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Hearing Disorders: HELP
Articles from Tel Aviv
Based on 162 articles published since 2010

These are the 162 published articles about Hearing Disorders that originated from Tel Aviv during 2010-2020.
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5 · 6 · 7

Stern, Daniel / Hilly, Ohad. ·Department of Otolaryngology-Head and Neck Surgery, Rabin Medical Center, Petach Tikva, Israel Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. ·Harefuah · Pubmed #29964378.

ABSTRACT: INTRODUCTION: The population of elderly patients in Israel is constantly increasing, and so is the prevalence of age-related morbidities. Age-related hearing loss is very common, affecting approximately a third of the population over 65 years of age. Dementia is also common in elderly patients, and its prevalence doubles every 5 years after the age of 65 years. Hearing loss has many negative effects, such as decreased quality of life, social isolation and depression. In the last two decades, researchers had shown a direct relationship between hearing loss and dementia. Successful hearing rehabilitation can mitigate these negative effects and recent studies demonstrated that rehabilitation with cochlear implants slowed cognitive decline. In most cases, hearing aids are used for rehabilitation. However, for many patients, conventional hearing aids are insufficient. Cochlear implants were found to be effective and safe for elderly patients with severe hearing loss, but commonly they do not refer for evaluation due to their age. In this review we present the scientific evidence regarding the negative effects of age-related hearing loss, the association between hearing loss and cognitive decline, and the role of cochlear implantation in improving quality of life and slowing cognitive decline.

2 Review Pseudoexfoliation: An Ocular Finding with Possible Systemic Implications. 2017

Aviv, Uri / Ben Ner, Daniel / Sharif, Nardeen / Gur, Zvi / Achiron, Asaf. ·Department of Ophthalmology, Wolfson Medical Center, Holon, affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. · Department of Ophthalmology, Soroka University Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel. ·Isr Med Assoc J · Pubmed #28457115.

ABSTRACT: BACKGROUND: Pseudoexfoliation syndrome (PES) is a common age-related disorder affecting 60-70 million people worldwide. Patients with PES have abnormal production and deposition of fibrillar material in the anterior chamber of the eye. These exfoliated fibrils, easily detected by ocular slit-lamp examination, have also been found to exist systematically in the skin, heart, lungs, liver and kidneys. Recently, myriad studies have associated PES with systemic conditions such as increased vascular risk, risk of dementia and inflammatory state. We review here the most current literature on the systemic implications of PES. Our aim is to encourage further studies on this important clinical entity.

3 Review Genetics of Hearing Loss: Syndromic. 2015

Koffler, Tal / Ushakov, Kathy / Avraham, Karen B. ·Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine and Sagol School of Neuroscience, Tel Aviv University, Tel Aviv 6997801, Israel. · Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine and Sagol School of Neuroscience, Tel Aviv University, Tel Aviv 6997801, Israel. Electronic address: karena@post.tau.ac.il. ·Otolaryngol Clin North Am · Pubmed #26443487.

ABSTRACT: Hearing loss (HL) is one of the most common birth defects in developed countries and is a diverse pathologic condition with different classifications. One of these is based on the association with other clinical features, defined as syndromic hearing loss (SHL). Determining the cause of the HL in these patients is extremely beneficial as it enables a personalized approach to caring for the individual. Early screening can further aid in optimal rehabilitation for a child's development and growth. The advancement of high-throughput sequencing technology is facilitating rapid and low-cost diagnostics for patients with SHL.

4 Review Critical period for first language: the crucial role of language input during the first year of life. 2015

Friedmann, Naama / Rusou, Dana. ·Language and Brain Lab, Tel Aviv University, Israel. Electronic address: naamafr@post.tau.ac.il. · Language and Brain Lab, Tel Aviv University, Israel. ·Curr Opin Neurobiol · Pubmed #26111432.

ABSTRACT: The critical period for language acquisition is often explored in the context of second language acquisition. We focus on a crucially different notion of critical period for language, with a crucially different time scale: that of a critical period for first language acquisition. We approach this question by examining the language outcomes of children who missed their critical period for acquiring a first language: children who did not receive the required language input because they grew in isolation or due to hearing impairment and children whose brain has not developed normally because of thiamine deficiency. We find that the acquisition of syntax in a first language has a critical period that ends during the first year of life, and children who missed this window of opportunity later show severe syntactic impairments.

5 Review Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options. 2015

Gordon, Karen / Henkin, Yael / Kral, Andrej. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Department of Otolaryngology-Head and Neck Surgery, University of Toronto, Toronto, Canada; karen.gordon@utoronto.ca. · Hearing, Speech, and Language Center, Sheba Medical Center, Tel Hashomer, Department of Communication Disorders, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; and. · Cluster of Excellence Hearing4all, Institute of AudioNeuroTechnology, Hannover, Germany; Department of Experimental Otology, ENT Clinics, School of Medicine, Hannover Medical University, Hannover, Germany; and School of Behavioral and Brain Sciences, The University of Texas at Dallas, Dallas, Texas. ·Pediatrics · Pubmed #26055845.

ABSTRACT: Deafness affects ∼2 in 1000 children and is one of the most common congenital impairments. Permanent hearing loss can be treated by fitting hearing aids. More severe to profound deafness is an indication for cochlear implantation. Although newborn hearing screening programs have increased the identification of asymmetric hearing loss, parents and caregivers of children with single-sided deafness are often hesitant to pursue therapy for the deaf ear. Delayed intervention has consequences for recovery of hearing. It has long been reported that asymmetric hearing loss/single-sided deafness compromises speech and language development and educational outcomes in children. Recent studies in animal models of deafness and in children consistently show evidence of an "aural preference syndrome" in which single-sided deafness in early childhood reorganizes the developing auditory pathways toward the hearing ear, with weaker central representation of the deaf ear. Delayed therapy consequently compromises benefit for the deaf ear, with slow rates of improvement measured over time. Therefore, asymmetric hearing needs early identification and intervention. Providing early effective stimulation in both ears through appropriate fitting of auditory prostheses, including hearing aids and cochlear implants, within a sensitive period in development has a cardinal role for securing the function of the impaired ear and for restoring binaural/spatial hearing. The impacts of asymmetric hearing loss on the developing auditory system and on spoken language development have often been underestimated. Thus, the traditional minimalist approach to clinical management aimed at 1 functional ear should be modified on the basis of current evidence.

6 Review Hearing disorders in multiple sclerosis. 2015

Furst, Miriam / Levine, Robert A. ·School of Electrical Engineering, Tel Aviv University, Tel Aviv, Israel. Electronic address: mira@eng.tau.ac.il. · Department of Ear, Nose and Throat and Head and Neck Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. ·Handb Clin Neurol · Pubmed #25726295.

ABSTRACT: Multiple sclerosis (MS) is a disease that is both a focal inflammatory and a chronic neurodegenerative disease. The focal inflammatory component is characterized by destruction of central nervous system myelin, including the spinal cord; as such it can impair any central neural system, including the auditory system. While on the one hand auditory complaints in MS patients are rare compared to other senses, such as vision and proprioception, on the other hand auditory tests of precise neural timing are never "silent." Whenever focal MS lesions are detected involving the pontine auditory pathway, auditory tests requiring precise neural timing are always abnormal, while auditory functions not requiring such precise timing are often normal. Azimuth sound localization is accomplished by comparing the timing and loudness of the sound at the two ears. Hence tests of azimuth sound localization must obligatorily involve the central nervous system and particularly the brainstem. Whenever a focal lesion was localized to the pontine auditory pathway, timing tests were always abnormal, but loudness tests were not. Moreover, a timing test that included only high-frequency sounds was very often abnormal, even when there was no detectable focal MS lesion involving the pontine auditory pathway. This test may be a marker for the chronic neurodegenerative aspect of MS, and, as such could be used to complement the magnetic resonance imaging scan in monitoring the neurodegenerative aspect of MS. Studies of MS brainstem lesion location and auditory function have led to advances in understanding how the human brain processes sound. The brain processes binaural sounds independently for time and level in a two-stage process. The first stage is at the level of the superior olivary complex (SOC) and the second at a level rostral to the SOC.

7 Review Tinnitus. 2015

Levine, Robert A / Oron, Yahav. ·Department of Ear, Nose and Throat and Head and Neck Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. Electronic address: ObbyLevine@Yahoo.com. · Department of Otolaryngology, Head and Neck Surgery, E. Wolfson Medical Centre, Holon, Israel. ·Handb Clin Neurol · Pubmed #25726282.

ABSTRACT: Tinnitus, the perception of sound in the absence of an external sound, usually results from a disorder of: (1) the auditory system (usually peripheral, rarely central); (2) the somatosensory system (head and neck); or (3) a combination of the two. Its cause can be determined through its characteristics. The history must include the tinnitus': (1) quality (including whether it can ever be pulsatile or have a clicking component); (2) location; (3) variability; (4) predominant pitch (low or high); and (5) whether the patient can do something to modulate the percept. In addition to the standard neuro-otologic examination, the exam should include inspection of the teeth for evidence of wear, listening around the ear and neck for sounds similar to the tinnitus, palpation of the craniocervical musculature for trigger points, and probing whether the tinnitus percept can be modulated with "somatic testing." All subjects should have a recent audiogram. Presently the most compelling tinnitus theory is the dorsal cochlear nucleus (DCN) hypothesis: both the auditory and somatosensory systems converge upon and interact within the DCN. If the activity of the DCN's somatosensory-interacting fusiform cells exceeds an individual's tinnitus threshold, then tinnitus results.

8 Review Cardiovascular risk factors as causes for hearing impairment. 2014

Oron, Yahav / Elgart, Katya / Marom, Tal / Roth, Yehudah. ·Department of Otolaryngology, Head and Neck Surgery, Edith Wolfson Medical Center, Sackler School of Medicine, Tel Aviv University, Holon, Israel. ·Audiol Neurootol · Pubmed #25073427.

ABSTRACT: The purpose of this paper is to provide a contemporary review of the correlation between cardiovascular risk factors (CVRFs) and hearing impairment (HI) . We conducted a comprehensive review of the literature in order to assess the effects of the different CVRFs on HI. We focused on the pathological findings in the inner ear and their correlation with cochlear function in population-based studies. We found that CVRFs adversely affect hearing acuity. HI diagnosis should be accompanied by detecting and treating CVRFs, according to the presented outline, which may augment hearing rehabilitation and improve the general health and the well-being of the patient. © 2014 S. Karger AG, Basel.

9 Review Tinnitus: diagnostic approach leading to treatment. 2013

Levine, Robert Aaron. ·Department of Otolaryngology, Tel Aviv Sourasky (Ichilov) Medical Center, Tel Aviv, Israel. ·Semin Neurol · Pubmed #24057829.

ABSTRACT: Optimal care for a patient with tinnitus begins with identifying the cause of the tinnitus. Once the cause has been identified then an appropriate treatment plan can be initiated. In this article, the author reviews how to identify the tinnitus etiology and its treatment.The workup begins with the patient's description of the percept because in some cases, the quality of the tinnitus will make the diagnosis (e.g., clicking, which is readily suppressed pharmacologically); in other cases, it will give direction in the diagnostic evaluation (e.g., pulsatile). With the exception of a small dural arteriovenous malformation, the source of objective pulsatile tinnitus can be determined without conventional cerebral angiography. If the diagnostic workup is unrevealing and the pulsations are not suppressed with somatic testing, then eighth nerve vascular compression becomes the likely etiology, especially if there is some clicking also heard, no matter how minor.The two major causes of tinnitus are hearing loss and myofascial disorders of the head and neck. Moreover, the two can combine and cause tinnitus even though either condition alone would not have caused tinnitus. Although the tinnitus of hearing loss is not easily treatable, the tinnitus from myofascial disorders is often responsive to an optimized myofascial treatment program. Hyperacusis, a frequent accompaniment of tinnitus, and its treatment are discussed.

10 Review Genomic advances for gene discovery in hereditary hearing loss. 2012

Avraham, Karen B / Kanaan, Moien. ·Department of Human Molecular Genetics and Biochemistry, Tel Aviv University, Tel Aviv, Israel. karena@post.tau.ac.il ·J Basic Clin Physiol Pharmacol · Pubmed #22962211.

ABSTRACT: High-throughput sequencing is changing the face of genetic diagnosis and counseling. While in the past, it would take on average 1 to 5 years to identify a mutation leading to deafness, today, the genetic basis for deafness can be determined within months in a child or adult with inherited hearing loss. Obstacles and challenges still remain, but the field is changing at a dramatic rate, making gene discovery a much easier and more efficient task than in the past.

11 Review microRNAs: the art of silencing in the ear. 2012

Rudnicki, Anya / Avraham, Karen B. ·Sackler Faculty of Medicine, Department of Human Molecular Genetics and Biochemistry, Tel Aviv University, Israel. ·EMBO Mol Med · Pubmed #22745034.

ABSTRACT: MicroRNAs (miRNAs) are small non-coding RNAs that regulate gene expression through the RNA interference (RNAi) pathway and by inhibition of mRNA translation. miRNAs first made their appearance in the auditory and vestibular systems in 2005, with the discovery of a triad of hair cell-specific miRNAs later found to be involved in both human and mouse deafness. Since then, miRNAs have been implicated in other medical conditions related to these systems, such as cholesteatomas, vestibular schwannomas and otitis media. Due to the limitations in studying miRNAs and their targets derived from human inner ears, animal models are vital in this field of research. Therefore their role in inner ear development and function has been demonstrated by studies in zebrafish and mice. Transcriptomic and proteomic approaches have been undertaken to identify miRNAs and their targets. Finally, it has been suggested that miRNAs may be used in the future in regeneration of inner ear hair cells and ultimately play a role in therapeutics.

12 Review High-throughput sequencing to decipher the genetic heterogeneity of deafness. 2012

Brownstein, Zippora / Bhonker, Yoni / Avraham, Karen B. ·Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 69978, Israel. ·Genome Biol · Pubmed #22647651.

ABSTRACT: Identifying genes causing non-syndromic hearing loss has been challenging using traditional approaches. We describe the impact that high-throughput sequencing approaches are having in discovery of genes related to hearing loss and the implications for clinical diagnosis.

13 Review Integration of human and mouse genetics reveals pendrin function in hearing and deafness. 2011

Dror, Amiel A / Brownstein, Zippora / Avraham, Karen B. ·Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. ·Cell Physiol Biochem · Pubmed #22116368.

ABSTRACT: Genomic technology has completely changed the way in which we are able to diagnose human genetic mutations. Genomic techniques such as the polymerase chain reaction, linkage analysis, Sanger sequencing, and most recently, massively parallel sequencing, have allowed researchers and clinicians to identify mutations for patients with Pendred syndrome and DFNB4 non-syndromic hearing loss. While thus far most of the mutations have been in the SLC26A4 gene coding for the pendrin protein, other genetic mutations may contribute to these phenotypes as well. Furthermore, mouse models for deafness have been invaluable to help determine the mechanisms for SLC26A4-associated deafness. Further work in these areas of research will help define genotype-phenotype correlations and develop methods for therapy in the future.

14 Review Hereditary hearing loss: from human mutation to mechanism. 2011

Lenz, Danielle R / Avraham, Karen B. ·Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. ·Hear Res · Pubmed #21664957.

ABSTRACT: The genetic heterogeneity of hereditary hearing loss is thus far represented by hundreds of genes encoding a large variety of proteins. Mutations in these genes have been discovered for patients with different modes of inheritance and types of hearing loss, ranging from syndromic to non-syndromic and mild to profound. In many cases, the mechanisms whereby the mutations lead to hearing loss have been partly elucidated using cell culture systems and mouse and other animal models. The discovery of the genes has completely changed the practice of genetic counseling in this area, providing potential diagnosis in many cases that can be coupled with clinical phenotypes and offer predictive information for families. In this review we provide three examples of gene discovery in families with hereditary hearing loss, all associated with elucidation of some of the mechanisms leading to hair cell degeneration and pathology of deafness.

15 Review Hearing loss: a common disorder caused by many rare alleles. 2010

Raviv, Dorith / Dror, Amiel A / Avraham, Karen B. ·Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. ·Ann N Y Acad Sci · Pubmed #21175685.

ABSTRACT: Perception of sound is a fundamental role of the auditory system. Traveling with the force of their mechanical energy, sound waves are captured by the ear and activate the sensory pathway of this complex organ. The hair cells, specialized sensory cells within the inner ear, transmit the mechanical energy into electrical nerve stimuli that reach the brain. A large number of proteins are responsible for the overarching tasks required to maintain the complex mechanism of sound sensation. Many hearing disorders are due to single gene defects inherited in a Mendelian fashion, thus enabling clinical diagnostics. However, at the same time, hearing impairment is genetically heterogeneous, with both common and rare forms occurring due to mutations in over 100 genes. The crosstalk between human and mouse genetics has enabled comprehensive studies on gene identification and protein function, taking advantage of the tools animal models have to offer. The aim of the following review is to provide background and examples of human deafness genes and the discovery of their function in the auditory system.

16 Review Hearing impairment: a panoply of genes and functions. 2010

Dror, Amiel A / Avraham, Karen B. ·Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. ·Neuron · Pubmed #20955936.

ABSTRACT: Research in the genetics of hearing and deafness has evolved rapidly over the past years, providing the molecular foundation for different aspects of the mechanism of hearing. Considered to be the most common sensory disorder, hearing impairment is genetically heterogeneous. The multitude of genes affected encode proteins associated with many different functions, encompassing overarching areas of research. These include, but are not limited to, developmental biology, cell biology, physiology, and neurobiology. In this review, we discuss the broad categories of genes involved in hearing and deafness. Particular attention is paid to a subgroup of genes associated with inner ear gene regulation, fluid homeostasis, junctional complex and tight junctions, synaptic transmission, and auditory pathways. Overall, studies in genetics have provided research scientists and clinicians with insight regarding practical implications for the hearing impaired, while heralding hope for future development of therapeutics.

17 Clinical Trial Multiple electrostimulation treatments to the promontory for tinnitus. 2015

Perez, Ronen / Shaul, Chanan / Vardi, Michael / Muhanna, Nidal / Kileny, Paul R / Sichel, Jean-Yves. ·*Department of Otolaryngology-Head and Neck Surgery, Shaare Zedek Medical Center affiliated with the Hebrew University Medical School, Jerusalem; †EStimME, Tel-Aviv, Israel; and ‡University of Michigan Health System, Otolaryngology-Head and Neck Surgery, Ann Arbor, Michigan, U.S.A. ·Otol Neurotol · Pubmed #24691511.

ABSTRACT: OBJECTIVES: To assess the safety and efficacy of multiple sessions of electrostimulation by a transtympanic needle electrode on the promontory for tinnitus relief. STUDY DESIGN: Prospective open, unblinded, uncontrolled clinical trial. SETTING: Tertiary academic referral center. PATIENTS: Ten patients (8 male and 2 female subjects), mean age 50.1 ± 12 years (range, 34-67) with severe unilateral tinnitus completed all stages of the study. Patients with tinnitus duration between 6 months to 3 years were included. INTERVENTION: The patients underwent 3 consecutive 30-minute sessions, every other day, of biphasic, charge balanced electrostimulation pulses to the promontory delivered by a transtympanic needle electrode. MAIN OUTCOME MEASURES: 1) Tinnitus loudness reported by visual analog scale (VAS) between 1 and 10, at baseline, before and after each treatment, and 1, 2, 3, and 4 weeks after the last treatment. 2) Tinnitus Handicap Inventory (THI) questionnaire at baseline and 4 weeks after treatment. 3) Basic audiometry and tinnitus specific tests such as minimum masking level, tinnitus loudness, and pitch. RESULTS: No long-term adverse safety outcomes were noted in physical examination or audiologic evaluation. VAS levels decreased by ≥2 levels in 5 patients (50%) and returned to baseline 4 weeks after treatment. The VAS decrease was found significant (p = 0.048) in those patients. A statistically significant decrease in THI score was noted 4 weeks after treatment. Tinnitus specific tests at that time were unchanged from baseline. CONCLUSION: Multiple sessions of electrostimulation to the promontory seem to be safe and may be beneficial for some tinnitus patients. Further clinical trials are warranted.

18 Article Hearing Loss and Ophthalmic Pathology in Children Diagnosed Before and after the Implementation of a Universal Hearing Screening Program. 2019

Gruber, Maayan / Brown, Colin / Mahadevan, Murali / Neeff, Michel. ·Paediatric Otolaryngology, Starship Children's Hospital, Auckland, New Zealand. · Pediatric Otolaryngology Service, Galilee Medical Center, Nahariya, Israel. · Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel. ·Isr Med Assoc J · Pubmed #31542906.

ABSTRACT: BACKGROUND: Ophthalmic pathologies may further complicate the sensory input of patients with congenital hearing loss; however, data on children with coexisting impairment of vision and hearing is outdated, from before universal implementation of hearing screening programs. OBJECTIVES: To examine the different ophthalmic pathologies among children with congenital sensorineural hearing loss (SNHL) before or after the introduction of a universal newborn hearing screening program (UNHSP). METHODS: Retrospective cohort study was conducted of 91 children diagnosed with congenital SNHL between 2005 and 2016 in a tertiary pediatric hospital. All patients completed an ophthalmologic examination, including assessment of visual acuity, refraction, ocular motility, slit lamp examination, and indirect funduscopy. Radiological assessment and genetic analysis were offered to all caregivers. RESULTS: Average age at diagnosis was 4.1 years. Nineteen children (21%) were diagnosed with an ophthalmic condition, of which the most common were refractive pathologies. Diagnosis of an ophthalmic pathology was twice as likely in the pre-UNHSP era (14 children, 27%) compared to the post-UNHSP era (5 children, 13%). Out of 91 children, 57 (63%) underwent a computed tomography scan and/or magnetic resonance imaging. Imaging was positive for structural abnormalities in 23 children (40%). There was no correlation between imaging and ophthalmic conditions. Genetic analysis was performed in 67 patients (74%). CONCLUSIONS: The ophthalmic assessment of babies and children with congenital SNHL may yield in significant numbers of children with concomitant ophthalmic pathologies. Implementation of a UNHSP allows early diagnosis and treatment of coexisting ophthalmic and hearing conditions.

19 Article Assessing and monitoring the impact of the national newborn hearing screening program in Israel. 2019

Wasser, Janice / Ari-Even Roth, Daphne / Herzberg, Orly / Lerner-Geva, Liat / Rubin, Lisa. ·Department of Maternal and Child Health, Public Health Services, Ministry of Health, Jerusalem, Israel. Janice.wasser@gmail.com. · Department of Communication Disorders, School of Health Professions, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. · Hearing, Speech and Language Center, Sheba Medical Centre, Tel HaShomer, Israel. · Chief Communication Disorders Clinician, Medical Directorate, Ministry of Health, Tel Aviv, Israel. · Women and Children's Health Research Unit, The Gertner Institute for Epidemiology and Health Policy, Tel HaShomer, Israel. · School of Public Health, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. · Department of Maternal and Child Health, Public Health Services, Ministry of Health, Jerusalem, Israel. · School of Public Health, University of Haifa, Haifa, Israel. ·Isr J Health Policy Res · Pubmed #30857547.

ABSTRACT: BACKGROUND: The Israeli Newborn Hearing Screening Program (NHSP) began operating nationally in January 2010. The program includes the Otoacoustic Emissions (OAE) test for all newborns and Automated Auditory Brainstem Response (A-ABR) test for failed OAE and infants at risk for auditory neuropathy spectrum disorders. NHSP targets are diagnosis of hearing impairment by age three months and initiation of habilitation by six months. OBJECTIVES: (1) Review NHSP coverage; (2) Assess NHSP impact on age at diagnosis for hearing impairment and age at initiation of habilitation; (3) Identify contributing factors and barriers to NHSP success. METHODS: (1) Analysis of screening coverage and referral rates for the NHSP; (2) Analysis of demographic data, results of coverage, age at diagnosis and initiation of habilitation for hearing impaired infants pre-implementation and post-implementation of NHSP from 10 habilitation centers; (3) Telephone interviews with parents whose infants failed the screening and were referred for further testing. RESULTS: The NHSP coverage was 98.7% (95.1 to 100%) for approximately 179,000 live births per year for 2014-2016 and average referral rates were under 3%. After three years of program implementation, median age at diagnosis was 3.7 months compared to 9.5 months prior to NHSP. The median age at initiation of habilitation after three years of NHSP was 9.4 months compared to 19.0 prior to NHSP. Parents (84% of 483 sampled) with infants aged 4-6 months participated in the telephone survey. While 84% of parents reported receiving a verbal explanation of the screening results, more than half of the parents reported not receiving written material. Parental report of understanding the test results and a heightened level of concern over the failed screen were associated with timely follow-up. CONCLUSIONS: The findings indicate high screening coverage. The program reduced ages at diagnosis and initiation of habilitation for hearing impaired infants. Further steps needed to streamline the NHSP are improving communication among caregivers to parents to reduce anxiety; increasing efficiency in transferring information between service providers using advanced technology while ensuring continuum of care; reducing wait time for follow-up testing in order to meet program objectives. Establishment of a routine monitoring system is underway.

20 Article Neurosyphilis presenting as cranial nerve palsy, an entity which is easy to miss. 2019

Piura, Yoav / Mina, Yair / Aizenstein, Orna / Gadoth, Avi. ·Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. · Radiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. ·BMJ Case Rep · Pubmed #30796075.

ABSTRACT: Neurosyphilis is a rare disease that until the 2000s was almost eradicated due to population awareness of HIV and efficient treatment. Since then, the prevalence of the entity is rising due to risk-associated behaviour such as unprotected intercourse. Neurosyphilis is still a difficult entity to diagnose especially when combined with acute HIV infection which can influence the usual clinical course of disease. In rare occasions, both acute HIV and early syphilis infection can present as mono or multiple cranial nerve palsies. This case demonstrates a rare manifestation of misdiagnosed early syphilis infection combined with acute HIV infection in a 34-year-old man with prior history of unprotected sex with men.

21 Article Nationwide Survey of Intratympanic Steroids for the Management of Sudden Sensorineural Hearing Loss. 2019

Levy, Einat / Sela, Eyal / Letichevsky, Vadim / Ronen, Ohad. ·Department of Otolaryngology - Head and Neck Surgery, Western Galilee Medical Center, Nahariya, Israel. · Azrieli Faculty of Medicine, Bar Ilan University, Safed, Israel. ·Isr Med Assoc J · Pubmed #30772961.

ABSTRACT: BACKGROUND: The currently accepted treatment for idiopathic sudden sensorineural hearing loss (ISSHL) is systemic steroids as first-line and intratympanic steroids as salvage therapy. Intratympanic (IT) treatment is applied worldwide in many different ways with no universally accepted protocol. OBJECTIVES: To present the current disparity in ISSHL management and to discuss the necessity for establishing a common national protocol. METHODS: In 2014 we conducted a national survey by sending questionnaires on ISSHL management to otologists in every otolaryngology department in the country. RESULTS: The majority of otolaryngology departments (56%) admit patients with sudden sensorineural hearing. Almost two-thirds (61%) of departments recommend supplementary initial treatment in addition to systemic steroids. None of the medical centers offer intratympanic steroid treatment as primary therapy, but 94% offer this treatment as a salvage therapy. Fewer than half the medical centers (44%) consider the maximal period for intratympanic therapy to be 4 weeks since hearing loss appears. Almost half (48%) the departments use intratympanic steroids once every 5-7 days, usually in an ambulatory setting. Almost half (44%) the medical centers tend to use not more than four courses of IT steroids. In 44% of departments an audiogram is performed at the beginning and at the end of the intratympanic course. CONCLUSIONS: Our results demonstrate a variability among Israeli medical centers in many aspects of intratympanic treatment. We believe this reinforces the need for a comparative international study in order to establish a standard protocol.

22 Article Atypical Auditory Brainstem Response and Protein Expression Aberrations Related to ASD and Hearing Loss in the Adnp Haploinsufficient Mouse Brain. 2019

Hacohen-Kleiman, Gal / Yizhar-Barnea, Ofer / Touloumi, Olga / Lagoudaki, Roza / Avraham, Karen B / Grigoriadis, Nikolaos / Gozes, Illana. ·The Lily and Avrahamo Gildor Chair for the Investigation of Growth Factors; The Elton Laboratory for Neuroendocrinology; Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Sagol School of Neuroscience and Adams Super Center for Brain Studies, Tel Aviv University, 69978, Tel Aviv, Israel. · Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine and Sagol School of Neuroscience, Tel Aviv University, Tel Aviv, Israel. · Department of Neurology, Laboratory of Experimental Neurology, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. · The Lily and Avrahamo Gildor Chair for the Investigation of Growth Factors; The Elton Laboratory for Neuroendocrinology; Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Sagol School of Neuroscience and Adams Super Center for Brain Studies, Tel Aviv University, 69978, Tel Aviv, Israel. igozes@tauex.tau.ac.il. ·Neurochem Res · Pubmed #30659505.

ABSTRACT: Autism is a wide spread neurodevelopmental disorder with growing morbidity rates, affecting more boys than girls worldwide. Activity-dependent neuroprotective protein (ADNP) was recently recognized as a leading gene accounted for 0.17% of autism spectrum disorder (ASD) cases globally. Respectively, mutations in the human ADNP gene (ADNP syndrome), cause multi-system body dysfunctions with apparent ASD-related traits, commencing as early as childhood. The Adnp haploinsufficient (Adnp

23 Article Tinnitus Perspectives among Israeli Ear, Nose and Throat Physicians: A Nationwide Survey. 2018

Tamir, Sharon Ovnat / Marom, Tal / Shushan, Sagit / Goldfarb, Abraham / Cinamon, Udi / Handzel, Ophir / Gluck, Ofer / Oron, Yahav. ·Department of Otolaryngology-Head and Neck Surgery, Edith Wolfson Medical Center, Tel Aviv University Sackler School of Medicine, Holon, Israel. · Department of Otolaryngology-Head and Neck Surgery, Assaf Harofeh Medical Center, Tel Aviv University Sackler School of Medicine, Zerifin, Israel. · Department of Otolaryngology-Head, Neck, and Maxillofacial Surgery, Tel-Aviv Sourasky Medical Center, Tel Aviv, Israel and Tel-Aviv University, Sackler School of Medicine, Tel Aviv, Israel. ·J Int Adv Otol · Pubmed #30541733.

ABSTRACT: OBJECTIVES: To study the compliance of ear, nose, and throat (ENT) physicians to the American Association Otolaryngology-Head - Neck Surgery (AAO-HNS) clinical practice guidelines (CPG) for tinnitus and to identify the disparity of both diagnosis and management options in the absence of a local protocol for the management of adult tinnitus. MATERIALS AND METHODS: A voluntary and anonymous questionnaire was emailed in a Google spread-out sheet format to all practicing ENT physicians across the country (n=370). Overall, 126 ENT physicians responded to the questionnaire (34% of the physicians to whom the questionnaire was sent). RESULTS: Medical history focuses on tinnitus characteristics and otological signs, and symptoms are often queried (80%-98%). Physicians routinely perform an otoscopic examination, whereas other relevant possible physical findings, such as temporomandibular joint disorders or neck trauma, are less frequently examined. Treating physicians have the most frequent recourse to sound therapy and cognitive behavioral therapy in accordance with AAO-HNS CPG. CONCLUSION: The publication of the AAO-HNS CPG for tinnitus is important, permitting a common approach for the diagnosis and management of primary tinnitus (PT). A diagnosis and management scheme that takes into consideration both the AAO-HNS CPG for tinnitus as well as physician diagnosis and management paradigms is suggested.

24 Article The Effect of Presentation Mode and Production Type on Word Memory for Hearing Impaired Signers. 2018

Taitelbaum-Swead, Riki / Mama, Yaniv / Icht, Michal. ·Department of Communication Disorders, Ariel University, Ariel, Israel. · Meuhedet Health Services, Tel Aviv, Israel. · Department of Behavioral Sciences and Psychology, Ariel University, Ariel, Israel. ·J Am Acad Audiol · Pubmed #30479260.

ABSTRACT: BACKGROUND: Production effect (PE) is a memory phenomenon referring to better memory for produced (vocalized) than for non-produced (silently read) items. Reading aloud was found to improve verbal memory for normal-hearing individuals, as well as for cochlear implant users, studying visually and aurally presented material. PURPOSE: The present study tested the effect of presentation mode (written or signed) and production type (vocalization or signing) on word memory in a group of hearing impaired young adults, sign-language users. RESEARCH DESIGN: A PE paradigm was used, in which participants learned lexical items by two presentation modes, written or signed. We evaluated the efficacy of two types of productions: vocalization and signing, using a free recall test. STUDY SAMPLE: Twenty hearing-impaired young adults, Israeli sign language (ISL) users, participated in the study, ten individuals who mainly use manual communication (MC) (ISL as a first language), and ten who mainly use total communication (TC). DATA COLLECTION AND ANALYSIS: For each condition, we calculated the proportion of study words recalled. A mixed-design analysis of variance was conducted, with learning condition (written-vocalize, written-signed, and manual-signed) and production type (production and no-production) as within-subject variables, and group (MC and TC) as a between-subject variable. RESULTS: Production benefit was documented across all learning conditions, with better memory for produced over non-produced words. Recall rates were higher when learning written words relative to signed words. Production by signing yielded better memory relative to vocalizing. CONCLUSIONS: The results are explained in light of the encoding distinctiveness account, namely, the larger the number of unique encoding processes involved at study, the better the memory benefit.

25 Article DNA methylation dynamics during embryonic development and postnatal maturation of the mouse auditory sensory epithelium. 2018

Yizhar-Barnea, Ofer / Valensisi, Cristina / Jayavelu, Naresh Doni / Kishore, Kamal / Andrus, Colin / Koffler-Brill, Tal / Ushakov, Kathy / Perl, Kobi / Noy, Yael / Bhonker, Yoni / Pelizzola, Mattia / Hawkins, R David / Avraham, Karen B. ·Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine and Sagol School of Neuroscience, Tel Aviv University, Tel Aviv, 6997801, Israel. · Division of Medical Genetics, Department of Medicine, Department of Genome Sciences, Institute for Stem Cell and Regenerative Medicine, University of Washington School of Medicine, Seattle, WA, 98195, USA. · Center for Genomic Science of IIT@SEMM, Fondazione Istituto Italiano di Tecnologia, Milano, 20139, Italy. · Division of Medical Genetics, Department of Medicine, Department of Genome Sciences, Institute for Stem Cell and Regenerative Medicine, University of Washington School of Medicine, Seattle, WA, 98195, USA. rdhawk@uw.edu. · Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine and Sagol School of Neuroscience, Tel Aviv University, Tel Aviv, 6997801, Israel. karena@tauex.tau.ac.il. ·Sci Rep · Pubmed #30478432.

ABSTRACT: The inner ear is a complex structure responsible for hearing and balance, and organ pathology is associated with deafness and balance disorders. To evaluate the role of epigenomic dynamics, we performed whole genome bisulfite sequencing at key time points during the development and maturation of the mouse inner ear sensory epithelium (SE). Our single-nucleotide resolution maps revealed variations in both general characteristics and dynamics of DNA methylation over time. This allowed us to predict the location of non-coding regulatory regions and to identify several novel candidate regulatory factors, such as Bach2, that connect stage-specific regulatory elements to molecular features that drive the development and maturation of the SE. Constructing in silico regulatory networks around sites of differential methylation enabled us to link key inner ear regulators, such as Atoh1 and Stat3, to pathways responsible for cell lineage determination and maturation, such as the Notch pathway. We also discovered that a putative enhancer, defined as a low methylated region (LMR), can upregulate the GJB6 gene and a neighboring non-coding RNA. The study of inner ear SE methylomes revealed novel regulatory regions in the hearing organ, which may improve diagnostic capabilities, and has the potential to guide the development of therapeutics for hearing loss by providing multiple intervention points for manipulation of the auditory system.