Use of real patients in teaching ENT diseases to undergraduate students and its effects on patient satisfaction: cross-sectional survey.

OBJECTIVES: To describe a method of using real patients in teaching ENT to undergraduates and to examine whether being a case patient affected patient satisfaction. METHODS: In a cross-sectional study, 68 teaching-involved patients (case patients) with a suspected common ENT illness and 68 matched (in terms of age, sex and region of complaint) control patients evaluated the health service and their encounter with the physician. The students saw the case patients first independently and then saw the patient with the teacher physician. The controls were treated in a normal way. RESULTS: Fifty-eight case patients (84 per cent) and 65 control patients (95 per cent) answered the questionnaire. The median duration of the visit was significantly longer for the case patients than the controls (115 vs 60 minutes). Almost all patients in both groups graded the overall quality of the health service, and the variables describing various aspects of the setting and the encounter with the physician, as either good or excellent. CONCLUSION: Patients who took part in the undergraduate teaching of ENT diseases were equally content with their primary visit as the control patients, even though their visit took a markedly longer time.

Parents' views on the quality of life of their children 2-3 years after cochlear implantation.

OBJECTIVE: Cochlear implants for children are known to have impact on the lives of recipients and their families in a variety of ways. To obtain a clearer picture of these benefits, we explored the quality of life of 36 Finnish children and their families 2-3 years after unilateral cochlear implantation. METHODS: The studied children were, on average 5 years old, and had received their implant at the median age of 2 years:5 months (range 1:6 to 12:3). Most (67%) of the children used speech, eight (22%) used speech and signs, and four (11%) used sign language as their main communication mode. A third of the children had concomitant problems in addition to their profound hearing impairment. A validated closed-set questionnaire "Children with cochlear implants: parental perspectives" (available, e.g., at http://www.earfoundation.org.uk/research/questionnaires.html) was used to find out parents' views and experiences on implantation and explore life after it. RESULTS: Parents were most satisfied with improved/expanded social relations, improved communication (the development of spoken language), general functioning with the help of hearing and improved self-reliance of the child. Benefit of cochlear implantation was also detected with the Categories of Auditory Performance (CAP), which was concordant with views of the parents on the progress of their child in the areas of communication and education. When deciding on implantation, the parents particularly expected auditory information to enhance their child's safety in traffic, joining socially the hearing world, and better employment prospects as adults. Concerning the process of implantation, parents especially valued the know-how and fluent services of the implant centre, positive attitude within the family and information received from other families during the time they were considering the implant decision. Parents also found it important that they have the possibility to influence the communication mode that is used in their child's educational setting. CONCLUSIONS: Parents report that cochlear implants affect their children in a wide variety of ways that cannot be summarized by a single scale. A broader descriptive framework is required to capture their experiences adequately.

Contribution of the N-acetyltransferase 2 polymorphism NAT2*6A to age-related hearing impairment.

BACKGROUND: Age-related hearing impairment (ARHI) is the most common sensory impairment in older people, affecting 50% of those aged 80 years. The proportion of older people is increasing in the general population, and as a consequence, the number of people affected with ARHI is growing. ARHI is a complex disorder, with both environmental and genetic factors contributing to the disease. The first studies to elucidate these genetic factors were recently performed, resulting in the identification of the first two susceptibility genes for ARHI, NAT2 and KCNQ4. METHODS: In the present study, the association between ARHI and polymorphisms in genes that contribute to the defence against reactive oxygen species, including GSTT1, GSTM1 and NAT2, was tested. Samples originated from seven different countries and were combined into two test population samples, the general European population and the Finnish population. Two distinct phenotypes for ARHI were studied, Z(low) and Z(high), representing hearing in the low and high frequencies, respectively. Statistical analysis was performed for single polymorphisms (GSTM1, GSTT1, NAT2*5A, NAT2*6A, and NAT2*7A), haplotypes, and gene-environment and gene-gene interactions. RESULTS: We found an association between ARHI and GSTT1 and GSTM1 in the Finnish population sample, and with NAT2*6A in the general European population sample. The latter finding replicates previously published data. CONCLUSION: As replication is considered the ultimate proof of true associations in the study of complex disorders, this study provides further support for the involvement of NAT2*6A in ARHI.

Familial aggregation of tinnitus: a European multicentre study.

INTRODUCTION AND AIM: Tinnitus is a common condition affecting approximately 20% of the older population. There is increasing evidence that changes in the central auditory system following cochlear malfunctioning are responsible for tinnitus. To date, few investigators have studied the influence of genetic factors on tinnitus. The present report investigates the presence of a familial effect in tinnitus subjects. METHODS: In a European multicentre study, 198 families were recruited in seven European countries. Each family had at least 3 siblings. Subjects were screened for causes of hearing loss other than presbyacusis by clinical examination and a questionnaire. The presence of tinnitus was evaluated with the question "Nowadays, do you ever get noises in your head or ear (tinnitus) which usually last longer than five minutes". Familial aggregation was tested using three methods: a mixed model approach, calculating familial correlations, and estimating the risk of a subject having tinnitus if the disorder is present in another family member. RESULTS: All methods demonstrated a significant familial effect for tinnitus. The effect persisted after correction for the effect of other risk factors such as hearing loss, gender and age. The size of the familial effect is smaller than that for age-related hearing impairment, with a familial correlation of 0.15. CONCLUSION: The presence of a familial effect for tinnitus opens the door to specific studies that can determine whether this effect is due to a shared familial environment or the involvement of genetic factors. Subsequent association studies may result in the identification of the factors responsible. In addition, more emphasis should be placed on the effect of role models in the treatment of tinnitus.

A 3-year clinical follow-up of adult patients with 3243A>G in mitochondrial DNA.

OBJECTIVE: To follow the clinical course of patients with the mitochondrial DNA mutation 3243A>G for 3 years. METHODS: Thirty-three adult patients with the 3243A>G mutation entered a 3-year follow-up study. They were clinically evaluated annually, audiometry was performed, and samples were drawn for the analysis of blood chemistry and mutation heteroplasmy in leukocytes. Holter recording was performed three times during the follow-up and echocardiography, neuropsychological assessment, and quantitative EEG and brain imaging conducted at entry and after 3 years. RESULTS: The incidence of new neurologic events was low during the 3-year follow-up. Sensorineural hearing impairment (SNHI) progressed, left ventricular wall thickness increased, mean alpha frequency in the occipital and parietal regions decreased, and the severity of disease index (modified Rankin score) progressed significantly. The rate of SNHI progression correlated with mutation heteroplasmy in muscle. The increase in left ventricular wall thickness was seen almost exclusively in diabetic patients. Seven patients died during the follow-up, and they were generally more severely affected than those who survived. CONCLUSIONS: Significant changes in the severity of disease, sensorineural hearing impairment, left ventricular hypertrophy, and quantitative EEG were seen in adult patients with 3243A>G during the 3-year follow-up.

Prevalence of large-scale mitochondrial DNA deletions in an adult Finnish population.

BACKGROUND: Large-scale mitochondrial DNA (mtDNA) deletions are associated with clinical conditions such as Kearns-Sayre syndrome and chronic progressive external ophthalmoplegia in adults and Pearson syndrome in children. Reported case series have suggested that deletions are not uncommon in the population, but their prevalence has not been documented. METHODS: The authors ascertained patients with clinical features associated with mtDNA deletions in a defined adult population in northern Finland. Buccal epithelial samples were requested from each patient fulfilling the selection criteria, and full-length mtDNA was amplified using the long PCR method. Deletion breakpoints were identified using sequencing. Patients with deletions were examined clinically. RESULTS: The authors identified four patients with single large-scale mtDNA deletions. The prevalence of deletions was calculated to be 1.6/100,000 in the adult population in the province of Northern Ostrobothnia (0.0 to 3.2; 95% CI). Analysis of incident cases from a neighboring province revealed two patients with deletions and yielded a similar population frequency. CONCLUSIONS: The frequency of large-scale mitochondrial DNA deletions is similar among populations, suggesting that there is a constant rate of new deletions.

Open-set speech perception in adult cochlear implant users with ossified cochleae.

This study describes open-set speech recognition in cochlear implant subjects with ossified cochleae and compares it to a control group with open cochleae. Twenty-one postlingually deafened adults with a Med-El Combi 40/40+GB split- electrode implant were matched to patients using a Med-El cochlear implant with a standard electrode. Speech recognition was assessed over an 18-month period. Split- electrode patients improved significantly over time, but their scores were significantly lower and increased significantly slower than those of controls. Of 14 patients with a duration of deafness less than 20 years, average sentence test scores were 50%, and average monosyllabic word test scores were 31%. This study provides evidence that cochlear implantation is beneficial to patients with ossified cochleae, but early implantation is advisable.

Provision of hearing aid services: a comparison between the Nordic countries and the United Kingdom.

About one in five adults has some form of hearing impairment. This paper reports on a survey which found significant variation in the provision of hearing aid services across Denmark, Finland, Norway, Sweden and the United Kingdom. Per capita expenditure levels were estimated to vary from under 3 euros in the UK to nearly 10 in Denmark and Norway. Annual figures for the number of people receiving at least one aid varied from around eight per 1,000 in the UK and Denmark to 2.2 in Finland. Over 50% of hearing aid users in Norway and Denmark are estimated to have bilateral aids, compared to under 15% in the UK. Estimates of the number of people who have a hearing aid in these countries are considerably lower than the number who have a hearing impairment. More research is needed to verify the true extent of these differences and their cause.

Hearing impairment among adults--extent of the problem and scientific evidence on the outcome of hearing aid rehabilitation.

Scientific surveys on current and estimated prevalence of hearing impairment (HI) in adult populations (> or = 18 years of age) in Denmark, Finland, Norway, Sweden and the United Kingdom, and scientific reports on the outcome of hearing aid (HA) rehabilitation worldwide were reviewed. Only a few of the studies meet strict scientific criteria, and many locally clinically relevant studies cannot be generalized to larger populations. Population-based studies indicate an increase in prevalence of HI with age, but because of differences in study populations and available national population statistics, the studies do not allow reliable comparisons between countries or estimation of future prevalence of HI. Studies on HA prescription or outcomes do not provide uniform data in favour of non-linear amplification, but they do show some subject preference for the newer technology. No conclusions can be drawn regarding the degree of HI and the effects of amplification. The literature review alone gives only limited information regarding the extent of the problem of HI in adult populations in the target countries. Similarly, only a few studies on HA outcome meet strict scientific criteria and even fewer studies correlate rehabilitation outcome with the degree of HI, disability or handicap.

Will hearing healthcare be affordable in the new millennium.

Calculations of the needs of healthcare, hearing healthcare included, are usually based on clinical data rather than on valid prevalence figures. According to our recent population study, the number of citizens needing hearing aids in Finland will be twice the figure usually presented, 280 000 for the country as a whole (population 5.1 million). The penetration of hearing aids was only 41%. Taking this and the ageing of the population into account, the economic challenges of hearing healthcare will be alarming in the new millennium.

Prognosis of hearing impairment in Ménière's disease.

The deterioration of hearing in Ménière's disease is known to be dependent on the individual and to be highly variable. In most cases, however, it leads to moderate hearing impairment. In order to be able to predict deterioration, we used multivariable modelling with a source population of definite cases of Ménière's disease diagnosed according to the latest recommended American Academy of Otolaryngology--Head and Neck Surgery criteria. The records of 205 patients were retrospectively analysed. Their earliest and latest pure-tone air conduction audiograms were used to evaluate the long-term effects of the disease on hearing. The effect of age and the follow-up period were calculated for different frequencies (0.125-8 kHz) and for the pure-tone average over the frequencies 0.5 to 4 kHz (PTA0.5-4 kHz). Deterioration in PTA0.5-4 kHz was approximately 1 dB/year due to the duration of the disease and approximately 0.5 dB/year due to aging. Hearing impairment in Ménière's disease appeared to increase linearly with the duration of the disease for patients < 50 years of age, but in the older subjects the effect of the duration of the disease decreased. Both aging and the duration of the disease affected the hearing level. The duration of the disease did not seem to have any statistically significant effect on the difference between the two ears. The difference that occurred at the onset of the disease remained until presbyacusis in the better ear reached the hearing level of the worse ear. The difference in hearing deterioration between men and women was not significant.

Speech perception and functional benefit after cochlear implantation: a multicentre survey in Finland.

This study was done to survey the effect of cochlear implantation on hearing level, speech perception and listening performance in Finnish-speaking adults. The subjects of the study comprise 67 adults. Pure-tone thresholds (0.125-8 kHz), word recognition and listening performance were studied before and after implantation. After switch-on of the implant, the median values of PTA(0.5-4 kHz) in the sound field were fairly stable across the evaluation period. Three months after switch-on of the implant, the mean word recognition score was 54%. There was clear improvement in the mean word recognition scores over a longer period of time, the mean score being 71% 24 months after switch-on. Six months after switch-on, the majority of subjects (40/48) were able to recognize some speech without speechreading, and 26 of these 48 subjects were able to use the telephone with a known speaker, gaining good functional benefit from the implantation.

Hearing impairment in patients with 3243A-->G mtDNA mutation: phenotype and rate of progression.

The relationship between the phenotype and the genotype is complex in diseases caused by mutations in mitochondrial DNA (mtDNA). The 3243A-->G mutation in mtDNA frequently leads to sensorineural hearing impairment (HI), a phenotype that can be assessed in severity by audiometry; hence, consecutive audiograms can give an estimate of the rate of HI progression. We examined the audiological phenotype of 38 patients (14 men, 24 women; mean age: 45+/-14 years) who possessed the 3243A-->G mutation and who belonged to a population-based cohort ascertained in the province of Northern Ostrobothnia, Finland. The subjects took part in an otorhinolaryngologic examination, including audiometry. Factors modulating the severity of HI were analyzed, and the rate of HI progression was calculated. The better ear hearing level (BEHL) at frequencies 0.5, 1, 2, and 4 kHz (BEHL0.5-4kHz) was greater than 20 dB suggesting HI in 28 patients (74%). A good correlation (r=0.428, P=0.009) was found between BEHL0.5-4kHz and the degree of the mutant heteroplasmy. BEHL0.5-4kHz was worse in men than in women, and women outnumbered men among patients with normal hearing or mild HI. In addition, 181 consecutive audiograms were reviewed from 24 patients with HI. The rate of HI progression was calculated to be 2.9 dB/year in men and 1.5 dB/year in women, being clearly faster than the rates that have been observed in the corresponding age group in the general population. A high degree of mutant heteroplasmy, male gender, and age were found to increase the severity of HI. Phenotypic difference by gender may thus be a more universal phenomenon in mitochondrial diseases, not only being associated with Leber's hereditary optic neuropathy. This study provides the first estimate of the rate of disease progression among patients with the 3243A-->G mutation.

Long-term outcome of early childhood hearing impairments in northern Finland.

Early childhood hearing impairment (HI) may have a marked negative effect on educational outcome and employment status in adulthood. The late outcome of 51 Finnish mildly to profoundly HId children born in the period 1965-1979 was studied using a questionnaire posted to the subjects. Subjects included in the study had an early childhood sensorineural, non-syndromal hearing impairment with no known associated handicaps. The response rate to the inquiry was as high as 88% (45/51). The subjects had qualified educationally at a somewhat lower level than their age peers according to nationwide statistics; 48% of the respondents belonging to the labour force were currently unemployed (versus 15% of all the 25-to 29-year-olds in Finland in 1997), but their employment status was not associated with the HI grade. In particular, the severely and profoundly HId often had needed special support from the employment authorities to find work.

On the construction of a Finnish audiometric sentence test.

To respond to the demands of clinical practice and the needs of rehabilitation, a Finnish audiometric sentence test is being developed. The test consists of 10 sets of 10 sentences, each set serving as an independent test. The test is scored by words (50 per set). Homogeneity between the sets was guaranteed by a number of linguistic and phonetic criteria, controlled by using the Virko Sentence Analyzer, a programme especially constructed for the purpose. Recognition tests for the validation of the sentence sets were made with young normally hearing adults (n = 70; age group 18-25 years). Psychometric recognition properties of the test are described. The selection process of the final 10 sentence sets is illustrated.

HI-SIMv1.0--towards the virtual reality of hearing impairments.

The virtual reality of hearing impairments has obvious practical applications in areas such as audiology, speech therapy and hearing aid technology and serves as an informational tool for the family members of the hearing impaired. To simulate hearing impairment, a CD-ROM with filtered speech material accessible through a graphical user-interface was produced; the user-interface was created with standard multimedia tools. The CD-ROM, HI-SIMv1.0, intended as an interactive educational tool, offers a virtual experience of the effects of a selection of common types of hearing impairment. The options available in this simulation include grade of hearing impairment, audiometric configuration and the type and level of background noise. Word recognition scores can be computed for standard Finnish audiometric material.

Speech perception and functional benefit after multichannel cochlear implantation.

This study was done to investigate the effect of a multichannel cochlear implant on speech perception and the functional benefit of cochlear implantation in Finnish-speaking postlingually deafened adults. Fourteen subjects were enrolled. Sentence and word recognition were studied with open-set tests auditorily only. One year after implantation, the listening performance was assessed by case histories and interviews. Before implantation for subjects with a hearing aid, the mean recognition score was 38% for sentences and 17% for words. One year after switching on the implant, the mean recognition score was 84% for sentences and 70% for words. Before implantation, the majority of the subjects were not aware of environmental sounds and only a few were able to recognize some environmental sounds. One year after switching on the implant, the majority of the subjects were able to use the telephone with a familiar speaker. All the subjects were able to recognize speech auditorily only and had thus gained good functional benefit from the implant.

Cochlear implant patients and quality of life.

During the last two decades, cochlear implants have been available for profoundly hearing-impaired patients who do not benefit from conventional hearing aids. The quality of life of these implantees has not been extensively studied, and has not been studied at all in Finland. To assess their quality of life, the Nottingham Health Profile questionnaire was sent to all adult implanted patients in Finland. Implantees tended to have more favourable average pain, emotional reaction and mobility indexes in each age group studied than the average population. Social-isolation seemed to be more common in the youngest and oldest age groups of implantees. Apparently, implantees are physically healthier than the average population. However, a bias caused by the strict selection of implant candidates cannot be excluded.