Christina Hederstierna

Hearing in women at menopause. Prevalence of hearing loss, audiometric configuration and relation to hormone replacement therapy

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.

Central auditory function in early Alzheimer's disease and in mild cognitive impairment.

OBJECTIVE to investigate auditory function in subjects with early Alzheimers 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. DESIGN AND SUBJECTS 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 Alzheimers disease (n = 43), mild cognitive impairment (n = 59) or with subjective memory complaints (n = 34), were included. METHODS 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. RESULTS 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 Alzheimers disease group performed significantly poorer than the other two groups, with the mild cognitive impairment group in an intermediate position. CONCLUSIONS our results demonstrate that central auditory processing dysfunction is highly evident in subjects with Alzheimers disease, and to a considerable extent even in subjects with mild cognitive impairment.

A longitudinal study of hearing decline in women with Turner syndrome

Conclusion: Young and middle-aged women with Turner syndrome (TS) have a progressive type of hearing impairment, deteriorating rapidly in adult age. The hearing decline seems to consist of two patterns: a mid-frequency dip, likely of a genetic origin, and a high-frequency loss resembling age-related hearing impairment – possibly influenced by estrogen deficiency. Objectives: This was a longitudinal study of hearing thresholds in a group of women with TS that also aimed to determine whether the factors initial age, initial hearing level, karyotype, and presence/absence of a mid-frequency dip influence the rate of decline and could serve as prognostic markers. Subjects and methods: Audiograms of air conduction thresholds in 69 women with TS (aged 28–62 years) were performed twice with an average interval of 10 years. Results: The rate of hearing decline is much higher in women with TS than in age-matched women from the general population. The decline rate is comparable to that seen in 70–90-year-old women, regardless of initial age, hearing level, karyotype, or presence of a mid-frequency dip. The rate of decline is especially high in the high-frequency region, 0.8–2.2 dB per year. The presence of a mid-frequency dip is an especially strong predictor for a future high rate of hearing decline with subsequent social consequences.

The menopause triggers hearing decline in healthy women

BACKGROUND Epidemiological studies have shown that women have better high-frequency thresholds than men in virtually all age groups, and that age-related hearing decline starts after 30 in men but not until after the age of 50 in women. This coincides with the menopausal transition in most women, thus leading us to hypothesize that the menopause triggers auditory deterioration, possibly due to reduced levels of endogenous estrogens, which are known to have protective effects on the auditory system. METHODS 104 women with a mean age 51.2 at baseline, were tested with pure tone audiometry twice with an average interval of 7.5 years. The age at the final menstrual period (FMP) was reported by all women. Hearing decline at individual frequencies was calculated. RESULTS Women with a FMP 0-4 years ago, had a rate of high frequency hearing decline of 0.9-1.5dB/year in the left ear, those with 5-7 years since the FMP had a corresponding rate of 1.1-1.5dB/year in the right ear, and 8-13 years after the FMP the decline was more subtle, 0.7-1.1dB/year in both ears. CONCLUSION The menopause appears to act as a trigger of a relatively rapid age-related hearing decline in healthy women, starting in the left ear.

Estrogen and hearing from a clinical point of view; characteristics of auditory function in women with Turner syndrome

Turner syndrome is a chromosomal aberration affecting 1:2000 newborn girls, in which all or part of one X chromosome is absent. This leads to ovarial dysgenesis and little or no endogenous estrogen production. These women have, among many other syndromal features, a high occurrence of ear and hearing problems, and neurocognitive dysfunctions, including reduced visual-spatial abilities; it is assumed that estrogen deficiency is at least partially responsible for these problems. In this, study 30 Turner women aged 40-67, with mild to moderate hearing loss, performed a battery of hearing tests aimed at localizing the lesion causing the sensorineural hearing impairment and assessing central auditory function, primarily sound localization. The results of TEOAE, ABR and speech recognition scores in noise were all indicative of cochlear dysfunction as the cause of the sensorineural impairment. Phase audiometry, a test for sound localization, showed mild disturbances in the Turner women compared to the reference group, suggesting that auditory-spatial dysfunction is another facet of the recognized neurocognitive phenotype in Turner women.

Hearing of 75-year old persons over three decades: Has hearing changed?

Abstract Objective: The state of hearing in 75-year old persons was measured in a population based epidemiological study with the aim of studying if hearing had changed during a time span of 29 years. Design: An epidemiological study of generational effects in three age cohorts. Study sample: Three age cohorts were included: cohort 1 (n: 267) born in 1976–77, cohort 4 (n: 197) in 1990–91, and cohort 6 (n: 570) in 2005. The same test procedures using pure-tone audiometry and a short questionnaire were applied to the three cohorts of 75-year old residents in the same city. Results: The hearing was essentially unchanged during the span of the investigation—almost three decades. Low-frequency hearing was up to about 10 dB poorer in the most recently studied cohort compared to the previously studied cohorts. The reason for this difference is considered to depend on methodological factors. Self-assessed hearing and tinnitus was mainly unchanged, or had minor changes both to the better and to the worse. Conclusions: The hearing, both measured with pure-tone audiometry and with a short questionnaire, of 75-year old persons has not changed at all, or only marginally, over three decades.

Hearing decline in menopausal women – a 10-year follow-up

Abstract Conclusions: 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.

Short-Term Longitudinal Study of Central Auditory Function in Alzheimer's Disease and Mild Cognitive Impairment

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 Alzheimers 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.

The Prevalence of Connexin 26 Mutations in the Swedish Population

Mutations in GJB2, the gene encoding the protein Connexin 26, have been shown to account for as much as 50% of autosomal recessive, non-syndromic childhood hearing loss (ARNSHL). Early, correct diagnosis and intervention have greatly improved the possibilities for these children in learning and developing language skills. In recent years, many reports from varied parts of the world have described the local scene of mutations in Connexin 26. The prevalence differs with geographic location and assessment procedures. Mutations in Connexin 26 have until recently been identified in very few cases in Sweden, Norway and Denmark, and nationwide population samples have not been studied in the Scandinavian countries. In this study we present the results of a large nationwide and a regional study of the prevalence of the 35delG, L90P and 167delT mutations of the GJB2 gene in Sweden. A total of 2052 samples (dried blood spots) were analysed. 1501 samples were collected representing all of Sweden and 551 samples from northern Sweden. The allele frequencies in Sweden were found to be 35delG, 0.83%; L90P, 0.30%; and 167delT, 0%. The prevalence of mutations in Connexin 26 in Sweden seems to be comparable to that in other northern European countries.

Age-related hearing decline in individuals with and without occupational noise exposure

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.