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Biomedical subjects

A Parving

Publications and source records attributed to A Parving.

At least 127 records · Page 7Linked to original sources
PubMed1977

Aniridia, cataract and gonadoblastoma in a mentally retarded girl with deletion of chromosome II. A clinicopathological case report.

A case of bilateral aniridia, cataract and mental deficiency is described in a girl with an interstitial deletion of the short arm of chromosome 11. The child died of pneumonia and on autopsy bilateral gonadoblastoma was detected, but no Wilms' tumour. Partial deletion of the short arm of chromosome 11 seems to be a clinically recognizable syndrome with aniridia, cataract and renal, gonadal or genital dysmorphogenesis.

Cataract↗
PubMed1976

Menière's disease in childhood.

Two cases of Menière's disease in childhood were found during one year from 850 examinations of children. The clinical and audiological findings are similar to those seen in adults.

Audiometry↗
PubMedDate not supplied

Evidence for diabetic encephalopathy.

Auditory brain stem responses were recorded in 20 normoacoustic long-duration Type 1 diabetic patients (duration of diabetes 26 (range 13-46) years, age 44 (25-66) years) with peripheral neuropathy and retinopathy and in 19 sex-matched normoacoustic short-duration Type 1 diabetic patients (duration of diabetes 2 (0-6) years, age 23 (18-50) years) without clinical signs of neuropathy or microangiopathy. Abnormal brain stem auditory evoked responses were demonstrated in 40% of the long-duration and in 5.3% of the short-duration diabetic patients (p less than 0.01). Interpeak latencies Jv-JI and JIII-JI were significantly prolonged in both patient groups compared with the non-diabetic control group (p less than 0.01). Magnetic resonance imaging was performed in 16 of the long-duration patients and in 40 age-matched healthy volunteers on a whole body MR-scanner. Subcortical and/or brain stem lesions with abnormally high signals were seen in 69% of the long-duration Type 1 patients and in 12% of the healthy volunteers (p less than 0.02). Neuropsychological examination including 17 tests for intelligence and cognition were performed in the 20 long-duration Type 1 diabetic patients. The results indicated a performance close to that seen in a control group of healthy age-matched control subjects. Our study demonstrates that a considerable proportion of long-duration Type 1 diabetic patients suffering from retinopathy and peripheral neuropathy additionally have signs but no symptoms of central nervous system affection, diabetic encephalopathy.

Adult↗
PubMedDate not supplied

Audiological and temporal bone findings in myxedema.

Fifteen patients with confirmed myxedema at a median age of 48 years (range 32 to 60 years) were referred for audiological evaluation before and after treatment with levothyroxine. The median interval between the pretreatment and posttreatment investigations was 18 months (range 9 to 27 months). In addition, 13 patients at a median age of 78 years (range 64 to 95 years) were audiologically reexamined after long-standing levothyroxine treatment. The observation period upon treatment with levothyroxine was 40 months (range 32 to 46 months). No improvement in hearing sensitivity could be demonstrated either in the younger patients or in the elderly. When compared to an age- and sex-matched unscreened population, the myxedematous patients did not demonstrate any different degree of hearing loss. Histological investigation of the temporal bones from an 83-year-old woman with myxedema, however, showed no morphological changes or deposition of glycosaminoglycans, changes which are compatible with true age-related hearing loss. It is concluded that no association exists between myxedema and hearing impairment and that no morphological or structural changes due to myxedema can be demonstrated in the temporal bones.

Adult↗
PubMedDate not supplied

Clinical findings and diagnostic problems in sensorineural low frequency hearing loss.

The otological and audiological findings in 39 patients with sensorineural low frequency hearing loss are reported. This type of perceptive hearing loss is difficult to distinguish from the true conductive hearing losses due to the air conduction audiogram shape and the invalidity of bone conduction determinations showing a false "air-bone gap". This may lead to surgical treatment of a perceptive hearing loss, as reported in the four case histories. By various audiological tests, contradictory information may be obtained. In our material, Bing's test and absent acoustic reflexes indicated a conductive disorder in 25% of the ears. The final differentiation may require cochleography. The hearing loss may be diagnosed as Meniere's disease. In our material only 17% complained of tinnitus and no patients had vertigo. Consequently, we find sensorineural low frequency hearing loss to differ from Meniere's disease. Our material comprises different etiological types of perceptive low frequency hearing loss. On type was inherited as an autosomal dominant trait, another type due to cochlear malformation probably also inherited, and a third group showing diverse audiological results. When the diagnosis is established, the patients may be treated successfully by specially constructed hearing aids.

Adolescent↗
PubMedDate not supplied

Reliability of Békésy threshold tracing in identification of carriers of genes for an X-linked disease with deafness.

Seven identified carriers and 20 potential carriers of Norris's disease have been examined by pure tone octave audiometry and Békésy audiometry. The investigation supports earlier results of Békésy threshold tracings performed in heterozygote carriers of genes for recessive hearing impairment. The sensitivity of the method is poor. The specificity of the Békésy threshold tracing is high, meaning that an absent dip cannot exclude the possibility of a subject being a carrier, whereas a present dip can be regarded as an indication of carrier. When comparing conventional octave audiometry and Békésy threshold tracing, the latter method is found to be more subtle in finding carriers of genes for recessive deafness. Therefore, Békésy threshold tracing may be of help in the genetic counselling of potential carriers of genes for recessive deafness.

Adolescent↗
PubMedDate not supplied

Congenital hearing loss and rubella infection.

In an examination of Danish children aged 0 to 4 years, with congenital sensorineural hearing loss, 60% (39/65) had rubella antibody, compared with only 23% (17/72) in an age-matched control group. Based upon these results and similar investigations reported in the literature, it is concluded that serological testing for rubella antibody has proved to be of major importance in the evaluation of etiological factors of congenital hearing loss. Although diagnostic conclusions cannot be drawn on the basis of serological testing per se in the individual child, it is important to estimate the incidence of fetal rubella infection as the cause of congenital hearing loss, since this type of hearing loss may be prevented by active immunization.

Antibodies, Viral↗
PubMedDate not supplied

Hearing sensitivity in patients with myxoedema before and after treatment with l-thyroxine.

Fifteen patients with confirmed myxoedema, median age 76 years, were consecutively referred for audiological evaluation. The diagnosis of myxoedema was based on the symptomatology, typical clinical appearance, increased TSH level and decreased T4. The audiological evaluation included routine ENT-examination, pure-tone octave audiometry, determination of speech reception threshold and discrimination score. The function of the middle ear was evaluated by impedance audiometry, indicating both middle ear pressure and stapedial reflex thresholds elicited by contralateral stimulation. All patients were evaluated in the myxoedematous state before treatment with l-thyroxine and reevaluated when treated and found euthyroid, both by the clinical investigation and as judged by chemical thyroid parameters. Bilateral symmetrical or nearly symmetrical sensorineural hearing loss was demonstrated in all patients before treatment. The results indicate that in elderly patients with myxoedema no improvement in hearing sensitivity can be demonstrated upon l-thyroxine medication. Patients with myxoedema at this age demonstrate neither more nor less degree of hearing loss when related to an age-matched group of hearing impaired patients. In patients with myxoedema the hearing impairment is found to be equal to that of an age and sex matched control group exhibiting true age-related hearing loss.

Acoustic Impedance Tests↗
PubMedDate not supplied

Inherited sensorineural low-frequency hearing impairment: some aspects of phenotype and epidemiology.

This contribution forms part of the HEAR project. It describes some phenotypes of inherited low-frequency sensorineural hearing impairment (LFSHI) and estimates the prevalence of this inherited hearing impairment (HI) based on a clinical series. During a 10-year period (1987-1996), 418 subjects (134 males and 284 females), with a median age of 68 years (range 4-98), had been examined with LFSHI, defined as hearing loss most pronounced in the low frequencies (i.e., 250 and 500 Hz > 20 dB HL with better hearing, i.e., > or =15-dB difference at 1 and/or 2 and/or 4 kHz with an air-bone gap <15 dB for the average of 0.5, 1, and 2 kHz). The 418 subjects comprising 0.6 per cent of the total number of subjects examined (N=69,309) were subdivided into four categories: category I positive genetic subjects (N=69); category II, probably genetic (N=339); category III, uncertain genetic (N=6); and category IV, subjects with contradictory audiological findings (N=4). The phenotype in category I demonstrated a symmetrical LFSHI, with a pattern of progression showing a slow deterioration in the high frequencies (i.e., 2, 4, and 8 kHz as a function of age)--the progression comprising 40-45 dB. In the low frequencies (i.e., 250, 500, and 1,000 Hz), a deterioration of 15-25 dB could be demonstrated from the youngest to the oldest age group. In category II, a symmetrical LFSHI was found in 179 subjects, showing the same pattern of progression as in category I. However, in the age group 20-39 years, a significantly poorer hearing was found in the low frequencies compared to category I, implying that several phenotypes may be present in LFSHI. A subgroup (A) in category II exhibited normal hearing in one ear with LFSHI in the opposite ear with the same pattern of progression as in category I. Three other subgroups with LFSHI and flat/sloping audiogram in the opposite ear and asymmetrical LFSHI also showed the same type of progression in the ear with LFSHI as in category I. A prevalence of 0.18/1,000 (95 per cent CI 0.13-0.22) of LFSHI was estimated based on the background population with a fairly constant prevalence throughout life. It is concluded that inherited nonsyndromal LFSHI is a rare disease and that the many different phenotypes of LFSHI probably are associated with pronounced genetic heterogeneity.

Acoustic Impedance Tests↗
PubMedDate not supplied

Dominantly inherited low-frequency hearing loss.

Two families with dominantly inherited low-frequency hearing loss are described. The two families are different in mode of transmission and in audiological findings. On the basis of these two differences, it is claimed that two types of inherited low-frequency hearing loss can be distinguished. One type is the dominantly inherited low-frequency hearing loss with fully penetrant abnormal genes. The other is also most probably due to an autosomal dominant gene, but with incomplete penetrance and probably represents a malformation in the middle ear combined with a defect in the apical part of the cochlear mechanism.

Audiometry↗
PubMedDate not supplied

Electrophysiological study of Norrie's disease. An X-linked recessive trait with hearing loss.

3 patients with Norrie's diseas were examined with electrocochleography and brain-stem-evoked responses. The examination revealed a hearing loss of pure cochlear origin with no involvement of the brain stem. The disease is inherited as an X-linked recessive trait only affecting males with unaffected female carriers. The results are thus in agreement with previous investigations of the topical lesion in genetic hearing loss.

Abnormalities, Multiple↗
PubMedDate not supplied

Rehabilitation of hearing-impaired children: intervention and outcome.

A cohort of 72 hearing-impaired children of a median age of 9.7 years, range 4.3-13.9, born during 1980-1990 and living in the health district of Copenhagen City was examined cross-sectionally in order to evaluate the interval from identification to the initial fitting of hearing aids (HAs) and children's use of HAs. As measures of outcome, the use of HAs at 1, 6, 12 months after fitting, and at the time of data collection was assessed along with the primary language of each child. One month after the fitting, 53% of the total sample used the HAs > 8 h/day, and no significant longitudinal changes in the proportion of time-related use were found. 56% of the children stayed in the same user category during the years, while the rest either increased (25%) or decreased (19%) the use of amplification from 1 month after fitting to the time of data collection. Children with better ear hearing level (BEHL) 0.5-4 kHz between 60 and 89 dB used the HAs significantly more than those with mild or profound hearing impairments, whereas age at onset (congenital, acquired, age at onset unknown) and age at intervention were unrelated to use of amplification. 72% of the children were oral, while 25% were manual (unknown 3%), and no differences in the use of HAs were found between these two groups. A subdivision of congenitally hearing-impaired children according to BEHL 0.5-4 kHz below/above 75 dB demonstrated a significantly larger proportion of manual children in the poorer hearing group despite a significantly younger amplification age. It is concluded that age at intervention is unrelated to primary language, although the number of children precludes dependable conclusions.

Adolescent↗
PubMedDate not supplied

The effect of neonatal universal hearing screening in a health surveillance perspective--a controlled study of two health authority districts.

The contribution describes the effect of a neonatal hearing screening program in terms of estimated prevalence rate of congenital hearing impairment and age at identification in two five-year cohorts born between 1990 and 1994 as a function of health authority districts (HADs). In addition, identically defined five-year birth cohorts from 1970 to 1974 and 1980 to 1984 living in the same HADs evaluated previously are used in the analysis, offering longitudinal data. In 1990 a non-targetted neonatal hearing screening program based on EOAE was introduced in the County-HAD, whereas the City-HAD continued its child hearing health surveillance program unchanged. Assuming an unchanged prevalence estimate of 1.5 per 1000 of congenital or early acquired (i.e. neonatal period) hearing disability, i.e. > or = 25 dB HL for the better ear at 0.5-4 kHz in both HADs, an underestimate of 68 per cent in the City and of 20 per cent in the County, respectively, was found at the time of data collection (January 1995). Significant longitudinal improvements in the early identification from the 1970-1974 cohort compared to the 1980-1984 cohort has been demonstrated, but the improvement in the proportion of children identified as a function of both 6 and 12 months of age in the 1990-1994 cohort is significantly greater, showing a median age at identification of 11 months in the County-HAD. The cross-sectional and longitudinal comparisons between the HADs imply that a neonatal universal hearing screening program with a 20 per cent coverage may add significantly to the early identification of children with early-onset hearing impairment.

Cohort Studies↗
PubMedDate not supplied

Clinical study of hearing instruments: a cross-sectional longitudinal audit based on consumer experiences.

The objective of the present audit was to document improvements/changes in hearing instrument (HI) benefit in terms of use and satisfaction throughout the 1990s and compare the benefit in consumers fitted with analogue (A), programmable (P), and digital signal processing (DSP) HIs. The audit data are based on a questionnaire mailed to subjects fitted with HIs 3 to 4 months after the fitting, including questions concerning satisfaction with the HI, use of the HI, ability to manage the HI, and satisfaction with the overall services in the department. Information was obtained from 32,694 subjects (i.e., 71.2 per cent of those fitted with HIs throughout the last decade), with a median age at fitting of 78 years (range, 18-102), with a preponderance of females. No significant differences in age, gender distribution, and hearing instrument distribution were found between the respondents and non-respondents. The audit shows that there are no significant differences in any of the benefit parameters throughout the decade, irrespective of the introduction of P-HIs in 1995 and of the DSP-HIs in 1998. Thus, from the consumers' perspective, no changes have appeared in the last decade. A comparative analysis of the A-HIs, P-HIs, and DSP-HIs shows that among those provided with P-HIs and DSP-HIs, only 69.6 per cent (CI 68.1-71.0) and 65.5 per cent (CI 60.2-78.8), respectively, were very satisfied/satisfied with the HI, which is significantly less than the 73.5 per cent (CI 72.4-74.5) in those fitted with A-HIs. The daily/weekly use of all three types of HIs was similar. However, the group fitted with DSP-HIs was significantly less satisfied with the general services compared with those fitted with A-HIs and P-HIs, respectively. Based on these audit data, it is concluded that, according to the consumers, the outcome from HIs has not changed over the last decade, and a comparison between the traditional A-HIs with both P-HIs and DSP-HIs is in favour of the traditional A-HIs.

Aged↗