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Hearing Disorders HELP
Based on 25,036 articles published since 2009
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These are the 25036 published articles about Hearing Disorders that originated from Worldwide during 2009-2019.
 
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5 · 6 · 7 · 8 · 9 · 10 · 11 · 12 · 13 · 14 · 15 · 16 · 17 · 18 · 19 · 20
401 Review [Thiamine-responsive megaloblastic anemia or Rogers syndrome: A literature review]. 2019

Lu, H / Lu, H / Vaucher, J / Tran, C / Vollenweider, P / Castioni, J. ·Service de médecine interne, centre hospitalier universitaire vaudois (CHUV), rue du Bugnon, 46, 1011 Lausanne, Suisse. Electronic address: henri.lu@chuv.ch. · Service des urgences adultes, centre hospitalier universitaire Antoine-Béclère, Assistance publique-Hôpitaux de Paris (AP-HP), 157, rue de la Porte de Trivaux, 92140 Clamart, France. · Service de médecine interne, centre hospitalier universitaire vaudois (CHUV), rue du Bugnon, 46, 1011 Lausanne, Suisse. · Service de médecine génétique, centre hospitalier universitaire vaudois (CHUV), rue du Bugnon, 46, 1011 Lausanne, Suisse. ·Rev Med Interne · Pubmed #30031565.

ABSTRACT: Thiamine-responsive megaloblastic anemia (TRMA), also known as Rogers syndrome, is a rare autosomal recessive disease characterized by three main components: megaloblastic anemia, diabetes mellitus and sensorineural deafness. Those features occur in infancy but may arise during adolescence. Diagnosis relies on uncovering genetic variations (alleles) in the SLC19A2 gene, encoding for a high affinity thiamine transporter. This transporter is essentially present in hematopoietic stem cells, pancreatic beta cells and inner ear cells, explaining the clinical manifestations of the disease. Based on a multidisciplinary approach, treatment resides on lifelong thiamine oral supplementation at pharmacological doses, which reverses anemia and may delay development of diabetes. However, thiamine supplementation does not alleviate already existing hearing defects.

402 Review Carrier frequencies of hearing loss variants in newborns of China: A meta-analysis. 2019

Fu, Yali / Zha, Shuwei / Lü, Nianqing / Xu, Haoqin / Zhang, Xuening / Shi, Wenhui / Zha, Ji. ·Jiangsu Family Planning Research Institute, Nanjing, P. R. China. · Children's Hospital of Philadelphia, Philadelphia, PA, USA. ·J Evid Based Med · Pubmed #29968368.

ABSTRACT: OBJECTIVE: The objective of this study was to review the carrier frequencies of hearing loss gene variants, such as GJB2, SLC26A4, and MT-RNR1 in newborns of China. DESIGN: PubMed, Embase, BioCentral, CNKI, WanFang, and VIP databases were used for searching relevant literature studies published during the period of January 2007 and January 2016. Meta-analysis was performed by using the R software. The estimated rate and its 95% confidence intervals (CI) of the relevant indexes in newborns were collected and calculated using a fixed-effects model or a random-effects model when appropriate. RESULTS: In total, 35 of 958 published literature studies in Chinese and English were selected. The overall results showed that in newborns of China, the carrier frequencies of GJB2 variants (235 delC, 299 delAT) were 1.64% (95% CI 1.52% to 1.77%) and 0.33% (95% CI 0.19% to 0.51%); SLC26A4 variants (IVS7-2 A > G, 2168 A > G) were 1.02% (95% CI 0.91% to 1.15%) and 0.14% (95% CI 0.06% to 0.25%); MT-RNR1 variants (1555 A > G, 1449 C > T) were 0.20% (95% CI 0.17% to 0.23%) and 0.03% (95% CI 0.02% to 0.05%). CONCLUSIONS: There are high carrier frequencies of GJB2 variants among newborns in China, followed by SLC26A4 and MT-RNR1 variants. In order to achieve "early detection, early diagnosis and early treatment" and reduce the incidence of hereditary hearing loss in offspring, a comprehensive combination of neonatal hearing screening and deafness gene detection should be recommended and implemented in China.

403 Review Neuromodulation for tinnitus treatment: an overview of invasive and non-invasive techniques. 2019

Peter, Nicole / Kleinjung, Tobias. ·Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Zürich, Zürich 8091, Switzerland. ·J Zhejiang Univ Sci B · Pubmed #29770647.

ABSTRACT: Tinnitus is defined as a perception of sound without any external sound source. Chronic tinnitus is a frequent condition that can affect the quality of life. So far, no causal cure for tinnitus has been documented, and most pharmacologic and psychosomatic treatment modalities aim to diminish tinnitus' impact on the quality of life. Neuromodulation, a novel therapeutic modality, which aims at alternating nerve activity through a targeted delivery of a stimulus, has emerged as a potential option in tinnitus treatment. This review provides a brief overview of the current neuromodulation techniques as tinnitus treatment options. The main intention is to provide updated knowledge especially for medical professionals counselling tinnitus patients in this emerging field of medicine. Non-invasive methods such as repetitive transcranial magnetic stimulation, transcranial electrical stimulation, neurofeedback, and transcutaneous vagus nerve stimulation were included, as well as invasive methods such as implanted vagus nerve stimulation and invasive brain stimulation. Some of these neuromodulation techniques revealed promising results; nevertheless, further research is needed, especially regarding the pathophysiological principle as to how these neuromodulation techniques work and what neuronal change they induce. Various studies suggest that individually different brain states and networks are involved in the generation and perception of tinnitus. Therefore, in the future, individually tailored neuromodulation strategies could be a promising approach in tinnitus treatment for achieving a more substantial and longer lasting improvement of complaints.

404 Review Blast-induced hearing loss. 2019

Mizutari, Kunio. ·Department of Otolaryngology, Head and Neck Surgery, National Defense Medical College, Saitama, 359-8513, Japan. ·J Zhejiang Univ Sci B · Pubmed #29770646.

ABSTRACT: The incidence of blast injury has increased recently. As the ear is the organ most sensitive to blast overpressure, the most frequent injuries seen after blast exposure are those affecting the ear. Blast overpressure affecting the ear results in sensorineural hearing loss, which is untreatable and often associated with a decline in the quality of life. Here, we review recent cases of blast-induced hearing dysfunction. The tympanic membrane is particularly sensitive to blast pressure waves, since such waves exert forces mainly at air-tissue interfaces within the body. However, treatment of tympanic membrane perforation caused by blast exposure is more difficult than that caused by other etiologies. Sensorineural hearing dysfunction after blast exposure is caused mainly by stereociliary bundle disruption on the outer hair cells. Also, a reduction in the numbers of synaptic ribbons in the inner hair cells and spiral ganglion cells is associated with hidden hearing loss, which is strongly associated with tinnitus or hyperacusis.

405 Review Atoh1 regulation in the cochlea: more than just transcription. 2019

Cheng, Yen-Fu. ·Department of Otology and Laryngology, Harvard Medical School, Boston, MA 02115, USA. · Eaton-Peabody Laboratory, Massachusetts Eye and Ear Infirmary, Boston, MA 02114, USA. · Department of Medical Research, Taipei Veterans General Hospital, Taipei 112, Taiwan, China. · Department of Otolaryngology-Head and Neck Surgery, Taipei Veterans General Hospital, Taipei 112, Taiwan, China. · School of Medicine, Yang-Ming University, Taipei 112, Taiwan, China. · Department of Speech Language Pathology and Audiology, Taipei University of Nursing and Health Science, Taipei 112, Taiwan, China. ·J Zhejiang Univ Sci B · Pubmed #29770645.

ABSTRACT: More than 80% of all cases of deafness are related to the death or degeneration of cochlear hair cells and the associated spiral ganglion neurons, and a lack of regeneration of these cells leads to permanent hearing loss. Therefore, the regeneration of lost hair cells is an important goal for the treatment of deafness. Atoh1 is a basic helix-loop-helix (bHLH) transcription factor that is critical in both the development and regeneration of cochlear hair cells. Atoh1 is transcriptionally regulated by several signaling pathways, including Notch and Wnt signalings. At the post-translational level, it is regulated through the ubiquitin-proteasome pathway. In vitro and in vivo studies have revealed that manipulation of these signaling pathways not only controls development, but also leads to the regeneration of cochlear hair cells after damage. Recent progress toward understanding the signaling networks involved in hair cell development and regeneration has led to the development of new strategies to replace lost hair cells. This review focuses on our current understanding of the signaling pathways that regulate Atoh1 in the cochlea.

406 Review BRI2 as an anti-Alzheimer gene. 2019

Matsuda, Shuji / Senda, Takao. ·Department of Anatomy, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu, Gifu, 501-1194, Japan. smatsuda@gifu-u.ac.jp. · Department of Anatomy, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu, Gifu, 501-1194, Japan. ·Med Mol Morphol · Pubmed #29687167.

ABSTRACT: There are several theories regarding the etiologies of Alzheimer disease (AD). Considering that all genes responsible for familial AD are amyloid protein precursor (APP) or APP metabolizing enzymes, surely aberrant APP metabolism is crucial to pathogenesis of AD. BRI2, a type II transmembrane protein, binds APP and inhibits all α, β, and γ pathways of APP proteolysis. Crossing AD model mice with BRI2 transgenic or BRI2 knockout mice confirmed that BRI2 is an anti-Alzheimer gene. Mutations of BRI2 are known to cause rare familial dementias in human. Analysis of knock-in mice harboring the disease mutation revealed the memory defect in the mice, attributable to loss of protective function of BRI2. Further studies are needed to decipher this anti-Alzheimer mechanism of BRI2 to develop a novel therapeutic application for AD. In this review, after describing basic assumptions in AD study, we focus on BRI2 as an anti-Alzheimer gene.

407 Review Superficial siderosis of central nervous system with unknown cause: report of 2 cases and review of the literature. 2019

Chen, Hao / Raza, Hafiz Khuram / Jing, Jia / Ye, Xinchun / Zhang, Zuohui / Hua, Fang / Cui, Guiyun. ·a Department of Neurology , The Affiliated Hospital of Xuzhou Medical University , Xuzhou , China. · b Department of Biology , Georgia State University , Atlanta , GA , USA. ·Br J Neurosurg · Pubmed #29160113.

ABSTRACT:

408 Review Hearing loss, lead (Pb) exposure, and noise: a sound approach to ototoxicity exploration. 2018

Carlson, Krystin / Neitzel, Richard L. ·a Department of Environmental Health Sciences , University of Michigan , Ann Arbor , MI , USA. ·J Toxicol Environ Health B Crit Rev · Pubmed #30663930.

ABSTRACT: To determine the state of the research on ototoxic properties of Pb, evaluate possible synergistic effects with concurrent noise exposure, and identify opportunities to improve future research, we performed a review of the peer-reviewed literature to identify studies examining auditory damage due to Pb over the past 50 years. Thirty-eight studies (14 animal and 24 human) were reviewed. Of these, 24 suggested potential ototoxicity due to Pb exposure, while 14 found no evidence of ototoxicity. More animal studies are needed, especially those investigating Pb exposure levels that are occupationally and environmentally relevant to humans. Further investigations into potential interactions of Pb in the auditory system with other hazards and compounds that elicit ototoxicity are also needed in animal models. To better assess the effects of Pb exposure on the human auditory system and the possibility of a synergism with noise, future epidemiological studies need to carefully consider and address four main areas of uncertainty: (1) hearing examination and quantification of hearing loss, (2) Pb exposure evaluation, (3) noise exposure evaluation, and (4) the personal characteristics of those exposed. Two potentially confounding factors, protective factors and mixtures of ototoxicants, also warrant further exploration.

409 Review Interplay between Nutrition and Hearing Loss: State of Art. 2018

Puga, Ana M / Pajares, María A / Varela-Moreiras, Gregorio / Partearroyo, Teresa. ·Department of Pharmaceutical and Health Sciences, Faculty of Pharmacy, CEU San Pablo University, 28668 Madrid, Spain. anamaria.pugagimenezazca@ceu.es. · Department of Structural and Chemical Biology, Centro de Investigaciones Biológicas (CSIC), 28040 Madrid, Spain. mapajares@cib.csic.es. · Molecular Hepatology Group, Hospital La Paz Institute for Health Research (IdiPAZ), 28046 Madrid, Spain. mapajares@cib.csic.es. · Department of Pharmaceutical and Health Sciences, Faculty of Pharmacy, CEU San Pablo University, 28668 Madrid, Spain. gvarela@ceu.es. · Department of Pharmaceutical and Health Sciences, Faculty of Pharmacy, CEU San Pablo University, 28668 Madrid, Spain. t.partearroyo@ceu.es. ·Nutrients · Pubmed #30586880.

ABSTRACT: Hearing loss has been recently ranked as the fifth leading cause of years lived with disability, ahead of many other chronic diseases such as diabetes, dementia, or chronic obstructive pulmonary disease. Moreover, according to the World Health Organization, moderate-to-profound hearing loss affects about 466 million people worldwide. Its incidence varies in each population segment, affecting approximately 10% of children and increasing to 30% of the population over 65 years. However, hearing loss receives still very limited research funding and public awareness. This sensory impairment is caused by genetic and environmental factors, and among the latter, the nutritional status has acquired relevance due its association to hearing loss detected in recent epidemiological studies. Several experimental models have proved that the onset and progression of hearing loss are closely linked to the availability of nutrients and their metabolism. Here, we have reviewed studies focused on nutrient effects on auditory function. These studies support the potential of nutritional therapy for the protection against hearing loss progression, which is especially relevant to the aging process and related quality of life.

410 Review [Recent immunology research of Meniere's disease]. 2018

Guo, S Y / Zhang, Y / Liu, B. ·Department of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing Institute of Otorhinolaryngology, Key Laboratory of Otolaryngology Head and Neck Surgery (Capital Medical University), Ministry of Education , Beijing 100730, China. ·Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi · Pubmed #30585012.

ABSTRACT: Inner ear disease is characterized by tinnitus, deafness and vertigo. The pathogenesis remains unknown. In the past, it is considered to be related to vascular disease such as thrombus and hemadostenosis.However, more and more researches indicate that it may be associated with multiple factors mediated by allergy. This paper reviews some possible pathogenesis of Meniere disease, including type Ⅰ allergy, autoimmunity, circulating immune complex and immune genetic.

411 Review Syphilis. 2018

Tsang, Stephen H / Sharma, Tarun. ·Jonas Children's Vision Care, Bernard & Shirlee Brown Glaucoma Laboratory, Columbia Stem Cell Initiative-Departments of Ophthalmology, Biomedical Engineering, Pathology & Cell Biology, Institute of Human Nutrition, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY, USA. · Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. · Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. ts3118@cumc.columbia.edu. ·Adv Exp Med Biol · Pubmed #30578519.

ABSTRACT: Syphilis is an infectious disease caused by a spirochete, Treponema pallidum; it is most commonly spread by sexual transmission. Syphilis is known as the "Great Imitator," as systemic manifestations are variable. It can involve any part of the eye, with syphilitic uveitis being the most common type. Congenital syphilis is characterized by Hutchinson's teeth, saddle nose deformity, deafness, and interstitial keratitis; pigmentary changes in the retina are varied and patchy.

412 Review Ciliopathy: Usher Syndrome. 2018

Tsang, Stephen H / Aycinena, Alicia R P / Sharma, Tarun. ·Jonas Children's Vision Care, Bernard & Shirlee Brown Glaucoma Laboratory, Columbia Stem Cell Initiative-Departments of Ophthalmology, Biomedical Engineering, Pathology & Cell Biology, Institute of Human Nutrition, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY, USA. · Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. · Department of Pediatrics, Division of Genetics, University of California San Francisco, San Francisco, CA, USA. alicia.aycinena@ucsf.edu. · Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. ts3118@cumc.columbia.edu. ·Adv Exp Med Biol · Pubmed #30578505.

ABSTRACT: Ciliopathies are a group of disorders caused by a defect in ciliogenesis, ciliary protein trafficking. Because nearly every cell in the body (including the photoreceptors) contains cilia, defects in ciliary proteins typically affect multiple organ systems. Usher syndrome is the most common syndromic cause of retinitis pigmentosa (RP) and accounts for 10-20% of cases of RP Inheritance is autosomal recessive, and the retinal dystrophy is usually rod-cone dystrophy (Figs. 32.1 and 32.2). These patients have RP with sensorineural hearing loss (partial or complete) since birth; some may have vestibular dysfunction. Most patients retain central vision of about 20/40 until about age 40. Usher Syndrome 1 (USH1): Profound congenital sensorineural hearing loss on audiometry, absent vestibular function, and typical RP (onset by 10 years of age); accounts for about 70% of all Usher cases. Patient may benefit from a cochlear implant. The retinitis pigmentosa occurs at an early age (childhood onset) and progress slowly. Usher Syndrome 2 (USH2): Moderate to severe congenital sensorineural hearing loss on audiometry (predominantly for higher frequencies), normal vestibular function, and typical RP (onset by 20 years of age); accounts for about 26% of all Usher cases. Usher Syndrome 3 (USH3): Progressive sensorineural hearing loss and typical RP (onset in second decade); accounts for about 4% of all Usher cases. Vestibular function is normal in about half of patients, but abnormal in the other half.

413 Review Mitochondrial Disorder: Maternally Inherited Diabetes and Deafness. 2018

Tsang, Stephen H / Aycinena, Alicia R P / Sharma, Tarun. ·Jonas Children's Vision Care, Bernard & Shirlee Brown Glaucoma Laboratory, Columbia Stem Cell Initiative-Departments of Ophthalmology, Biomedical Engineering, Pathology & Cell Biology, Institute of Human Nutrition, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY, USA. · Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. · Department of Pediatrics, Division of Genetics, University of California San Francisco, San Francisco, CA, USA. alicia.aycinena@ucsf.edu. · Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. ts3118@cumc.columbia.edu. ·Adv Exp Med Biol · Pubmed #30578504.

ABSTRACT: Patients with maternally inherited diabetes and deafness (MIDD) have insulin-dependent diabetes with relatively low BMI; usually the onset of the diabetes is during the third or fourth decade of life and it is associated with progressive neurosensory deafness. The fundus shows circumferentially oriented but discontinuous patches of RPE and choriocapillaris (CC) atrophy around the macula, within the arcades (Figs. 31.1 and 31.2). Sometimes even hyperpigmentation can be seen, also around the optic nerve, or pattern-like dystrophy may occur. Vision is usually good, about 20/40 or better. Fundus autofluorescence (FAF) shows decreased AF in the areas of RPE atrophy, surrounded by a zone of speckled AF. No generalized retinal dysfunction is seen on full-field electroretinography (ERG), but pattern ERG or multifocal ERG shows abnormal function. Asymptomatic maternal relatives harboring the mutation may show pigmentary changes, hearing loss, and in some, diabetes. Another disorder associated with the same A3243G mitochondrial DNA (mtDNA) mutation as MIDD is Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like episodes (MELAS). The stroke-like episode (such as hemiplegia) occurs at about 5-15 years of age, and severe encephalopathy may cause death at a young age. Patients may also experience headache, vomiting, visual field defects, or cortical blindness.

414 Review Efficacy of hearing conservation education programs for youth and young adults: a systematic review. 2018

Khan, Khalid M / Bielko, Sylvanna L / McCullagh, Marjorie C. ·Department of Environmental Health, School of Public Health, Indiana University-Bloomington, 1025 E Seventh Street, Room 025E, Bloomington, IN, 47405, USA. kmkhan@indiana.edu. · Department of Environmental Science, Public Health, and Sustainable Development, School of Applied and Natural Sciences, Taylor University, Upland, IN, USA. · Department of Systems, Populations and Leadership, University of Michigan School of Nursing, Ann Arbor, MI, USA. ·BMC Public Health · Pubmed #30466413.

ABSTRACT: BACKGROUND: Many youth and young adults experience high noise exposure compounded by lack of access to hearing health education. Although the need for hearing health education programs is evident, the efficacy of these programs for youth is unclear. We evaluated the literature for efficacy of various hearing conservation programs aimed at youth and young adults, and analyzed their strengths and limitations. METHODS: Studies reporting results of hearing conservation or hearing loss prevention programs with youth or young adults, using randomized controlled trials, quasi-experimental designs, experimental design, or qualitative research, and published in peer-reviewed journals in English between 2001 and 2018 were included. Studies were found through searches of selected literature databases (i.e., PubMed, Google Scholar, NIOSH Toxline, and Scopus). Identified publications were assessed for relevance, and data were extracted from the studies deemed relevant. RESULTS: A total of 10 studies were included. Very little evidence of efficacy of hearing conservation educational programs was found in these studies. Several methodological limitations including lack of rigorous study designs, inadequate power, and application of inappropriate statistical analysis were noted. Some use of technology in programs (e.g., smartphone apps, mobile phone text messages, and computers) was observed, but conclusions as to the effectiveness of these tools were limited by the small number of studies and small sample sizes. CONCLUSIONS: The number of studies of educational hearing conservation programs for youth and young adults was low. The efficacy of the program was not reported in most studies, and it is difficult to draw public health conclusions from these studies due to their multiple methodological limitations. While use of technology in hearing conservation educational programs offers promise, its effectiveness has not been studied.

415 Review [Gene therapy progress: hopes for Usher syndrome]. 2018

Calvet, Charlotte / Lahlou, Ghizlene / Safieddine, Saaid. ·Institut Pasteur, Unité de génétique et physiologie de l'audition, 25, rue du Docteur Roux, 75724 Paris, Cedex 15, France - Inserm UMRS 1120, 75015 Paris, France - Sorbonne Universités, 75005 Paris, France. · Institut Pasteur, Unité de génétique et physiologie de l'audition, 25, rue du Docteur Roux, 75724 Paris, Cedex 15, France - Inserm UMRS 1120, 75015 Paris, France - Sorbonne Universités, 75005 Paris, France - CNRS, UMRS 1120, 75015 Paris, France. ·Med Sci (Paris) · Pubmed #30451679.

ABSTRACT: Hearing and balance impairment are major concerns and a serious public health burden, as it affects millions of people worldwide, but still lacks an effective curative therapy. Recent breakthroughs in preclinical and clinical studies using viral gene therapy suggest that such an approach might succeed in curing many genetic diseases. Our actual understanding and the comprehensive analysis of the molecular bases of genetic deafness forms have provided the multiple bridges toward gene therapy to correct, replace, or modify the expression of defective endogenous genes involved in deafness. The aim of this review article is to summarize the recent advances in the restoration of cochlear and vestibular functions by local gene therapy in mouse models of Usher syndrome, the leading genetic cause of deafness associated with blindness in the world. We focus herein on therapeutic approaches with the highest potential for clinical application.

416 Review Bilateral Cochlear Implantation: A Health Technology Assessment. 2018

Anonymous4611168. · ·Ont Health Technol Assess Ser · Pubmed #30443278.

ABSTRACT: Background: Sensorineural hearing loss occurs as a result of damage to the hair cells in the cochlea, or to the auditory nerve. It negatively affects learning and development in children, and employment and economic attainment in adults. Current policy in Ontario is to provide unilateral cochlear implantation for patients with bilateral severe to profound sensorineural hearing loss. However, hearing with both ears as a result of bilateral cochlear implantation may offer added benefits. Methods: We completed a health technology assessment, which included an evaluation of clinical benefits and harms, value for money, budget impact, and patient preferences related to bilateral cochlear implantation. We performed a systematic literature search for studies on bilateral cochlear implantation in adults and children from inception to March 2017. We conducted a cost-utility analysis with a lifetime horizon from a public payer perspective and analyzed the budget impact of publicly funding bilateral cochlear implantation in adults and children in Ontario for the next 5 years. Finally, we conducted interviews with adults who have sensorineural hearing loss and unilateral or bilateral cochlear implants, and with parents of children with bilateral cochlear implants. Results: We included 24 publications (10 in adults, 14 in children) in the clinical evidence review. Compared with unilateral cochlear implantation, bilateral cochlear implantation improved sound localization, speech perception in noise, and subjective benefits of hearing in adults and children with severe to profound sensorineural hearing loss (GRADE: moderate to high). Bilateral cochlear implantation also allowed for better language development and more vocalization in preverbal communication in children (GRADE: moderate). The safety profile was acceptable.Bilateral cochlear implantation was more expensive and more effective than unilateral cochlear implantation. The incremental cost-effectiveness ratio was $48,978/QALY in adults and between $27,427/QALY and $30,386/QALY in children. Cost-effectiveness was highly dependent on the quality-of-life values used. We estimated that the net budget impact of publicly funding bilateral cochlear implantation for adults in Ontario would be between $510,000 and $780,000 per year for the next 5 years.Patients described the social and emotional effects of hearing loss, and the benefits and challenges of using cochlear implants. Conclusions: Based on evidence of moderate to high quality, we found that bilateral cochlear implantation improved hearing in adults and children with severe to profound sensorineural hearing loss. Bilateral cochlear implantation was potentially cost-effective compared to unilateral cochlear implantation in adults and children. Patients with sensorineural hearing loss reported the positive effects of cochlear implants, and patients with unilateral cochlear implants generally expressed a desire for bilateral implants.

417 Review Towards personalized medicine in Ménière's disease. 2018

Lopez-Escamez, Jose Antonio / Batuecas-Caletrio, Angel / Bisdorff, Alexandre. ·Otology & Neurotology Group CTS495, Department of Genomic Medicine, Centro de Genómica e Investigación Oncológica, Pfizer/Universidad de Granada/Junta de Andalucía (GENYO), Granada, Spain. · Luxembourg Centre for Systems Biomedicine (LCSB), University of Luxembourg, Esch-sur-Alzette, Luxembourg. · Department of Otolaryngology, Instituto de Investigación Biosanitaria, ibs.GRANADA, Hospital Universitario Virgen de las Nieves, Universidad de Granada, Granada, Spain. · Department of Otolaryngology, Hospital Universitario de Salamanca, IBSAL, Salamanca, Spain. · Clinique du Vertige, Centre Hospitalier Emile Mayrisch, Esch-sur-Alzette, Luxembourg. ·F1000Res · Pubmed #30430003.

ABSTRACT: Ménière's disease (MD) represents a heterogeneous group of relatively rare disorders with three core symptoms: episodic vertigo, tinnitus, and sensorineural hearing loss involving 125 to 2,000 Hz frequencies. The majority of cases are considered sporadic, although familial aggregation has been recognized in European and Korean populations, and the search for familial MD genes has been elusive until the last few years. Detailed phenotyping and cluster analyses have found several clinical predictors for different subgroups of patients, which may indicate different mechanisms, including genetic and immune factors. The genes associated with familial MD are

418 Review Clinical protocol for Hearing Health Services for the care of adults and elderly. 2018

Rodrigues-Sato, Lyvia Christina Camarotto Battiston / Almeida, Katia de. ·Faculdade de Ciências Médicas da Santa Casa de São Paulo - FCMSCSP - São Paulo (SP), Brasil. ·Codas · Pubmed #30427378.

ABSTRACT: PURPOSE: To develop a clinical protocol for patient care in the selection, verification, and validation process of hearing aids; to verify the viability of the protocol during its use by specialists in the field; to establish the graphical representation of the protocol by means of a flowchart with algorithms. METHODS: We conducted a literature review to collect the procedures required for developing clinical protocols in healthcare services and the main procedures at each step along the process of fitting hearing aids. Subsequently, we developed the protocol, which was evaluated by eight audiologists in terms of its content and ease of use. We considered the issues raised by the professionals and then drew up a final document, as well as a flowchart with process algorithms. RESULTS: A protocol after having conducted an extensive survey of the literature was developed; all audiologists reported that the use of the instrument was of great value in their clinical practice; finally, we created the flowchart with algorithms after having developed the protocol and, by extension, we also created the Standard Operational Procedure for the selection, verification and validation process of hearing aids. CONCLUSION: The clinical protocol for the care of patients in the selection, verification and validation process of hearing aids was developed and validated by means of its use by professionals. The information and data we collected allowed a graphical representation of the protocol and its steps as a flowchart with algorithms.

419 Review Hearing Loss and Failed Newborn Hearing Screen. 2018

Dedhia, Kavita / Graham, Elise / Park, Albert. ·Department of Pediatric Otolaryngology, Emory University, 2015 Uppergate Drive, Atlanta, GA 30324, USA. Electronic address: kavi.dedhia@emory.edu. · Department of Pediatric Otolaryngology, University of Utah, 100 North Mario Capercchi Drive, Salt Lake City, UT 84113, USA. ·Clin Perinatol · Pubmed #30396409.

ABSTRACT: Hearing loss is the most common congenital defect. With early diagnosis and intervention, we are able to improve speech and language outcomes in this population. In this article, we discuss the implications of the newborn hearing screen, as well as diagnostic interventions, management, and intervention, and the increasing role of congenital cytomegalovirus screening.

420 Review Management of Vestibular Schwannoma (Including NF2): Facial Nerve Considerations. 2018

Kaul, Vivian / Cosetti, Maura K. ·Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, New York Eye and Ear of Mount Sinai, Floor 6, 310 East 14th Street, New York, NY 10003, USA. · Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, New York Eye and Ear of Mount Sinai, Floor 6, 310 East 14th Street, New York, NY 10003, USA. Electronic address: Maura.Cosetti@mountsinai.org. ·Otolaryngol Clin North Am · Pubmed #30390772.

ABSTRACT: Current consensus on optimal treatment of vestibular schwannoma remains poorly established; treatment options include observation, stereotactic radiosurgery, microsurgical resection, medical therapy, or a combination of these. Treatment should be individualized and incorporate the multitude of patient- and tumor-specific characteristics known to affect outcome. Treatment paradigms for sporadic and neurofibromatosis type 2-related tumors are distinct and decision-making in neurofibromatosis type 2 is uniquely challenging. In all cases, treatment should maximize tumor control and minimize functional deficit.

421 Review Hearing loss in the trenches - a hidden morbidity of World War I. 2018

Conroy, K / Malik, V. ·Department of Otolaryngology,Royal Preston Hospital,UK. · Department of Otolaryngology,Blackpool Victoria Hospital,UK. ·J Laryngol Otol · Pubmed #30370887.

ABSTRACT: BACKGROUND: One hundred years ago, millions of British and Allied troops were fighting in the trenches of the Great War. With a tenth of soldiers losing their lives, hearing loss seemed a low priority; however, vast numbers of troops sustained significant hearing loss. METHOD: A review was conducted of literature published between 1914 and 1925. RESULTS: Soldiers were exposed to up to 185 dB of sustained noise from new, high-energy weapons, which caused 'labyrinthine concussion'. Traumatic injuries, non-organic hearing loss and malingering were also common. One source estimated that 2.4 per cent of the army was disabled by hearing loss. However, many British doctors viewed this 'soldier's deafness' as a temporary affliction, resulting in soldiers being labelled as malingerers or 'hysterical'. CONCLUSION: Today, one can recognise that a scant evidence base and misconceptions influenced the mismanagement of hearing loss by otolaryngologists in World War I. However, noise-induced hearing loss is still very much a feature of armed conflict.

422 Review Targeted next generation sequencing reveals OTOF mutations in auditory neuropathy spectrum disorder. 2018

Chen, Kaitian / Liu, Min / Wu, Xuan / Zong, Ling / Jiang, Hongyan. ·Department of Otorhinolaryngology, The First Affiliated Hospital, Sun Yat-sen University and Institute of Otorhinolaryngology, Sun Yat-sen University, Guangzhou, 510080, PR China. · Department of Otorhinolaryngology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510260, PR China. · Department of Otorhinolaryngology, Hainan General Hospital, Haikou, 570311, PR China. Electronic address: jhongy@mail.sysu.edu.cn. ·Int J Pediatr Otorhinolaryngol · Pubmed #30368385.

ABSTRACT: OBJECTIVE: To study the genetic etiology of auditory neuropathy spectrum disorder (ANSD) in a Chinese family and perform a literature review of OTOF mutations and cochlear implantation (CI). METHODS: Sequential targeted next generation sequencing (NGS) and CI was performed for the proband. Further, 50 DNA samples from unrelated families with nonsyndromic deafness were examined for frequency determination. The impact of OTOF mutations on hearing recovery after CI was assessed through the literature survey. RESULTS: In the proband, the targeted NGS panel revealed five suspected variants in four genes (OTOF, EYA4, PCDH15, and GIPC3), of which two mutations-c.5098G > C (p.Glu1700Gln) and c.1702C > T (p.Arg568Trp)-in the OTOF gene were found to be correlated with ANSD. The c.5098G > C allele was identified in only one child from the 50 unrelated participants. The proband's hearing and speech abilities were restored 2 years after the surgery. Most ANSD patients (90.9%; 30/33) with OTOF mutations have acceptable surgical outcomes, as indicated by existing reports. CONCLUSIONS: Our results support the feasibility of CI for patients with ANSD and OTOF mutations, and this hypothesis was supported by the review of existing data. A larger number of cases studies is required to determine possible modifies on the prognosis of surgery.

423 Review Transcranial direct current stimulation for the treatment of tinnitus: a review of clinical trials and mechanisms of action. 2018

Yuan, Tifei / Yadollahpour, Ali / Salgado-Ramírez, Julio / Robles-Camarillo, Daniel / Ortega-Palacios, Rocío. ·Shanghai Key Laboratory of Psychotic Disorders, Shanghai Mental Health Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China. · Co-innovation Center of Neuroregeneration, Nantong University, Nantong, Jiangsu, China. · Department of Medical Physics, School of Medicine, Ahvaz Jundishapur University of Medical Sciences, Golestan Blvd, Ahvaz, 61357-33118, Iran. Yadollahpour.a@gmail.com. · Biomedical Engineering Department, Polytechnic University of Pachuca, Zempoala, Mexico. · Graduate and Research Department, Polytechnic University of Pachuca, Zempoala, Mexico. ·BMC Neurosci · Pubmed #30359234.

ABSTRACT: BACKGROUND: Tinnitus is the perception of sound in the absence of any external acoustic stimulation. Transcranial direct current stimulation (tDCS) has shown promising though heterogeneous therapeutic outcomes for tinnitus. The present study aims to review the recent advances in applications of tDCS for tinnitus treatment. In addition, the clinical efficacy and main mechanisms of action of tDCS on suppressing tinnitus are discussed. METHODS: The study was performed in accordance with the PRISMA guidelines. The databases of the PubMed (1980-2018), Embase (1980-2018), PsycINFO (1850-2018), CINAHL, Web of Science, BIOSIS Previews (1990-2018), Cambridge Scientific Abstracts (1990-2018), and google scholar (1980-2018) using the set search terms. The date of the most recent search was 20 May, 2018. The randomized controlled trials that have assessed at least one therapeutic outcome measured before and after tDCS intervention were included in the final analysis. RESULTS: Different tDCS protocols were used for tinnitus ranging single to repeated sessions (up to 10) consisting of daily single session of 15 to 20-min and current intensities ranging 1-2 mA. Dorsolateral prefrontal cortex (DLPFC) and auditory cortex are the main targets of stimulation. Both single and repeated sessions showed moderate to significant treatment effects on tinnitus symptoms. In addition to improvements in tinnitus symptoms, the tDCS interventions particularly bifrontal DLPFC showed beneficial outcomes on depression and anxiety comorbid with tinnitus. Heterogeneities in the type of tinnitus, tDCS devices, protocols, and site of stimulation made the systematic reviews of the literature difficult. However, the current evidence shows that tDCS can be developed as an adjunct or complementary treatment for intractable tinnitus. TDCS may be a safe and cost-effective treatment for tinnitus in the short-term application. CONCLUSIONS: The current literature shows moderate to significant therapeutic efficacy of tDCS on tinnitus symptoms. Further randomized placebo-controlled double-blind trials with large sample sizes are needed to reach a definitive conclusion on the efficacy of tDCS for tinnitus. Future studies should further focus on developing efficient disease- and patient-specific protocols.

424 Review Otolaryngology for the Internist: Hearing Loss. 2018

Nieman, Carrie L / Reed, Nicholas S / Lin, Frank R. ·Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, 601 North Caroline Street, Baltimore, MD 21287, USA; Cochlear Center for Hearing and Public Health, Johns Hopkins Bloomberg School of Public Health, 2024 East Monument Street, Suite 2-700, Baltimore, MD 21205, USA. Electronic address: cnieman1@jhmi.edu. · Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, 601 North Caroline Street, Baltimore, MD 21287, USA; Cochlear Center for Hearing and Public Health, Johns Hopkins Bloomberg School of Public Health, 2024 East Monument Street, Suite 2-700, Baltimore, MD 21205, USA. · Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, 601 North Caroline Street, Baltimore, MD 21287, USA; Cochlear Center for Hearing and Public Health, Johns Hopkins Bloomberg School of Public Health, 2024 East Monument Street, Suite 2-700, Baltimore, MD 21205, USA; Department of Medicine, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Baltimore, MD 21234, USA; Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, 615 North Wolfe Street, Baltimore, MD 21205, USA. ·Med Clin North Am · Pubmed #30342615.

ABSTRACT: Hearing loss is the third most common chronic condition but can be secondary to a range of conditions encountered in primary care. This article reviews the presentation, evaluation, differential diagnosis, and associated treatments for both acute and chronic causes of hearing loss. As the most common cause of hearing loss, this article provides an overview of age-related hearing loss, its consequences, and pragmatic treatment approaches, including over-the-counter hearing technology.

425 Review Tinnitus. 2018

Chari, Divya A / Limb, Charles J. ·Department of Otolaryngology-Head and Neck Surgery, University of California San Francisco, 2233 Post Street, 3rd Floor, San Francisco, CA 94115, USA. · Department of Otolaryngology-Head and Neck Surgery, University of California San Francisco, 2233 Post Street, 3rd Floor, San Francisco, CA 94115, USA. Electronic address: Charles.Limb@ucsf.edu. ·Med Clin North Am · Pubmed #30342610.

ABSTRACT: Objective and subjective tinnitus can often be differentiated based on comprehensive history, physical examination, and audiogram. Examples of objective tinnitus include vascular abnormalities, palatal myoclonus, patulous eustachian tube, and stapedial/tensor tympani muscle spasm. Subjective tinnitus is usually associated with hearing loss. Rarely, tinnitus is the result of an underlying condition. In these cases, imaging and additional testing may be indicated. Classification of the type, quality, and intensity of tinnitus is helpful in the work-up and treatment of tinnitus. Treatment modalities include cognitive behavioral therapy, tinnitus retraining therapy, sound therapy, hearing aids, cochlear implants, pharmacotherapy, and brain stimulation.

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