Relations Between Self-Reported Daily-Life Fatigue, Hearing Status, and Pupil Dilation During a Speech Perception in Noise Task.

Objective: People with hearing impairment are likely to experience higher levels of fatigue because of effortful listening in daily communication. This hearing-related fatigue might not only constrain their work performance but also result in withdrawal from major social roles. Therefore, it is important to understand the relationships between fatigue, listening effort, and hearing impairment by examining the evidence from both subjective and objective measurements. The aim of the present study was to investigate these relationships by assessing subjectively measured daily-life fatigue (self-report questionnaires) and objectively measured listening effort (pupillometry) in both normally hearing and hearing-impaired participants. Design: Twenty-seven normally hearing and 19 age-matched participants with hearing impairment were included in this study. Two self-report fatigue questionnaires Need For Recovery and Checklist Individual Strength were given to the participants before the test session to evaluate the subjectively measured daily fatigue. Participants were asked to perform a speech reception threshold test with single-talker masker targeting a 50% correct response criterion. The pupil diameter was recorded during the speech processing, and we used peak pupil dilation (PPD) as the main outcome measure of the pupillometry. Results: No correlation was found between subjectively measured fatigue and hearing acuity, nor was a group difference found between the normally hearing and the hearing-impaired participants on the fatigue scores. A significant negative correlation was found between self-reported fatigue and PPD. A similar correlation was also found between Speech Intelligibility Index required for 50% correct and PPD. Multiple regression analysis showed that factors representing "hearing acuity" and "self-reported fatigue" had equal and independent associations with the PPD during the speech in noise test. Less fatigue and better hearing acuity were associated with a larger pupil dilation. Conclusions: To the best of our knowledge, this is the first study to investigate the relationship between a subjective measure of daily-life fatigue and an objective measure of pupil dilation, as an indicator of listening effort. These findings help to provide an empirical link between pupil responses, as observed in the laboratory, and daily-life fatigue. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Three-Dimensional Analysis of the Fundus of the Human Internal Acoustic Canal.

Objectives: Documentation of the nerve components in the internal acoustic canal is essential before cochlea implantation surgery. Interpretations may be challenged by wide anatomical variations of the VIIIth nerve and their ramifications. Malformations may further defy proper nerve identification. Design: Using microcomputed tomography, we analyzed the fundus bone channels in an archival collection of 113 macerated human temporal bones and 325 plastic inner molds. Data were subsequently processed by volume-rendering software using a bony tissue algorithm. Three-dimensional reconstructions were made, and through orthogonal sections, the topographic anatomy was established. Results: The technique provided additional information regarding the anatomy of the nerve foramina/channels of the human fundus region, including variations and destinations. Channel anastomosis were found beyond the level of the fundus. A foramen of the transverse crest was identified. Conclusions: Three-dimensional reconstructions and cropping outlined the bone canals and demonstrated the highly variable VIIIth nerve anatomy at the fundus of the human inner acoustic canal. Myriad channel interconnections suggested an intricate system of neural interactive pathways in humans. Particularly striking was the variable anatomy of the saccule nerve channels. The results may assist in the preoperative interpretation of the VIIIth nerve anatomy. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Relations Between Self-Reported Daily-Life Fatigue, Hearing Status, and Pupil Dilation During a Speech Perception in Noise Task.

Objective: People with hearing impairment are likely to experience higher levels of fatigue because of effortful listening in daily communication. This hearing-related fatigue might not only constrain their work performance but also result in withdrawal from major social roles. Therefore, it is important to understand the relationships between fatigue, listening effort, and hearing impairment by examining the evidence from both subjective and objective measurements. The aim of the present study was to investigate these relationships by assessing subjectively measured daily-life fatigue (self-report questionnaires) and objectively measured listening effort (pupillometry) in both normally hearing and hearing-impaired participants. Design: Twenty-seven normally hearing and 19 age-matched participants with hearing impairment were included in this study. Two self-report fatigue questionnaires Need For Recovery and Checklist Individual Strength were given to the participants before the test session to evaluate the subjectively measured daily fatigue. Participants were asked to perform a speech reception threshold test with single-talker masker targeting a 50% correct response criterion. The pupil diameter was recorded during the speech processing, and we used peak pupil dilation (PPD) as the main outcome measure of the pupillometry. Results: No correlation was found between subjectively measured fatigue and hearing acuity, nor was a group difference found between the normally hearing and the hearing-impaired participants on the fatigue scores. A significant negative correlation was found between self-reported fatigue and PPD. A similar correlation was also found between Speech Intelligibility Index required for 50% correct and PPD. Multiple regression analysis showed that factors representing "hearing acuity" and "self-reported fatigue" had equal and independent associations with the PPD during the speech in noise test. Less fatigue and better hearing acuity were associated with a larger pupil dilation. Conclusions: To the best of our knowledge, this is the first study to investigate the relationship between a subjective measure of daily-life fatigue and an objective measure of pupil dilation, as an indicator of listening effort. These findings help to provide an empirical link between pupil responses, as observed in the laboratory, and daily-life fatigue. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Three-Dimensional Analysis of the Fundus of the Human Internal Acoustic Canal.

Objectives: Documentation of the nerve components in the internal acoustic canal is essential before cochlea implantation surgery. Interpretations may be challenged by wide anatomical variations of the VIIIth nerve and their ramifications. Malformations may further defy proper nerve identification. Design: Using microcomputed tomography, we analyzed the fundus bone channels in an archival collection of 113 macerated human temporal bones and 325 plastic inner molds. Data were subsequently processed by volume-rendering software using a bony tissue algorithm. Three-dimensional reconstructions were made, and through orthogonal sections, the topographic anatomy was established. Results: The technique provided additional information regarding the anatomy of the nerve foramina/channels of the human fundus region, including variations and destinations. Channel anastomosis were found beyond the level of the fundus. A foramen of the transverse crest was identified. Conclusions: Three-dimensional reconstructions and cropping outlined the bone canals and demonstrated the highly variable VIIIth nerve anatomy at the fundus of the human inner acoustic canal. Myriad channel interconnections suggested an intricate system of neural interactive pathways in humans. Particularly striking was the variable anatomy of the saccule nerve channels. The results may assist in the preoperative interpretation of the VIIIth nerve anatomy. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Relations Between Self-Reported Daily-Life Fatigue, Hearing Status, and Pupil Dilation During a Speech Perception in Noise Task.

Objective: People with hearing impairment are likely to experience higher levels of fatigue because of effortful listening in daily communication. This hearing-related fatigue might not only constrain their work performance but also result in withdrawal from major social roles. Therefore, it is important to understand the relationships between fatigue, listening effort, and hearing impairment by examining the evidence from both subjective and objective measurements. The aim of the present study was to investigate these relationships by assessing subjectively measured daily-life fatigue (self-report questionnaires) and objectively measured listening effort (pupillometry) in both normally hearing and hearing-impaired participants. Design: Twenty-seven normally hearing and 19 age-matched participants with hearing impairment were included in this study. Two self-report fatigue questionnaires Need For Recovery and Checklist Individual Strength were given to the participants before the test session to evaluate the subjectively measured daily fatigue. Participants were asked to perform a speech reception threshold test with single-talker masker targeting a 50% correct response criterion. The pupil diameter was recorded during the speech processing, and we used peak pupil dilation (PPD) as the main outcome measure of the pupillometry. Results: No correlation was found between subjectively measured fatigue and hearing acuity, nor was a group difference found between the normally hearing and the hearing-impaired participants on the fatigue scores. A significant negative correlation was found between self-reported fatigue and PPD. A similar correlation was also found between Speech Intelligibility Index required for 50% correct and PPD. Multiple regression analysis showed that factors representing "hearing acuity" and "self-reported fatigue" had equal and independent associations with the PPD during the speech in noise test. Less fatigue and better hearing acuity were associated with a larger pupil dilation. Conclusions: To the best of our knowledge, this is the first study to investigate the relationship between a subjective measure of daily-life fatigue and an objective measure of pupil dilation, as an indicator of listening effort. These findings help to provide an empirical link between pupil responses, as observed in the laboratory, and daily-life fatigue. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Three-Dimensional Analysis of the Fundus of the Human Internal Acoustic Canal.

Objectives: Documentation of the nerve components in the internal acoustic canal is essential before cochlea implantation surgery. Interpretations may be challenged by wide anatomical variations of the VIIIth nerve and their ramifications. Malformations may further defy proper nerve identification. Design: Using microcomputed tomography, we analyzed the fundus bone channels in an archival collection of 113 macerated human temporal bones and 325 plastic inner molds. Data were subsequently processed by volume-rendering software using a bony tissue algorithm. Three-dimensional reconstructions were made, and through orthogonal sections, the topographic anatomy was established. Results: The technique provided additional information regarding the anatomy of the nerve foramina/channels of the human fundus region, including variations and destinations. Channel anastomosis were found beyond the level of the fundus. A foramen of the transverse crest was identified. Conclusions: Three-dimensional reconstructions and cropping outlined the bone canals and demonstrated the highly variable VIIIth nerve anatomy at the fundus of the human inner acoustic canal. Myriad channel interconnections suggested an intricate system of neural interactive pathways in humans. Particularly striking was the variable anatomy of the saccule nerve channels. The results may assist in the preoperative interpretation of the VIIIth nerve anatomy. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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An Objective Method to Determine the Electrically Evoked Stapedius Reflex Threshold During Cochlea Implantation.

Objective: In cochlea implantation, assessment of the electrically evoked stapedius reflex threshold (eSRT) provides information for postoperative adjustment of the speech processor. This is used to prevent undesired overstimulation. To this day, the reflex is visually identified by the surgeon. However, this method may be prone to error due to ventilation-associated situs excursions, blinking, or other distractions. Therefore, the aim of this study was to establish an objective and automatized method to determine the eSRT. Methods: Video material documenting the intraoperative measurements was analyzed offline applying a tracking software to quantify stapes head movements. Moreover, a computer program (TrackerAnalyzer) was developed to detect reflex associated movements automatically. Results: Reflex responses seen visually by the surgeon compared with visual registration on the video material postoperatively and to results of the automatized method with TrackerAnalyzer showed a high inter-rater agreement (Cohen's kappa coefficient = 0.47-0.59). 82% of events in which the TrackerAnalyzer registered reflexes that were not seen by the surgeon had values close but lower to the intraoperatively determined eSRT (mean -1.02 current units). Conclusion: We demonstrated the feasibility of an objective and automatized method to determine electrically evoked stapedius reflex thresholds in cochlea implantation. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Korean Version of the Voice Symptom Scale for Patients with Thyroid Operation, and Its Use in a Validation and Reliability Study

Publication date: Available online 8 November 2017
Source:Journal of Voice
Author(s): Hee Young Son, Chang-Yoon Lee, Kyung A. Kim, Sina Kim, Hee Seok Jeong, Jin Pyeong Kim
ObjectivesThe Voice Symptom Scale (VoiSS) questionnaire is a self-reported measure of voice function. Compared with previous voice-rating tools, the VoiSS focuses more on communication difficulties, pharyngeal symptoms, and psychosocial distress. This study aimed to translate the VoiSS into the Korean language, validate it, and assess its reliability.Study DesignThis is a prospective patient study utilizing questionnaires and acoustic analysis.MethodsA recognized methodology was used to translate the questionnaires. The final Korean version was used in 31 patients scheduled for thyroidectomy between November 2013 and February 2015 for preoperative voice assessment, and at 2 weeks, 3 months, and 6 months postoperatively. The participants included had no specific vocal disorders before surgery and no vocal cord paralysis after surgery, and completed the Korean versions of the VoiSS and Voice Handicap Index (VHI), and acoustic analysis.ResultsThe Korean version of the VoiSS demonstrated high internal consistency (α = 0.97) and test-retest reliability of its subscales. There was a significant correlation between the VoiSS and VHI scores in the total thyroidectomy group at each time-point. With regard to subjective symptoms, the VoiSS appeared to be more accurate than the VHI in terms of physical and functional subscales.ConclusionsThe Korean version of the VoiSS is ready for use for the assessment of voice dysfunction in Korean patients. It is an applicable and useful supplementary tool for evaluating patients' perceptions of voice dysfunction after thyroidectomy, for identifying multiple factors affecting patients' voices, and for measuring treatment efficacy before and after therapeutic intervention.



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Kallmann Syndrome: Phenotype and Genotype of Hypogonadotropic Hypogonadism.

Related Articles

Kallmann Syndrome: Phenotype and Genotype of Hypogonadotropic Hypogonadism.

Metabolism. 2017 Nov 03;:

Authors: Stamou MI, Georgopoulos NA

Abstract
Isolated Gonadotropin-Releasing Hormone (GnRH) Deficiency (IGD) IGD is a genetically and clinically heterogeneous disorder. Mutations in many different genes are able to explain ~40% of the causes of IGD, with the rest of cases remaining genetically uncharacterized. While most mutations are inherited in X-linked, autosomal dominant, or autosomal recessive pattern, several IGD genes are shown to interact with each other in an oligogenic manner. In addition, while the genes involved in the pathogenesis of IGD act on either neurodevelopmental or neuroendocrine pathways, a subset of genes are involved in both pathways, acting as "overlap genes". Thus, some IGD genes play the role of the modifier genes or "second hits", providing an explanation for incomplete penetrance and variable expressivity associated with some IGD mutations. The clinical spectrum of IGD includes a variety of disorders including Kallmann Syndrome (KS), i.e. hypogonadotropic hypogonadism with anosmia, and its normosmic variation normosmic idiopathic hypogonadotropic hypogonadism (nIHH), which represent the most severe aspects of the disorder. Apart from these disorders, there are also "milder" and more common reproductive diseases associated with IGD, including hypothalamic amenorrhea (HA), constitutional delay of puberty (CDP) and adult-onset hypogonadotropic hypogonadism (AHH). Interestingly, neurodeveloplmental genes are associated with the KS form of IGD, due to the topographical link between the GnRH neurons and the olfactory placode. On the other hand, neuroendocrine genes are mostly linked to nIHH. However, a great deal of clinical and genetic overlap characterizes the spectrum of the IGD disorders. IGD is also characterized by a wide variety of non- reproductive features, including midline facial defects such as cleft lip and/ or palate, renal agenesis, short metacarpals and other bone abnormalities, hearing loss, synkinesia, eye movement abnormalities, poor balance due to cerebellar ataxia, etc. Therefore, genetic screening should be offered in patients with IGD, as it can provide valuable information for genetic counseling and further understanding of IGD.

PMID: 29108899 [PubMed - as supplied by publisher]

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Kallmann Syndrome: Phenotype and Genotype of Hypogonadotropic Hypogonadism.

Related Articles

Kallmann Syndrome: Phenotype and Genotype of Hypogonadotropic Hypogonadism.

Metabolism. 2017 Nov 03;:

Authors: Stamou MI, Georgopoulos NA

Abstract
Isolated Gonadotropin-Releasing Hormone (GnRH) Deficiency (IGD) IGD is a genetically and clinically heterogeneous disorder. Mutations in many different genes are able to explain ~40% of the causes of IGD, with the rest of cases remaining genetically uncharacterized. While most mutations are inherited in X-linked, autosomal dominant, or autosomal recessive pattern, several IGD genes are shown to interact with each other in an oligogenic manner. In addition, while the genes involved in the pathogenesis of IGD act on either neurodevelopmental or neuroendocrine pathways, a subset of genes are involved in both pathways, acting as "overlap genes". Thus, some IGD genes play the role of the modifier genes or "second hits", providing an explanation for incomplete penetrance and variable expressivity associated with some IGD mutations. The clinical spectrum of IGD includes a variety of disorders including Kallmann Syndrome (KS), i.e. hypogonadotropic hypogonadism with anosmia, and its normosmic variation normosmic idiopathic hypogonadotropic hypogonadism (nIHH), which represent the most severe aspects of the disorder. Apart from these disorders, there are also "milder" and more common reproductive diseases associated with IGD, including hypothalamic amenorrhea (HA), constitutional delay of puberty (CDP) and adult-onset hypogonadotropic hypogonadism (AHH). Interestingly, neurodeveloplmental genes are associated with the KS form of IGD, due to the topographical link between the GnRH neurons and the olfactory placode. On the other hand, neuroendocrine genes are mostly linked to nIHH. However, a great deal of clinical and genetic overlap characterizes the spectrum of the IGD disorders. IGD is also characterized by a wide variety of non- reproductive features, including midline facial defects such as cleft lip and/ or palate, renal agenesis, short metacarpals and other bone abnormalities, hearing loss, synkinesia, eye movement abnormalities, poor balance due to cerebellar ataxia, etc. Therefore, genetic screening should be offered in patients with IGD, as it can provide valuable information for genetic counseling and further understanding of IGD.

PMID: 29108899 [PubMed - as supplied by publisher]

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Variants in CIB2 cause DFNB48 and not USH1J.

Variants in CIB2 cause DFNB48 and not USH1J.

Clin Genet. 2017 Nov 07;:

Authors: Booth KT, Kahrizi K, Babanejad M, Daghagh H, Bademci G, Arzhangi S, Zareabdollahi D, Duman D, El-Amraoui A, Tekin M, Najmabadi H, Azaiez H, Smith RJ

Abstract
The genetic, mutational and phenotypic spectrum of deafness-causing genes shows great diversity and pleiotropy. The best examples are the group of genes, which when mutated can either cause non-syndromic hearing loss (NSHL) or the most common dual sensory impairment, Usher Syndrome (USH). Variants in the CIB2 gene have been previously reported to cause of hearing loss at the DFNB48 locus and deaf-blindness at the USH1J locus. In this study, we characterize the phenotypic spectrum in a multiethnic cohort with autosomal recessive non-syndromic hearing loss (ARNSHL) due to variants in the CIB2 gene. Of the 6 families we ascertained, 3 segregated novel loss-of-function variants, 2 families segregated missense variants (one novel) and 1 family segregated a previously reported pathogenic variant in trans with a frameshift variant. This report is the first to show that biallelic loss of function variants in CIB2 cause ARNSHL and not USH. In the era of precision medicine, providing the correct diagnosis (NSHL vs USH) is essential for patient care as it impacts potential intervention and prevention options for patients. Here we provide evidence disqualifying CIB2 as an USH-causing gene.

PMID: 29112224 [PubMed - as supplied by publisher]

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An Application of NGS for Molecular Investigations in Perrault Syndrome: Study of 14 Families and Review of the Literature.

Related Articles

An Application of NGS for Molecular Investigations in Perrault Syndrome: Study of 14 Families and Review of the Literature.

Hum Mutat. 2016 Dec;37(12):1354-1362

Authors: Lerat J, Jonard L, Loundon N, Christin-Maitre S, Lacombe D, Goizet C, Rouzier C, Van Maldergem L, Gherbi S, Garabedian EN, Bonnefont JP, Touraine P, Mosnier I, Munnich A, Denoyelle F, Marlin S

Abstract
Perrault syndrome (PS) is a rare autosomal recessive condition characterized by deafness and gonadic dysgenesis. Recently, mutations in five genes have been identified: C10orf2, CLPP, HARS2, HSD17B4, and LARS2. Probands included are presented with sensorineural deafness associated with gonadic dysgenesis. DNA was sequenced using next-generation sequencing (NGS) with a panel of 35 deafness genes including the five Perrault genes. Exonic variations known as pathogenic mutations or detected with <1% frequency in public databases were extracted and subjected to segregation analysis within each family. Both mutations and low coverage regions were analyzed by Sanger sequencing. Fourteen female index patients were included. The screening in four cases has been extended to four family members presenting with PS phenotype. For four unrelated patients (28.6%), causative mutations were identified: three homozygous mutations in C10orf2, CLPP, and HARS2, and one compound heterozygous mutation in LARS2. Three additional heterozygous mutations in LARS2 and HSD17B4 were found in three independent familial cases. All these missense mutations were verified by Sanger sequencing. Familial segregation analyses confirmed the molecular diagnosis in all cases carrying biallelic mutations. Because of NGS, molecular analysis confirmed the clinical diagnosis of PS in 28.6% of our cohort and four novel mutations were found in four Perrault genes. For the unsolved cases, exome sequencing should be performed to search for a sixth unknown PS gene.

PMID: 27650058 [PubMed - indexed for MEDLINE]

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