10/24/2016 Auditory Neuropathy/Auditory Dyssynchrony Christine Yo shina g a -I ta no , Ph.D. Unive rsity o f Co lo ra do , Bo ulde r De pa rtme nt o f Spe e c h, L a ng ua g e & He a ring Sc ie nc e s Alb e rt Me hl, M.D. K a ise r Pe rma ne nte , Co lo ra do Cha pte r Cha mpio n Vic kie T ho mpso n, Ph.D. Unive rsity o f Co lo ra do Me dic a l Ce nte r Ma rio n Do wns Ce nte r E HDI Sta te Co o rdina to r K ristin Uhle r, Ph.D. Unive rsity o f Co lo ra do De nve r Me dic a l Ca mpus Childre n’ s Ho spita l • T he a utho rs ha ve no c o nflic t o f inte re sts to disc lo se Learning Objectives • 1. I de ntify the pre va le nc e o f Co lo ra do c hildre n ide ntifie d with a udito ry ne uro pa thy/ a udito ry dyssync hro ny (AN/ AD) a fte r unive rsa l ne wb o rn he a ring sc re e ning . • 2. De sc rib e the c ha ra c te ristic s o f c hildre n with AN/ AD: NI CU ve rsus we ll-b a b y, unila te ra l ve rsus b ila te ra l (N==67) • 3.De sc rib e the de ve lo pme nta l o utc o me s o f a po pula tio n o f a Co lo ra do po pula tio n o f c hildre n with b ila te ra l AN/ AD (N=37) • 4. I de ntify c ha ra c te ristic s o f c hildre n with AN/ AD a nd CI suc c e ss a nd c hildre n with AN/ AD a nd CI s who a re una b le to de ve lo p spo ke n la ng ua g e . 1
10/24/2016 2002 ‐ 2009 State of Colorado • I nc ide nc e o f HL in 610,829 infa nts sc re e ne d fro m a b irth po pula tio n o f 626,701 • T he sc re e ning ra te wa s 97.5% a nd the fo llo w-thro ug h ra te ra ng e d fro m 83 to 89% • 873 infa nts we re dia g no se d with SNHL in this time pe rio d • 67 infa nts we re dia g no se d with AN • Of c hildre n with SNHL the inc ide nc e o f AN wa s 8.7% • Pre va le nc e o f AN wa s a b o ut 1 in 10,000 • Pre va le nc e o f SNHL wa s 1.6 pe r 1000 2
10/24/2016 Children with AN • 21% we re Unila te ra l AN (14/ 67) • 79% or 53 we re bila te ra l (53/ 67) NICU (Newborn Intensive Care Unit • 60 we re b o rn in the NI CU (90%) • Most infa nts from NICU ha ve bila te r a l AN o 11 we re unila te ra l (18%) o 49 we re b ila te ra l (82%) Well baby nursery • 7 we re b o rn in the we ll- b a b y nurse ry (10%) o 3 we re unila te ra l (43%) o 4 we re b ila te ra l (57%) 3
10/24/2016 Developmental data about ANSD • 39 o f the c hildre n in this b irth c o ho rt ha ve de ve lo pme nta l da ta in the b irth thro ug h thre e a g e ra ng e • 2 o f the 11 (18%) a re c hildre n with unila te ra l UANSD • 37 o f 53 c hildre n ha ve b ila te ra l ANSD (70%) Proportion of Children with significant cognitive disability (32%) • 32% (12/ 37) ha ve sig nific a nt c og nitive disa bility in a ddition to he a r ing loss o Almo st 1 in e ve ry 3 c hildre n o 9 o f the se 12 c hildre n o r 75% ha ve sig nific a nt c o g nitive a nd ne uro lo g ic a l invo lve me nt with de ve lo pme nta l q uo tie nts ra ng ing fro m 10 to 55 (9/ 37 o r 1 in 4) o 3/ 4 o f the c hildre n with c o g nitive disa b ility ha ve se ve re / pro fo und invo lve me nt a nd multiple o the r issue s Additional disabilities including cognitive disabilities ‐ 57% • 9 a dditio na l c hildre n ha d no rma l c o g nitive de ve lo pme nt b ut o the r disa b ilitie s inc luding visio n, mo to r/ o rtho pe dic , se ve re he a lth issue s • 57% (21/ 37) of the popula tion of c hildre n with bila te ra l AN ha ve a dditiona l disa bilitie s a nd the va st pro po rtio n o f the se issue s a re se ve re / pro fo und c o g nitive de la ys, in ma ny c a se s a lso ne uro lo g ic a l issue s. 4
10/24/2016 Normal cognitive function – 68% • 25 of 37 or 68% of the c hildre n ha d c og nitive de ve lopme nta l quotie nts within the norma l r a ng e . • 9/ 37 ha d no rma l c o g nitive q uo tie nts a nd a dditio na l disa b ilitie s (24%) • 9/ 25 Nine c hildre n o f the 25 with c o g nitive de ve lo pme nta l q uo tie nts in the no rma l ra ng e o r 36% o f c hildre n with no rma l c o g nitio n ha d a dditio na l disa b ilitie s Language Quotients • 21/ 37 c hildre n ha d la ng ua g e q uo tie nts within the no rma l ra ng e Re c a ll tha t 25/ 37 c hildre n ha d no rma l c o g nitive q uo tie nts • 57% of the c hildre n ha d la ng ua g e quotie nts within the norma l ra ng e • Re c a ll tha t 57% o f the c hildre n ha d he a ring lo ss plus a dditio na l disa b ilitie s • 37% o f the se c hildre n with a dditio na l disa b ilitie s ha d c o g nitive disa b ilitie s • Appro xima te ly 16% o f the c hildre n with no rma l c o g nitive sc o re s did no t ha ve la ng ua g e q uo tie nts in the no rma l ra ng e - Hispanic/Latino • 32% (12/ 37) c hildre n ide ntify a s Hispa nic / L a tino • 83% or 10 of the se 12 c hildre n a re Spa nish- spe a king in the home indic a ting tha t o ne o f e ve ry fo ur c hildre n with b ila te ra l AN in the sta te o f Co lo ra do is b o rn into a fa mily tha t do e s no t spe a k E ng lish a s a na tive la ng ua g e . 5
10/24/2016 Gender • 35% a re fe ma le (13/ 37) • 65% a re ma le (24/ 37) • T he inc ide nc e o f b ila te ra l AN wa s two time s g re a te r fo r ma le s tha n fo r fe ma le s. Cochlear Implants • 4/ 37 o r 10.8% o f the c hildre n re c e ive d a c o c hle a r impla nt/ s • 5/ 37 we re no t a mplifie d – 14% • 28 or 76% use d he a ring a ids Early Intervention Services • All 37 fa milie s re c e ive d we e kly e a rly inte rve ntio n se rvic e s fro m a n e a rly inte rve ntio n pro vide r with e ithe r a de a f e duc a tio n, spe e c h/ la ng ua g e pa tho lo g y o r a n a udio lo g y de g re e . 6
10/24/2016 Sign Language • 31 o f 38 fa milie s c ho se to re c e ive we e kly sig n la ng ua g e instruc tio n fro m a de a f o r ha rd o f he a ring na tive / flue nt sig n la ng ua g e instruc to r (82% ) • 1 o f the c hildre n use d Cue d Spe e c h PHYSIOLOGY – Gardner ‐ Berry, 2012 • N=142 CI s o ve r 10 ye a r pe rio d • Sydne y Co c hle a r I mpla nt Ce nte r, • 16% ha d a bnorma litie s on the ir CT sc a ns, whic h inc lude d Mo ndini de fo rmitie s, wide inte rna l a udito ry me a tus, dyspla stic a pic a l turn, a nd a b no rma l ve stib ule a nd la te ra l se mi-c irc ula r c a na ls. • 20% of the c hildre n with bila te ra l AN a nd 6% of the c hildr e n with unila te r a l AN ha d c ompr omise d a uditory ne rve s. • A tota l of 43% of the c hildr e n ha d disa bilitie s in a ddition to HL . The Infant Monitor of vocal Production ( IMP IMP ) Ro b yn Ca ntle Mo o re , PhD RI DBC Re nwic k Ce ntre / Unive rsity o f Ne wc a stle Sydne y, Austra lia . 7
10/24/2016 IMP IMP Précis • Crite rio n re fe re nc e d instrume nt • Clinic a l e va lua tio n & pa re nt e duc a tio n to o l • Suc c e ssive me a sure o f infa nt, pre -ling uistic vo c a l c o mpe te nc e • Dia g no stic a id to ha b ilita tio n pro g ra mming Typical infant vocal development Universal speech perception Phonetic contrasts all languages Language specific speech perception Language specific Language specific sound combinations vowels Native language consonants Perception * Visual attention shift to mouth seeks redundant audio-visual speech cues Social attention Visual-social attention to eyes shift back to eyes 1 2 3 4 5 6 7 8 9 10 11 12 MONTHS Production First words Language specific speech production ‘Canonical babbling’ Vowel-like sounds Non-speech sounds Universal speech production ( Kuhl, 2004; Lewkowicz & Hansen ‐ Tift, 2012 * First words Kuhl (2004) Stages of Language specific speech production Infant Vocal ‘Canonical babbling’ Production Vowel-like sounds Non-speech sounds Adapted from: Oller (2000) Nathani, Ertmer, & Stark (2006) 8
10/24/2016 IMP IMP probe ‐ questions Interpreting the IMP IMP TYPICAL IMP IMP : infant (ANSD) Case Study 102 “Lily”” 11.5 Innate Transition to A-P Integrity of A-P Loop Loop 9
10/24/2016 IMP IMP : infant (ANSD) Case Study 102 IMP IMP : infant (ANSD) Case Study 36 “Mary”” 9 Innate Transition to A-P Integrity of A-P Loop Loop IMP IMP : infant (ANSD) Case Study 36 “Mary” 9 10
10/24/2016 IMP IMP : infant (ANSD) Case Study 35 “Ian” 1 7 19 19 14 14 Innate Transition to A-P Integrity of A-P Loop Loop IMP IMP : infant (ANSD) Case Study 35 “Ian” 1 7 IMP IMP : infant (ANSD) Case Study 135 “Cameron” 1 1 Innate Transition to A-P Integrity of A-P Loop Loop 11
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