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deafness

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1. Childhood deafness associated with marriage/relationships between first cousins

factors for childhood sensorineural hearing loss in the Oxford Region. Br J Audiol 1997;31:39-54. (Oxford – 2.5 times greater risk) 6. Turner S. Children under five with sensorineural hearing loss from ethnic minority families a survey into provision by educational services for pre-school hearing impaired children in England 1995, Manchester: Internal report, Centre for audiology, education of the deaf and speech pathology, University of Manchester, 1996. 7. Vanniasegaram I, Tungland O P, Bellman S (...) , but would not have a hearing loss themselves. 2.6 Sometimes a gene mutation is a one-off that simply occurred by chance. This means that, although the hearing loss has a genetic cause, it has happened for the first time in that person and has not been inherited. However, it could be passed onto future generations. 2.7 We all carry several altered genes – probably about three or four on average. For many genes having a second working copy of the gene is enough to make up for an altered copy so

2018 National Deaf Children's Society

2. Genetics stem cell therapy and deafness

that a hearing loss will worsen over time and therefore more frequent hearing checks may be needed. It might also mean that hearing aids will not provide a child with access to sound and that families should consider cochlear implants for their child, particularly for children with some types of auditory neuropathy. 3.15. WGS can also give families information on ‘additional findings’ in their genome. These are not related to the deafness that led them to have the testing. ‘Additional findings’ are genomic (...) Genetics stem cell therapy and deafness POSITION STATEMENT: MEDICAL ADVANCEMENTS IN GENETICS AND INTERVENTIONS TO PREVENT OR REDUCE HEARING LOSS Medical advancements in genetics & interventions position statement 20180324 - ED PaC 1 of 11 Owner/s: Director Policy and Campaigns Author/s: Head of Policy and Research Development Manager (Audiology & Health) Issuing Team/Department: Policy and Campaigns Version No: V3 Date Approved: ? EDs ? Committees ? Trustees 13 February 2018 1 March 2018 24

2018 National Deaf Children's Society

3. Emotional wellbeing and mental health of deaf children and young people

a) Early support: Parents and the wider family are crucial in the promotion of positive mental health for deaf children. However, 90% of deaf children are born to hearing parents who have little or no experience of deafness. Parents therefore need access to immediate support and impartial information about deafness following diagnosis of their child. Parents should be enabled to be key partners and empowered to make informed choices over how they can best support their child. Parent to parent support (...) is vital and can be encouraged through, for example, family weekends and local groups. b) Communication within the family: Deaf children will have different communication and language needs from hearing children, consequently the development of communication and language skills in some deaf children can be delayed significantly. This can impact on a deaf child’s interpersonal and social skills. To ensure that parents are able to communicate with their child from the point of diagnosis, they need access

2017 National Deaf Children's Society

4. Early Identification of Deafness in Childhood

alternative recommendations, the National Deaf Children’s Society supports government policy in England, Wales and Northern Ireland to ensure all children receive hearing screening at school between the ages of 4-7 years. 2. Introduction Permanent hearing loss 2.1. Newborn hearing screening completed roll-out across the UK by 2006. During its period of implementation the programme has proved very successful in reducing the age of diagnosis for babies born deaf, and provides the opportunity for early (...) at the age of 18 years 6 . Temporary hearing loss 2.2. Temporary deafness caused by ‘otitis media with effusion’ (a common childhood condition known as ‘glue ear’) affects up to 80% of children before the age of 10 years at some point 7 . Glue ear usually resolves spontaneously within three months. However, 30-40% of children have recurrent episodes and 5-10% of these episodes last for more than a year. During this time, and without appropriate awareness and support, glue ear can also have a significant

2017 National Deaf Children's Society

5. Communication Support For Deaf Children Who Use British Sign Language (BSL)

Communication Support For Deaf Children Who Use British Sign Language (BSL) 1 of 3 Communication support for deaf children in early years settings, schools and colleges who use BSL to access teaching and learning position statement Owner/s: Director Policy and Campaigns Author/s: Head of Policy and Research Issuing Team/Dept: Policy and Campaigns Version No: V1 Date Approved: ? EDs ? Committee ? Trustee Board N/A 5 November 2016 N/A Review Frequency: Three years Next Review Date: 2019/20 (...) Circulation (primary location): Website 1. Introduction 1.1 Good language and communication skills are at the heart of deaf children being able to make friends and succeed in education. 1.2 It is logical that the quality of a deaf child’s communication support as well as the quality of teaching directly contributes to that child’s ability to learn and achieve their potential in an educational environment. The role of the communication support worker is to support deaf learners in and outside the classroom

2016 National Deaf Children's Society

6. Children deafness - 0 to 6 years

approach The first approach, itself divided into two, has as an essential principle the will to stimulate auditory function and thereby enable the deaf child to develop a socially useful spoken language. It is proposed that this approach be called audiophonic, aiming to stimulate hearing early in order to encourage the development of spoken language (comprehension then expression). Practical implementation of this first approach is based on data from auditory assessments, which must be obtained (...) whatever age the child is. According to the level of hearing, auditory aids, then, if indicated, cochlear implants are used to stimulate the auditory pathways of the deaf child. During the last decade, significant technological progress has been made in stimulation methods. The educational environment proposed to parents to take advantage of auditory stimulation, and to promote the development of spoken language in the deaf child uses two methods from early intervention programme implementation

2010 HAS Guidelines

7. Hearing loss in adults

onset or slowly progressive. Conductive hearing loss occurs due to abnormalities of the outer or middle ear which impair sound wave conduction to the cochlea in the inner ear. Sensorineural hearing loss occurs due to abnormalities in the cochlea, auditory nerve or other structures in the neural pathway from the inner ear to the auditory cortex. Classification of hearing loss as mild, moderate, severe or profound is based on the quietest sound (in decibels) which can be discerned on pure tone (...) Hearing loss in adults Hearing loss in adults | Topics A to Z | CKS | NICE Search CKS… Menu Hearing loss in adults Hearing loss in adults Last revised in September 2019 Hearing loss is common at any age, it can be temporary, permanent, sudden onset or progressive, conductive or sensorineural. Diagnosis Management Background information Hearing loss in adults: Summary Hearing loss is common and can occur at any age — depending on the underlying cause it can be temporary or permanent; of sudden

2019 NICE Clinical Knowledge Summaries

8. Hearing Loss and/or Vertigo

without IV contrast Usually Not Appropriate O MRA head without and with IV contrast Usually Not Appropriate O MRA head without IV contrast Usually Not Appropriate O ACR Appropriateness Criteria ® 3 Hearing Loss and/or Vertigo Variant 5: Congenital hearing loss or total deafness or cochlear implant candidate. Surgical planning. Procedure Appropriateness Category Relative Radiation Level CT temporal bone without IV contrast Usually Appropriate ??? MRI head and internal auditory canal without and with IV (...) and internal auditory canal without IV contrast, or without and with IV contrast, is recommended for evaluating patients with acquired sensorineural hearing loss. ? Variant 4: Mixed conductive and sensorineural hearing loss can be evaluated with either CT temporal bone without IV contrast, or MRI head and internal auditory canal without IV contrast, or without and with IV contrast. ? Variant 5: Congenital hearing loss, total deafness, or cochlear implant is best assessed with either CT temporal bone

2018 American College of Radiology

9. Sudden Hearing Loss (Full text)

obtain, or refer to a clinician who can obtain, audiometry as soon as possible (within 14 days of symptom onset) to confirm the diagnosis of sudden sensorineural hearing loss. (KAS 6) Clinicians should evaluate patients with sudden sensorineural hearing loss for retrocochlear pathology by obtaining magnetic resonance imaging or auditory brainstem response. (KAS 10) Clinicians should offer, or refer to a clinician who can offer, intratympanic steroid therapy when patients have incomplete recovery from (...) is emphasized. KAS 5—New studies were added to confirm the lack of benefit of nontargeted laboratory testing in sudden sensorineural hearing loss. KAS 6—Audiometric follow-up is excluded as a reasonable workup for retrocochlear pathology. Magnetic resonance imaging, computerized tomography scan if magnetic resonance imaging cannot be done, and, secondarily, auditory brainstem response evaluation are the modalities recommended. A time frame for such testing is not specified, nor is it specified which

2019 American Academy of Otolaryngology - Head and Neck Surgery PubMed abstract

10. Hearing loss in adults: assessment and management

not to remove earwax or clean their ears by inserting small objects, such as cotton buds, into the ear canal. Explain that this could damage the ear canal and eardrum, and push the wax further down into the ear. Hearing loss in adults: assessment and management (NG98) © NICE 2019. All rights reserved. Subject to Notice of rights (https://www.nice.org.uk/terms-and- conditions#notice-of-rights). Page 8 of 201.3 Investigation using MRI 1.3.1 Offer MRI of the internal auditory meati to adults with hearing loss (...) and localising symptoms or signs (such as facial nerve weakness) that might indicate a vestibular schwannoma or CPA (cerebellopontine angle) lesion, irrespective of pure tone thresholds. 1.3.2 Consider MRI of the internal auditory meati for adults with sensorineural hearing loss and no localising signs if there is an asymmetry on pure tone audiometry of 15 dB or more at any 2 adjacent test frequencies, using test frequencies of 0.5, 1, 2, 4 and 8 kHz. 1.4 Treating idiopathic sudden sensorineural hearing loss

2018 National Institute for Health and Clinical Excellence - Clinical Guidelines

11. American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss

American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss 347 © American College of Medical Genetics and Genomics ACMG PrACtiCe Guidelines DEFINITIONS Deaf: a community with a distinct culture and language shaped by the experience of being deaf or hard of hear- ing, which may include deaf, hard-of-hearing, and hearing individuals deaf: an auditory phenotype characterized by a total or near- total loss of the ability to hear (...) hard of hearing: an auditory phenotype characterized by a par- tial loss of the ability to hear hearing loss: an auditory phenotype characterized by any degree of loss of the ability to hear; depending on cause, hearing loss can be temporary or permanent—this guideline focuses on permanent hearing loss Submitted 6 January 2014; accepted 6 January 2014; advance online publication 20 March 2014. doi:10.1038/gim.2014.2 Genet Med 00 00 2014 Genetics in Medicine 10.1038/gim.2014.2 ACMG Practice

2014 American College of Medical Genetics and Genomics

12. Occupational therapy for people with Parkinson's disease

the British Library. While every effort is made to ensure accuracy, the College of Occupational Therapists shall not be liable for any loss or damage directly or indirectly resulting from the use of this publication. ISBN 978-1-905944-16-3 Typeset by Servis Filmsetting Ltd, Stockport, Cheshire Digitally printed on demand in Great Britain by The Lavenham Press, Suffolk PARKINSON'S ALL TEXT.indd ii 27/4/10 11:44:33 iii College of Occupational Therapists Contents Foreword iv Part 1 Guideline development

2010 Publication 1554

14. Peripheral venous cannulation of children

. Indications of occluded arterial supply include: loss of colour, compromised pulse and pain. Loosen and remove the tourniquet immediately if any indication of occluded arterial supply is observed. Lightly tapping the vein or instructing the child to clench or pump the fist can encourage further venous filling ( ). Palpate the intended vein by placing one or two fingers over the vein and pressing lightly ( ). Disinfect the skin at the site at which it is intended to insert the needle, with 2% chlorhexidine (...) features. Rationale 17: Many families will have had previous experiences of cannulation or how their child copes with a stressful situation. Rationale 18: Thumb sucking will be an important coping strategy during the stressful experience of hospitalisation ( ). Rationale 19: This would represent a betrayal of the child’s trust ( ). Rationale 20: Increased risk of extravasation and phlebitis ( ). Rationale 21: Sixty minutes will achieve analgesia to pin prick but not loss of sensation or touch

2017 Publication 1593

15. Recovery: care of the child/young person

needs blood loss/fluid replacement blood components used/available intravenous fluids given and future requirements antibiotics given and when the next dose is due urine output during the procedure and expected output for the next few hours patient's anxiety level and preoperative psychological problems further investigations required how much oxygen is required and how to administer it monitoring required in the recovery room and on return to the ward possible language barrier if a throat pack has (...) to recovery staff should include: ( ) care and placement of drains precautions about dressings special nursing requirements, such as positioning of the child details of pre-operative skin integrity and any visible changes post -surgery ensuring the correct charts accompany the patient noting that personal belongings such as hearing aids and comforters, toys etc are present ( any isolation precautions and reason required Surgical handover should be included if there are specific surgical instructions

2015 Publication 1593

16. Peripheral venous cannulation of children

. Indications of occluded arterial supply include: loss of colour, compromised pulse and pain. Loosen and remove the tourniquet immediately if any indication of occluded arterial supply is observed. Lightly tapping the vein or instructing the child to clench or pump the fist can encourage further venous filling ( ). Palpate the intended vein by placing one or two fingers over the vein and pressing lightly ( ). Disinfect the skin at the site at which it is intended to insert the needle, with 2% chlorhexidine (...) features. Rationale 17: Many families will have had previous experiences of cannulation or how their child copes with a stressful situation. Rationale 18: Thumb sucking will be an important coping strategy during the stressful experience of hospitalisation ( ). Rationale 19: This would represent a betrayal of the child’s trust ( ). Rationale 20: Increased risk of extravasation and phlebitis ( ). Rationale 21: Sixty minutes will achieve analgesia to pin prick but not loss of sensation or touch

2014 Publication 1593

17. Height: measuring a child/young person

scapula, wherever possible, against the backboard arms loosely at their side The child/young person’s head must be positioned with the lower margins of the orbit in the same horizontal plane as the external auditory meatus, i.e. the corner of the eyes horizontal to the middle of the ear . The headboard of the apparatus should be weighted with a 500gram wipeable beanbag and placed carefully on the child/young person’s head . Ensure the child/young person is in the correct position and hold (...) the child/young person’s head with the lower margins of the orbit in the same horizontal plane as the external auditory meatus, i.e. the corner of the eyes horizontal to the middle of the ear . The other person should position the child/young person with their: feet together heels touching the back plate of the measuring instrument legs straight and in alignment with the body buttocks against the backboard scapula, wherever possible, against the backboard The patient’s ankles should be supported

2014 Publication 1593

18. External ventricular drainage

. This is to prevent the filters within the drip chamber becoming wet which could affect the efficiency of the drainage ( ). It is the responsibility of the neurosurgeon to give instructions on the level at which the drain is to be set or the amount of drainage required each hour and document in the patient’s medical records. The level of the ventricles must be estimated to create a zero reference point: Draw an imaginary line between the outer aspect of the child’s eye and the external auditory meatus (...) records. If the EVD continues to not drain, the child may need to return to theatre for a new drain to be inserted. The frequency of observations increased (every fifteen minutes may be required dependant on the child's neurological status). Any changes in neurological status must be highlighted to the neurosurgical team promptly. Drain management: fluid and electrolyte balance CSF contains approximately 120mmol/l of sodium. The child should have their electrolytes measured regularly, daily if losses

2015 Publication 1593

19. Pregnancy and Renal Disease

, (Piccoli et al., 2018, #13860) which equates to between 15,000-20,000 pregnancies per year in England. The prevalence of CKD in pregnancy is predicted to rise in the future due to increasing maternal age and obesity. Although CKD is not a barrier to reproduction in most women, the risk of adverse pregnancy outcomes is increased in women with CKD including pre-eclampsia, fetal growth restriction, preterm delivery and accelerated loss of maternal renal function. CKD impacts on communication, decision

2019 Renal Association

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