09/07/2015. Chear Ltd: independent audiology centre Hyperacusis: Assessment and Management in Children MULTIPLE CHOICE QUESTION 1

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1 Hyperacusis: Assessment and Management in Children Josephine Marriage PhD BAA Student Conference 3rd July 2015 Chear Ltd: independent audiology centre Independent centre for second opinion of children s hearing assessment and hearing aid review Set up in 2001 Adult hearing aid dispensing Management of hyperacusis Research Now London centre Bermondsey MULTIPLE CHOICE QUESTION 1 Q1. What is hyperacusis? A) Hyper acute hearing thresholds ~ -10 dbhl B) Dislike of sounds like chalk on black board C) Abnormal loudness growth or recruitment D) Inability to tolerate some everyday sounds Choose one answer and write it down What is Hyperacusis? Intolerance of sounds that would be acceptable to most listeners Collapse of loudness tolerance Avoidance of situations where sounds may occur (cf phonophobia) Hyperacusis infers a cochlear pathology but NO evidence of hyper-acute hearing levels on audiogram Not the same as recruitment which is feature of increased growth in loudness with hearing loss MULTIPLE CHOICE QUESTION 2 Q2. The most appropriate test for hyperacusis is: A) Oto-acoustic emissions B) Audiogram C) Questionnaire D) Loudness discomfort levels using pure tones E) Sound level meter measure of noise levels Choose one answer and write it down How can we test for hyperacusis? There is NO objective test for hyperacusis Subjective condition, need validated questionnaire Children may not be able to describe sensations Behavioural symptom profile (crying, cowering, covering ears, lashing out, running away) Parent description of behaviours Can develop into phonophobia with very similar behavioural profile So, how do we know it has a physiological basis? 1

2 Hyperacusis: When is loudness discomfort outside normal range? My interest is from PhD on Hyperacusis in Williams Syndrome (WS) Hyperacusis in WS is: Consistent finding with WS (~90%) Early onset of sound aversion following birth Not related to elastin abnormality Sensitivity specific to auditory modality, unlike Fragile X or autism (ASD) No evidence of hearing loss Not due to hyper-acute threshold of hearing on audiogram, Some evidence for central hyper-excitability Katie with WS describes hyperacusis Other groups with Hyperacusis? Autism and ASD Tinnitus and hyperacusis (research ++) Migraine, increased auditory perception Benzodiazepine withdrawal Chronic fatigue syndrome, ME Post traumatic stress disorder Depression Hydrocephalus / CP Hypermobility syndromes Is there a common mechanism for sound intolerance in ASD? Sound Sensitivity recognised feature of Autism-Related disorders (ASD) n= 17,000 ASD children 40% of parents report sound sensitivity Gilberg includes abnormality in sensory perception (esp auditory) as 4 th symptom for ASD Is this physiologically-based difference in sensory perception from typically developing controls? Or psycho-emotional differences based on fear and anxiety? Hyper-reactivity It depends on who you ask! 2

3 Hyperacusis / Tinnitus literature in Adult population Probably physiological basis to hyperacusis Studies show threshold for ART reduces after ear plugs (Formby et al, Munro et al, 2009) Maintained by fear/anxiety (amygdala) Enhanced auditory vigilance Needs effective management of all aspects Acoustic enhancement reduces anxiety and auditory sensitivity. Tyler reports 2/3 children benefit Plugging of ears increases auditory sensitivity. Do not use ear plugs for hyperacusis What treatment approaches might be practical and helpful in hyperacusis? Management Strategy 1. Profile of the problem 2. Impact of problem 3. Understanding of condition by all carers 4. Behavioural desensitisation not in severe ASD 5. Auditory desensitisation enhanced auditory stimulation 6. Re-assessment and follow up MULTIPLE CHOICE QUESTION 3 Why do families ask for an appointment for sound sensitivity? Reaction of child to some sounds is extreme Panic or flight response Very difficult to calm or intervene for child Looks very aversive, or like bad behaviour, to onlookers Generally family have already tried strategies to calm, reassure, de-sensitise fear, and maintain noise levels in environment Q3. The type of sounds that cause aversion behaviours are: A) High pitched B) Low pitched C) Loud sounds D) Wide band frequency range E) Sudden Choose one answer and write it down What parents say: My child hates the sound of 3

4 What treatment approaches might be practical and helpful in children? Management Strategy 1. Profile of the problem 2. Impact of problem 3. Understanding of condition by all carers 4. Behavioural desensitisation not in severe ASD 5. Auditory desensitisation enhanced auditory stimulation 6. Re-assessment and follow up Hyperacusis Case History Profile of problems Types and location of sounds Frequency of occurrence Behavioural Reaction Effect on child Effect on others Coping strategies used How effective are these Use of a single week diary to get baseline of aversion behaviours Fill in diary sheet of sound aversion incidents: over a full week More specific information around sound or situation example: Hoovering Time Sound type Effect on child 0-10 Coping strategy Sunday Monday Tuesday Wed.. Please can you circle a number between 0 to 10 giving the overall impact of sound sensitivity now Sound/Situation date Morning Afternoon evening Loudness of sound to you Severity of child s response Comments Your response in this situation Soft Medium Loud Very loud 1 no distress Made me turn hoover off Turned off hoover 2 slight distress I panicked and shouted Reassured and calmed T 3 moderate 4 great distress Child s responses Becomes hysterical and hides under the bed He has bat ears now from folding his ears over his ear holes Becomes so distressed that he vomits he becomes aggressive and hits me So overwhelmed with sound he bit another child Cries and becomes very anxious He has hit our baby when she cries Can t go to a public toilet as there may be a hand drier, he won t go in Effect on the family - I had to take him out of nursery as he became very distressed by the other children so he is loosing out now on learning and socialising at nursery - We cannot take public transport and therefore his experiences are very limited - It impacts on my other children in so many ways - We cannot go out for a meal he may get hysterical and scream and hide under the table - We can t have friends round. We can t meet other people.. We don t go out.. 4

5 Information to classroom and other carers for consistent handling Research literature: Untreated hearing loss may exacerbate auditory sensitivity 1. Check hearing levels as possible: Audiogram, Auditory brainstem response (ABR) Speech discrimination testing: computerised Middle ear status for glue ear, wax etc Hearing loss needs proactive management and amplification Hyperacusis is not a contra-indication for hearing aids or surgery What treatment approaches might be practical and helpful in ASD? Management Strategy 1. Profile of the problem 2. Impact of problem 3. Understanding of condition by all carers 4. Behavioural desensitisation not in severe ASD 5. Auditory desensitisation enhanced auditory stimulation 6. Re-assessment and follow up Auditory Desensitisation Hyperacusis Masking Programme Consists of: Use of BTE white noise generators (WNG) Tinnitus ball or i-pod with environmental sounds Medium to long-term plan (approx 18 months) Aiming to improve: Tolerance immediately in aversive situations Improve tolerance / sensitivity to sound over time Reduce anxiety and fear-potentiated sound aversion 5

6 Need to have: Open earmoulds made for both ears, or open slim tube fitting Tinnitus maskers or white noise sound generators (WNG). NHS has contracts for these. Standard use with tinnitus patients Devices have manual volume control Can be part of hearing aid device if needed eg Zen tones Management - White Noise Generator WNG or Maskers are fitted with open ear moulds or slim tube fittings Adjustable volume for sound level output of device Initially set at just audible level in quiet Over first week become used to devices Over second week Build up use of maskers to 6 hours+ per day Monitor for about 6 weeks Gradually increase the level of the noise generator Or encourage individual to change volume as necessary * Check that child can understand quiet speech with masker in and on Increase noise level over time until child is able to tolerate all daily situations without difficulty Redo the specific sounds list to re-evaluate child s response with these sounds Then: Slowly reduce the volume of the WNG output over time Reduce hours or situation of use as possible until no longer used routinely Do diary re-assessment over one week once the maskers are stopped Parents have great ideas on how to help support use of maskers Allow parents to keep maskers in case necessary for specific situations. In several cases over time re-started use of maskers josephine@chears.co.uk 6

7 Professionals need to keep an open mind on cause, impact and treatment of hyperacusis 50% of what we have been taught will turn out to be wrong! We just don t know which 50%, so we need to constantly upskill so We need to keep learning through our professional lives Our best learning comes from listening to patients and children Questions? Comments? 7