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Hearing Disorders: HELP
Articles from Toronto
Based on 256 articles published since 2010
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These are the 256 published articles about Hearing Disorders that originated from Toronto during 2010-2020.
 
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5 · 6 · 7 · 8 · 9 · 10 · 11
1 Guideline Clinical Practice Guideline: Sudden Hearing Loss (Update). 2019

Chandrasekhar, Sujana S / Tsai Do, Betty S / Schwartz, Seth R / Bontempo, Laura J / Faucett, Erynne A / Finestone, Sandra A / Hollingsworth, Deena B / Kelley, David M / Kmucha, Steven T / Moonis, Gul / Poling, Gayla L / Roberts, J Kirk / Stachler, Robert J / Zeitler, Daniel M / Corrigan, Maureen D / Nnacheta, Lorraine C / Satterfield, Lisa. ·1 ENT & Allergy Associates, LLP, New York, New York, USA. · 2 Zucker School of Medicine at Hofstra-Northwell, Hempstead, New York, USA. · 3 Icahn School of Medicine at Mount Sinai, New York, New York, USA. · 4 Kaiser Permanente, Walnut Creek, California, USA. · 5 Virginia Mason Medical Center, Seattle, Washington, USA. · 6 University of Maryland School of Medicine, Baltimore, Maryland, USA. · 7 The Hospital for Sick Children, Toronto, Canada. · 8 Consumers United for Evidence-Based Healthcare, Baltimore, Maryland, USA. · 9 Ear, Nose & Throat Specialists of Northern Virginia, PC, Manassas, Virginia, USA. · 10 University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA. · 11 Gould Medical Group-Otolaryngology, Stockton, California, USA. · 12 Columbia University Medical Center, New York, New York, USA. · 13 Mayo Clinic, Rochester, Minnesota, USA. · 14 StachlerENT, West Bloomfield, Michigan, USA. · 15 American Academy of Otolaryngology-Head and Neck Surgery Foundation, Alexandria, Virginia, USA. ·Otolaryngol Head Neck Surg · Pubmed #31369359.

ABSTRACT: OBJECTIVE: Sudden hearing loss is a frightening symptom that often prompts an urgent or emergent visit to a health care provider. It is frequently but not universally accompanied by tinnitus and/or vertigo. Sudden sensorineural hearing loss affects 5 to 27 per 100,000 people annually, with about 66,000 new cases per year in the United States. This guideline update provides evidence-based recommendations for the diagnosis, management, and follow-up of patients who present with sudden hearing loss. It focuses on sudden sensorineural hearing loss in adult patients aged ≥18 years and primarily on those with idiopathic sudden sensorineural hearing loss. Prompt recognition and management of sudden sensorineural hearing loss may improve hearing recovery and patient quality of life. The guideline update is intended for all clinicians who diagnose or manage adult patients who present with sudden hearing loss. PURPOSE: The purpose of this guideline update is to provide clinicians with evidence-based recommendations in evaluating patients with sudden hearing loss and sudden sensorineural hearing loss, with particular emphasis on managing idiopathic sudden sensorineural hearing loss. The guideline update group recognized that patients enter the health care system with sudden hearing loss as a nonspecific primary complaint. Therefore, the initial recommendations of this guideline update address distinguishing sensorineural hearing loss from conductive hearing loss at the time of presentation with hearing loss. They also clarify the need to identify rare, nonidiopathic sudden sensorineural hearing loss to help separate those patients from those with idiopathic sudden sensorineural hearing loss, who are the target population for the therapeutic interventions that make up the bulk of the guideline update. By focusing on opportunities for quality improvement, this guideline should improve diagnostic accuracy, facilitate prompt intervention, decrease variations in management, reduce unnecessary tests and imaging procedures, and improve hearing and rehabilitative outcomes for affected patients. METHODS: Consistent with the American Academy of Otolaryngology-Head and Neck Surgery Foundation's "Clinical Practice Guideline Development Manual, Third Edition" (Rosenfeld et al. RESULTS: The guideline update group made DIFFERENCES FROM PRIOR GUIDELINE: Incorporation of new evidence profiles to include quality improvement opportunities, confidence in the evidence, and differences of opinion Included 10 clinical practice guidelines, 29 new systematic reviews, and 36 new randomized controlled trials Highlights the urgency of evaluation and initiation of treatment, if treatment is offered, by emphasizing the time from symptom occurrence Clarification of terminology by changing potentially unclear statements; use of the term

2 Guideline Clinical Practice Guideline: Sudden Hearing Loss (Update) Executive Summary. 2019

Chandrasekhar, Sujana S / Tsai Do, Betty S / Schwartz, Seth R / Bontempo, Laura J / Faucett, Erynne A / Finestone, Sandra A / Hollingsworth, Deena B / Kelley, David M / Kmucha, Steven T / Moonis, Gul / Poling, Gayla L / Roberts, J Kirk / Stachler, Robert J / Zeitler, Daniel M / Corrigan, Maureen D / Nnacheta, Lorraine C / Satterfield, Lisa / Monjur, Taskin M. ·1 ENT & Allergy Associates, LLP, New York, New York, USA. · 2 Zucker School of Medicine at Hofstra-Northwell, Hempstead, New York, USA. · 3 Icahn School of Medicine at Mount Sinai, New York, New York, USA. · 4 University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA. · 5 Virginia Mason Medical Center, Seattle, Washington, USA. · 6 University of Maryland School of Medicine, Baltimore, Maryland, USA. · 7 The Hospital for Sick Children, Toronto, Canada. · 8 Consumers United for Evidence-based Healthcare (CUE), Baltimore, Maryland, USA. · 9 Ear, Nose & Throat Specialists of Northern Virginia, P.C., Manassas, Virginia, USA. · 10 Gould Medical Group-Otolaryngology, Stockton, California, USA. · 11 Columbia University Medical Center, New York, New York, USA. · 12 Mayo Clinic, Rochester, Minnesota, USA. · 13 StachlerENT, West Bloomfield, Michigan, USA. · 14 American Academy of Otolaryngology-Head and Neck Surgery Foundation, Alexandria, Virginia, USA. ·Otolaryngol Head Neck Surg · Pubmed #31369349.

ABSTRACT: OBJECTIVE: Sudden hearing loss is a frightening symptom that often prompts an urgent or emergent visit to a health care provider. It is frequently, but not universally, accompanied by tinnitus and/or vertigo. Sudden sensorineural hearing loss affects 5 to 27 per 100,000 people annually, with about 66,000 new cases per year in the United States. This guideline update provides evidence-based recommendations for the diagnosis, management, and follow-up of patients who present with sudden hearing loss. It focuses on sudden sensorineural hearing loss in adult patients aged 18 and over and primarily on those with idiopathic sudden sensorineural hearing loss. Prompt recognition and management of sudden sensorineural hearing loss may improve hearing recovery and patient quality of life. The guideline update is intended for all clinicians who diagnose or manage adult patients who present with sudden hearing loss. PURPOSE: The purpose of this guideline update is to provide clinicians with evidence-based recommendations in evaluating patients with sudden hearing loss and sudden sensorineural hearing loss, with particular emphasis on managing idiopathic sudden sensorineural hearing loss. The guideline update group recognized that patients enter the health care system with sudden hearing loss as a nonspecific primary complaint. Therefore, the initial recommendations of this guideline update address distinguishing sensorineural hearing loss from conductive hearing loss at the time of presentation with hearing loss. They also clarify the need to identify rare, nonidiopathic sudden sensorineural hearing loss to help separate those patients from those with idiopathic sudden sensorineural hearing loss, who are the target population for the therapeutic interventions that make up the bulk of the guideline update. By focusing on opportunities for quality improvement, this guideline should improve diagnostic accuracy, facilitate prompt intervention, decrease variations in management, reduce unnecessary tests and imaging procedures, and improve hearing and rehabilitative outcomes for affected patients. METHODS: Consistent with the American Academy of Otolaryngology-Head and Neck Surgery Foundation's RESULTS: The guideline update group made strong recommendations for the following: clinicians should distinguish sensorineural hearing loss from conductive hearing loss when a patient first presents with sudden hearing loss (KAS 1); clinicians should educate patients with sudden sensorineural hearing loss about the natural history of the condition, the benefits and risks of medical interventions, and the limitations of existing evidence regarding efficacy (KAS 7); and clinicians should counsel patients with sudden sensorineural hearing loss who have residual hearing loss and/or tinnitus about the possible benefits of audiological rehabilitation and other supportive measures (KAS 13). These strong recommendations were modified from the initial clinical practice guideline for clarity and timing of intervention. The guideline update group made strong recommendation against the following: clinicians should DIFFERENCES FROM PRIOR GUIDELINE: Incorporation of new evidence profiles to include quality improvement opportunities, confidence in the evidence, and differences of opinion Included 10 clinical practice guidelines, 29 new systematic reviews, and 36 new randomized controlled trials Highlights the urgency of evaluation and initiation of treatment, if treatment is offered, by emphasizing the time from symptom occurrence Clarification of terminology by changing potentially unclear statements; use of the term

3 Editorial Eriksholm Workshop on Hearing Impairment and Cognitive Energy. 2016

Pichora-Fuller, M Kathleen / Kramer, Sophia E. ·Department of Psychology, University of Toronto, Mississauga, Ontario, Canada, k.pichora.fuller@utoronto.ca Department of Otolaryngology-Head and Neck Surgery, Ear and Hearing Section and the EMGO Institute for Health and Care Research, VU University Medical Center, Amsterdam, The Netherlands. ·Ear Hear · Pubmed #27355766.

ABSTRACT: -- No abstract --

4 Editorial Nepal after the disaster. Insider points of view for the future of critical care medicine. 2015

Aryal, Diptesh / Acharya, Subhash P / Shrestha, Gentle S / Goffi, Alberto / Hawryluck, Laura. ·1 Department of Anesthesiology Tribhuvan University Teaching Hospital Kathmandu, Nepal. · 2 Interdepartmental Division of Critical Care Medicine University of Toronto Toronto, Ontario, Canada. ·Am J Respir Crit Care Med · Pubmed #26176763.

ABSTRACT: -- No abstract --

5 Review Endovascular treatment of debilitating tinnitus secondary to cerebral venous sinus abnormalities: a literature review and technical illustration. 2019

Yang, I-Hsiao / Pereira, Vitor M / Lenck, Stephanie / Nicholson, Patrick / Orru, Emanuele / Klostranec, Jesse M / Krings, Timo / Tsang, Anderson Chun On. ·Department of Medical Imaging, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan. · Medical Imaging, Toronto Western Hospital, Toronto, Ontario, Canada. · Division of Neuroradiology, Groupe Hospitalier Pitié Salpêtrière, University Paris Sorbonne, Paris, France. · Neuroradiology, Toronto Western Hospital, Toronto, Ontario, Canada. · Department of Neuroradiology, Beaumont Hospital, Dublin, Ireland. · Division of Neuroradiology, Joint Department of Medical Imaging, Toronto Western Hospital, Toronto, Ontario, Canada. · Division of Neuroradiology, Department of Medical Imaging and Division of Neurosurgery, Department of Surgery, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada. · Department of Surgery, The University of Hong Kong, Hong Kong. ·J Neurointerv Surg · Pubmed #30872352.

ABSTRACT: BACKGROUND AND OBJECTIVE: Pulsatile tinnitus (PT) can be debilitating and lead to significant morbidity. Cerebral venous sinus lesions, such as venous sinus stenosis, diverticula, and high-riding jugular bulb, are uncommon causes of PT, for which there is no standard treatment. Endovascular interventions have shown promising results for PT secondary to idiopathic intracranial hypertension, and may be a valid therapeutic option for isolated venous PT. METHODS: We conducted a systematic literature review on the outcome and safety of endovascular treatment for patients with isolated, debilitating venous PT. The venous lesion characteristics, endovascular techniques, complications, and clinical outcomes were assessed. In addition, an illustrative case of endovascular stenting for PT caused by venous sinus stenosis was included. RESULTS: A total of 41 patients (90.2% female) from 26 papers were included. The median age was 46 years (IQR 23; range 25-72 years). Focal venous sinus stenosis (20 patients) and sinus diverticula (14 patients) were the most common culprit lesions. Endovascular treatment included venous sinus stenting in 35 patients, 11 of whom had adjuvant coil embolization, and coil embolization alone in six patients. Complete resolution of the tinnitus was achieved in 95.1% of patients. There was one complication of cerebellar infarct, and no procedure-related mortality. CONCLUSIONS: In patients with debilitating PT secondary to venous sinus lesions, endovascular treatment by stenting and/or coil embolization appears to be safe and effective. Prospective randomized studies with objective outcome assessments are needed to confirm the treatment benefits.

6 Review Hearing, Emotion, Amplification, Research, and Training Workshop: Current Understanding of Hearing Loss and Emotion Perception and Priorities for Future Research. 2018

Picou, Erin M / Singh, Gurjit / Goy, Huiwen / Russo, Frank / Hickson, Louise / Oxenham, Andrew J / Buono, Gabrielle H / Ricketts, Todd A / Launer, Stefan. ·1 Vanderbilt University School of Medicine, Nashville, TN, USA. · 2 Phonak Canada, Mississauga, ON, Canada. · 3 Department of Speech-Language Pathology, University of Toronto, ON, Canada. · 4 Department of Psychology, Ryerson University, Toronto, ON, Canada. · 5 School of Health and Rehabilitation Sciences, University of Queensland, Brisbane, Australia. · 6 Department of Psychology, University of Minnesota, MN, USA. · 7 Sonova AG, Stäfa, Switzerland. ·Trends Hear · Pubmed #30270810.

ABSTRACT: The question of how hearing loss and hearing rehabilitation affect patients' momentary emotional experiences is one that has received little attention but has considerable potential to affect patients' psychosocial function. This article is a product from the Hearing, Emotion, Amplification, Research, and Training workshop, which was convened to develop a consensus document describing research on emotion perception relevant for hearing research. This article outlines conceptual frameworks for the investigation of emotion in hearing research; available subjective, objective, neurophysiologic, and peripheral physiologic data acquisition research methods; the effects of age and hearing loss on emotion perception; potential rehabilitation strategies; priorities for future research; and implications for clinical audiologic rehabilitation. More broadly, this article aims to increase awareness about emotion perception research in audiology and to stimulate additional research on the topic.

7 Review Approach to tinnitus management. 2018

Wu, Vincent / Cooke, Bonnie / Eitutis, Susan / Simpson, Matthew T W / Beyea, Jason A. ·Medical student in the Department of Otolaryngology at Queen's University in Kingston, Ont, at the time of writing. · Audiologist in the Department of Speech Language Pathology and Audiology at Hotel Dieu Hospital in Kingston. · Family physician in the Department of Family Medicine at Queen's University. · Otologist, neurotologist, and cranial base surgeon, Clinician Scientist, and Assistant Professor in the Department of Otolaryngology at Queen's University, and Adjunct Scientist at the Institute for Clinical Evaluative Sciences Queen's. jason.beyea@queensu.ca. ·Can Fam Physician · Pubmed #30002023.

ABSTRACT: OBJECTIVE: To provide family physicians with an evidence-based and practical approach to managing patients with tinnitus. SOURCES OF INFORMATION: MEDLINE was searched for English-language tinnitus guidelines and reviews. All such articles published between 1980 and 2016 were reviewed, with most providing level II and III evidence. MAIN MESSAGE: Tinnitus affects more than 40% of Canadians at least once in their lifetimes, most commonly older adults. Tinnitus is the perception of sound without external stimulation. It can greatly affect a patient's physical and psychological quality of life. Clinical history taking is directed at eliciting whether symptoms have a pulsatile or nonpulsatile quality, whether symptoms are unilateral or bilateral, and whether there is associated hearing loss. For tinnitus that is pulsatile or unilateral, referral to an otolaryngologist is recommended, as these qualities might be associated with more serious underlying conditions. Most patients with tinnitus can be managed with reassurance, conservative measures, and hearing aids if substantial hearing loss exists. CONCLUSION: Family physicians play the primary role in managing patients with tinnitus and are well situated to address both the physiologic and the psychological manifestations. As tinnitus is very common, helping patients cope with the symptoms through conservative measures and reassurance can prove to have the best outcomes.

8 Review Implications and attitudes of audiologists towards smartphone integration in hearing healthcare. 2018

Kimball, Suzanne H / Singh, Gurjit / John, Andrew B / Jenstad, Lorienne M. ·Department of Communication Sciences and Disorders, College of Allied Health, University of Oklahoma Health Sciences Center, USA. Electronic address: suzanne-kimball@ouhsc.edu. · Phonak, AG, Stäfa, Switzerland; Department of Speech-Language Pathology, University of Toronto, Toronto, Canada; Department of Psychology, Ryerson University, Toronto, Canada; Toronto Rehabilitation Institute-University Health Network, Toronto, Canada. · Department of Communication Sciences and Disorders, College of Allied Health, University of Oklahoma Health Sciences Center, USA. · School of Audiology & Speech Sciences, University of British Columbia, Vancouver, Canada. ·Hear Res · Pubmed #29945802.

ABSTRACT: In a relatively short period of time, modern societies have been transformed by the ubiquitous uptake of advanced and portable mobile communication, computation, and sensors available on smartphones. Looking forward, it is anticipated that smartphones will have an increasingly important role in health management including the delivery of hearing healthcare and operation of hearing instruments. OBJECTIVE: This paper provides a brief overview of the role of smartphones in audiologic rehabilitation and hearing research and reports on the findings of a survey assessing attitudes of audiologists towards smartphone integration in hearing healthcare. DESIGN: A total of 258 audiologists working in the United States completed the 10-item survey. RESULTS: The key finding from the survey is that practitioners generally expressed a high willingness to integrate smartphone technology in patient care. Counterintuitively, it was observed that clinicians with the least number of years of experience had relatively more negative attitudes toward smartphone integration in hearing healthcare than clinicians with comparatively more years of experience. CONCLUSIONS: The findings suggest that the attitudes of audiologists likely do not represent a barrier regarding smartphone integration in audiologic rehabilitation.

9 Review A protocol for a network meta-analysis of interventions to treat patients with sudden sensorineural hearing loss. 2018

Ahmadzai, Nadera / Kilty, Shaun / Wolfe, Dianna / Bonaparte, Jamie / Schramm, David / Fitzpatrick, Elizabeth / Lin, Vincent / Cheng, Wei / Skidmore, Becky / Moher, David / Hutton, Brian. ·The Ottawa Hospital Research Institute, Center for Practice Changing Research, 501 Smyth Road, Box 201, Ottawa, Ontario, K1H 8L6, Canada. · Department of ENT, The Ottawa Hospital, Ottawa, Canada. · Faculty of Health Sciences, University of Ottawa, Ottawa, Canada. · Children's Hospital of Eastern Ontario Research Institute, Ottawa, Canada. · Department of Otolaryngology-Head & Neck Surgery, Sunnybrook Health Sciences Centre, Sunnybrook Research Institute, Toronto, Canada. · Faculty of Medicine, University of Toronto, Toronto, Canada. · The Ottawa Hospital Research Institute, Center for Practice Changing Research, 501 Smyth Road, Box 201, Ottawa, Ontario, K1H 8L6, Canada. bhutton@ohri.ca. · The University of Ottawa School of Epidemiology, Public Health and Preventive Medicine, Ottawa, Canada. bhutton@ohri.ca. ·Syst Rev · Pubmed #29769117.

ABSTRACT: BACKGROUND: Hearing loss is one of the leading causes of disability worldwide, with greater than 20% of Canadian adults having measurable hearing loss in at least one ear. Patients with hearing loss experience impaired quality of life, and emotional and financial consequences that affect themselves and their families. Sudden sensorineural hearing loss (SSNHL) is a common but difficult to treat form of hearing loss that has a sudden onset of ≤ 72 h associated with various etiologies, with the majority of cases being idiopathic. Some patients may partially or completely recover hearing ability, but for 32 to 65% of patients whose hearing does not recover, feelings of social isolation elevate the risk of anxiety and depression. Hearing loss is also associated with poorer functional status, including difficulty with sound localization and hearing in noise. There exists a wide range of therapeutic options; however, treatment of idiopathic SSNHL is controversial because some patients recover spontaneously. The planned systematic review and network meta-analysis (NMA) will assess the relative effects of competing treatments for management of idiopathic SSNHL in adults. METHODS: Electronic search strategies were developed by an experienced medical information specialist in consultation with the review team. We will search MEDLINE, Embase, and the Cochrane Library with no date or language restrictions. Key clinical trial registries will also be searched for in-progress and completed trials. Two reviewers will independently screen the literature using pre-specified eligibility criteria, and assess the quality of included studies using the Cochrane Risk of Bias tool. Disagreements will be resolved through consensus or third party adjudication. Bayesian NMAs will be pursued to compare interventions in terms of their effects on hearing (including audiometric thresholds and speech recognition scores), extent of hearing recovery, quality of life, and incidence of harms (including vestibular dysfunction, incidence of infections, and withdrawals due to adverse events). DISCUSSION: This systematic review and NMA will offer new and informative evaluations of current therapies for SSNHL. The results will inform clinicians as to the relative benefits of the currently available interventions for managing this difficult condition, provide optimal clinical treatment strategies, establish evidence gaps, and identify promising treatments for evaluation in future trials. SYSTEMATIC REVIEW REGISTRATION: PROSPERO registration number: CRD 42017073756 .

10 Review Hearing, self-motion perception, mobility, and aging. 2018

Campos, Jennifer / Ramkhalawansingh, Robert / Pichora-Fuller, M Kathleen. ·Department of Psychology, University of Toronto, 100 St. George Street, Toronto, ON, M5S 3G3, Canada; Toronto Rehabilitation Institute, University Health Network, 550 University Avenue, Toronto, ON, M5G 2A2, Canada. Electronic address: Jennifer.Campos@uhn.ca. · Department of Psychology, University of Toronto, 100 St. George Street, Toronto, ON, M5S 3G3, Canada; Toronto Rehabilitation Institute, University Health Network, 550 University Avenue, Toronto, ON, M5G 2A2, Canada. · Department of Psychology, University of Toronto, 100 St. George Street, Toronto, ON, M5S 3G3, Canada. ·Hear Res · Pubmed #29661612.

ABSTRACT: Hearing helps us know where we are relative to important events and objects in our environment and it allows us to track our changing position dynamically over space and time. Auditory cues are used in combination with other sensory inputs (visual, vestibular, proprioceptive) to help us perceive our own movements through space, known as self-motion perception. Whether we are maintaining standing balance, walking, or driving, audition can provide unique and important information to help optimize self-motion perception, and consequently to support safe mobility. Recent epidemiological and experimental studies have provided evidence that hearing loss is associated with greater walking difficulties, poorer overall physical functioning, and a significantly increased risk of falling in older adults. Importantly, the mechanisms underlying the associations between hearing status and mobility are poorly understood. It is also critical to consider that age-related hearing loss is often concomitant with declines in other sensory, motor, and cognitive functions and that these declines may interact, particularly during realistic, everyday tasks. Overall, exploring the role of auditory cues and the effects of hearing loss on self-motion perception specifically, and mobility more generally, are important to both building fundamental knowledge about the perceptual processes underlying the ability to perceive our movements through space, as well as to optimizing mobility-related interventions for those with hearing loss so that they can function better when confronted by everyday, real-world, sensory-motor challenges. The goal of this paper is to explore the role of hearing in self-motion perception across a range of mobility-related behaviors. First, we briefly review the ways in which auditory cues are used to perceive self-motion and how sound inputs affect behaviors such as standing balance, walking, and driving. Next, we consider age-related changes in auditory self-motion perception and the potential consequences to performance on mobility-related tasks. We then describe how hearing loss is associated with declines in mobility-related abilities and increased adverse outcomes such as falls. We describe age-related changes to other sensory and cognitive functions and how these may interact with hearing loss in ways that affect mobility. Finally, we briefly consider the implications of the hearing-mobility associations with respect to applied domains such as screening for mobility problems and falls risk in those with hearing loss and developing interventions and training approaches targeting safe and independent mobility throughout the lifespan.

11 Review Binaural integration: a challenge to overcome for children with hearing loss. 2017

Gordon, Karen A / Cushing, Sharon L / Easwar, Vijayalakshmi / Polonenko, Melissa J / Papsin, Blake C. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, Ontario, Canada. ·Curr Opin Otolaryngol Head Neck Surg · Pubmed #29049038.

ABSTRACT: PURPOSE OF REVIEW: Access to bilateral hearing can be provided to children with hearing loss by fitting appropriate hearing devices to each affected ear. It is not clear, however, that bilateral input is properly integrated through hearing devices to promote binaural hearing. In the present review, we examine evidence indicating that abnormal binaural hearing continues to be a challenge for children with hearing loss despite early access to bilateral input. RECENT FINDINGS: Behavioral responses and electrophysiological data in children, combined with data from developing animal models, reveal that deafness in early life disrupts binaural hearing and that present hearing devices are unable to reverse these changes and/or promote expected development. Possible limitations of hearing devices include mismatches in binaural place, level, and timing of stimulation. Such mismatches could be common in children with hearing loss. One potential solution is to modify present device fitting beyond providing audibility to each ear by implementing binaural fitting targets. SUMMARY: Efforts to better integrate bilateral input could improve spatial hearing in children with hearing loss.

12 Review Linking Deafness and Dementia: Challenges and Opportunities. 2017

Lin, Vincent Y W / Black, Sandra E. ·*Department of Otolaryngology-Head & Neck Surgery †Department of Medicine, Division of Neurology, Sunnybrook Health Sciences Centre ‡Hurvitz Brain Sciences Program, Sunnybrook Research Institute, Faculty of Medicine, University of Toronto, Toronto, Canada. ·Otol Neurotol · Pubmed #28806332.

ABSTRACT: : The rising incidence of dementia locally and worldwide has now reached a critical level. The associated costs associated with these individuals will ultimately be untenable to most societies. Furthermore there is a paucity of highly effective treatments. However, the recent discovery of the association of hearing loss and dementia may open many potential opportunities. Although we still are acquiring knowledge on the pathophysiology of this association, clinicians are hopeful that our current highly effective treatments of hearing loss may ultimately be shown to have a positive effect on those with dementia.

13 Review Cochlear Implants Meet Regenerative Biology: State of the Science and Future Research Directions. 2017

Dabdoub, Alain / Nishimura, Koji. ·*Biological Sciences, Sunnybrook Research Institute †Department of Otolaryngology-Head & Neck Surgery ‡Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada §Shiga Medical Center Research Institute, Moriyama, Shiga, Japan. ·Otol Neurotol · Pubmed #28806331.

ABSTRACT: : The cochlear implant, the first device to restore a human sense, is an electronic substitute for lost mechanosensory hair cells. It has been successful at providing hearing to people with severe to profound hearing loss and as of 2012, an estimated 324,000 patients worldwide have received cochlear implants. Users of cochlear implants however, suffer from difficulties in processing complex sounds such as music and in discriminating sounds in noisy environments. Recent advances in regenerative biology and medicine are opening new avenues for enhancing the efficacy of cochlear implants by improving the neural interface in the future and offer the possibility of an entirely biological solution for hearing loss in the long term. This report comprises the latest developments presented in the first Symposium on cochlear implants and regenerative biology, held at the 14th International Conference on Cochlear Implants in 2016 in Toronto, Canada.

14 Review Diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS). 2017

Kuemmerle-Deschner, Jasmin B / Ozen, Seza / Tyrrell, Pascal N / Kone-Paut, Isabelle / Goldbach-Mansky, Raphaela / Lachmann, Helen / Blank, Norbert / Hoffman, Hal M / Weissbarth-Riedel, Elisabeth / Hugle, Boris / Kallinich, Tilmann / Gattorno, Marco / Gul, Ahmet / Ter Haar, Nienke / Oswald, Marlen / Dedeoglu, Fatma / Cantarini, Luca / Benseler, Susanne M. ·Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany. · Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey. · Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada. · Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France. · Translational Autoinflammatory Disease Section, NIAMS/NIH, Bethesda, Maryland, USA. · National Amyloidosis Centre, University College London Medical School, London, UK. · Haematologie, Onkologie und Rheumatologie, Universitaetsklinikum Heidelberg, Heidelberg, Germany. · University of California at San Diego, San Diego, California, USA. · Kinderrheumatologische Ambulanz, Universitaetsklinikum Eppendorf, Hamburg, Germany. · German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany. · Department of Rheumatology, Charité, University Medicine Berlin, Berlin, Germany. · UO Pediatria 2, G. Gaslini Institute, Genoa, Italy. · Istanbul University, Istanbul, Turkey. · Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. · Department of Rheumatology, Boston Children's Hospital, Boston, Massachusetts, USA. · Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA. · Rheumatology Unit, Policlinico Le Scotte, University of Sienna, Italy. · Rheumatology, Department of Paediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Alberta, Canada. ·Ann Rheum Dis · Pubmed #27707729.

ABSTRACT: Cryopyrin-associated periodic syndrome (CAPS) is a rare, heterogeneous disease entity associated with

15 Review Patient-Reported Measures of Hearing Loss and Tinnitus in Pediatric Cancer and Hematopoietic Stem Cell Transplantation: A Systematic Review. 2016

Stark, Daniel / Rosenberg, Abby R / Johnston, Donna / Knight, Kristin / Caperon, Lizzie / Uleryk, Elizabeth / Frazier, A Lindsay / Sung, Lillian. ·Section of Oncology and Cancer Research, Leeds Institute of Molecular Medicine, University of Leeds, United Kingdom. · Hematology/Oncology, Seattle Children's Hospital, WA. · Division of Paediatric Haematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, Canada. · Pediatric Audiology, Oregon Health and Science University, Portland. · Scholarly Communications and Researcher Skills, University of Leeds, United Kingdom. · E. M. Uleryk Consulting, Toronto, Ontario, Canada. · Dana-Farber/Boston Children's Cancer Center, MA. · Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada. ·J Speech Lang Hear Res · Pubmed #27603148.

ABSTRACT: Purpose: We identified studies that described use of any patient-reported outcome scale for hearing loss or tinnitus among children and adolescents and young adults (AYAs) with cancer or hematopoietic stem cell transplantation (HSCT) recipients. Method: In this systematic review, we performed electronic searches of OvidSP MEDLINE, EMBASE, and PsycINFO to August 2015. We included studies if they used any patient-reported scale of hearing loss or tinnitus among children and AYAs with cancer or HSCT recipients. Only English language publications were included. Two reviewers identified studies and abstracted data. Results: There were 953 studies screened; 6 met eligibility criteria. All studies administered hearing patient-reported outcomes only once, after therapy completion. None of the studies described the psychometric properties of the hearing-specific component. Three instruments (among 6 studies) were used: Health Utilities Index (Barr et al., 2000; Fu et al., 2006; Kennedy et al., 2014), Hearing Measurement Scales (Einar-Jon et al., 2011; Einarsson et al., 2011), and the Tinnitus Questionnaire for Auditory Brainstem Implant (Soussi & Otto, 1994). All had limitations, precluding routine use for hearing assessment in this population. Conclusions: We identified few studies that included hearing patient-reported measures for children and AYA cancer and HSCT patients. None are ideal to take forward into future studies. Future work should focus on the creation of a new psychometrically sound instrument for hearing outcomes in this population.

16 Review Hearing and vision screening tools for long-term care residents with dementia: protocol for a scoping review. 2016

McGilton, Katherine S / Höbler, Fiona / Campos, Jennifer / Dupuis, Kate / Labreche, Tammy / Guthrie, Dawn M / Jarry, Jonathan / Singh, Gurjit / Wittich, Walter. ·Department of Research, Toronto Rehabilitation Institute-University Health Network, Toronto, Ontario, Canada Lawrence S. Bloomberg Faculty of Nursing, University of Toronto, Ontario, Canada. · Department of Research, Toronto Rehabilitation Institute-University Health Network, Toronto, Ontario, Canada Department of Speech-Language Pathology, University of Toronto, Toronto, Ontario, Canada. · Department of Research, Toronto Rehabilitation Institute-University Health Network, Toronto, Ontario, Canada Department of Psychology, University of Toronto, Toronto, Ontario, Canada. · Department of Psychology, University of Toronto, Toronto, Ontario, Canada Department of Audiology, Baycrest Health Sciences, Toronto, Ontario, Canada. · Centre for Sight Enhancement, School of Optometry and Vision Science, University of Waterloo, Waterloo, Ontario, Canada. · Department of Kinesiology & Physical Education and Health Sciences, Faculty of Science, Wilfrid Laurier University, Ontario, Canada. · School of Optometry, University of Montréal, Montréal, Quebec, Canada. · Department of Research, Toronto Rehabilitation Institute-University Health Network, Toronto, Ontario, Canada Department of Speech-Language Pathology, University of Toronto, Toronto, Ontario, Canada Phonak AG, Stäfa, Switzerland Department of Psychology, Ryerson University, Toronto, Ontario, Canada. · School of Optometry, University of Montréal, Montréal, Quebec, Canada School of Physical and Occupational Therapy, McGill University, Montréal, Quebec, Canada CRIR/Centre de réadaptation MAB-Mackay du CIUSSS du Centre-Ouest-de-l'Île-de-Montréal. Montréal, Quebec, Canada CRIR/Institut Nazareth et Louis-Braille du CISSS de la Montérégie-Centre, Montréal, Quebec, Canada. ·BMJ Open · Pubmed #27466242.

ABSTRACT: INTRODUCTION: Hearing and vision loss among long-term care (LTC) residents with dementia frequently goes unnoticed and untreated. Despite negative consequences for these residents, there is little information available about their sensory abilities and care assessments and practices seldom take these abilities or accessibility needs into account. Without adequate knowledge regarding such sensory loss, it is difficult for LTC staff to determine the level of an individual's residual basic competence for communication and independent functioning. We will conduct a scoping review to identify the screening measures used in research and clinical contexts that test hearing and vision in adults aged over 65 years with dementia, aiming to: (1) provide an overview of hearing and vision screening in older adults with dementia; and (2) evaluate the sensibility of the screening tools. METHODS AND ANALYSIS: This scoping review will be conducted using the framework by Arksey and O'Malley and furthered by methodological enhancements from cited researchers. We will conduct electronic database searches in CENTRAL, CINAHL, EMBASE, MEDLINE and PsycINFO. We will also carry out a 'grey literature' search for studies or materials not formally published, both online and through interview discussions with healthcare professionals and research clinicians working in the field. Our aim is to find new and existing hearing and vision screening measures used in research and by clinical professionals of optometry and audiology. Abstracts will be independently reviewed twice for acceptance by a multidisciplinary team of researchers and research clinicians. ETHICS AND DISSEMINATION: This review will inform health professionals working with this growing population. With the review findings, we aim to develop a toolkit and an algorithmic process to select the most appropriate hearing and vision screening assessments for LTC residents with dementia that will facilitate accurate testing and can inform care planning, thereby improving residents' quality of life.

17 Review Aging and Hearing Health: The Life-course Approach. 2016

Davis, Adrian / McMahon, Catherine M / Pichora-Fuller, Kathleen M / Russ, Shirley / Lin, Frank / Olusanya, Bolajoko O / Chadha, Shelly / Tremblay, Kelly L. ·University College London. AD Cave Solutions. · Department of Linguistics, Macquarie University, Sydney, New South Wales, Australia. · Department of Psychology, University of Toronto, Ontario, Canada. · UCLA Center for Healthier Children, Families and Communities, Los Angeles, California. · Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University, Baltimore, Maryland. · Centre for Healthy Start Initiative, Ikoyi, Lagos, Nigeria. · World Health Organization, Geneva, Switzerland. · Department of Speech and Hearing Sciences, University of Washington, Seattle. tremblay@uw.edu. ·Gerontologist · Pubmed #26994265.

ABSTRACT: Sensory abilities decline with age. More than 5% of the world's population, approximately 360 million people, have disabling hearing loss. In adults, disabling hearing loss is defined by thresholds greater than 40 dBHL in the better hearing ear.Hearing disability is an important issue in geriatric medicine because it is associated with numerous health issues, including accelerated cognitive decline, depression, increased risk of dementia, poorer balance, falls, hospitalizations, and early mortality. There are also social implications, such as reduced communication function, social isolation, loss of autonomy, impaired driving ability, and financial decline. Furthermore, the onset of hearing loss is gradual and subtle, first affecting the detection of high-pitched sounds and with difficulty understanding speech in noisy but not in quiet environments. Consequently, delays in recognizing and seeking help for hearing difficulties are common. Age-related hearing loss has no known cure, and technologies (hearing aids, cochlear implants, and assistive devices) improve thresholds but do not restore hearing to normal. Therefore, health care for persons with hearing loss and people within their communication circles requires education and counseling (e.g., increasing knowledge, changing attitudes, and reducing stigma), behavior change (e.g., adapting communication strategies), and environmental modifications (e.g., reducing noise). In this article, we consider the causes, consequences, and magnitude of hearing loss from a life-course perspective. We examine the concept of "hearing health," how to achieve it, and implications for policy and practice.

18 Review Clinical Practice Recommendations for the Management and Prevention of Cisplatin-Induced Hearing Loss Using Pharmacogenetic Markers. 2016

Lee, Jong W / Pussegoda, Kusala / Rassekh, Shahrad R / Monzon, Jose G / Liu, Geoffrey / Hwang, Soomi / Bhavsar, Amit P / Pritchard, Sheila / Ross, Colin J / Amstutz, Ursula / Carleton, Bruce C / Anonymous7720860. ·*Center for Molecular Medicine and Therapeutics, Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada; †Pharmaceutical Outcomes Programme, British Columbia Children's Hospital, Vancouver, British Columbia, Canada; ‡Child and Family Research Institute, Vancouver, British Columbia, Canada; §Department of Pediatrics, Division of Pediatric Hematology/Oncology/Bone Marrow Transplant, British Columbia Children's Hospital, Vancouver, British Columbia, Canada; ¶Department of Medical Oncology, Tom Baker Cancer Center, University of Calgary, Calgary, Alberta, Canada; ‖Department of Medical Oncology, Princess Margaret Cancer Center, University of Toronto, Toronto, Ontario, Canada; **Faculty of Pharmaceutical Sciences, University of British Columbia, Vancouver, British Columbia, Canada; ††Division of Translational Therapeutics, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada; and ‡‡Institute of Clinical Chemistry, Inselspital Bern University Hospital, University of Bern, Bern, Switzerland. ·Ther Drug Monit · Pubmed #26960170.

ABSTRACT: Currently no pharmacogenomics-based criteria exist to guide clinicians in identifying individuals who are at risk of hearing loss from cisplatin-based chemotherapy. This review summarizes findings from pharmacogenomic studies that report genetic polymorphisms associated with cisplatin-induced hearing loss and aims to (1) provide up-to-date information on new developments in the field, (2) provide recommendations for the use of pharmacogenetic testing in the prevention, assessment, and management of cisplatin-induced hearing loss in children and adults, and (3) identify knowledge gaps to direct and prioritize future research. These practice recommendations for pharmacogenetic testing in the context of cisplatin-induced hearing loss reflect a review and evaluation of recent literature, and are designed to assist clinicians in providing optimal clinical care for patients receiving cisplatin-based chemotherapy.

19 Review Preoperative steroids for hearing preservation cochlear implantation: A review. 2016

Kuthubutheen, Jafri / Smith, Leah / Hwang, Euna / Lin, Vincent. ·a Department of Otolaryngology - Head and Neck Surgery , University of Toronto, Sunnybrook Health Sciences Centre , Ontario , Canada. · b Department of Otolaryngology - Head and Neck Surgery , School of Surgery, University of Western Australia , Perth , Australia. ·Cochlear Implants Int · Pubmed #26913646.

ABSTRACT: Preoperative steroids have been shown to be beneficial in reducing the hearing loss associated with cochlear implantation. This review article discusses the mechanism of action, effects of differing routes of administration, and side effects of steroids administered to the inner ear. Studies on the role of preoperative steroids in animal and human studies are also examined and future directions for research in this area are discussed.

20 Review Bilateral cochlear implants in children: Effects of auditory experience and deprivation on auditory perception. 2016

Litovsky, Ruth Y / Gordon, Karen. ·University of Wisconsin-Madison, 1500 Highland Ave, Madison, WI, 53705, United States. Electronic address: litovsky@waisman.wisc.edu. · University of Toronto, Canada. ·Hear Res · Pubmed #26828740.

ABSTRACT: Spatial hearing skills are essential for children as they grow, learn and play. These skills provide critical cues for determining the locations of sources in the environment, and enable segregation of important sounds, such as speech, from background maskers or interferers. Spatial hearing depends on availability of monaural cues and binaural cues. The latter result from integration of inputs arriving at the two ears from sounds that vary in location. The binaural system has exquisite mechanisms for capturing differences between the ears in both time of arrival and intensity. The major cues that are thus referred to as being vital for binaural hearing are: interaural differences in time (ITDs) and interaural differences in levels (ILDs). In children with normal hearing (NH), spatial hearing abilities are fairly well developed by age 4-5 years. In contrast, most children who are deaf and hear through cochlear implants (CIs) do not have an opportunity to experience normal, binaural acoustic hearing early in life. These children may function by having to utilize auditory cues that are degraded with regard to numerous stimulus features. In recent years there has been a notable increase in the number of children receiving bilateral CIs, and evidence suggests that while having two CIs helps them function better than when listening through a single CI, these children generally perform worse than their NH peers. This paper reviews some of the recent work on bilaterally implanted children. The focus is on measures of spatial hearing, including sound localization, release from masking for speech understanding in noise and binaural sensitivity using research processors. Data from behavioral and electrophysiological studies are included, with a focus on the recent work of the authors and their collaborators. The effects of auditory plasticity and deprivation on the emergence of binaural and spatial hearing are discussed along with evidence for reorganized processing from both behavioral and electrophysiological studies. The consequences of both unilateral and bilateral auditory deprivation during development suggest that the relevant set of issues is highly complex with regard to successes and the limitations experienced by children receiving bilateral cochlear implants. This article is part of a Special Issue entitled .

21 Review Sign Language and Spoken Language for Children With Hearing Loss: A Systematic Review. 2016

Fitzpatrick, Elizabeth M / Hamel, Candyce / Stevens, Adrienne / Pratt, Misty / Moher, David / Doucet, Suzanne P / Neuss, Deirdre / Bernstein, Anita / Na, Eunjung. ·Faculty of Health Sciences and Children's Hospital of Eastern Ontario Research Institute, Ottawa, Ontario, Canada; elizabeth.fitzpatrick@uottawa.ca. · Centre for Practice-Changing Research, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada; · Centre for Practice-Changing Research, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada; Translational Research in Biomedicine Graduate Program, University of Split School of Medicine, Split, Croatia; · Centre for Practice-Changing Research, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada; School of Epidemiology, Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada; · Consultant, Moncton, New Brunswick; · Children's Hospital of Eastern Ontario Research Institute, Ottawa, Ontario, Canada; Audiology Clinic, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada; and. · Voice for Hearing-Impaired Children, Toronto, Ontario, Canada. · Faculty of Health Sciences and Children's Hospital of Eastern Ontario Research Institute, Ottawa, Ontario, Canada; ·Pediatrics · Pubmed #26684476.

ABSTRACT: CONTEXT: Permanent hearing loss affects 1 to 3 per 1000 children and interferes with typical communication development. Early detection through newborn hearing screening and hearing technology provide most children with the option of spoken language acquisition. However, no consensus exists on optimal interventions for spoken language development. OBJECTIVE: To conduct a systematic review of the effectiveness of early sign and oral language intervention compared with oral language intervention only for children with permanent hearing loss. DATA SOURCES: An a priori protocol was developed. Electronic databases (eg, Medline, Embase, CINAHL) from 1995 to June 2013 and gray literature sources were searched. Studies in English and French were included. STUDY SELECTION: Two reviewers screened potentially relevant articles. DATA EXTRACTION: Outcomes of interest were measures of auditory, vocabulary, language, and speech production skills. All data collection and risk of bias assessments were completed and then verified by a second person. Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) was used to judge the strength of evidence. RESULTS: Eleven cohort studies met inclusion criteria, of which 8 included only children with severe to profound hearing loss with cochlear implants. Language development was the most frequently reported outcome. Other reported outcomes included speech and speech perception. LIMITATIONS: Several measures and metrics were reported across studies, and descriptions of interventions were sometimes unclear. CONCLUSIONS: Very limited, and hence insufficient, high-quality evidence exists to determine whether sign language in combination with oral language is more effective than oral language therapy alone. More research is needed to supplement the evidence base.

22 Review Mandibulofacial Dysostosis with Microcephaly: Mutation and Database Update. 2016

Huang, Lijia / Vanstone, Megan R / Hartley, Taila / Osmond, Matthew / Barrowman, Nick / Allanson, Judith / Baker, Laura / Dabir, Tabib A / Dipple, Katrina M / Dobyns, William B / Estrella, Jane / Faghfoury, Hanna / Favaro, Francine P / Goel, Himanshu / Gregersen, Pernille A / Gripp, Karen W / Grix, Art / Guion-Almeida, Maria-Leine / Harr, Margaret H / Hudson, Cindy / Hunter, Alasdair G W / Johnson, John / Joss, Shelagh K / Kimball, Amy / Kini, Usha / Kline, Antonie D / Lauzon, Julie / Lildballe, Dorte L / López-González, Vanesa / Martinezmoles, Johanna / Meldrum, Cliff / Mirzaa, Ghayda M / Morel, Chantal F / Morton, Jenny E V / Pyle, Louise C / Quintero-Rivera, Fabiola / Richer, Julie / Scheuerle, Angela E / Schönewolf-Greulich, Bitten / Shears, Deborah J / Silver, Josh / Smith, Amanda C / Temple, I Karen / Anonymous80847 / van de Kamp, Jiddeke M / van Dijk, Fleur S / Vandersteen, Anthony M / White, Sue M / Zackai, Elaine H / Zou, Ruobing / Anonymous90847 / Bulman, Dennis E / Boycott, Kym M / Lines, Matthew A. ·The Children's Hospital of Eastern Ontario Research Institute, University of Ottawa, Ottawa, Ontario, Canada. · Department of Pediatrics, University of Ottawa, Ottawa, Ontario, Canada. · Department of Genetics, The Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada. · Division of Medical Genetics, A. I. duPont Hospital for Children, Wilmington, Delaware. · Clinical Genetics Department, Belfast City Hospital, Belfast, UK. · Department of Pediatrics and Human Genetics, David Geffen School of Medicine, University of California, Los Angeles, California. · Division of Genetic Medicine, Department of Pediatrics, University of Washington, Seattle, Washington. · Center for Integrative Brain Research, Seattle Children's Research Institute, Seattle, Washington. · Department of Medical Genetics, Westmead Hospital, Sydney, Australia. · The Fred A. Litwin Family Centre in Genetic Medicine, University Health Network and Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada. · Department of Clinical Genetics, Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, Brazil. · Hunter Genetics, Newcastle, Waratah, Australia. · University of Newcastle, Newcastle - School of Medicine and Public Health, Faculty of Health, Callaghan, Australia. · Department of Clinical Genetics, Aarhus University Hospital, Aarhus, Denmark. · Department of Genetics, Permanente Medical Group, Roseville, California. · Division of Human Genetics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. · The Perelman School of Medicine of the University of Pennsylvania, Philadelphia, Pennsylvania. · Shodair Children's Hospital, Helena, Montana. · Medical Geneticist, Ottawa, Ontario, Canada. · Clinical Genetics and Metabolism, Floating Hospital for Children, Tufts Medical Center, Boston, Massachusetts. · West of Scotland Clinical Genetics Service, South Glasgow University Hospital, Glasgow, UK. · Harvey Institute for Human Genetics, Greater Baltimore Medical Center, Baltimore, Maryland. · Department of Clinical Genetics, Oxford University Hospitals NHS Trust, Oxford, UK. · Department of Medical Genetics, Alberta Children's Hospital Research Institute, University of Calgary, Calgary, Alberta, Canada. · Sección de Genética Médica, Servicio de Pediatría, Hospital Clínico Universitario Virgen de la Arrixaca, IMIB-Arrixaca, Murcia, Spain. · Grupo Clínico Vinculado al Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III (ISCIII), Madrid, Spain. · Department of Genetics, Sacramento Medical Center, Sacramento, California. · NSW Health Pathology, Newcastle, Australia. · West Midlands Regional Genetics Service, Birmingham Women's Hospital, Birmingham, UK. · Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania. · Department of Pathology and Laboratory Medicine, UCLA Clinical Genomics Center, David Geffen School of Medicine, University of California, Los Angeles, California. · Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas. · Genetic Counselling Clinic Kennedy Center, Copenhagen University Hospital, Rigshospitalet, Glostrup, Denmark. · Oxford Regional Genetics Service, The Churchill Hospital, Oxford University Hospitals NHS Trust, Oxford, UK. · Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UK. · Wessex Clinical Genetics Service, Princess Anne Hospital, University Hospital Southampton NHS Foundation Trust, Southampton, UK. · Department of Clinical Genetics, VU Medical Center, Amsterdam, The Netherlands. · Maritime Medical Genetics Service, IWKHealth Centre, Halifax, Nova Scotia, Canada. · Victoria Clinical Genetics Service, Murdoch Children's Research Institute, Melbourne, Australia. · Department of Paediatrics, University of Melbourne, Melbourne, Australia. · Newborn Screening Ontario, The Children's Hospital of Eastern Ontario, Ottawa, Canada. · Metabolics and Newborn Screening, Department of Pediatrics, The Children's Hospital of Eastern Ontario, Ottawa, Canada. ·Hum Mutat · Pubmed #26507355.

ABSTRACT: Mandibulofacial dysostosis with microcephaly (MFDM) is a multiple malformation syndrome comprising microcephaly, craniofacial anomalies, hearing loss, dysmorphic features, and, in some cases, esophageal atresia. Haploinsufficiency of a spliceosomal GTPase, U5-116 kDa/EFTUD2, is responsible. Here, we review the molecular basis of MFDM in the 69 individuals described to date, and report mutations in 38 new individuals, bringing the total number of reported individuals to 107 individuals from 94 kindreds. Pathogenic EFTUD2 variants comprise 76 distinct mutations and seven microdeletions. Among point mutations, missense substitutions are infrequent (14 out of 76; 18%) relative to stop-gain (29 out of 76; 38%), and splicing (33 out of 76; 43%) mutations. Where known, mutation origin was de novo in 48 out of 64 individuals (75%), dominantly inherited in 12 out of 64 (19%), and due to proven germline mosaicism in four out of 64 (6%). Highly penetrant clinical features include, microcephaly, first and second arch craniofacial malformations, and hearing loss; esophageal atresia is present in an estimated ∼27%. Microcephaly is virtually universal in childhood, with some adults exhibiting late "catch-up" growth and normocephaly at maturity. Occasionally reported anomalies, include vestibular and ossicular malformations, reduced mouth opening, atrophy of cerebral white matter, structural brain malformations, and epibulbar dermoid. All reported EFTUD2 mutations can be found in the EFTUD2 mutation database (http://databases.lovd.nl/shared/genes/EFTUD2).

23 Review Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation. 2015

Harrison, Robert V / Gordon, Karen A / Papsin, Blake C / Negandhi, Jaina / James, Adrian L. ·Department of Otolaryngology - HNS, Program in Neuroscience and Mental Health, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8; Department of Otolaryngology - Head and Neck Surgery, University of Toronto, 190 Elizabeth Street, Toronto, Ontario, Canada MG5 2N2. Electronic address: rvh@sickkids.ca. · Department of Otolaryngology - HNS, Program in Neuroscience and Mental Health, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8; Department of Otolaryngology - Head and Neck Surgery, University of Toronto, 190 Elizabeth Street, Toronto, Ontario, Canada MG5 2N2. · Department of Otolaryngology - HNS, Program in Neuroscience and Mental Health, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8. ·Int J Pediatr Otorhinolaryngol · Pubmed #26545793.

ABSTRACT: We discuss issues related to cochlear implantation in children with auditory neuropathy spectrum disorder (ANSD). We describe the varied nature of this disease category including the numerous potential causes of auditory neuropathy. The most prevalent etiology for infants with ANSD is associated with prolonged neonatal intensive care unit (NICU) stay. We discuss the potential contribution of cochlear hypoxia to this etiology. The second part of this review describes in detail our own experience at the Hospital for Sick Children in Toronto, with cochlear implantation of children diagnosed with ANSD. We outline the detection, diagnosis, and referral routes for our patients. We provide an overview of our "standard operation procedures" regarding candidacy, and discuss some of the special considerations that need to be applied to children with ANSD. This includes decisions to implant children with better audiometric thresholds that are standard in non-ANSD patients, concerns about the possibility of spontaneous remission and the appropriate timing of implantation. Finally we review an extensive published literature in outcomes after cochlear implantation (CI) in ANSD. This is not a systematic review but rather an exercise to distill out some important reoccurring themes and the general consensus of opinion to date. Our conclusion is that the hearing loss category ANSD, together with its numerous co-morbidities, is far too heterogeneous to make definitive statements about prognosis with CI.

24 Review Taking the History and Performing the Physical Examination in a Child with Hearing Loss. 2015

Cushing, Sharon L / Papsin, Blake C. ·Archie's Cochlear Implant Laboratory, Department of Otolaryngology Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. Electronic address: sharon.cushing@sickkids.ca. · Archie's Cochlear Implant Laboratory, Department of Otolaryngology Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. ·Otolaryngol Clin North Am · Pubmed #26409821.

ABSTRACT: Hearing loss is one of the most common childhood disorders and has far reaching effects on communication and socialization in children. Language acquisition, the most commonly sought and measured outcome, is tightly linked to age at diagnosis of the hearing loss and the speed with which rehabilitation is instituted. Treatment is often not affected by the underlying cause of the hearing loss and should be initiated at the time of initial identification. History-taking and physical examination in the setting of pediatric hearing loss are straightforward and should include an assessment of motor milestones, balance, and vestibular function.

25 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.

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