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Vicki Kirwin @KidsAudiologist
Going to tweet some info about Auditory Neuropathy Spectrum Disorder #ANSD There's quite a lot - ignore me for a while if not interested!
Vicki Kirwin @KidsAudiologist
Auditory neuropathy & dys-synchrony renamed auditory neuropathy spectrum disorder at Global Consensus Conference, Lake Como, 2008 #ANSD
Vicki Kirwin @KidsAudiologist
#ANSD is term used to describe hearing disorder characterised by normal outer hair cell function and abnormal neural function
Vicki Kirwin @KidsAudiologist
Typical sensorineural deafness is caused by damage to, or a lack of working outer hair cells within the cochlea.... #ANSD
Vicki Kirwin @KidsAudiologist
….#ANSD is caused by damaged inner hair cells, faulty connections between inner hair cells and auditory nerve, or damage to nerve itself.
Vicki Kirwin @KidsAudiologist
Listen to simulation of mild, moderate, severe, and profound #ANSD http://ow.ly/5z9sF
Vicki Kirwin @KidsAudiologist
Parents describes #ANSD comparing it to a frayed wire between radio and the speaker system. Full description here http://t.co/dJZUsmD
Vicki Kirwin @KidsAudiologist
#ANSD hearing levels can range from mild to profound deafness
Vicki Kirwin @KidsAudiologist
Speech perception and discrimination difficulties over and above what we would expect from the level of hearing loss alone #ANSD
Vicki Kirwin @KidsAudiologist
Diagnosis #ANSD - evidence of outer hair cell function (OAEs and/or cochlear microphonics) with absent or abnormal ABR
Vicki Kirwin @KidsAudiologist
#ANSD behavioural testing – very variable, can fluctuate, can be misleading
Vicki Kirwin @KidsAudiologist
#ANSD encompasses a range of conditions which differ in site and pathology (Cone-Wesson & Rance, 2000, Starr et al, 2000) incl…
Vicki Kirwin @KidsAudiologist
….Delayed maturation / myelination of the auditory nerve (maturational or transient #ANSD)...
Vicki Kirwin @KidsAudiologist
… NICU/SCBU populations (eg prematurity, ventilator dependency, hyperbilirubinaemia, ototoxicity)… #ANSD
Vicki Kirwin @KidsAudiologist
… absent or thin auditory nerve, genes (eg Otoferlin), other neurological disorders (eg cerebral palsy)… #ANSD
Vicki Kirwin @KidsAudiologist
… or hereditary neuropathies (eg Charcot Marie Tooth syndrome) #ANSD
Vicki Kirwin @KidsAudiologist
In pre-verbal child we cannot predict who will be able to access enough linguistically useful input to learn language spontaneously #ANSD
Vicki Kirwin @KidsAudiologist
Therefore recommend child w/ #ANSD rec's visual comms support + spoken language until we know more about speech dev't eg SSE, Cued speech
Vicki Kirwin @KidsAudiologist
#ANSD Reduce background noise reduction as much as possible. Use personal FM / Soundfield w/ or w/out h/aids / CI
Vicki Kirwin @KidsAudiologist
#ANSD children need specialist deaf EYs support & close MDT work to determine functional profile & evaluate dev't of S&L skills
Vicki Kirwin @KidsAudiologist
~50% #ANSD children can benefit from h/aids. Others find they amplify distortion and not helpful.
Vicki Kirwin @KidsAudiologist
Fit hearing aids for #ANSD conservatively to behavioural thresholds
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