Hearing loss is one of the major medical concerns in girls and women with Turner syndrome (TS) and has a negative effect on well-being and quality of everyday life. Sensorineural hearing loss is the ...most common type of hearing loss, affecting more than half of adults with TS. Karyotypes with a loss of the short p-arm on the X-chromosome are more prone to ear and hearing problems. The importance of detecting, investigating, and treating hearing loss with hearing aids cannot be emphasized enough. The pathophysiology of the sensorineural hearing loss in TS is not known, but theories regarding estrogen deficiency, the cell cycle delay hypothesis, IGF-1 deficiency and the possible role of the KDM6A gene are discussed. Due to the diversity of symptoms and conditions within the same karyotype, a combination of genetic factors altered by epigenetic and/or hormonal effects is probable. Further research is needed regarding the pathophysiology of ear and hearing problems in TS to develop new treatment methods.
Objective:
To investigate hearing thresholds in children born with cleft palate and in children with otitis media with effusion but no cleft palate.
Design:
Prospective longitudinal group comparison ...study.
Setting:
University hospital.
Participants:
Sixteen children born with nonsyndromic cleft palate with or without cleft lip (CP±L) and 15 age-matched children with otitis media with effusion (OME) but without cleft.
Main Outcome Measures:
Hearing was tested at repeated occasions beginning with neonatal auditory brainstem response (ABR) at 1-4 months of age, and age-appropriate hearing tests from 9 to 36 months of age.
Results:
The median ABR thresholds in both groups were elevated but did not differ significantly. At 12 months of age, the median 4 frequency averages at 500-1000-2000-4000 Hz (4FA) were indicative of mild hearing loss but significantly better in the CP±L-group than in the group without cleft (P < .01). There were no significant group-wise differences regarding the median 4FA at 24 and 36 months of age, and at 36 months, the median 4FA were normal in both groups. Both groups exhibited a significant improvement over time from the neonatal ABR thresholds to the 4FA at 36 months (CP±L-group P < .05; without CP±L-group P < .01).
Conclusion:
The hearing loss in children with CP±L was not more severe than among children with OME but without cleft palate; rather, at 12 months of age, the thresholds were significantly better in the CP±L-group than those in the group without cleft. The air conduction thresholds improved with age in both groups.
This study was conducted to compare the pattern of age-related hearing decline in individuals with and without self-reported previous occupational noise exposure. This was a prospective, ...population-based, longitudinal study of individuals aged 70-75 years, from an epidemiological investigation, comprising three age cohorts. In total there were 1013 subjects (432 men and 581 women). Participants were tested with pure tone audiometry, and they answered a questionnaire to provide information regarding number of years of occupational noise exposure. There were no significant differences in hearing decline, at any frequency, for those aged 70-75 years between the noise-exposed (N= 62 men, 22 women) and the nonexposed groups (N = 96 men, 158 women). This study supports the additive model of noise-induced hearing loss (NIHL) and age-related hearing loss (ARHL). The concept of different patterns of hearing decline between persons exposed and not exposed to noise could not be verified.
Background/Aim: Central auditory processing disorder (CAPD) might precede the onset of Alzheimer’s disease (AD). A method of evaluating CAPD is the dichotic digits test (DDT). The aim was to address ...this in a longitudinal setting. Methods: A total of 136 individuals were assessed with peripheral and central hearing tests at baseline and at 5-year follow-up. Results: Subjects with AD showed a significant decline in DDT scores of the right ear from baseline to follow-up. The other groups retained high DDT scores. Peripheral auditory function declined as expected according to age. Conclusions: Our study indicates that DDT performance reflects an ongoing process resulting in dementia.
Conclusion. Hormone replacement therapy (HRT) may have a protective effect on hearing impairment in postmenopausal women. New guidelines for classification of audiometric configuration in age-related ...hearing loss are suggested. Objectives. To describe prevalence of hearing loss and audiometric configuration in a group of middle-aged women with respect to menopausal stage and HRT. Subjects and methods. A total of 143 women around menopause were sampled through the Swedish population register. The mean hearing threshold levels were compared according to menopausal status. The audiograms in the 57 women with hearing loss were classified according to audiometric configuration. Results. In all, 57 women (40%) had any kind of hearing loss; 42 had very minute hearing loss; 15 had a 4FA (average of thresholds at 0.5, 1, 2, and 4 kHz) of at least 20-39 dB HL in at least one ear. Two of these had a 4FA of 40-69 dB HL in at least one ear. The most common configurations were: gently sloping (47%), steeply sloping (14%), and high-frequency U-shaped (14%). The postmenopausal women who were not on HRT had poorer hearing mainly at 2 and 3 kHz, compared with pre- and perimenopausal women, and postmenopausal women on HRT.
Background/Aims: Central auditory function can be studied to monitor the progression of mild cognitive impairment to dementia. Our aim was to address this issue in a prospective longitudinal setting. ...Methods: Tests of central hearing function were performed on 70 subjects with either Alzheimer's disease (AD) or mild cognitive impairment, and in controls with subjective memory complaints but normal cognition. The time span until follow-up was 1.5 years. Results: The dichotic digit free recall test showed a significant decline in the AD group compared with the controls (left ear). Conclusion: The short time span was long enough to disclose a central auditory processing decline in AD.
to investigate auditory function in subjects with early Alzheimer's disease, mild cognitive impairment and with subjective memory complaints, in search of signs of central auditory processing ...dysfunction even in early stages of cognitive impairment.
a consecutive group of men and women, referred to the Memory Clinic at the Karolinska University Hospital, was approached for inclusion in this prospective study. One hundred and thirty-six subjects, mean age 64 years (range 50-78 years), diagnosed with Alzheimer's disease (n = 43), mild cognitive impairment (n = 59) or with subjective memory complaints (n = 34), were included.
auditory function was assessed with pure tone audiometry, speech perception in quiet and in background noise and dichotic digits tests with two or three digits.
pure tone audiometry and speech perception scores in quiet and in background noise were normal for age and without between-group differences. Dichotic digits tests showed strongly significant differences between the three groups, where the Alzheimer's disease group performed significantly poorer than the other two groups, with the mild cognitive impairment group in an intermediate position.
our results demonstrate that central auditory processing dysfunction is highly evident in subjects with Alzheimer's disease, and to a considerable extent even in subjects with mild cognitive impairment.
Abstract Objective Children with cleft lip and palate have a high prevalence of otitis media with effusion (OME) which is often associated with a fluctuating, conductive hearing loss in the low and ...mid-frequencies and a risk for permanent hearing loss in the higher frequencies. Although common, there is no consensus on the treatment of OME with ventilation tubes. The aim of this study is to document if the risk for permanent hearing loss and acquired cholesteatoma increases due to treatment with ventilation tubes (VT-treatments) during childhood in a group of children with cleft lip and palate. Methods A retrospective medical chart review of 33 children (25 boys and 8 girls) born with unilateral cleft lip and palate (UCLP) was completed. Audiological data (results of hearing sensitivity tests, the total number of hearing tests, and number of VT treatments) were extracted from medical records from when the children were 4-7 and >7-10 years of age. Results The hearing thresholds in the speech frequencies improved with age (p < 0,05) but a minority of the children continued to present with elevated hearing thresholds in the higher frequencies at >7-10 years of age. There were no significant correlations between number of VTtreatments and hearing thresholds at >7-10 years. Four of the 33 children presented with complications: two children exhibited perforations of the ear drum (6. 1%) and two children developed unilateral cholesteatoma (6. 1%). Conclusion In the current study, the hearing sensitivity of children with cleft lip and palate improved with age. However, this improvement was not seen in the higher frequencies. Twelve percent of the children experienced complications following VT treatments. Due to these complications, it is recommended that all children with cleft palate should have routine follow-ups by an ENT doctor and audiologist. As part of the routine follow-up care, hearing assessments should be performed before and after VT-treatments.
AbstractConclusions: An unexpected rapid hearing decline remained after the 10-year follow up, similar to the hearing decline in 70-year-old women in reference materials. No clear changes concerning ...hearing in the peri- and postmenopausal period were noted. Objective: To assess whether hearing decline correlates with menopause and/or cortisol blood levels. Methods: A prospective individual longitudinal study of peri-menopausal women followed for 10 years was performed at baseline, and after 2, 7 and 10 years, respectively. With a starting age of around 51 years, 100 women remained in the study after 10 years. Pure-tone audiometry and cortisol blood testing were performed at all visits. Results: A continuous hearing decline, at all frequencies, was found during the follow-up time. The rate of decline during the menopausal period was higher than compared with reference materials for the same age group. The correlation with time for menopause is most apparent at 1 and 3 kHz where the hearing decline is more rapid after menopause than before. Serum cortisol levels did not correlate with rate of hearing decline.
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