Is Hearing Loss Associated with Poorer Health in Older Adults Who Might Benefit from Hearing Screening?.

Objectives: Hearing screening programs may benefit adults with unacknowledged or unaddressed hearing loss, but there is limited evidence regarding whether such programs are effective at improving health outcomes. The objective was to determine if poorer audiometric hearing thresholds are associated with poorer cognition, social isolation, burden of physical or mental health, inactivity due to poor physical or mental health, depression, and overnight hospitalizations among older American adults with unacknowledged or unaddressed hearing loss. Design: The authors performed a cross-sectional population-based analysis of older American adults with normal hearing or unacknowledged or unaddressed hearing loss. Data was obtained from the 1999 to 2010 cycles of the National Health and Nutrition Examination Survey. Participants with a pure-tone average (PTA in the better hearing ear of thresholds at 0.5, 1, 2, and 4 kHz) > 25 dB HL who self-reported their hearing ability to be "good" or "excellent" were categorized as having "unacknowledged" hearing loss. Those who had a PTA > 25 dB HL and who self-reported hearing problems but had never had a hearing test or worn a hearing aid were categorized as having "unaddressed" hearing loss. Multivariate regression was performed to account for confounding due to demographic and health variables. Results: A 10 dB increase in PTA was associated with a 52% increased odds of social isolation among 60- to 69-year-olds in multivariate analyses (p = 0.001). The average Digit Symbol Substitution Test score dropped by 2.14 points per 10 dB increase in PTA (p = 0.03), a magnitude equivalent to the drop expected for 3.9 years of chronological aging. PTA was not associated significantly with falls, hospitalizations, burden of physical or mental health, or depression, or social isolation among those aged 70 years or older in these samples. Conclusion: Unacknowledged or unaddressed hearing loss was associated with a significantly increased risk of social isolation among 60- to 69-year-olds but not those 70 years or older. It was also associated with lower cognitive scores on the Digit Symbol Substitution Test among 60- to 69-year-olds. This study differs from prior studies by focusing specifically on older adults who have unacknowledged or unaddressed hearing loss because they are the most likely to benefit from pure-tone hearing screening. The finding of associations between hearing loss and measures of social isolation and cognition in these specific samples extends previous findings on unrestricted samples of older adults including those who had already acknowledged hearing problems. Future randomized controlled trials measuring the effectiveness of adult hearing screening programs should measure whether interventions have an effect on these measures in those who have unacknowledged or unaddressed pure-tone hearing loss. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Is Hearing Loss Associated with Poorer Health in Older Adults Who Might Benefit from Hearing Screening?.

Objectives: Hearing screening programs may benefit adults with unacknowledged or unaddressed hearing loss, but there is limited evidence regarding whether such programs are effective at improving health outcomes. The objective was to determine if poorer audiometric hearing thresholds are associated with poorer cognition, social isolation, burden of physical or mental health, inactivity due to poor physical or mental health, depression, and overnight hospitalizations among older American adults with unacknowledged or unaddressed hearing loss. Design: The authors performed a cross-sectional population-based analysis of older American adults with normal hearing or unacknowledged or unaddressed hearing loss. Data was obtained from the 1999 to 2010 cycles of the National Health and Nutrition Examination Survey. Participants with a pure-tone average (PTA in the better hearing ear of thresholds at 0.5, 1, 2, and 4 kHz) > 25 dB HL who self-reported their hearing ability to be "good" or "excellent" were categorized as having "unacknowledged" hearing loss. Those who had a PTA > 25 dB HL and who self-reported hearing problems but had never had a hearing test or worn a hearing aid were categorized as having "unaddressed" hearing loss. Multivariate regression was performed to account for confounding due to demographic and health variables. Results: A 10 dB increase in PTA was associated with a 52% increased odds of social isolation among 60- to 69-year-olds in multivariate analyses (p = 0.001). The average Digit Symbol Substitution Test score dropped by 2.14 points per 10 dB increase in PTA (p = 0.03), a magnitude equivalent to the drop expected for 3.9 years of chronological aging. PTA was not associated significantly with falls, hospitalizations, burden of physical or mental health, or depression, or social isolation among those aged 70 years or older in these samples. Conclusion: Unacknowledged or unaddressed hearing loss was associated with a significantly increased risk of social isolation among 60- to 69-year-olds but not those 70 years or older. It was also associated with lower cognitive scores on the Digit Symbol Substitution Test among 60- to 69-year-olds. This study differs from prior studies by focusing specifically on older adults who have unacknowledged or unaddressed hearing loss because they are the most likely to benefit from pure-tone hearing screening. The finding of associations between hearing loss and measures of social isolation and cognition in these specific samples extends previous findings on unrestricted samples of older adults including those who had already acknowledged hearing problems. Future randomized controlled trials measuring the effectiveness of adult hearing screening programs should measure whether interventions have an effect on these measures in those who have unacknowledged or unaddressed pure-tone hearing loss. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Is Hearing Loss Associated with Poorer Health in Older Adults Who Might Benefit from Hearing Screening?.

Objectives: Hearing screening programs may benefit adults with unacknowledged or unaddressed hearing loss, but there is limited evidence regarding whether such programs are effective at improving health outcomes. The objective was to determine if poorer audiometric hearing thresholds are associated with poorer cognition, social isolation, burden of physical or mental health, inactivity due to poor physical or mental health, depression, and overnight hospitalizations among older American adults with unacknowledged or unaddressed hearing loss. Design: The authors performed a cross-sectional population-based analysis of older American adults with normal hearing or unacknowledged or unaddressed hearing loss. Data was obtained from the 1999 to 2010 cycles of the National Health and Nutrition Examination Survey. Participants with a pure-tone average (PTA in the better hearing ear of thresholds at 0.5, 1, 2, and 4 kHz) > 25 dB HL who self-reported their hearing ability to be "good" or "excellent" were categorized as having "unacknowledged" hearing loss. Those who had a PTA > 25 dB HL and who self-reported hearing problems but had never had a hearing test or worn a hearing aid were categorized as having "unaddressed" hearing loss. Multivariate regression was performed to account for confounding due to demographic and health variables. Results: A 10 dB increase in PTA was associated with a 52% increased odds of social isolation among 60- to 69-year-olds in multivariate analyses (p = 0.001). The average Digit Symbol Substitution Test score dropped by 2.14 points per 10 dB increase in PTA (p = 0.03), a magnitude equivalent to the drop expected for 3.9 years of chronological aging. PTA was not associated significantly with falls, hospitalizations, burden of physical or mental health, or depression, or social isolation among those aged 70 years or older in these samples. Conclusion: Unacknowledged or unaddressed hearing loss was associated with a significantly increased risk of social isolation among 60- to 69-year-olds but not those 70 years or older. It was also associated with lower cognitive scores on the Digit Symbol Substitution Test among 60- to 69-year-olds. This study differs from prior studies by focusing specifically on older adults who have unacknowledged or unaddressed hearing loss because they are the most likely to benefit from pure-tone hearing screening. The finding of associations between hearing loss and measures of social isolation and cognition in these specific samples extends previous findings on unrestricted samples of older adults including those who had already acknowledged hearing problems. Future randomized controlled trials measuring the effectiveness of adult hearing screening programs should measure whether interventions have an effect on these measures in those who have unacknowledged or unaddressed pure-tone hearing loss. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Decoding four different sound-categories in the auditory cortex using functional near-infrared spectroscopy

Publication date: Available online 29 January 2016
Source:Hearing Research
Author(s): Keum-Shik Hong, Hendrik Santosa
The ability of the auditory cortex in the brain to distinguish different sounds is important in daily life. This study investigated whether activations in the auditory cortex caused by different sounds can be distinguished using functional near-infrared spectroscopy (fNIRS). The hemodynamic responses (HRs) in both hemispheres using fNIRS were measured in 18 subjects while exposing them to four sound categories (English-speech, non-English-speech, annoying sounds, and nature sounds). As features for classifying the different signals, the mean, slope, and skewness of the oxy-hemoglobin (HbO) signal were used. With regard to the language-related stimuli, the HRs evoked by understandable speech (English) were observed in a broader brain region than were those evoked by non-English speech. Also, the magnitudes of the HbO signals evoked by English-speech were higher than those of non-English speech. The ratio of the peak values of non-English and English speech was 72.5%. Also, the brain region evoked by annoying sounds was wider than that by nature sounds. However, the signal strength for nature sounds was stronger than that for annoying sounds. Finally, for brain-computer interface (BCI) purposes, the linear discriminant analysis (LDA) and support vector machine (SVM) classifiers were applied to the four sound categories. The overall classification performance for the left hemisphere was higher than that for the right hemisphere. Therefore, for decoding of auditory commands, the left hemisphere is recommended. Also, in two-class classification, the annoying vs. nature sounds comparison provides a higher classification accuracy than the English vs. non-English speech comparison. Finally, LDA performs better than SVM.



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Decoding four different sound-categories in the auditory cortex using functional near-infrared spectroscopy

Publication date: Available online 29 January 2016
Source:Hearing Research
Author(s): Keum-Shik Hong, Hendrik Santosa
The ability of the auditory cortex in the brain to distinguish different sounds is important in daily life. This study investigated whether activations in the auditory cortex caused by different sounds can be distinguished using functional near-infrared spectroscopy (fNIRS). The hemodynamic responses (HRs) in both hemispheres using fNIRS were measured in 18 subjects while exposing them to four sound categories (English-speech, non-English-speech, annoying sounds, and nature sounds). As features for classifying the different signals, the mean, slope, and skewness of the oxy-hemoglobin (HbO) signal were used. With regard to the language-related stimuli, the HRs evoked by understandable speech (English) were observed in a broader brain region than were those evoked by non-English speech. Also, the magnitudes of the HbO signals evoked by English-speech were higher than those of non-English speech. The ratio of the peak values of non-English and English speech was 72.5%. Also, the brain region evoked by annoying sounds was wider than that by nature sounds. However, the signal strength for nature sounds was stronger than that for annoying sounds. Finally, for brain-computer interface (BCI) purposes, the linear discriminant analysis (LDA) and support vector machine (SVM) classifiers were applied to the four sound categories. The overall classification performance for the left hemisphere was higher than that for the right hemisphere. Therefore, for decoding of auditory commands, the left hemisphere is recommended. Also, in two-class classification, the annoying vs. nature sounds comparison provides a higher classification accuracy than the English vs. non-English speech comparison. Finally, LDA performs better than SVM.



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Decoding four different sound-categories in the auditory cortex using functional near-infrared spectroscopy

Publication date: Available online 29 January 2016
Source:Hearing Research
Author(s): Keum-Shik Hong, Hendrik Santosa
The ability of the auditory cortex in the brain to distinguish different sounds is important in daily life. This study investigated whether activations in the auditory cortex caused by different sounds can be distinguished using functional near-infrared spectroscopy (fNIRS). The hemodynamic responses (HRs) in both hemispheres using fNIRS were measured in 18 subjects while exposing them to four sound categories (English-speech, non-English-speech, annoying sounds, and nature sounds). As features for classifying the different signals, the mean, slope, and skewness of the oxy-hemoglobin (HbO) signal were used. With regard to the language-related stimuli, the HRs evoked by understandable speech (English) were observed in a broader brain region than were those evoked by non-English speech. Also, the magnitudes of the HbO signals evoked by English-speech were higher than those of non-English speech. The ratio of the peak values of non-English and English speech was 72.5%. Also, the brain region evoked by annoying sounds was wider than that by nature sounds. However, the signal strength for nature sounds was stronger than that for annoying sounds. Finally, for brain-computer interface (BCI) purposes, the linear discriminant analysis (LDA) and support vector machine (SVM) classifiers were applied to the four sound categories. The overall classification performance for the left hemisphere was higher than that for the right hemisphere. Therefore, for decoding of auditory commands, the left hemisphere is recommended. Also, in two-class classification, the annoying vs. nature sounds comparison provides a higher classification accuracy than the English vs. non-English speech comparison. Finally, LDA performs better than SVM.



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Decoding four different sound-categories in the auditory cortex using functional near-infrared spectroscopy

Publication date: Available online 29 January 2016
Source:Hearing Research
Author(s): Keum-Shik Hong, Hendrik Santosa
The ability of the auditory cortex in the brain to distinguish different sounds is important in daily life. This study investigated whether activations in the auditory cortex caused by different sounds can be distinguished using functional near-infrared spectroscopy (fNIRS). The hemodynamic responses (HRs) in both hemispheres using fNIRS were measured in 18 subjects while exposing them to four sound categories (English-speech, non-English-speech, annoying sounds, and nature sounds). As features for classifying the different signals, the mean, slope, and skewness of the oxy-hemoglobin (HbO) signal were used. With regard to the language-related stimuli, the HRs evoked by understandable speech (English) were observed in a broader brain region than were those evoked by non-English speech. Also, the magnitudes of the HbO signals evoked by English-speech were higher than those of non-English speech. The ratio of the peak values of non-English and English speech was 72.5%. Also, the brain region evoked by annoying sounds was wider than that by nature sounds. However, the signal strength for nature sounds was stronger than that for annoying sounds. Finally, for brain-computer interface (BCI) purposes, the linear discriminant analysis (LDA) and support vector machine (SVM) classifiers were applied to the four sound categories. The overall classification performance for the left hemisphere was higher than that for the right hemisphere. Therefore, for decoding of auditory commands, the left hemisphere is recommended. Also, in two-class classification, the annoying vs. nature sounds comparison provides a higher classification accuracy than the English vs. non-English speech comparison. Finally, LDA performs better than SVM.



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Decoding four different sound-categories in the auditory cortex using functional near-infrared spectroscopy

Publication date: Available online 29 January 2016
Source:Hearing Research
Author(s): Keum-Shik Hong, Hendrik Santosa
The ability of the auditory cortex in the brain to distinguish different sounds is important in daily life. This study investigated whether activations in the auditory cortex caused by different sounds can be distinguished using functional near-infrared spectroscopy (fNIRS). The hemodynamic responses (HRs) in both hemispheres using fNIRS were measured in 18 subjects while exposing them to four sound categories (English-speech, non-English-speech, annoying sounds, and nature sounds). As features for classifying the different signals, the mean, slope, and skewness of the oxy-hemoglobin (HbO) signal were used. With regard to the language-related stimuli, the HRs evoked by understandable speech (English) were observed in a broader brain region than were those evoked by non-English speech. Also, the magnitudes of the HbO signals evoked by English-speech were higher than those of non-English speech. The ratio of the peak values of non-English and English speech was 72.5%. Also, the brain region evoked by annoying sounds was wider than that by nature sounds. However, the signal strength for nature sounds was stronger than that for annoying sounds. Finally, for brain-computer interface (BCI) purposes, the linear discriminant analysis (LDA) and support vector machine (SVM) classifiers were applied to the four sound categories. The overall classification performance for the left hemisphere was higher than that for the right hemisphere. Therefore, for decoding of auditory commands, the left hemisphere is recommended. Also, in two-class classification, the annoying vs. nature sounds comparison provides a higher classification accuracy than the English vs. non-English speech comparison. Finally, LDA performs better than SVM.



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Brain's 'amplifier' compensates for lost inner ear function

Researchers from Massachusetts Eye and Ear/Harvard Medical School have described, for the first time, the adult brain's ability to compensate for a near-complete loss of auditory nerve fibers that...

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Brain's 'amplifier' compensates for lost inner ear function

Researchers from Massachusetts Eye and Ear/Harvard Medical School have described, for the first time, the adult brain's ability to compensate for a near-complete loss of auditory nerve fibers that...

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A Challenging Form of Non-autoimmune Insulin-Dependent Diabetes in a Wolfram Syndrome Patient with a Novel Sequence Variant.

A Challenging Form of Non-autoimmune Insulin-Dependent Diabetes in a Wolfram Syndrome Patient with a Novel Sequence Variant.

J Diabetes Metab. 2015 Jun;6(7):1-5

Authors: Paris LP, Usui Y, Serino J, Sá J, Friedlander M

Abstract
Wolfram syndrome type 1 is a rare, autosomal recessive, neurodegenerative disorder that is diagnosed when insulin-dependent diabetes of non-auto-immune origin and optic atrophy are concomitantly present. Wolfram syndrome is also designated by DIDMOAD that stands for its most frequent manifestations: diabetes insipidus, diabetes mellitus, optic atrophy and deafness. With disease progression, patients also commonly develop severe neurological and genito-urinary tract abnormalities. When compared to the general type 1 diabetic population, patients with Wolfram Syndrome have been reported to have a form of diabetes that is more easily controlled and with less microvascular complications, such as diabetic retinopathy. We report a case of Wolfram syndrome in a 16-year-old male patient who presented with progressive optic atrophy and severe diabetes with very challenging glycemic control despite intensive therapy since diagnosis at the age of 6. Despite inadequate metabolic control he did not develop any diabetic microvascular complications during the 10-year follow-up period. To further investigate potential causes for this metabolic idiosyncrasy, we performed genetic analyses that revealed a novel combination of homozygous sequence variants that are likely the cause of the syndrome in this family. The identified genotype included a novel sequence variant in the Wolfram syndrome type 1 gene along with a previously described one, which had initially been associated with isolated low frequency sensorineural hearing loss (LFSNHL). Interestingly, our patient did not show any abnormal findings with audiometry testing.

PMID: 26819810 [PubMed - as supplied by publisher]

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Identification of a recurrent mitochondrial mutation in a Japanese family with palmoplantar keratoderma, nail dystrophy, and deafness.

Related Articles

Identification of a recurrent mitochondrial mutation in a Japanese family with palmoplantar keratoderma, nail dystrophy, and deafness.

Eur J Dermatol. 2015 Jan-Feb;25(1):79-81

Authors: Hayashi R, Fujiwara H, Morishita M, Ito M, Shimomura Y

PMID: 25513986 [PubMed - indexed for MEDLINE]

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MEKK4 Signaling Regulates Sensory Cell Development and Function in the Mouse Inner Ear.

MEKK4 Signaling Regulates Sensory Cell Development and Function in the Mouse Inner Ear.

J Neurosci. 2016 Jan 27;36(4):1347-61

Authors: Haque K, Pandey AK, Zheng HW, Riazuddin S, Sha SH, Puligilla C

Abstract
UNLABELLED: Mechanosensory hair cells (HCs) residing in the inner ear are critical for hearing and balance. Precise coordination of proliferation, sensory specification, and differentiation during development is essential to ensure the correct patterning of HCs in the cochlear and vestibular epithelium. Recent studies have revealed that FGF20 signaling is vital for proper HC differentiation. However, the mechanisms by which FGF20 signaling promotes HC differentiation remain unknown. Here, we show that mitogen-activated protein 3 kinase 4 (MEKK4) expression is highly regulated during inner ear development and is critical to normal cytoarchitecture and function. Mice homozygous for a kinase-inactive MEKK4 mutation exhibit significant hearing loss. Lack of MEKK4 activity in vivo also leads to a significant reduction in the number of cochlear and vestibular HCs, suggesting that MEKK4 activity is essential for overall development of HCs within the inner ear. Furthermore, we show that loss of FGF20 signaling in vivo inhibits MEKK4 activity, whereas gain of Fgf20 function stimulates MEKK4 expression, suggesting that Fgf20 modulates MEKK4 activity to regulate cellular differentiation. Finally, we demonstrate, for the first time, that MEKK4 acts as a critical node to integrate FGF20-FGFR1 signaling responses to specifically influence HC development and that FGFR1 signaling through activation of MEKK4 is necessary for outer hair cell differentiation. Collectively, this study provides compelling evidence of an essential role for MEKK4 in inner ear morphogenesis and identifies the requirement of MEKK4 expression in regulating the specific response of FGFR1 during HC development and FGF20/FGFR1 signaling activated MEKK4 for normal sensory cell differentiation.
SIGNIFICANCE STATEMENT: Sensory hair cells (HCs) are the mechanoreceptors within the inner ear responsible for our sense of hearing. HCs are formed before birth, and mammals lack the ability to restore the sensory deficits associated with their loss. In this study, we show, for the first time, that MEKK4 signaling is essential for the development of normal cytoarchitecture and hearing function as MEKK4 signaling-deficient mice exhibit a significant reduction of HCs and a hearing loss. We also identify MEKK4 as a critical hub kinase for FGF20-FGFR1 signaling to induce HC differentiation in the mammalian cochlea. These results reveal a new paradigm in the regulation of HC differentiation and provide significant new insights into the mechanism of Fgf signaling governing HC formation.

PMID: 26818521 [PubMed - in process]

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MEKK4 Signaling Regulates Sensory Cell Development and Function in the Mouse Inner Ear.

MEKK4 Signaling Regulates Sensory Cell Development and Function in the Mouse Inner Ear.

J Neurosci. 2016 Jan 27;36(4):1347-61

Authors: Haque K, Pandey AK, Zheng HW, Riazuddin S, Sha SH, Puligilla C

Abstract
UNLABELLED: Mechanosensory hair cells (HCs) residing in the inner ear are critical for hearing and balance. Precise coordination of proliferation, sensory specification, and differentiation during development is essential to ensure the correct patterning of HCs in the cochlear and vestibular epithelium. Recent studies have revealed that FGF20 signaling is vital for proper HC differentiation. However, the mechanisms by which FGF20 signaling promotes HC differentiation remain unknown. Here, we show that mitogen-activated protein 3 kinase 4 (MEKK4) expression is highly regulated during inner ear development and is critical to normal cytoarchitecture and function. Mice homozygous for a kinase-inactive MEKK4 mutation exhibit significant hearing loss. Lack of MEKK4 activity in vivo also leads to a significant reduction in the number of cochlear and vestibular HCs, suggesting that MEKK4 activity is essential for overall development of HCs within the inner ear. Furthermore, we show that loss of FGF20 signaling in vivo inhibits MEKK4 activity, whereas gain of Fgf20 function stimulates MEKK4 expression, suggesting that Fgf20 modulates MEKK4 activity to regulate cellular differentiation. Finally, we demonstrate, for the first time, that MEKK4 acts as a critical node to integrate FGF20-FGFR1 signaling responses to specifically influence HC development and that FGFR1 signaling through activation of MEKK4 is necessary for outer hair cell differentiation. Collectively, this study provides compelling evidence of an essential role for MEKK4 in inner ear morphogenesis and identifies the requirement of MEKK4 expression in regulating the specific response of FGFR1 during HC development and FGF20/FGFR1 signaling activated MEKK4 for normal sensory cell differentiation.
SIGNIFICANCE STATEMENT: Sensory hair cells (HCs) are the mechanoreceptors within the inner ear responsible for our sense of hearing. HCs are formed before birth, and mammals lack the ability to restore the sensory deficits associated with their loss. In this study, we show, for the first time, that MEKK4 signaling is essential for the development of normal cytoarchitecture and hearing function as MEKK4 signaling-deficient mice exhibit a significant reduction of HCs and a hearing loss. We also identify MEKK4 as a critical hub kinase for FGF20-FGFR1 signaling to induce HC differentiation in the mammalian cochlea. These results reveal a new paradigm in the regulation of HC differentiation and provide significant new insights into the mechanism of Fgf signaling governing HC formation.

PMID: 26818521 [PubMed - in process]

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