Effects of genetic correction on the differentiation of hair cell-like cells from iPSCs with MYO15A mutation.

Effects of genetic correction on the differentiation of hair cell-like cells from iPSCs with MYO15A mutation.

Cell Death Differ. 2016 Feb 26;

Authors: Chen JR, Tang ZH, Zheng J, Shi HS, Ding J, Qian XD, Zhang C, Chen JL, Wang CC, Li L, Chen JZ, Yin SK, Shao JZ, Huang TS, Chen P, Guan MX, Wang JF

Abstract
Deafness or hearing loss is a major issue in human health. Inner ear hair cells are the main sensory receptors responsible for hearing. Defects in hair cells are one of the major causes of deafness. A combination of induced pluripotent stem cell (iPSC) technology with genome-editing technology may provide an attractive cell-based strategy to regenerate hair cells and treat hereditary deafness in humans. Here, we report the generation of iPSCs from members of a Chinese family carrying MYO15A c.4642G>A and c.8374G>A mutations and the induction of hair cell-like cells from those iPSCs. The compound heterozygous MYO15A mutations resulted in abnormal morphology and dysfunction of the derived hair cell-like cells. We used a CRISPR/Cas9 approach to genetically correct the MYO15A mutation in the iPSCs and rescued the morphology and function of the derived hair cell-like cells. Our data demonstrate the feasibility of generating inner ear hair cells from human iPSCs and the functional rescue of gene mutation-based deafness by using genetic correction.Cell Death and Differentiation advance online publication, 26 February 2016; doi:10.1038/cdd.2016.16.

PMID: 26915297 [PubMed - as supplied by publisher]

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Vestibular Assessment and Rehabilitation: Ten-Year Survey Trends of Audiologists' Opinions and Practice.

Related Articles

Vestibular Assessment and Rehabilitation: Ten-Year Survey Trends of Audiologists' Opinions and Practice.

J Am Acad Audiol. 2016 Feb;27(2):126-40

Authors: Nelson MD, Akin FW, Riska KM, Andresen K, Mondelli SS

Abstract
BACKGROUND: The past decade has yielded changes in the education and training of audiologists and technological advancements that have become widely available for clinical balance function testing. It is unclear if recent advancements in vestibular instrumentation or the transition to an AuD degree have affected audiologists' vestibular clinical practice or opinions.
PURPOSE: The purpose of this study was to examine predominant opinions and practices for vestibular assessment (VA) and vestibular rehabilitation (VR) over the past decade and between master's- and AuD-level audiologists.
METHOD: A 31-question survey was administered to audiologists via U.S. mail in 2003 (N = 7,500) and electronically in 2014 (N = 9,984) with a response rate of 12% and 10%, respectively.
RESULTS: There was an increase in the number of audiologists providing vestibular services in the past decade. Most respondents agreed that audiologists were the most qualified professionals to conduct VA. Less than half of the surveyed audiologists felt that graduate training was adequate for VA. AuD-level audiologists were more satisfied with graduate training and felt more comfortable performing VA compared to master's-level audiologists. Few respondents agreed that audiologists were the most qualified professionals to conduct VR or that graduate training prepared them to conduct VR. The basic vestibular test battery was unchanged across surveys and included: calorics, smooth pursuit, saccades, search for spontaneous, positional, gaze and optokinetic nystagmus, Dix-Hallpike, case history, and hearing evaluation. There was a trend toward greater use of air (versus water) calorics, videonystagmography (versus electronystagmography), and additional tests of vestibular and balance function.
CONCLUSIONS: VA is a growing specialty area in the field of audiology. Better training opportunities are needed to increase audiologists' knowledge and skills for providing vestibular services. The basic tests performed during VA have remained relatively unchanged over the past 10 yr.

PMID: 26905532 [PubMed - in process]

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A Distributed Network for Social Cognition Enriched for Oxytocin Receptors.

A Distributed Network for Social Cognition Enriched for Oxytocin Receptors.

J Neurosci. 2016 Feb 24;36(8):2517-35

Authors: Mitre M, Marlin BJ, Schiavo JK, Morina E, Norden SE, Hackett TA, Aoki CJ, Chao MV, Froemke RC

Abstract
UNLABELLED: Oxytocin is a neuropeptide important for social behaviors such as maternal care and parent-infant bonding. It is believed that oxytocin receptor signaling in the brain is critical for these behaviors, but it is unknown precisely when and where oxytocin receptors are expressed or which neural circuits are directly sensitive to oxytocin. To overcome this challenge, we generated specific antibodies to the mouse oxytocin receptor and examined receptor expression throughout the brain. We identified a distributed network of female mouse brain regions for maternal behaviors that are especially enriched for oxytocin receptors, including the piriform cortex, the left auditory cortex, and CA2 of the hippocampus. Electron microscopic analysis of the cerebral cortex revealed that oxytocin receptors were mainly expressed at synapses, as well as on axons and glial processes. Functionally, oxytocin transiently reduced synaptic inhibition in multiple brain regions and enabled long-term synaptic plasticity in the auditory cortex. Thus modulation of inhibition may be a general mechanism by which oxytocin can act throughout the brain to regulate parental behaviors and social cognition.
SIGNIFICANCE STATEMENT: Oxytocin is an important peptide hormone involved in maternal behavior and social cognition, but it has been unclear what elements of neural circuits express oxytocin receptors due to the paucity of suitable antibodies. Here, we developed new antibodies to the mouse oxytocin receptor. Oxytocin receptors were found in discrete brain regions and at cortical synapses for modulating excitatory-inhibitory balance and plasticity. These antibodies should be useful for future studies of oxytocin and social behavior.

PMID: 26911697 [PubMed - in process]

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Vestibular Assessment and Rehabilitation: Ten-Year Survey Trends of Audiologists' Opinions and Practice.

Related Articles

Vestibular Assessment and Rehabilitation: Ten-Year Survey Trends of Audiologists' Opinions and Practice.

J Am Acad Audiol. 2016 Feb;27(2):126-40

Authors: Nelson MD, Akin FW, Riska KM, Andresen K, Mondelli SS

Abstract
BACKGROUND: The past decade has yielded changes in the education and training of audiologists and technological advancements that have become widely available for clinical balance function testing. It is unclear if recent advancements in vestibular instrumentation or the transition to an AuD degree have affected audiologists' vestibular clinical practice or opinions.
PURPOSE: The purpose of this study was to examine predominant opinions and practices for vestibular assessment (VA) and vestibular rehabilitation (VR) over the past decade and between master's- and AuD-level audiologists.
METHOD: A 31-question survey was administered to audiologists via U.S. mail in 2003 (N = 7,500) and electronically in 2014 (N = 9,984) with a response rate of 12% and 10%, respectively.
RESULTS: There was an increase in the number of audiologists providing vestibular services in the past decade. Most respondents agreed that audiologists were the most qualified professionals to conduct VA. Less than half of the surveyed audiologists felt that graduate training was adequate for VA. AuD-level audiologists were more satisfied with graduate training and felt more comfortable performing VA compared to master's-level audiologists. Few respondents agreed that audiologists were the most qualified professionals to conduct VR or that graduate training prepared them to conduct VR. The basic vestibular test battery was unchanged across surveys and included: calorics, smooth pursuit, saccades, search for spontaneous, positional, gaze and optokinetic nystagmus, Dix-Hallpike, case history, and hearing evaluation. There was a trend toward greater use of air (versus water) calorics, videonystagmography (versus electronystagmography), and additional tests of vestibular and balance function.
CONCLUSIONS: VA is a growing specialty area in the field of audiology. Better training opportunities are needed to increase audiologists' knowledge and skills for providing vestibular services. The basic tests performed during VA have remained relatively unchanged over the past 10 yr.

PMID: 26905532 [PubMed - in process]

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Higher prevalence of autoimmune diseases and longer spells of vertigo in patients affected with familial Ménière's disease: A clinical comparison of familial and sporadic Ménière's disease.

Related Articles

Higher prevalence of autoimmune diseases and longer spells of vertigo in patients affected with familial Ménière's disease: A clinical comparison of familial and sporadic Ménière's disease.

Am J Audiol. 2014 Jun;23(2):232-7

Authors: Hietikko E, Sorri M, Männikkö M, Kotimäki J

Abstract
PURPOSE This study compared clinical features, predisposing factors, and concomitant diseases between sporadic and familial Ménière's disease (MD). METHOD Retrospective chart review and postal questionnaire were used. Participants were 250 definite patients with MD (sporadic, n =149; familial, n = 101) who fulfilled the American Academy of Otorhinolaryngology-Head and Neck Surgery (1995) criteria. RESULTS On average, familial patients were affected 5.6 years earlier than sporadic patients, and they suffered from significantly longer spells of vertigo (p = .007). The prevalence of rheumatoid arthritis (p = .002) and other autoimmune diseases (p = .046) was higher among the familial patients, who also had more migraine (p = .036) and hearing impairment (p = .002) in their families. CONCLUSION The clinical features of familial and sporadic MD are very similar in general, but some differences do exist. Familial MD patients are affected earlier and suffer from longer spells of vertigo.

PMID: 24686733 [PubMed - indexed for MEDLINE]

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Cerebral Processing of Emotionally Loaded Acoustic Signals by Tinnitus Patients

This exploratory study determined the activation pattern in nonauditory brain areas in response to acoustic, emotionally positive, negative or neutral stimuli presented to tinnitus patients and control subjects. Ten patients with chronic tinnitus and without measurable hearing loss and 13 matched control subjects were included in the study and subjected to fMRI with a 1.5-tesla scanner. During the scanning procedure, acoustic stimuli of different emotional value were presented to the subjects. Statistical analyses were performed using statistical parametric mapping (SPM 99). The activation pattern induced by emotionally loaded acoustic stimuli differed significantly within and between both groups tested, depending on the kind of stimuli used. Within-group differences included the limbic system, prefrontal regions, temporal association cortices and striatal regions. Tinnitus patients had a pronounced involvement of limbic regions involved in the processing of chimes (positive stimulus) and neutral words (neutral stimulus), strongly suggesting improperly functioning inhibitory mechanisms that were functioning well in the control subjects. This study supports the hypothesis about the existence of a tinnitus-specific brain network. Such a network could respond to any acoustic stimuli by activating limbic areas involved in stress reactivity and emotional processing and by reducing activation of areas responsible for attention and acoustic filtering (thalamus, frontal regions), possibly reinforcing negative effects of tinnitus.
Audiol Neurotol 2016;21:80-87

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Music Lovers and Hearing Aids

Some people cannot imagine life without music - it relaxes, inspires, and for many, completes their lives. Music has a different meaning for each person, and there are individual preferences, but most would agree that music listening should be enjoyable. This seemingly simple task easily can be undermined when hearing loss is present, as listening to just a limited part of the dynamics, or a reduced frequency range of our favorite music could significantly reduce the enjoyment of the experience. Moreover, in several cases, this problem cannot be fixed with modern hearing aids.

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