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S P R I N G 2 0 2 0
HEARING LOSS SPECIAL
I N T H I S I S S U E
BACD NEWSThe official newsletter of the British Academy of Childhood Disability
Welcome to our new look!Dr Yasmin DeAlwis, EditorWelcome to our new online edition of the newsletter. I hope you will agree with me that this change has beeninevitable given concerns with climate change. In addition to saving the planet, we now have the ability to expand thenewsletter so that important articles are published in a timely manner.
We are very pleased to run a chapter on ‘Childhood hearing impairment’ in keeping with the theme of this year’s ASM.These articles are very comprehensive and written by members of BAPA. They will undoubtedly update yourknowledge in the subject. I always find myself looking up which investigations to do at what age and aim to keep thesearticles as a reference for my practice. The BACD inaugural Gala dinner was held prior to the Annual Scientific Meeting (ASM) at the Crown Plaza Sheffield,where many achievements were celebrated. Congratulations to Jill Cadwgan and Belinda Crowe for organising such aninspiring and fun night. We have captured some of the highlights from the night on page 3. We hope you found the ASM useful opportunity to learn as well network with colleagues. I'd like to thank CatherineTuffrey for putting together such a great programme with fantastic speakers. A review of the ASM will appear in thesummer edition of the newsletter.
(c) Imelda Bell of Photography by Imelda
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R E G I O N A L R E P R E S E N T A T I V E SE a s t e r n M e r s e y N o r t h e r n N o r t h e r n I r e l a n d N o r t h W e s tO x f o r d S c o t l a n d S o u t h W e s t T h a m e s N E T h a m e s N W T h a m e s S E T h a m e s S W W a l e s W e s s e x W e s t M i d l a n d s Y o r k s h i r e
T R U S T E E SC h a i r V i c e C h a i r H o n . S e c r e t r a y H o n . T r e a s u r e r E X E C U T I V E C O M M I T T E EA c a d e m i c C o n v e n o r A u d i t L e a d D e p u t y A c . C o n v e n o rG o v e r n a n c e L e a d N e w s l e t t e r E d i t o r P a s t C h a i r S p e c i a l t y T r a i n i n gS t r a t e g i c R e s e a r c h G r o u p T r a i n e e R e p s A D M I N I S T R A T O R - K e l l y R o b i n s o nk e l l y . r o b i n s o n @ r c p c h . a c . u kb a c d @ r c p c h . a c . u k
BACDBritish Academy of Childhood Disability
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www.bacdis.org.uk
Discounts on BACD ConferencesQuarterly newsletter and monthly e-newsDiscounts on Paediatric Disability DistanceLearning CoursesReduced subscription to DevelopmentalMedicine & Child Neurology journal20% discount on all Mac Keith publications
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THIS IS AN OPEN ACCESS, SHORTER VERSION OF THE SPRING 2020 BACD NEWS.
TO READ THE FULL VERSION:
BACD is registered in England andWales under charity number 1177868
V a c a n tD r Z a b y B a s s iD r M o r a g A n d r e wD r C l i o n a C u m m i n g s & C l a i r e K e r rD r M e l a n i e M c M a h o nD r M a n d y R o s e & D r N i l u s h i k a W e e r a p p e r u m aD r A l e x a n d r a B a x t e r & D r V a l e r i e O r rD r T o m A l l p o r t & D r C a r o l i n e B o d e yD r T a n j a S a t t e r t h w a i t eD r M a d h u m i t a M u k h e r j e eD r S a m e e n a S h a k o o rD r S e t h u W a r i y a rD a w n F o r b e s & R i n a v a n d e r W a l tD r C a t h e r i n e T u f f r e yD r J a n e W a l t e rD r P a m G h o s h
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D r T o n i W o l f fD r I a n M a l eD r J i l l C a d w g a nD r G a b b y W h i t l i n g u m D r C a t h e r i n e T u f f r e yD r J e n n i f e r M c A n u f fD r M a d h u D a s a r a t h iD r B e n K oD r Y a s m i n D e A l w i sD r H i l a r y C a s sD r K a t h e r i n e M a r t i nP r o f J e r e m y P a r rD r K a t e H a r v e y &D r K a t y W o o d
PROMOTING THE HIGHESTPOSSIBLE STANDARD OF CLINICAL
CARE FOR DISABLED CHILDREN ANDYOUNG PEOPLE, AND THEIR
FAMILIES
Lifetime Achievement Award - Dr Val HarpinMany thanks to the BACD for giving me one ofthe first of these exciting honours. When I learntof the award I was prompted to think aboutmy career. I realise that I was fortunate in manyways. I was able to include research andteaching in my full time NHS clinical role; bothof which taught me a great deal and hugelyenriched my experience and I believe that of mypatients and their families. When Professor Sir David Hall suggested weneeded to support training for doctors workingwith disabled children and young people I wasthe lucky colleague given the challenge. WhenNeurodisability was becoming recognised as apaediatric speciality, I was fortunate to beelected the first chair of the BACD. I have been very lucky to be in the right places atthe right times with the right colleagues, here inSheffield and previously in Nottingham andOxford. I am grateful to all the amazing medicalsecretaries, therapists, nurses, psychologists,education professionals, social workers,administrators, managers, doctors and othercolleagues who supported me through goodtimes and sometimes really bad times. MDTsreally do work. A massive thank you too to all the children,young people and families with whom I have hadthe privilege to work for over more than 30years. You taught me so much. It has been such apleasure and support to share my passion andenthusiasm with my friends in the BACD. Congratulations on becoming a Charity. Thankyou from the bottom of my heart. Val
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BACD Gala Dinner and Achievement Awards
We were delighted that so many friends attended the Gala Dinnerand the first ever Achievement awards to formally celebrate BACDbecoming a charity, as well as inspirational achievements fromcolleagues, and children, young people and their families affectedby disability. The summer edition of the newsletter will showcase the winnersof the inaugural Achievement Awards.
Award Winners
Lifetime Achievement Dr Val Harpin and Prof Gillian BairdInspirational Young Person Naveed HafeezStar Quality Ethan PeacockInspirational Professional Susie TurnerInspirational Parent Carer Carlie GulInspirational Sibling Liam and OliverBest Photo that captures ethos of BACD (see front page)Imelda Bell's photo of Caitlin Goodsell
Otoacoustic emissions (OAEs)OAE’s describe sounds produced by the outer haircells of the cochlea in response to a stimulus soundwhich are transmitted back to the outer ear.Recording is affected by middle ear problems andnoise.Transient Evoked Otoacoustic Emissions (TEOAE’s)present at multiple frequencies suggest thathearing is better than 30dBHL
Auditory Evoked Potentials (Automated AuditoryBrainstem Response- ABR, Auditory BrainstemResponse, Auditory Steady State Responses, Middlelatency responses, Cortical Evoked Responses)
ABR, the most commonly used evoked potentialtest assesses the auditory nerve and brainstemresponse to acoustic stimuli.
LEVELS OF HEARING LOSS The British Society of Audiology describe the differentlevels of hearing loss as below: Degree of hearing loss Normal hearing Mild Moderate Severe Profound Even a mild hearing loss has a significant impact on achild’s ability to hear and discriminate speech,particularly in noisy situations such as a classroom. Auseful hearing loss simulator: ww.youtube.com/watch?v=TD5E88fFnxE HOW CAN WE CLASSIFY THE DIFFERENT TYPES OFHEARING TESTS?Hearing tests can be classified as objective or subjectivebased on whether the results depend on aresponse from the child or not. A Screening test give apass / fail for a specific parameter and Diagnostic testinforms of specific hearing levels, degree and type ofhearing loss. A Freefield or Soundfield test inform aboutchild’s hearing when both ears are working together,more closely reflecting day-to-day life. Speech testinginform about functional difficulties in hearing within aclassroom setting. There are specific tests for auditoryprocessing disorder. Objective tests of hearing
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Why do we need so many hearing tests?Dr Rosa Crunkhorn, Specialty trainee in Audiovestibular Medicine and Dr Shankar Rangan,Consultant Audiovestibular Physician, Wirral University Teaching Hospital
INTRODUCTIONThere is an ever-expanding range of diagnostic andscreening tests available both for paediatric hearing andvestibular assessment. This article aims to demystify thedifferent hearing tests available for children, as well asclarify the rationale for different approaches. How hearing works – a brief reminder
Conductive – blockage to the passage of soundthrough the outer and middle ear, commonly “glueear”. Usually temporary but can be permanent e.g.ossicular abnormalities.Sensorineural – inner ear usually within the cochlea,which is a permanent hearing loss.Mixed – a combination of conductive andsensorineural hearing lossCentral – problems with central processing of soundwith normal peripheral hearing assessmentAuditory neuropathy spectrum disorder - aheterogenous group characterised by dysfunction ofthe inner hair cells of the cochlea, the synapsesand/or the auditory nerve itself. This leads todifficulties with auditory perception.
National Deaf Children’s Society The outer ear funnels sound waves down the ear canaltowards the tympanic membrane, causing vibration ofthe ear drum which is conducted through the middleear by the three ossicles and into the inner ear via theoval window, causing the endolymph and hair cellswithin the cochlea to move. This movement istransduced into electrical energy which travels via thecochlear nerve to the brain, where it is perceived assound. TYPES OF HEARING LOSSProblems in different parts of the hearing pathwayscause the different type of hearing losses:
≤ 20 dB HL21 – 40 dB HL41 – 70 dB HL71 – 95 dB HL> 95 dB HL
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Acoustic immitance studies (Tympanometry andAcoustic reflexes)
Tympanometry provides information about middleear status, e.g. glue ear or eustachian tubedysfunctionAcoustic reflexes assess the reflex pathway(afferent pathway cranial nerve VIII; efferentpathway cranial nerve VII) in response to loudsound. Stimulating the ear leads to a reflexcontraction of the stapedial muscle which can berecorded at screening or diagnostic levels givinginformation both ipsi- and contralaterally
Behavioural observation audiometry (BOA): This testuses observation of an infant, usually aged 3-6months, during exposure to sounds. Infants may showa startle response at suprathreshold sounds or mayshow more subtle responses such as changes insucking or blinking for sounds closer to the hearingthreshold.
Visual reinforcement audiometry (VRA): This test iscommonly used in children from age 6 months to 2 ½years. The test conditions the child to turn when theyhear a sound, by presenting a visual reward, forexample a video screen with cartoon pictures.Different frequencies are tested either in soundfield(ears working together) or with inserts (ear specifictesting). Bone conduction levels can also be measured.
o ABR can be checked at different frequencies to givea representation of the child’s overall hearing. ABR isalso used in the diagnosis of auditory neuropathy.
Objective testing plays a vital role in the newborn hearingscreen programme (NHSP) in the UK, specifically with theuse of OAE’s and ABR6. If there is no clear response on two
tests of OAE’s, babies are assessed with automated ABRtesting. If no clear response is recorded on automated ABR,
babies are referred for diagnostic ABR which must beperformed within 4 weeks of birth as early intervention in
hearing loss leads to better outcomes for the child. Targeted follow up of hearing is also arranged for certainhigh-risk groups for example babies with Down’ssyndrome, certain congenital infections, Craniofacialabnormality, confirmed syndrome associated withhearing loss, etc. Subjective tests of hearingBehavioural tests of hearing assess the child’s wholeauditory pathway and require a child’s response tohearing a sound. The different tests are illustrated brieflybelow, with links to video’s which demonstrate muchmore clearly how the tests are performed.
www.youtube.com/watch?v=S45H3i2ulto
Distraction testing: This tests the ability of a childfrom 6-8 months to turn and localise to a sound. Thechild’s attention is held in front by a distractor. Asecond tester presents different sounds from behind.A positive response is a clear turn towards the soundon 2 out of 3 presentations. This test is not earspecific but can be very useful to give an idea offunctional hearing levels.
Performance audiometry / Play / Pure ToneAudiometry: children aged 2 ½ - 3 years areconditioned to complete a task when they hear asound, e.g. put a man into a boat. Threshold levels aremeasured by seeing the quietest response sound thechild can respond to. Slightly older children canprogress to play audiometry, wearing headphoneswith the same game. Pure tone audiometry (PTA) isthe gold standard hearing test in older children. PTAfollows the same principle as play audiometry –wearing headphones and responding each time thesound is heard. As a child gets older masking can beperformed if needed.
Speech tests: Assessments looking at speechdiscrimination, speech understanding, and wordrecognition can be used to give further functionalhearing information, and to support the findings ofbehavioural testing. There are a number of ageappropriate tests available, for example theMcCormick Toy Test can be used in children from theage of approximately 2 ½ years.
www.youtube.com/watch?v=LK5ExH4KwBI
www.youtube.com/watch?v=rr2m4fmirAM
Testing the more complex childWhich test to use depends on whether the child isdevelopmentally ready to turn their head to localise asound, whether vision limits the use of visual rewards, achild’s attention and concentration and other healthconditions and factors on the day rather than age alone. A routine hearing check to assess whether the child canhear speech sounds adequately for their speech andlanguage development a freefield assessment of hearingcan be sufficient. Explaining to parents that this test maynot detect unilateral hearing loss is essential. Hearing test may be modified for children withneurodevelopmental difficulties such as autism, ADHD orlearning difficulties. Children may not respond as theyare just not interesting, particularly at quieter levels. Usewhatever methods will gain as much information aspossible.
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Virus - part of the Herpes familyVery common – many adults are seropositive (CMVcauses a mild, febrile illness)Spread via close contact- urine, blood, breast milk,faecesInfected babies can shed virus for many yearsIf primary infection occurs during pregnancytransmission rate to foetus is 30-40%10% of infected foetuses will be symptomatic(ventriculomegaly, periventicular calcification,cataracts, petechiae/ purpura, hepatosplenomegaly,IUGR )90% are “asymptomatic” at birth – but up to 15% ofthis group will develop hearing loss
Late onset hearing loss is common- more than 50% ofchildren diagnosed with cCMV associated hearingloss have normal hearing at birth and pass their newborn screening.Deterioration in hearing in cCMV is a commonfinding- unilateral loss can become a bilateral loss, ormild losses can become severe.Deterioration in hearing has been reported up to latechildhood but most common in under 8 yearolds.Type of hearing loss can be variable.Balance difficulties are also common.
Congenital Cytomegalovirusand Hearing lossDr Martina Stones, Associate Specialist,Community Paediatrics, Tayside WHAT DO WE KNOW?Congenital CMV infection is the most common, nongenetic cause of permanent hearing loss. WHAT IS CYTOMEGALOVIRUS (CMV)?
Hearing loss in Congenital CMV (cCMV)
Congenital CMV is diagnosed at birth in symptomaticchildren- urine/saliva viral culture or PCR.In children identified with hearing loss from thenewborn hearing screen
if day 21 or before then urine/saliva viral detectionif over 21 days of age then CMV IgG and, if positive,CMV PCR on neonatal stored heel prick sample toconfirm presence of virus at birth and not a postnatal infection.
Anti viral treatment for symptomatic cCMV -Recommended 6 months of oral treatment -16mg/kg/dose valganciclovir oral solution twice dailyIf hearing loss diagnosed and cCMV confirmed priorto 1 month consider treatment Antiviral treatments have been shown to reduce therisk of hearing deterioration. Known side effects areshort-term neutropenia.
Try to not share food, cups or cutlery, or put child'sdummy in your mouth. Give kisses on the head ratherthan mouth.Clean hands after touching a child's urine or saliva.Wash hands well for 15-20 seconds with soap andwater.Wash items that may have been in contact with achild's saliva or urine.
DIAGNOSIS
Note local variation in length of time blood spots arestored for- may affect ease of diagnosis. MANAGEMENTIn discussion with local virology/ infectious diseasepaediatricians:
What can be done to reduce incidence of cCMVhearing loss?Infected babies continue to shed virus for many months.Therefore education of pregnant mums with toddlers,pregnant women working in nursery/ child caresettings, workers of child bearing age in high riskworkplaces is vital. These groups should be advised to:
For further information visit www.cmvaction.org.uk
The British Association of Paediatricians in Audiology(BAPA) was inaugurated in 2007 as an association ofpaediatricians practising audio-vestibular medicine.BAPA has its roots in the former British Association ofCommunity Doctors in Audiology (BACDA) anorganisation begun in 1985 by a small group of cliniciansdedicated to the development of high quality hearingassessments for children.
www.bapa.uk.com
PAUL POLANI AWARD 2020This year's award was held in conjunction with theChartered Society of Physiotherapists Charitable Trust,which co-funded the £60,000 award with the RoyalCollege of Paediatrics and Child Health. The Award called for a study of clinically relevantphysiotherapy interventions for children withneurodevelopmental conditions, disability, and theirfamilies. The 2020 Award has been awarded to Dr SarahCrombie, Expert Clinical Academic Physiotherapist andResearch Fellow, Chailey Clinical Services, SussexCommunity NHS Foundation Trust (SCFT) and MDTcolleagues for the study: Breathe-Easy: a pilot study to examine the acceptability
and feasibility of a novel night-time posturalmanagement night-time intervention (PMN-TI) to
improve respiratory health of children with complexneuro-disability
We hope to bring you a summary of the project in theJune newsletter.
THERAPY PRIORITY SETTING WORKSHOPSMembers will recall that BACD funded the prioritysetting partnership to identify the top 10 unansweredquestions in childhood disability research. The numberone question identified was therapy interventions. Todate, no studies have been funded relating to thispriority. With funding from the Castang Foundation, the BACDStrategic Research Group (SRG) delivered a therapy-specific priority setting process, including twoworkshops in London and Leeds with therapist delegatesand NIHR HTA in September 2019. Five clear priorities relating to speech and languagetherapy, physiotherapy, and occupational therapy wereidentified, as well as a longer list of other importanttopics. This has been reviewed by NIHR and worked upas research questions/PICOS by the SRG, which havenow entered the NIHR HTA development pipeline. It ishoped that they will become the subject of multiplecommissioned calls. Lindsay Pennington has writtenreport with other SRG therapists (especially SarahCrombie, Chailey and Rob Brooks, Leeds). BACD-CASTANG FELLOWSHIP WORKSHOPSCohort 1 Fellows have been awarded £1.5 million in grantfunding since the Fellowship programme commenced.The Castang Foundation is positive about the impact andis interested in potentially supporting further awards/related initiatives. Cohort 2, year 2 was delivered by Jennifer McAnuff,Jeremy Parr, and others from SRG, along with invitedclinical academics for two days of workshops prior to theBACD annual scientific meeting in March 2020. TheCohort 2 Fellows are progressing well and feedback hasbeen positive.
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Research UpdateProfessor Jeremy Parr, Chair, BACD Strategic Research Group
Strategic Research Group Castang Fellowship programmeAwardsPriority Setting Partnerships
There is a dedicated RESEARCH sectionon the BACD website, where you can find
information on:
www.bacdis.org.uk/pages/16- research
