Ear and hearing problems in 44 middle-aged women with Turner's syndrome

Hultcrantz, Malou; Sylvén, Lisskulla; Borg, Erik

doi:10.1016/0378-5955(94)90094-9

Cited by 90 publications

(80 citation statements)

References 16 publications

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“…Auditory processing, as assessed by auditory brainstem responses (ABRs), is more efficient in females relative to males (Jerger and Hall, 1980). Furthermore, women with Turner's syndrome, who are deficient in E2, exhibit hearing disorders and lower auditory processing efficiency (Hultcrantz et al, 1994;Hultcrantz and Sylvén, 1997;Güngör et al, 2000). These findings are consistent with results obtained in rodents and monkeys, in which ovariectomy alters ABRs (Coleman et al, 1994;Golub et al, 2004); after E2 replacement, however, normal ABRs are reinstated (Coleman et al, 1994).…”

Section: Introductionsupporting

confidence: 81%

Estradiol Shapes Auditory Processing in the Adult Brain by Regulating Inhibitory Transmission and Plasticity-Associated Gene Expression

Tremere¹,

Jeong²,

Pinaud³

2009

J. Neurosci.

137

219

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Estradiol impacts a wide variety of brain processes, including sex differentiation, mood, and learning. Here we show that estradiol regulates auditory processing of acoustic signals in the vertebrate brain, more specifically in the caudomedial nidopallium (NCM), the songbird analog of the mammalian auditory association cortex. Multielectrode recordings coupled with local pharmacological manipulations in awake animals reveal that both exogenous and locally generated estradiol increase auditory-evoked activity in NCM. This enhancement in neuronal responses is mediated by suppression of local inhibitory transmission. Surprisingly, we also found that estradiol is both necessary and sufficient for the induction of multiple mitogen-activated protein kinase (MAPK)-dependent genes thought to be required for synaptic plasticity and memorization of birdsong. Specifically, we show that local blockade of estrogen receptors or aromatase activity in awake birds decrease song-induced MAPK-dependent gene expression. Infusions of estradiol in acoustically isolated birds induce transcriptional activation of these genes to levels comparable with song-stimulated animals. Our results reveal acute and rapid nongenomic functions for estradiol in central auditory physiology and suggest that such roles may be ubiquitously expressed across sensory systems.

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Section: Introductionsupporting

confidence: 81%

Estradiol Shapes Auditory Processing in the Adult Brain by Regulating Inhibitory Transmission and Plasticity-Associated Gene Expression

Tremere¹,

Jeong²,

Pinaud³

2009

J. Neurosci.

137

219

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“…However, the present investigation did not confirm the hypothesis that E alone has a protective effect on the auditory system (10,11,23,24) because no differences were observed between the hearing abilities of females taking E relative to those who had never taken HRT (i.e., NHRT).…”

Section: Discussioncontrasting

confidence: 53%

“…Some studies suggest a protective effect of E, reporting that estrogen replacement therapy may delay hearing loss in postmenopausal females and that there is an association between low serum E levels and hearing loss (8,9). Others, investigating hearing problems in Turner's syndrome (10,11), suggested that the lack of E may influence hearing in those patients and may induce premature aging of the auditory system.…”

mentioning

confidence: 99%

Progestin negatively affects hearing in aged women

Guimaraes

Frisina²,

Mapes³

et al. 2006

Proc. Natl. Acad. Sci. U.S.A.

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Female hormone influences on auditory system aging are not completely understood. Because of widespread clinical use of hormone replacement therapy (HRT), it is critical to understand HRT effects on sensory systems. The present study retrospectively analyzed and compared hearing abilities among 124 postmenopausal women taking HRT, treated with estrogen and progestin (E؉P; n ‫؍‬ 32), estrogen alone (E; n ‫؍‬ 30), and a third [non-hormone replacement therapy (NHRT; n ‫؍‬ 62)] control group. Subjects were 60 -86 years old and were matched for age and health status. All had relatively healthy medical histories and no significant noise exposure, middle-ear problems, or major surgeries. Hearing tests included pure-tone audiometry, tympanometry, distortion-product otoacoustic emissions (DPOAEs), transient otoacoustic emissions, and the hearing-in-noise test (HINT). The HINT tests for speech perception in background noise, the major complaint of hearing-impaired persons. Pure-tone thresholds in both ears were elevated (poorer) for the E؉P relative to the E and control groups. For DPOAEs, the E؉P group presented with lower (worse) levels than the E and control groups, with significant differences for both ears. For the HINT results, the E؉P group had poorer speech perception than the E and control groups across all background noise speaker locations and in quiet. These findings suggest that the presence of P as a component of HRT results in poorer hearing abilities in aged women taking HRT, affecting both the peripheral (ear) and central (brain) auditory systems, and it interferes with the perception of speech in background noise.estrogen ͉ hearing loss ͉ hormone replacement therapy ͉ presbycusis ͉ progesterone A ge-related hearing loss (presbycusis) is the number one communication disorder, and it is one of the top three chronic medical conditions of elderly persons. Because of the widespread prescription of hormone replacement therapy (HRT), it is critical to determine the effects of HRT on sensory systems in postmenopausal females. Sensory function declines with age, yet the effects of HRT on hearing, balance, vision, and the chemical senses are not assessed in HRT drug development.Estrogen (E) and progestin (P) actions have been linked to key sensory and CNS processes and disorders such as cognition, memory, dementia (Alzheimer's disease), epilepsy, depression, and others. For instance, Rice et al.(1) reported differences between E alone and EϩP in rates of cognitive decline, showing E alone as beneficial and the presence of P as detrimental. Shumaker et al. (2) reported that the use of EϩP increased the risk for dementia in elderly females. Klaiber et al. (3,4) demonstrated that the effects of P seem opposed to those of E for mood changes. Stein and Hoffman (5) reported opposite effects of E and P in the treatment of acute brain trauma, attributing to P overall qualities of a neuroprotection agent. Klein et al. (6) suggested a protective effect of E on eye-lens opacities.The effects of sex hormones on hearing and ...

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“…In addition, a progressive midfrequency sensorineural hearing loss often occurs, which necessitates the use of hearing aids in some children 49 and more than 25% of women by their mid-forties. 50 …”

Section: Hearing Lossmentioning

confidence: 99%

Health Supervision for Children With Turner Syndrome

Frías¹,

Davenport²

2003

Pediatrics

109

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ABSTRACT. This report is designed to assist the pediatrician in caring for the child in whom the diagnosis of Turner syndrome has been confirmed by karyotyping. The report is meant to serve as a supplement to the American Academy of Pediatrics' "Recommendations for Preventive Pediatric Care" and emphasizes the importance of continuity of care and the need to avoid its fragmentation by ensuring a medical home for every girl with Turner syndrome. The pediatrician's first contact with a child with Turner syndrome may occur during infancy or childhood. This report also discusses interactions with expectant parents who have been given the prenatal diagnosis of Turner syndrome and have been referred for advice.

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Ear and hearing problems in 44 middle-aged women with Turner's syndrome

Cited by 90 publications

References 16 publications

Estradiol Shapes Auditory Processing in the Adult Brain by Regulating Inhibitory Transmission and Plasticity-Associated Gene Expression

Estradiol Shapes Auditory Processing in the Adult Brain by Regulating Inhibitory Transmission and Plasticity-Associated Gene Expression

Progestin negatively affects hearing in aged women

Health Supervision for Children With Turner Syndrome

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