20/09/2018 1 Management of Hyperacusis in Children Veronica Kennedy and Rosa Crunkhorn BACCH 10 th September 2018 Case 1 » 3 year old » Dislikes hand driers, hoovers, motorbikes » Covers ears with hands – Flinches, panics » Otherwise well, normal development
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Microsoft PowerPoint - BACCH Sept 2018 Hyperacusis 2Veronica Kennedy and Rosa Crunkhorn BACCH 10th September 2018 Case 1 » Covers ears with hands 20/09/2018 3 Definitions – Range of different descriptions may describe different sensations • Likely different underlying mechanisms and aetiologies » Phonophobia – fear of sound, anticipatory anxiety » Misophonia – dislike for selected sounds Prevalence Coelho, Sanchez, Tyler 2007 • 506 school children (512yrs) • 42% bothered by sounds • 3.2% had hyperacusis–of whom 50% tinnitus • 9% phonophobia 20/09/2018 4 Prevalence • 3.7% children reported hyperacusis. Bolton Case Review – Review of notes 1 3 0 5 10 15 20 1y 2y 3y 4y 5y 6y 7y 8y 9y 10y 11y 15y N um be r of c hi ld re n Male:Female 1.65:1. (in order of frequency) Number of children (%) Vacuum cleaner Hair/hand dryer Siren/alarms Washing machine Clapping 20/09/2018 6 24% N u m b er o f ch ild re n ( % ) Reported Behavioural Responses • Cover ears 62% • General dislike 13% • Hates parties, family had to whisper happy birthday song • Avoids going to school dinner hall as noisy • Dreads school bell ringing • Family had to keep stopping to let noisy vehicles pass while walking Assoc medical conditions in relation to age < 5 yrs ≥ 5yrs A: Active middle ear problem 11 4 B: Neurodevelopmental condition 4 10 C: Both of the above 11 3 D: Past history glue ear 2 3 E: Sensorineural hearing loss 0 1 F: None of the above 8 4 A AB Autistic spectrum disorder Cerebral Palsy Attention deficit hyperactivity disorder Down’s syndrome Hydrocephalus Epilepsy Williams syndrome Leigh’s syndrome Microdeletion 15q13.3 Possible Syndrome (facial dysmorphism, developmental delay, congenital heart defect) 8 (13%) 3 2 2 2 2 2 1 1 1 1 1 1 1 Results Summary • Hearing loss present in about onethird • Middle ear problems/history in nearly half of them (OME: 11, Hx OME: 8) • Tinnitus reported in 13% of children • Neurodevelopmental/significant behavioural problems in more than half 55% (ASD 18%) • Overlap seen in 22% patients between OME and neurodevelopmental/behavioural problem 20/09/2018 9 Assessment • History Severity of child’s response 1 no distress 3 m od er at e di str es s 4 ve ry di str es se d 5 Maximum distress Comments Started crying. Had to leave the park. Stopped crying only once inside the car. Your response in this situation Picked him up/cuddled him. Felt very anxious. Also wanted to leave the park, hurried off to the car park immediately. • Hearing thresholds and middle ear status • Age appropriate techniques should be used in an suitable paediatric setting. • Allow plenty of time and reassurance • (No ULLs) Level 1 Cover Level 3 Avoid 2. Temporary auditory deprivation 4. Disorder of sensory processing 1. Immature but normally developing auditory system • As most children with hypersensitivity in the pre school group, ?hypothesise that with progressive maturation of the auditory system, the ability to process the troublesome sounds improve. 20/09/2018 13 • Progressive maturation of the auditory system, • myelination of the thalamic fibres of the thalamocortical auditory system 14 yr old with further axonal myelination up to 10 years Moore (2002) • contributes to improvements in auditory sensitivity thresholds • maturation of intensity discrimination through at least 10 years of age Fior & Bolzonello (1982) • Development of sensory gating between 7– 13 years (Davies, Chang, & Gavin, 2009) • Sensory gating is the process that inhibits ‘distracting’ or non relevant auditory information. An inability to inhibit an influx or distracting information can cause difficulty in processing a sensory stimulus • With maturation of the frontal cortex, decrease in susceptibility to interference from irrelevant environmental stimuli (Marshall, BarHaim & Fox 2004). 20/09/2018 14 (2001) : the limbic system and the amygdala • Respond to somatosensory as well as auditory stimuli • fight or flight response to aversive sounds • involvement of these pathways diminishes with increasing age 2. Temporary auditory deprivation • 1/3 in our study had history of hearing loss at some point mostly secondary to OME. • Post grommets 47% (Nigam, 1994) • Temporary effect on the central auditory gain system and threshold of the middle ear reflex (Formby et al. (2003). Munro (2014)). • Conductive hearing loss at an early age can impair sound intolerance by reducing GABA inhibition in inferior colliculus Sun Wei 2011 – Rat study 20/09/2018 15 3. Permanent Auditory disorder • For some, the disorder in the auditory system may be permanent rather than temporary. • Individuals with William’s syndrome frequently have some degree of hypersensitivity to sounds. • Possible causes a. Hyperactive auditory efferent system b. Lack of acoustic reflexes c. Also high incidence of OME in William’s syndrome ?auditory deprivation may be an additional factor here.(Klien 1990, Gothelf et al 2006). 4. Disorder of sensory processing • Sensory over responsitivity (SOR) reported in children with developmental and learning disorders. • defined as a child’s exaggerated, intense, and/or frequent behavioural response to sensory stimulation. (Carter 2011). 20/09/2018 16 Sensory difficulties • BenSasson & Carter, 2009 prevalence of Sensory over responsitivity at levels interfering with routine activities in 516% in school children • Adamson et al 2006: 70% of children with ASD showed abnormal sensory reactivity with highest difficulty in auditory filtering. Sensory modulation difficulties in children with ASD independent of age. • Sensory modulation difficulties have also been reported in children with other developmental disorders eg ADHD, Fragile X syndrome Neurodevelopmental issues • Hyperacusis may be the most visible part of a wider difficulty with integrating sensory information in the environment. • Children with ASD are often much less able to recognise and describe their emotional reactions 20/09/2018 17 Hyperacusis and ASD • contrary behaviours of hypersensitivity and unawareness to sound in ASD may be due to difficulties in attention control Funabikki et al 2012 • • Anxiety has the same physical correlates to anger. • Anger is easier to tolerate for most children and the behavioural correlates of anger also come with the added bonus of allowing the child to escape from the anxiety stimulus therefore reinforcing the anxiety, anger and behaviour 20/09/2018 18 increased auditory increased anxiety The child with ASD often experiences an increased level of emotional arousal. This is because the social environment contains threats for these children that neuro typical children are oblivious to. So how can we help? 20/09/2018 19 Management • Possible different approaches • Sound therapy • Systematic desensitisation May need different approaches at the same time across these levels Modify a/c age, ability May need different skills • Engage with child to explore sound • Validation • How long does it last? • Instead of dwelling on the distress, explore the enjoyable aspect of the sound eg the colourful lights of the fireworks, dessert! • Be competitive Reassurance • • Don’t force a child to stay in a situation that is obviously causing them distress • Children may feel less anxious if they can leave the situation, or know they can, even for a short time, should the noise become distressing to them. 20/09/2018 21 • ‘the dog is going to be good and not bark’ • ’naughty dog is barking’ • (Note how others caring for the child react – mixed messages) Simple strategies • Preparation/Setting the scene 20/09/2018 23 Managing control issues • Often the sounds that children find distressing are those over which they have no control. • The child's reaction to the sound will often get better if she/he can exercise some control over the noise eh ‘helping’ with the vacuuming. • For some children, it may be helpful to increase their interest, or get them involved, in the noisy activity that bothers them. It can be helpful to engage the child’s attention in the enjoyable part of the activity. Safety Behaviours • Overt or covert behaviours which allow the user to escape the experience of anxiety and therefore prevent habituation. • Safety behaviours prevent the user from learning that the feared catastrophe does not happen (the avoidance of catastrophe is attributed to the safety behaviour). • Habituation will not occur if the child, or carer, is using covert safety behaviours 20/09/2018 24 Diversion/Direction • For some children, it may be helpful to increase their interest, or get them involved, in the noisy activity that bothers them. • engage the child’s attention in the enjoyable part of the activity. • sitting towards the front of school assembly/play where their interest may be maintained longer • giving them a fun but noisy task to do within the noisy activity eg keeping beat with a drum or tambourine. Sound therapy • Aim is to improve the tolerance to problematic sounds by reducing the sensitivity of the ear. • Increase background sounds • The use of sound generators can help improve a child’s sense of control over a situation, such as school, where the option of moving away from the sound source is usually not possible • lack of evidence to support this • Can be helpful in children with ASD 20/09/2018 25 Systematic desensitisation • Repeated gentle exposure to the problem noise while the child is playing may help to reduce the child's anxiety and help him/her to get used to the sound. • It can also help to prepare the child before going into noisy situations as sudden or unexpected noises e.g. fireworks can be distressing. • But must be something that child is on board with and can see benefit Systematic desensitisation • Step two: Learn relaxation and coping strategies • Age/ability 20/09/2018 26 Systematic desensitisation • Step three: graded, step by step exposure to the sound or feared stimuli. (Bouchard, Mendlowitz, Coles and Franklin (2004). Important to • make treatment fun and playful, need a strong rapport with the child, • ensure that the child is motivated to undertake this challenging work, and rewarded for progress made • Know your limits professionally Conclusion • Hypersensitivity to sounds can be distressing for children and their families • Many children/parents can be helped with explanation and strategies • Helpful strategies – need to know • Awareness of possible pathogeneses • Developmental and otological history • Prospective studies in children looking at pathogeneses and management needed 20/09/2018 27 The James Lind Alliance Priority Setting Partnership, a non-profit making initiative, specialise in bringing patients and clinicians together to identify and prioritise uncertainties, or 'unanswered questions' • 2018: Top Ten hyperacusis research uncertainties • Managing a child with hyperacusis • Managing hyperacusis in a child with autism JLA Further information • Hyperacusis in children: A clinical profile. Myne S, Kennedy V. Int J Pediatr Otorhinolaryngol. 2018 Apr;107:8085. • Increased Sound Sensitivity in Children. Veronica Kennedy, Claire Benton, and Rosie Kentish. Chapter 13 in Hyperacusis and Disorders of Sound Intolerance Clinical and Research Perspectives Ed: Marc Fagelson, David M. Baguley 2018 ISBN13: 9781944883287 20/09/2018 28 References • Moore, D. R. (2002). Auditory development and the role of experience. Br. Med. Bull. 63(1),171–181. • Fior, R., & Bolzonello, P. (1988). An investigation on the maturation of hearing abilities in children. Ear and Hearing, 8(6), 347–349. • Marshall, P. J., BarHaim, Y., & Fox, N. A. (2004). The development of P50 suppression in the auditory eventrelated potential. Int. J. Psychophysiol., 51(2), 135–141. • Davies, P. L., Chang, W., & Gavin, W. J. (2009). Maturation of sensory gating performance in children with and without sensory processing disorders Int. J. Psychophysiol., 72(2), 187–197. Nigam A, Samuel PR. Hyperacusis and Williams syndrome. J Laryngol Otol 1994;108(6):494496. • Sun, W., Manohar, S., Jayaram, A., Kumaraguru, A., Fu, Q., Li, J., & Allman, B. (2011). Early age conductive hearing loss causes audiogenic seizure and hyperacusis behaviour. Hearing Research, 282(1–2), 178–183. Klein AJ, Armstrong BL, Greer MK, Brown FR 3rd. Hyperacusis and otitis media in individuals with Williams syndrome. J Speech Hear Disord 1990;55(2):339344. • Gothelf, D., Farber, N., Raveh, E., Apter, A., & Attias, J. (2006). Hyperacusis in Williams syndrome: Characteristics and associated neuroaudiologic abnormalities. Neurology, 66(3), 390–395. • Carter, A. S., BenSasson, A., & BriggsGowan, M. J. (2011). Sensory overresponsivity, psychopathology, and family impairment in schoolaged children. J. Am. Acad. Child Adolesc. Psychiatry., 50(12), 1210–1219. • BenSasson A, Carter AS, BriggsGowanMJ. Sensory overresponsivity in elementary school: prevalence and socialemotional correlates. J Abnorm Child Psychol 2009;37(5):705716. • Adamson A, O'Hare A, Graham C. Impairments in Sensory Modulation in Children with Autistic Spectrum Disorder. Br J Occup Ther 2006;69(8):357364.