Hearing loss but not bone-regulating hormones predicts fractures in older women-a 17-year follow-up of the Gothenburg BEDA study.

High-frequency hearing loss and S-Ca, but not hormones related to bone structure and strength, or lifestyle factors, predicted incident fractures during 17 years of follow-up in women up to 97 years of age. INTRODUCTION: The fracture risk increases and inner ear function deteriorates with increasing age. The aim of this study was to investigate whether hearing loss was of greater importance than bone-regulating hormones for the risk of fracture in elderly women. METHODS: In 1997, a random population sample of 63-82-year-old women, n = 552, underwent a physical examination, audiometry and blood sampling for analyses of serum albumin-adjusted calcium (S-Ca), parathyroid hormone (PTH), 25(OH) vitamin D and insulin-like growth factor-1 (IGF-1). Data on medication, lifestyle, previous fractures, hearing, vision and dizziness were obtained using questionnaires. Data on subsequent fractures were retrieved, and censored at death, through December 2013. RESULTS: In 1997, 228 women (41%) reported a previous fracture, most commonly of the wrist (18%). During the following 17 years, 323 fractures occurred in 207 women (38%). Hip fractures were the most frequent, in 96 women (17%). In a Cox regression analysis adjusted for age and previous fractures, hearing loss, reflected by a high pure tone average ≥ 59 dB, almost doubled the risk of a subsequent fracture (hazard ratio (HR) 1.81, 95% CI 1.25; 2.61, p = 0.002). S-Ca (HR 1.21 (1.02; 1.44) p = 0.028) also predicted future fractures, whereas PTH, IGF-1, 25(OH) vitamin D, hormone replacement therapy, smoking, degree of physical activity, impaired vision and dizziness did not. CONCLUSION: Hearing loss and higher S-Ca, but not bone-regulating hormones, medication or lifestyle factors predicted incident fractures, mainly caused by falling, during 17 years of follow-up in women up to 97 years of age.

Turner syndrome isochromosome karyotype correlates with decreased dental crown width.

The aim of this project was to study possible influences of Turner syndrome (TS) karyotype and the number of X chromosomes with intact short arm (p-arm) on dental crown width. Primary and permanent mesio-distal crown width was measured on plaster casts from 112 TS females. The influence on crown width of four karyotypes: 1. monosomy (45,X), 2. mosaic (45,X/46,XX), 3. isochromosome, and 4. other, and the number of intact X chromosomal p-arms were investigated. In comparisons between karyotypes, statistically significant differences were found for isochromosome karyotype maxillary second premolars, canines, laterals, mandibular first premolars, and canines, indicating that this karyotype was the most divergent as shown by the most reduced crown width. When each karyotype group were compared versus controls, all teeth in the isochromosome group were significantly smaller than controls (P < 0.01-0.001). The 45,X/46,XX karyotype expressed fewer and smaller differences from controls, while 45,X individuals seemed to display an intermediate tooth width compared with 45,X/46,XX and isochromosomes. No significant difference in crown width was found comparing the groups with one or two intact X chromosomal p-arms. Both primary and permanent teeth proved to have a significantly smaller crown width in the entire group of TS females compared to healthy females. We conclude that the isochromosome group deviates most from other karyotypes and controls, exhibiting the smallest dental crown width, while individuals with 45,X/46,XX mosaicism seemed to have a less affected crown width. An influence of the number of intact p-arms on crown width could not be demonstrated in this study.

Obstetric outcomes in women with Turner karyotype.

CONTEXT: Women with Turner syndrome (TS) have high risk of cardiovascular complications and hypertensive disorders. Few studies have analyzed obstetric outcome in women with TS. OBJECTIVE: This study compared obstetric outcome in women with TS karyotype with women in the general population. DESIGN: The Swedish Genetic Turner Register was cross-linked with the Swedish Medical Birth Register between 1973 and 2007. Obstetric outcome in singletons was compared with a reference group of 56,000 women from the general population. Obstetric outcome in twins was described separately. RESULTS: A total of 202 singletons and three sets of twins were born to 115 women with a TS karyotype that was unknown in 52% at time of pregnancy. At first delivery, TS women of singletons were older than controls (median 30 vs. 26 yr, P < 0.0001). Preeclampsia occurred in 6.3 vs. 3.0% (P = 0.07). Aortic dissection occurred in one woman. Compared with the general population, the gestational age was shorter in children born by TS women (-6.4 d, P = 0.0067), and median birth weight was lower (-208 g, P = 0.0012), but sd scores for weight and length at birth were similar. The cesarean section rate was 35.6% in TS women and 11.8% in controls (P < 0.0001). There was no difference in birth defects in children of TS women as compared with controls. CONCLUSIONS: Obstetric outcomes in women with a TS karyotype were mostly favorable. Singletons of TS women had shorter gestational age, but similar size at birth, adjusted for gestational age and sex. Birth defects did not differ between TS and controls.

Women who gave birth to girls with Turner syndrome: maternal and neonatal characteristics.

BACKGROUND: The aim was to identify maternal risk factors in women giving birth to girls with Turner syndrome (TS) and to describe the characteristics of newborns with TS. METHODS: The Swedish Genetic Turner Register was cross-linked with the Swedish Medical Birth Register. Between 1973 and 2005, 494 children with TS were born. Maternal age, parity, height, smoking habits and neonatal characteristics; mode of delivery, gestational age, size at birth and Apgar score, were compared with women in the general population who gave birth to girls during the same period. RESULTS: More women with advanced maternal age (40+) delivered girls with TS, 3.2% when compared with 1.8% in the general population [OR 1.83, 95% confidence interval (CI) 1.09-3.08, after adjustment for year of birth]. Maternal height was inversely associated with TS pregnancies (P = 0.005). Late preterm birth occurred in newborns with TS in 10.5% when compared with 4.8% in the general population (OR 2.23; 95% CI: 1.67-2.97, after adjustment for year of birth and maternal age). Newborns with TS had birthweight less than -2SD in 17.8% and birth length less than -2SD in 21.0% when compared with 3.5 and 3.4%, in the general population (OR 6.55; 95% CI: 5.12-8.38 and OR 8.69; 95% CI: 6.89-10.97, after adjustment for year of birth and maternal age). CONCLUSION: Advanced maternal age and short stature were risk factors for giving birth to a girl with TS. More TS girls were born late preterm and were smaller for gestational age than non-TS girls in the general population.

The benefit method: fitting hearing aids in noise.

The most common complaint among individuals with hearing impairment is the inability to follow a conversation when several people are talking simultaneously, a noisy listening situation which is completely different from the quiet surrounding of the conventional pure tone audiometry used as basis for the hearing aid settings. The purpose of this report was to present important characteristics of the BeneFit Method (BFM), a procedure that fits the hearing aid under simulated conditions of competing speech and also a clinical pilot evaluation study comparing the BFM to the NAL-R recommendations and also to the Logic procedure, a GN resound proprietary fitting algorithm representing a modern digital hearing aid fitting procedure. Speech recognition scores in noise (SRSN) using monosyllabic words presented under different background noise levels were evaluated on 21 randomly selected subjects with hearing impairment. The subjects were fitted with the same type of hearing aid Danalogic 163D according to the BFM procedure as well as the logic procedure, the latter developed and recommended by the manufacturer. A comparison of the SRSN when using the subjects' current hearing aid fitted according to the NAL-R procedure was also made. Only the BFM procedure provided a significant SRSN improvement compared to the unaided condition (P< 0.01) in a signal/speech-noise level of 75/65 dB corresponding to a normal cocktail party condition. Moreover, patients performed significantly higher SRSN when fitted according to the BFM, than when fitted according the Logic or NAL-R procedures. The BFM procedure, which is based on individual and functional detection of hearing thresholds in noise levels corresponding to a cocktail party condition, can improve SRSN significantly. Hearing aids should be fitted under conditions similar to those when the hearing disability is perceived the most, i.e, in an environment with background noise.

Genetic analysis of mosaicism in 53 women with Turner syndrome.

Mosaicism involving the sex chromosomes is a common finding in women with Turner syndrome (TS). It is especially important to detect Y-chromosomal material, since this is a risk factor for the development of gonadoblastoma. Recent studies have also indicated that the frequency of 45,X cells may be used to predict prognosis. As part of an ongoing multi-disciplinary study, we have examined the extent of Y-chromosomal material and sex chromosomal mosaicism and its tissue specificity in 53 women with TS. The results of lymphocyte karyotyping were compared with the use of interphase X/Y fluorescence in situ hybridisation (FISH) analysis of lymphocytes and buccal mucosal cells. As could be expected, an extended FISH analysis detected more Y-chromosomal material than karyotyping (in 15% vs. 11% of the women, respectively) and also detected more X-chromosomal mosaicism among the TS women (in 70% vs. 45 % of the women, respectively). In half of the women, tissue-specific differences between lymphocytes and buccal mucosal cells were found. Based on these results, we suggest the use of X/Y interphase FISH as a complement to karyotyping in order to obtain a more complete knowledge of the chromosome constitution of each individual with TS.

Ototoxic interaction between noise and pheomelanin: distortion product otoacoustic emissions after acoustical trauma in chloroquine-treated red, black, and albino guinea pigs.

This study provides further evidence of an ototoxic interaction between red pheomelanin pigmentation and noise-induced hearing loss. Red, black, and albino guinea pigs were treated with a low, a high, or no dose of chloroquine. The 2f1-f2 distortion product otoacoustic emission (DPOE) measurements were measured before, immediately after, and 1 month after noise exposure to a 1-kHz tone at 105 dB SPL for 72 hours. In red guinea pigs, the DPOE was severely affected by noise trauma when treated even by a low single dose of chloroquine, whereas in both albino and black guinea pigs, the chloroquine effect on the DPOE was temporary and present only when the drug was given in a high single dose. The structure most likely to be responsible for the severe loss of DPOE in chloroquine-treated red animals is the strial melanocyte. The damage may be triggered by an ototoxic noise-induced production of radical oxygen species from pheomelanin, for example, by the Fenton reaction or due to the increased variability of the melanocyte 1 receptor gene as in red-haired individuals.

Predictive factors for the severity of tinnitus.

The majority of patients with tinnitus experience a lessening of their symptoms during an 18-month period after their first consultation. The exception to this rule is severe incapacitating tinnitus, the sometimes very troublesome symptoms of which show no sign of diminishing with time. The aim of the present study was to investigate risk factors for incapacitating tinnitus, as measured by absence from work related to tinnitus (AWT) of more than 1 month during an 18-month period after the first visit to the tinnitus clinic. Audiometric data and the scores from the Tinnitus Severity Questionnaire and the Nottingham Health Profile at the first visit to the clinic were correlated to AWT by a logistic regression model. By using this risk model, an individual risk score for AWT could be estimated. Depression and physical immobility were strong predictors of AWT, and hearing loss was moderate. Based on these results, we suggest that the aetiology of tinnitus severity could be described as depression-/anxiety-related, somatic, and auditory tinnitus.

The influence of hearing and age on speech recognition scores in noise in audiological patients and in the general population.

OBJECTIVE: To describe the influence of pure-tone audiometry and age on the speech recognition score in noise, both in audiological patients and also in a random population sample. DESIGN: In a cross-sectional study, speech recognition scores (SRS) using monosyllabic words presented in a fixed background noise were evaluated on 1895 audiological patients of both genders with normal hearing or sensorineural hearing losses. The background noise was speech weighted and presented with a signal to noise ratio of +4 dB. In 291 participants, SRS in quiet was estimated as well. A female random population sample also was tested (N = 513). RESULTS: The major predictor for the SRS-noise was high-frequency hearing thresholds. If hearing was normal, age had no effect on speech recognition. Young persons with hearing loss had higher SRS-noise than older persons with the same degree of hearing loss. The difference between young and old persons became larger the greater the hearing loss. Predictive SRS-noise with consideration taken to hearing function and age are presented. SRS-noise correlated stronger with pure-tone audiometry and age than SRS-quiet. Controls performed better (by 10 to 20%) than their same-aged peers with similar hearing loss. CONCLUSION: It is recommended that speech recognition tests be performed in background noise. SRS-noise is a valuable tool for audiologists and audiological physicians to identify patients in need of pedagogic rehabilitation programs or further diagnostic investigations.

The influence of karyotype on the auricle, otitis media and hearing in Turner syndrome.

The study has investigated the relationship between the chromosomal aberration and ear and/or hearing disorders in 115 girls/women with Turner syndrome (TS). A dose-response relationship was found between the karyotype and hearing function. Hearing deteriorated more rapidly with increasing age in TS women lacking the whole p-arm of chromosome X (i.e. monosomy 45,X, or isochromosome cases 46,X,i(Xq)) as compared to women having a partial deletion of the p-arm (structural deletions or mosaicism cases), who, in turn, had poorer hearing than a female random population sample (46,XX) (P<0.001). Moreover, TS subjects having total deletion of the p-arm were three times more likely to have auricular anomalies or conductive hearing loss due to otitis media than subjects with partial deletion (P<0. 05). The results support the hypothesis that lack of growth-regulating genes such as the short stature homeobox-containing gene (SHOX), which is located within the pseudo-autosomal region on the p-arm of the X chromosome, may increase the occurrence of auricular malformations and otitis media and also induce an earlier loss of hearing function. Accordingly, the ear and hearing disorders in TS may be a result of growth disturbances of the auricle, the mastoid, the Eustachian tube and the organ of Corti during development. It is suggested that karyotype may be used as a predictor for future ear and hearing problems in TS.

Hair cell loss from acoustic trauma in chloroquine-treated red, black and albino guinea pigs.

In order to further elucidate the relationship between noise-induced hearing loss and pigmentation, a two-factor study was designed. Albino, red and black guinea pigs were divided into controls and chloroquine-treated groups and exposed to 1 kHz noise, 105 dB SPL, for 72 hours. One month later the animals were sacrificed and the loss of hair cells evaluated. The red guinea pigs developed a greater hair cell loss (OHC) in all three OHC rows than black or albino animals. Black and albino groups showed equal amounts of OHC loss. A high dosage of chloroquine seemed to reduce the OHC loss in albino, but not in black or red guinea pigs. The greater OHC loss in red compared with black animals is in accordance with the original hypothesis that melanin protects the inner ear against noise trauma. However, as red guinea pigs developed greater OHC loss than albinos, it is obvious that the original hypothesis needs to be modified to consider also the different melanin types, i.e., the black eumelanin and the red pheomelanin. The present results are interpreted as a toxic interaction in the strial melanocytes between pheomelanin and noise. It is suggested that the pathophysiology of noise-induced hearing loss involves cochlear mechanisms related to radical oxygen species (ROS) as melanin both generates and neutralizes ROS. A hypothesis about a linkage between dopamine, noise trauma and the cochlear melanocyte system is discussed.

The effect of low level acoustic stimulation on susceptibility to noise in blue- and brown-eyed young human subjects.

OBJECTIVE: The aim of this study was to investigate the influence of pigmentation on the reduction in temporary noise-induced threshold shift (TTS) due to low level acoustic stimulation (LLAS). A pigmentation-dependent LLAS effect on TTS could be interpreted as a strial melanocyte involvement in LLAS. It could not be explained by cochlear sensorineural structure changes only. DESIGN: Teenagers were classified according to eye color (n = 6 + 6) and exposed to music at 70 dBA 6 h per day for 9 days (LLAS). TTS was measured before, during, and after the LLAS period. RESULTS: It was shown that LLAS reduced TTS significantly more in blue-eyed than in brown-eyed subjects. The difference in TTS remained for at least 1 wk after the cessation of LLAS. CONCLUSIONS: It is suggested that the observed difference in LLAS effect is due to strial melanocyte differences in free radical defense. It is also possible that other cochlear antioxidant enzyme systems, responsible for inactivation of harmful oxygen radicals and simultaneously involved in melanin synthesis such as the thioredoxin reductase/thioredoxin electron transfer system are activated.

Coping with noise-induced hearing loss: experiences from the perspective of middle-aged male victims.

The primary aim of this study was to describe coping with noise-induced hearing loss (NIHL) from the perspective of middle-aged men. An additional aim was to gain a deeper insight in their experiences of suffering from NIHL. Taped interviews were conducted with 53 consecutive patients. The interviews focused on the subjects' own descriptions of demanding auditory situations and what they did, thought and felt in these situations. The verbatim transcribed interview protocols were analysed by a method influenced by the tradition of grounded theory (Glaser and Strauss, 1967). The first important finding was that the coping model, consisting of controlling and avoiding strategies, described by Hallberg and Carlsson (1991), was validated by the present data. Second, it was shown that avoiding strategies dominated among men with NIHL, although situational circumstances, interactional conditions, social closeness and degrees of priority, awareness and acceptance sometimes changed the behaviour towards the more controlling strategies. Identification of these five factors should be regarded as an extension of the original model of coping, a third important contribution of this study. The driving force for coping was the hearing impaired person's striving to avoid definition as a deviant in social interactions in order to maintain a positive (normal) self-image. Health professionals are requested to encourage men with NIHL to recognize, confirm and actively seek solutions to problems caused by their hearing loss rather than to reinforce their denial of hearing difficulties.

Group rehabilitation of middle-aged males with noise-induced hearing loss and their spouses: evaluation of short- and long-term effects.

This study aims at evaluating the short- and long-term effects of a group rehabilitation programme, designed for males with noise-induced hearing loss and their spouses. The rehabilitation programme, including four 3 h group sessions per week, intended to offer adequate information, psychosocial support and training in effective coping strategies. Fifty-three males were consecutively selected and randomly divided into an experimental and a control group. Twenty-seven males were invited to participate in the group sessions together with their spouses. Although an informed consent to participate was given by the males initially, 15 couples (55.6%) refused to participate, and 12 couples agreed to participate in the group sessions. The short-term effects of the programme showed that the couples felt supported by meeting other couples in the same position. Further, the spouses' awareness of the effects of the hearing impairment was increased, which facilitated the understanding of problems related to their husbands' hearing disability. A reduction in perceived handicap, measured by the handicap sections of the Hearing Measurement Scale (P = 0.017) and by the disability/handicap factor of the Hearing Handicap and Support scale (P = 0.054), was found immediately after the rehabilitation programme in the participants compared to the controls. Four months later, no significant difference in any of the variables measured was found. Interestingly, both from a short-term and a long-term perspective, the drop-outs from the experimental group (n = 15) scored significantly higher on perceived handicap (P = 0.034 and P = 0.031, respectively) compared to the participants in the group rehabilitation programme.

The influence of a melanin-binding drug on temporary threshold shift in humans.

To evaluate the hypothesis that inner-ear melanin protects the sensory cells against harmful noise, noise-induced temporary threshold shift (TTS) was measured in 12 subjects during treatment with the melanin-binding drug, chloroquine, and after cessation of the drug. It was demonstrated that susceptibility to TTS was increased by chloroquine. This increase was most pronounced in young individuals, with dark complexion, and diminished with age. The chloroquine-induced increase in TTS in individuals with a light complexion was small and seemed unaffected by age. It could be argued that patients should avoid noise exposure during treatment with melanin-binding drugs.

Living with a male with noise-induced hearing loss: experiences from the perspective of spouses.

The aim of this inductive study was to describe, from the perspective of the spouses, their experiences of living close to a male with severe noise-induced hearing loss. An additional aim was to generate the first step of a theory of how these spouses managed their daily life. The study sample consisted of 10 strategically selected females who differed in age, educational status, number of children and years of marital relationship. Verbatim transcribed thematized interviews were analysed according to a method influenced by the constant comparative method for grounded theory. Two main variables, or core concepts, were identified: the husband's reluctance to acknowledge hearing difficulties and the impact of hearing loss on the intimate relationship. Combinations of these main variables were related to four qualitatively different strategies used by the spouses: co-acting, minimizing, mediating and distancing strategies. It is suggested that the type of strategy chosen by the spouse influences the outcome of the audiological rehabilitation.

The development of melanin in the stria vascularis of the gerbil.

One factor that influences noise susceptibility is pigmentation. The aim of this study was to investigate the development of melanocytes, other melanin-containing cells and the amount of melanin in stria vascularis from birth to adult age in the gerbil which has a uniform pigmentation of the fur and eyes, is born without hearing but establishes hearing function at 14-18 days after birth. Changes in the melanin morphology, concentration and distribution have been correlated to the development of the inner ear and to the time period during which hearing function is established, which indicates that the melanocytes in stria vascularis are of importance for the hearing function.

The influence of eye colour on susceptibility to TTS in humans.

In order to investigate the function of the inner ear melanin, noise-induced temporary hearing loss (temporary threshold shift, TTS) was studied in humans with either blue or brown iris colour. Sixty-eight normally hearing teenage boys participated in this study. Hearing thresholds before and after exposure were established with a computerized sweep frequency audiometer in the frequency range 0.8-8 kHz. The noise exposure consisted of a 1/3 octave band-filtered noise with centre frequency 2 kHz at 105 dB SPL for 10 min. The mean TTS in the frequency range 2-8 kHz showed a significant difference with the brown-eyed subjects developing least TTS, and the blue-eyed subjects most TTS.

The influence of inner ear melanin on susceptibility to TTS in humans.

In order to investigate the function of the inner ear melanin, the relationship between skin pigmentation and noise-induced temporary hearing loss (TTS) was studied. Forty-four normal-hearing Caucasian subjects were divided into three groups according to their sun sensitivity. Hearing thresholds before and after exposure were ascertained with a computerized sweep frequency audiometer in the frequency range 2-8 kHz. The noise exposure consisted of a 1/3-octave band-filtered noise with a centre frequency of 2 kHz at 105 dB SPL for 10 min. The mean TTS in the frequency range 2-8 kHz showed statistically significant differences between the three groups, i.e. the most pigmented subjects developed least TTS, and the least pigmented subjects most TTS.