency characteristics were compared with the individual's middle ear dynamic characteristics. TEOAE status as a function of hearing threshold and middle ear dynamic characteristics was also examined. The results showed that the middle ear dynamic characteristics in patients with middle ear disorders correlated with the TEOAE frequency characteristics and amplitudes. The hearing level and middle ear mobility were the controlling factors affecting TEOAE status. Using both non-linear and linear stimulus modes, larger TEOAE responses were obtained when the hearing level was better than 20 dB HL, and there was moderately good middle ear mobility. Moreover, TEOAEs were absent using the non-linear mode when the hearing level was worse than 30 dB HL, whereas with the linear mode. TEOAEs were recordable even with hearing losses of up to 40 dB HL in patients with middle ear disorders. A higher incidence of recordable TEOAEs was found in the subgroups with tympanic membrane abnormalities and secretory otitis media when compared with the other subgroups. No TEOAEs were recordable in patients with chronic otitis media.

Acoustic Impedance Tests↗

King-Kopetzky syndrome in the light of an ecological conceptual framework.

The principal symptom of subjects suffering from King-Kopetzky syndrome is a perceived difficulty in recognizing and understanding speech in noisy backgrounds. For some patients, minor disturbances in auditory function, e.g. a deteriorated signal-to-noise ratio for speech, can be demonstrated; for others, all measurements of hearing are normal. A conceptual framework developed for the analysis of communication disorders is applied to King-Kopetzky syndrome in the present article. The usefulness of the framework is evaluated, and an extended system for the analysis of King-Kopetzky syndrome patients is suggested. It is emphasized that changes in demands resulting from external conditions and altered perception and evaluation of the conditions may generate the symptoms. A change in the subjects' desired self-image (preferendum) and a change in social role, new, unfamiliar vocabulary, as well as minor dysfunctions in peripheral and central auditory processing, might generate the characteristic features of the syndrome. The framework is related to the current literature, and arguments for the usefulness of the framework in the planning of support and rehabilitation are presented. Several new questions are generated for further analysis of King-Kopetzky syndrome.

Auditory Perceptual Disorders↗

The role of positive experiences in living with acquired hearing loss.

This paper presents findings from our most recent research investigating positive experiences associated with acquired hearing loss. This work builds upon our earlier qualitative studies but now takes a more quantitative approach. The findings of two separate quantitative questionnaire studies are reported. The aim of the first study was to incorporate and build upon the qualitative work to design a quantitative questionnaire tool. This work produced a 48-item questionnaire measuring the frequency with which people recognized specific positive experiences. The questionnaire was distributed to 174 consecutive patients. The results of principal component analysis identified eight factors comprising 57% of the total variance. The primary factors were labelled 'Cognitive changes to self-perception', 'Successful communication behaviours', 'Using hearing aids to self-advantage', 'Effort in communication', 'Social support', 'Social contact', and 'Resignation'. The aim of the second study was to reduce and refine the quantitative tool to 20 items. This second tool was distributed to a second sample of 173 consecutive patients The questionnaires distributed included the revised 20-item positive questionnaire in addition to four additional outcome measures. A principal component analysis was performed, and six factors emerged, accounting for 60.5% of the total variance. These factors broadly reflect those previously identified, and were labelled (1) 'Cognitive changes to self-perception', (2) 'Using hearing impairment to self-advantage', (3) 'Successful communication behaviour', (4) 'Resignation', (5) 'Effort in communication', and (6) 'Technical facilitators'. The first second, second and fourth of these may be regarded as non-auditory factors, and the others as auditory factors. There was little relationship between the two types of factors, and a complex set of relationships with the other outcome measures.

Activities of Daily Living↗

Investigating lifestyle factors affecting hearing aid candidature in the elderly.

Within this presentation, the authors consider briefly the published data on the effects of hearing impairment on lifestyle in the elderly, and suggest that the World Health Organization's International Classification of Functioning, Disability and Health (ICF) would provide a framework for standardizing the study of effects of impairments. This is followed by two studies. In the first study, we asked a consecutive sample of elderly patients (hearing aid candidates and users) to list the activities, transactions and interactions in which they participated, and classified these using the ICF. We then took the main categories, and, in a second study, explored how often individuals participated in them, how much difficulty they had because of their hearing loss, and how much they enjoyed them. This highlighted the considerable difficulties that most such individuals experience in common activities and communication situations, and which seem to be little influenced by whether or not they use hearing aids.

Aged↗

Mutations in the WFS1 gene that cause low-frequency sensorineural hearing loss are small non-inactivating mutations.

Hereditary hearing impairment is an extremely heterogeneous trait, with more than 70 identified loci. Only two of these loci are associated with an auditory phenotype that predominantly affects the low frequencies (DFNA1 and DFNA6/14). In this study, we have completed mutation screening of the WFS1 gene in eight autosomal dominant families and twelve sporadic cases in which affected persons have low-frequency sensorineural hearing impairment (LFSNHI). Mutations in this gene are known to be responsible for Wolfram syndrome or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), which is an autosomal recessive trait. We have identified seven missense mutations and a single amino acid deletion affecting conserved amino acids in six families and one sporadic case, indicating that mutations in WFS1 are a major cause of inherited but not sporadic low-frequency hearing impairment. Among the ten WFS1 mutations reported in LFSNHI, none is expected to lead to premature protein truncation, and nine cluster in the C-terminal protein domain. In contrast, 64% of the Wolfram syndrome mutations are inactivating. Our results indicate that only non-inactivating mutations in WFS1 are responsible for non-syndromic low-frequency hearing impairment.

Audiometry↗

Absence of hearing impairment in adult onset facioscapulohumeral muscular dystrophy.

Hearing impairment has long been associated with the rarer forms of severe childhood and infantile facioscapulohumeral muscular dystrophy. Recent studies have suggested a high prevalence in classic, adult or adolescent, onset cases as well. We undertook detailed pure tone audiometric examination of 21 adult onset facioscapulohumeral muscular dystrophy cases. Patient results were compared with normative data obtained from the (United Kingdom) National Study of Hearing (The prevalence and distribution of hearing impairment and reported hearing disability in the MRC Institute of Hearing Research's National Study of Hearing, Whurr, 1995). There was no significant difference in the prevalence of hearing impairment in facioscapulohumeral muscular dystrophy patients at any single or averaged frequency tested. In this group of patients, pure tone audiometric thresholds were consistently better in the facioscapulohumeral muscular dystrophy patients, although these reached statistical significance only at 4 and 6 kHz. Age of onset, disease duration, clinical severity, degree of facial weakness and double-digest fragment size made no significant difference to mean hearing thresholds. We conclude that hearing impairment is not more common in adult onset facioscapulohumeral muscular dystrophy, and according to this data, may even, be less prevalent than in the normal population.

Adolescent↗

Middle ear dynamic characteristics in patients with otosclerosis.

OBJECTIVE: To investigate the middle ear dynamic characteristics in patients with otosclerosis using the sweep frequency impedance meter (SFI test) and conventional tympanometry, and also to evaluate the diagnostic efficiency of the SFI test for otosclerosis. DESIGN: The study was designed to collect a total of 25 (36 ears) consecutive patients with otosclerosis. All subjects followed a clinical protocol, which consisted of a hearing problem questionnaire, otoscopic examination, and audiometric measurements. These included pure tone audiometry, conventional tympanometry, and SFI test. RESULTS: In the SFI test, the middle ear dynamic characteristics were measured in terms of the resonance frequency and middle ear mobility. Three distinct categories of middle ear dynamic characteristics were found in patients with otosclerosis, i.e., high stiffness, normal stiffness, and low stiffness middle ear status. On comparison of the results of SFI with conventional tympanometry, a significantly higher percentage of abnormal stiffness was found when using the SFI test than that when using conventional tympanometry. CONCLUSIONS: The present findings confirm the advantage of the SFI test over conventional tympanometry in detecting middle ear status and mechanics in patients with otosclerosis. Moreover, different middle ear dynamic characteristics in patients with otosclerosis are most likely to be related to the different stages of the pathological changes.

Acoustic Impedance Tests↗

Opting for two hearing aids: a predictor of long-term use among adult patients fitted after screening.

This study is a follow-up of patients fitted with one or two hearing aids, approximately 10 years previously. Our patients were identified through population screening for hearing difficulties when they were aged 50-65 years. At the time, they had a trial of unilateral and bilateral fittings, and then they made their own choice to keep one or two aids. We found that 10 of the 12 who had opted for two aids and were available for follow-up 10 years later continued using at least one aid (83%, 95% CI 55-95). Six of the 17 who opted for unilateral fitting and were available for follow-up used a hearing aid in the long term (35%, 95% CI: 17-59%). Therefore, this study suggests that people who are identified through population screening, are bilaterally impaired, are willing to try bilateral fitting, and choose to continue with two aids, are likely to become long-term users of (one or two) hearing aids. Those who choose to continue with one aid after a trial of bilateral fitting are at high risk of rejecting the aid in the long term. Our sample is small, and we took a number of steps in the analysis in order to clarify whether these findings are reliable. We could not identify any confounding factors. The patients' preference for continuing with bilateral or unilateral aids after fitting was the only predictor of long-term use in our sample. Remarkably, bilateral preference was a better predictor of long-term use than the degree of hearing impairment. We discuss how this study provides useful information for planning population screening for hearing difficulties in middle age.

Aged↗

The short form 36 (SF36) in a specialist tinnitus clinic.

Tinnitus is an extremely common condition which is known to have an effect on quality of life. This study reports the health profile of tinnitus sufferers who attend a specialist tinnitus clinic. This cross-sectional study uses the self-report questionnaire SF36. Two hundred consecutive new patients completed the questionnaire as part of their assessment in a specialist tinnitus clinic in Wales. In all eight scales of the SF36 the mean scores were below that of the general population. Compared to a reference population patients referred to a specialist tinnitus clinic show a reduction in their age/sex-adjusted health profile which is comparable to that of people reporting low back pain. The findings also lend weight to the suggestion that tinnitus sufferers may have a general response bias.

Adult↗

The International Outcome Inventory for Hearing Aids (IOI-HA) and its relationship to the Client-oriented Scale of Improvement (COSI).

The International Outcome Inventory-Hearing Aids (IOI-HA) was administered to 161 consecutive patients who had been fitted with hearing aids just prior to their being discharged from the clinic. Most also completed the Client-oriented Scale of Improvement (COSI). Very few questions (<2%) were not completed. Analyses indicated two subscales of IOI-HA, one of which could be defined as a 'benefit' subscale and the other a 'residual problems' subscale. Both elements of COSI correlated with the 'benefit' subscale, but only the 'residual' measure of COSI related to the 'residual problems' subscale. There were no consistent relationships between the IOI-HA and a range of demographic factors.

Adult↗