Management of Hyperacusis in Children Veronica Kennedy and Rosa Crunkhorn BACCH 10 th September 2018

Transcription

1 Management of Hyperacusis in Children Veronica Kennedy and Rosa Crunkhorn BACCH 10 th September 2018» 3 year old» Dislikes hand driers, hoovers, motorbikes» Covers ears with hands Flinches, panics» Otherwise well, normal development Case 1 1

2 Case 2» 8 year old girl» Multiple difficulties: Babies crying Dog barking Birthday parties / family gatherings» Foetal valproate syndrome Outcomes» Know what hyperacusis is» How common hyperacusis is» Understanding of management of hyperacusis 2

3 Definitions» Hyperacusis disturbed loudness function (Phillips &Carr 1998) 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 Defeating deafness 6% child population oversensitive to noise Coelho, Sanchez, Tyler school children (5 12yrs) 42% bothered by sounds 3.2% had hyperacusis of whom 50% tinnitus 9% phonophobia 3

4 Prevalence ALSPAC (Hall et al 2016) year old children 3.7% children reported hyperacusis. more likely: Male>females where higher maternal education where readmission to hospital in first four weeks» Retrospective study Bolton Case Review All children with troublesome sensitivity to sounds Review of notes Age, gender Clinical presentation Hearing levels Associated medical conditions 4

5 Age when first reported (n=61) (31%) Number of children (24.5%) Male:Female 1.65: y 2y 3y 4y 5y 6y 7y 8y 9y 10y 11y 15y Age at presentation (y=years) Which sounds are troublesome? Sounds Number of children (in order of frequency) (%) Vacuum cleaner 28(45%) Noisy crowd/children shouting 22(36%) Music related sounds 17(28%) Hair/hand dryer 13(21%) Siren/alarms 9(15%) Traffic/vehicle noise 7 Ringing noise/door bells 5 Washing machine 5 Clapping 4 Drilling noise 4 Dog barking 3 5

6 How many sounds are troublesome? 35% 30% 33% 31% Number of children (%) 25% 20% 15% 10% 24% 7% 5% 3% 2% 0% Number of troublesome sounds reported Reported Behavioural Responses Cover ears 62% Cry, scream, stress, panic 38% General dislike 13% Pain (Ears/head hurt) 9% Runaway/hide 4% Teeth grinding 2% 6

7 Some examples Covers ears before he shouts 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 Number of children (n=61) A B E 0 < 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 C D F A B C D E F 7

8 Neurodevelopmental Conditions Number of children (28/61) Autistic spectrum disorder 8 (13%) Cerebral Palsy 3 Attention deficit hyperactivity disorder 2 Down s syndrome 2 Hydrocephalus 2 Epilepsy 2 Sensory processing problems 2 Dyspraxia 1 Klinefelter s syndrome 1 Microcephaly,severe learning difficulties 1 Williams syndrome 1 Leigh s syndrome 1 Microdeletion 15q Possible Syndrome (facial dysmorphism, 1 developmental delay, congenital heart defect) Results Summary Hearing loss present in about one third 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 8

9 Assessment History Sounds / sensations Circumstance/setting Impact / reactions Support by others Successful or unsuccessful strategies Developmental and otological history Date: m aft evening SOUND SENSITIVITY DIARY or er Child s Name: ni no Address: ng on Example: Sound or Situation: Dog barking in the park Severity of child s response 1 no distress 2 sli gh t di str es s 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. 9

10 Common Findings Control Unpredictability Spectrum: Hyperacusis Phobic response Safety behaviours Assessment Full audiological assessment (where possible) 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) 10

11 Management Tailored Management Likely mechanism Depends on impact of hyperacusis Level 1 Cover Level 2 Paddy make it stop Level 3 Avoid Age/Developmental stage of child Cognitive ability 11

12 Possible mechanisms Kennedy Classification 1. Immature but normally developing auditory system 2. Temporary auditory deprivation 3. Disorder within the auditory system 4. Disorder of sensory processing 1. Immature but normally developing auditory system As most children with hypersensitivity in the preschool group,?hypothesise that with progressive maturation of the auditory system, the ability to process the troublesome sounds improve. 12

13 Normally development of auditory system Progressive maturation of the auditory system, myelination of the thalamic fibres of the thalamocortical auditory system 1 4 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 nonrelevant 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, Bar Haim & Fox 2004). 13

14 Emotions, flight or fight Non classical auditory pathways Moller & Rollins (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 14

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). 15

16 Sensory difficulties Ben Sasson & Carter, 2009 prevalence of Sensory over responsitivity at levels interfering with routine activities in 5 16% 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 16

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 children with autistic spectrum disorder had ineffective inhibitory control of sensory processing. Orekhova et al Dunn (2007) Difficulty regulating sensory information hyper sensitivity to sensory stimuli leading to sensory avoidance ie shying away or intensely reacting to loud sounds, bright lights, being held etc Anxiety in ASD 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 17

18 Anxiety can exacerbate hyperacusis and vice versa 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. increased anxiety increased vigilance to sound increased auditory gain So how can we help? 18

19 Don t even think about it. Management Possible different approaches Level 1 : Explanation Reassurance Level 2 Simple strategies Calming strategies eg relaxation Level 3 Address safety behaviours Sound therapy Systematic desensitisation May need different approaches at the same time across these levels Modify a/c age, ability May need different skills 19

20 Explanation Age and ability dependant Engage with child to explore sound Validation Why does it have to be loud? 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 Where the child becomes distressed by a sound, it may be helpful to gently move him/her away from the sound (if possible) and comfort them. (Better to act on early signs before responses entrenched) 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

21 Avoid labelling Avoid negative labels or blaming re sound sources the dog is going to be good and not bark naughty dog is barking (Note how others caring for the child react mixed messages) Acknowledgment Diversion/direction Control Simple strategies Identify/address safety behaviours Aim for planned rather than reactionary behaviour, permissions Preparation/Setting the scene 21

22 Relaxation Relaxation techniques Finger cued breathing Imagining favourite place Sensory grounding techniques Mood/emotion regulation exercises Websites: Cosmic kids, Relax kids 22

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 23

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 24

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 1: Identify hierarchy of troublesome or feared sounds Step two: Learn relaxation and coping strategies Age/ability Relax kids website 25

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 26

27 JLA 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 Further information Hyperacusis in children: A clinical profile. Myne S, Kennedy V. Int J Pediatr Otorhinolaryngol Apr;107: 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:

28 Thank you for listening References Moore, D. R. (2002). Auditory development and the role of experience. Br. Med. Bull. 63(1), Fior, R., & Bolzonello, P. (1988). An investigation on the maturation of hearing abilities in children. Ear and Hearing, 8(6), Marshall, P. J., Bar Haim, Y., & Fox, N. A. (2004). The development of P50 suppression in the auditory event related potential. Int. J. Psychophysiol., 51(2), 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), Nigam A, Samuel PR. Hyperacusis and Williams syndrome. J Laryngol Otol 1994;108(6): 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), 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): Gothelf, D., Farber, N., Raveh, E., Apter, A., & Attias, J. (2006). Hyperacusis in Williams syndrome: Characteristics and associated neuroaudiologic abnormalities. Neurology, 66(3), Carter, A. S., Ben Sasson, A., & Briggs Gowan, M. J. (2011). Sensory over responsivity, psychopathology, and family impairment in school aged children. J. Am. Acad. Child Adolesc. Psychiatry., 50(12), Ben Sasson A, Carter AS, Briggs Gowan MJ. Sensory over responsivity in elementary school: prevalence and social emotional correlates. J Abnorm Child Psychol 2009;37(5): Adamson A, O'Hare A, Graham C. Impairments in Sensory Modulation in Children with Autistic Spectrum Disorder. Br J Occup Ther 2006;69(8): Bouchard, S., Mendlowitz, S. L., Coles, M.E., & Franklin, M. (2004). Considerations in the use of exposure with children. Cognitive and Behavioural Practice, 11,