Language Impairment

Language problems

•  Several conditions are related to difficulties in spoken language acquisition:

•  Hearing impairment •  Intellectual impairments •  Autism/PDD •  Specific language impairment

A note of compassion, and a note of skepticism

•  Keep in mind that: •  Something being wrong with your child is

frightening--parents are motivated to seek solutions

•  Any therapy costs $money$--other people are motivated to provide solutions

•  The notion of evidence-based practice: if it really works, it should work in an actual experiment, not just for one person who writes a testimonial on a web site

Hearing impairment

•  1 million kids in US, 90% to hearing parents

•  Most prelingually deaf •  Trouble developing oral language •  This cascades into difficulty in language

comprehension, reading •  Difficulty is usually related to how profound

the hearing loss is

Hearing impairment •  Problems in language development •  Phonology

–  Not very intelligible, even with training –  CI: more intelligible, but still not great articulation

•  Language development, lexicon –  Reading skills, vocab max out @ 4th grade level

•  Grammar –  Poor grasp of English syntax (e.g. passives, etc. that

are hard for non-deaf children) –  Better to teach use of [written] language to

communicate effectively

•  Identification by three months

•  Amplification by six months

Universal Newborn Hearing Screening

Hearing impairment •  Congenital or pre-lingual HL good candidates for

Auditory Habilitation •  Concern is communication development •  How can we deliver language to the child?

• Make spoken language louder • Deliver higher quality speech signal •  Provide language through another modality

(e.g., signed language)

Hearing impairment •  Can we just fix it? •  Not exactly. •  Cochlear implants show some efficacy*

–  Stimulate auditory nerve directly, past site of problem –  Still not the same as actual speech input –  Requires speech-language therapy –  Active area of research

How Does a Cochlear Implant Work?

1.  Sound waves enter through the microphone and are converted by the sound processor a distinctive digital code.

How Does a Cochlear Implant Work?

3.  The internal device delivers the sound to the electrodes.

4.  The electrodes stimulate the hearing nerve, and the hearing nerve sends the signal to the brain for processing.

Photos provided courtesy of Cochlear™ Americas, ©2009

Cochlear Americas

1.  Sound waves enter through the microphone and are converted by the sound processor into a distinctive digital code.

2.  The electrically coded signal is transmitted across the skin through the headpiece to the internal portion of the device.

Hearing impairment •  Just lip-read? •  Not as easy as it sounds. •  Some sounds not visible (Ex.: /l/, /g/, /k/) •  Some sounds look alike (/m/, /b/, /p/)

Hearing impairment

•  Educational philosophies •  Oral/aural (auditory-verbal therapy)

–  Can be effective if cochlear implant, residual hearing; resurgence due to implants

–  Cued speech: handshapes to supplement lips •  Total communication

–  Response to old oral/aural ed and bad outcomes –  Make up sign systems yoked to English (SEE)

•  Bilingual/bicultural (bi-bi) –  Learn ASL first, then English as second language –  Good in principle, limited by teachers’ expertise

Hearing impairment

•  Cochlear implants: controversy •  See improvements in language skills, esp.

if implanted young, but •  Not like real hearing •  Still need lots of educational support •  Threat to Deaf culture •  Best solution: learn both

CochlearImplants

Intellectual disabilities

•  Down syndrome (poor language) •  Williams syndrome (preserved language?) •  Fragile X syndrome

•  Range of cognitive difficulties •  Sometimes, physical difficulties •  Poorer categorization, generalization

•  Early intervention!!

Intellectual disabilities

•  Down syndrome •  Trisomy 21 •  Achievement related to working memory skills •  Look like younger TD children, mostly

–  Behind by 20 mos at age 3 years; 24 at age 4 years –  Big trouble with English morphosyntax –  Later, pretty good narrative, pragmatic skills

•  10%+ diagnosed with autism

Intellectual disabilities •  Williams syndrome •  Deletion on chromosome 7,

including elastin protein •  Characteristic appearance, health

issues •  Old claim: bad cognition, good

language (reverse of Downs) –  Language module idea

•  New claim (see Karmiloff-Smith’s work): language abilities in keeping with overall cognitive abilities; still behind for chrono age

h0ps://theconversa4on.com/explainer-what-is-williams-syndrome-26142

Intellectual disabilities

•  Williams syndrome

h"ps://www.youtube.com/watch?v=z57jSDU1PFM

Intellectual disabilities •  Williams syndrome •  Deletion on chromosome 7, including elastin protein •  Characteristic appearance, health issues •  Old claim: bad cognition, good language (reverse of

Downs) –  Language module idea

•  New claim (see Karmiloff-Smith’s work): language abilities in keeping with overall cognitive abilities; still behind for chrono age

•  Interesting holistic/analytic split w/Downs

h"ps://theconversaCon.com/explainer-what-is-williams-syndrome-26142

LanguagecharacterisCcsinWSComplexlanguageprofile•  RelaCvestrengths

–  Concretevocabulary–  Phonologicalprocessing–  Verbalshort-termmemory

•  RelaCveweaknesses–  RelaConal/conceptuallanguage–  Readingcomprehension–  PragmaCcs–  Complexgrammar

Intellectual disabilities

•  Down syndrome (poor language?) •  Williams syndrome (preserved language?) •  Fragile X syndrome

•  Range of cognitive difficulties •  Sometimes, physical difficulties •  Poorer categorization, generalization

•  Early intervention!!

Intellectual disabilities •  Fragile X •  Problem with FMR1*geneonX chromosome

–  Mostly boys affected; biggest source of ID w/known source in boys

–  Specifics: over 200 CGG repeats (normal: ~30) –  Premutation: 55-200 repeats

•  Long face, high forehead •  Delayed language onset •  Poor oral-motor skills, articulation •  A little better than DS for language skills •  Perseveration in language •  25% also have autism (more, by some estimates)

h"p://medgen.geneCcs.utah.edu/photographs/pages/fragile_x.htm

Intellectual disabilities •  All of these: therapy of all types (speech, occupational,

behavioral) •  Language instruction (DS especially): •  Catch it early •  Teach real-life language skills •  Teach so as to generalize skills •  Train learning/rehearsal of new info •  If severe, try augmentative or alternative communication

(AAC) –  Symbols/pictures –  Signed system (complex but can mold a handshape better than

a speech sound) •  For DS, keep teaching throughout adolescence (still

developing)

Autism

•  Partofa“spectrum”ofdisorders,incl.:– Aspergersyndrome– Re"syndrome– AuCsm[spectrum]

•  CategorieschangefromCmetoCme•  IsAspergerdifferentfromhigh-funcConingauCsm?DSM-V:samething

•  Areallcasesfromsamesource[s]?•  Nominalfallacy

Autism

•  Diagnostic criteria: •  Highly delayed onset/absence of language •  Impaired conversation starting, responding •  Use lg skills in stereotyped, repetitive way •  Lack of age-appropriate pretend-play or socially-

imitative play

Autism •  Age of discovery: ~2 years •  Signs may appear earlier

–  Aberrant eye gaze –  Lack of responsiveness to name –  Hand flapping, toe walking –  Pierce et al. 2011: visual preference for geometric

patterns > people •  About 1/3 reported to show regression •  Seeming increase in cases over time

–  MMR vaccine? No—original article discredited –  Corresponding decrease in ID diagnoses –  Get more intervention services for child if autism

diagnosis

Autism

•  Likely genetic basis –  Siblings at risk –  Families tend to have some of the behaviors –  Heritability likely, a little hard to tell b/c don’t have kids

•  Many brain differences –  Accelerated growth during time when symptoms first

ID’ed

Autism: Social & communicative problems

•  Poor joint attention –  On 1st birthday, lack of pointing/showing –  Didn�t look @ faces, respond to name

•  Symbol use is difficult –  Conventionalized gestures (wave, point) –  Banana phone

•  Theory of mind is underdeveloped –  Understanding others’ intents, perspectives –  Alternatively: impaired executive function or weak

central coherence

Autism & language

Language outcome strongly linked to

•  Age of diagnosis •  Initial cognitive profile •  Initial language profile •  Amount of speech-

language intervention 5 word vocab Receptive & expressive deficits Structure OK but poor pragmatics

Autism & language •  Receptive, expressive

correlated, both related to IQ •  Phonological skills better than

other skills •  Problems with intonation and

stress •  Confuse you and I •  Difficulty imitating (but echolalia) •  Hard to tell about really early

language problems, b/c diagnosis is quite late

•  Not catalyzed by desire to socially interact

5 word vocab Receptive & expressive deficits Structure OK but poor pragmatics

Autism & language •  Echolalia: repeating things you’ve heard •  Just another self-stimulatory behavior? Doesn’t tend to

covary with other ones •  Also, typically-developing kids do it (early in

development; Bloom et al., 1976) •  Immediate vs. delayed •  Exact vs. mitigated •  A lot of echolalia is mitigated

–  Communicative strategy? –  Tends to disappear as they acquire more spontaneous speech –  May be �stepping stone�

•  Break down larger pieces

Autism & language

Wodka et al. (2013) studied language delay •  535 children with autism who were

–  Age 8+ –  Had no phrases by age 4 (37% of 8+-y-o’s in database)

•  253 (47%) attained fluent speech after age 4 •  372 (70%) could at least produce phrases •  Non-verbal IQ, social engagement were predictors •  Stereotypies, sensory sensitivities: not predictive •  Good news! Late language ≠ no language

Autism treatment

•  Intervention guidelines –  Early –  Intensive (up to 25 h/wk!) –  Involve family –  Social, pragmatic communication –  Systematic but customized to child –  Generalization

•  Evaluation –  Until recently, mostly �small N� studies

Autism treatment

Modifybehaviors

Developsocial/pragmaCc,communicaConskills

DifferenCalReinforcement…(DRA/I/O)Reinforcement(R+)

ResponseinterrupCon/redirecCon(RIR)Taskanalysis(TA)

PictureExchangeCommunicaConSystem(PECS)SocialnarraCves(SN)

Socialskillstraining(SST)

Currently27evidence-basedintervenConsh"p://auCsmpdc.fpg.unc.edu/evidence-based-pracCces

Autism treatment Currently27evidence-basedintervenCons

h"p://auCsmpdc.fpg.unc.edu/evidence-based-pracCces

Wongetal.

Autism treatment •  Not just treating language, of course •  ABA: quite good for reducing harmful behaviors

–  If can communicate better, aggression, inappropriate behavior may drop

–  Functional analysis: what’s motivation/function of bad behavior?

•  Shape into better behavior

•  Medication –  Often, undesirable side effects (weight gain, dyskinesias)

Autism treatment •  Things that don’t work •  Auditory integration therapy (AIT)

–  �uses filtered and modulated music to help stimulate the audiological and neurological systems�

•  Facilitated communication (FC) –  �Facilitator� offers physical support (usually to type) –  May have helped small handful of people (4ish) –  Most scientists think authorship is facilitator�s

AndsomeCmes…•  Itgoesaway!Feinetal.(2013)•  Kidswithsymptomsatage5orearlier

–  Delayedlanguage–  RepeCCvebehaviors

•  Symptomsnowgone–  NormalrangesocialinteracCon–  Language–  FacerecogniCon

•  Onlydifferencefromkidswithhigh-funcConingASD:– Mildersocialsymptomsearlyon

•  Perhaps~10%ofdiagnosedkidsenduplosingdiagnosis–  Outgrow?IntervenConworks?Wedon’tunderstandauCsmitself?

Language problems

•  Several conditions are related to difficulties in spoken language acquisition:

•  Hearing impairment •  Intellectual impairments •  Autism/PDD •  Specific language impairment

SLI: The big picture

•  Delayed (not deviant) learning •  May originate in toddlerhood (or earlier) with late

talkers –  Some late talkers end up with language in normal range

•  Esp. if receptive language is okay (L. Rescorla, 2011) –  25-50% of late talkers end up getting SLI diagnosis

•  Family history •  Less likely to be caught than articulation problems

(David Sedaris’ �s�) •  Likely to cascade into reading difficulty

What does SLI look like? •  Badatmappingwordstomeanings•  CircumlocuCons

–  SLIkid:“SomethingroundandEnglish”forbreakfast–  Troubleaccessingwords?[Likeanomicadults!]

•  SyntacCcproblems(bigidenCfier)–  ToughCmeusinggrammaCcalsyntax

•  Herun•  Mewantcookie

–  Evenwhenyoucontrolforlanguageagew/MLU!•  PragmaCcproblems

– WhichcancascadeintosocialdifficulCes•  *Differentsymptomsindifferentlanguages

Where does it come from? •  Tip end of distribution? (Hard to say) •  The Brain? •  Genes? (KE family; Crago & Gopnik, 1994)

–  Lots of SLI kids tested, no FOXP2 mutation –  In mice:

•  Difficulty in motor learning (French et al., 2007, Genesis) •  Aberrant vocalizations (Gaub et al., 2010) •  Poor sound-behavior associations (Kurt et al., 2012, PLOS One)

– Differences in brain development in multiple regions

Where does it come from?

•  Deficits in temporal auditory processing (Tallal) •  Surface Hypothesis: Hard time processing

grammatical morphemes with low salience (they don’t get stressed in the speech signal)

•  Trouble with linguistic rules (modular-ish) •  Generalized Slowing Hypothesis: slower at

executing a lot of perceptual/motor tasks

SLI interventions

•  Imitation – Repeat the therapist

•  Modeling – Kid makes new version of what therapist says

•  Recasting – Restating what kid says

•  (Some others too) •  As always, generalization is big challenge

Other speech disorders

•  Cerebral palsy –  Impaired motor control – Comprehension, cognition often OK

•  Cleft palate – Physically impossible to produce certain sounds – Again, cognition and comprehension fine

•  Functional articulation disorders (NOS) – Otitis media? (ear infections)

Other speech disorders •  Stuttering

–  Starts after speech sounds acquired

–  Part-word repetitions •  Not just �And…and this

one time� •  But also �t-t-t-t-time�

–  Children often aware (unlike other disorders)

–  Many resolve in a few years

–  Talking to an external rhythm may help

h"ps://www.youtube.com/watch?v=U6vsz996OcQ

Clues to language disorders •  Nobabblingby12months•  NoconvenConalizedgesturesby12months•  Nospokenwordsby18months•  Lessthan50words,no2-wordcombosby24months•  Speechorlanguageregression

•  Firststeps:–  Isitahearingproblem?(audiologist)–  EvaluaCon,possibletherapy(speech-languagepathologist)–  TesCng:lotsofopCons,varyingincontentvalidity–  Watchoutforlanguagevaria2onthat’snotadisorder(differentdialects)

Brain lesions

•  Studies of children who had prenatal or perinatal brain injury –  Before language production started –  Not necessarily before getting language input

•  Range of lesion locations and sizes •  Big questions: does L or R hemisphere damage

matter for language ability? •  Is impairment related to lesion size?

Brain lesions •  Methodological problems:

–  Small N –  Huge variability in

•  Timing of lesion •  Underlying cause (Stroke? Seizures?)

–  Sensitivity of tests •  Extreme views of language specialization:

–  Equipotentiality –  Determinism –  Compromise: emergentism

Brain lesions •  Early work: Bates and colleagues •  Large set of kids with similar etiology •  By about age 6, most had recovered to low-

normal levels •  No relationship to hemisphere damaged •  Lesion size x performance:

–  U-function: tiny and large did better, medium did worse (consistent with animal lit--�fresh start� H)

–  Later, U-function did not hold

Brain lesions long-term

•  The happy story: •  Temporary impairment •  Then recover to normal by about 6 •  BUT •  Studies after this age show overall below-normal

IQ (verbal and performance) •  Artifact of more impaired kids continuing to see

doctors?

Brain lesions long-term •  After initial recovery of function, is there a

decline in IQ later in development for kids with unilateral focal lesions?

•  Levine et al. examined 15 kids who were IQ-tested pre- age 7 and post- age 7 –  Unusual--a longitudinal study

•  12 of 15 showed an IQ decline •  Equivalent declines in verbal and performance

Brain lesions long-term

•  *Side note: what does a decline in IQ mean? •  IQ tests have age-based norms

–  A 2-y-o who performs like a UCSD undergrad has a higher IQ than that undergrad*

•  Not gaining skills as rapidly •  (Still improving, but not as fast as normal)

Brain lesions long-term

•  Relationship to lesion size? –  Smaller is worse

•  Relationship to earlier IQ? –  Higher IQ early on meant bigger decline

•  Why does this happen? –  Not enough processing power to keep up –  Artifact--IQ tests start demanding abstract reasoning

(maybe a deficit all along)

Comparisons across language difficulties

SLI vs FL vs WS

•  Reilly et al. used narratives – Look at this picture book and explain story – Tests both formal language (e.g., syntax) and

cognitive abilities (understanding the story) •  Do SLI kids look like

– FL (�focal lesion�) kids? – WS (Williams syndrome) kids?

SLI vs FL vs WS •  Study 1: SLI vs. early lesions •  Measures:

–  Story length –  Complexity of sentences –  Rate of morphological errors

•  Assessment –  Count total # of propositions –  Count errors, divide by prop. count –  Assess diversity of syntactic constructions

SLI vs FL vs WS

•  SLI, FL below typical at age 4-6

•  Lesion group approached normal at 10-12

SLI vs FL vs WS

•  SLI, FL below typical at age 4-6

•  Lesion group approached normal at 10-12

SLI vs FL vs WS

•  SLI, FL below typical at age 4-6

•  Lesion group approached normal at 10-12

SLI vs FL vs WS

•  SLI,FLbelowtypicalatage4-6

•  Lesiongroupapproachednormalat10-12

•  ImplicaCon:SLIworsethanmissingpartofbrain!!

SLI vs FL vs WS

•  Study 2: SLI vs. Williams (WS)

SLI vs FL vs WS

•  Study 2: SLI vs. Williams (WS) •  Measures:

– Story length – Morphological errors – Narrative coherence (major points, theme)

SLI vs FL vs WS

•  Study 2: SLI vs. Williams (WS) •  SLI: more errors at 4-6 than WS •  Comparable on complex syntax

SLI vs FL vs WS

•  Study 2: SLI vs. Williams (WS) •  But WS has harder time maintaining

structure of narrative (boy looks for frog)

TD:Theylookedinthebeehiveandintheholebutcouldnotfindthefrog.SLI:Thedogistryingforthebeesandtheboy’slookingforthefrog.WS:Andthenallofasuddenthedogfindssomebeesflying.

SLI vs FL vs WS

•  Study 2: SLI vs. Williams (WS) •  WS use more social evaluation devices

•  Voices, sound effects, exclamations--audience attention

TD:Theylookedinthebeehiveandintheholebutcouldnotfindthefrog.SLI:Thedogistryingforthebeesandtheboy’slookingforthefrog.WS:Andthenallofasuddenthedogfindssomebeesflying.

SLI vs FL vs WS

•  Overall picture for SLI •  Grammatical competence really poor

– Worse than focal brain injury •  Narrative skills not so bad