Comorbidity in children with hearing impairment and a multilingual background Institutt for Spesialpedagogikk, 2017-11-24

Ulrika Löfkvist, Försteamanuens, Med Dr, Leg. Logoped, LSLS cert. AVEd Universitetet i Oslo, Karolinska Institutet

“It’s not the hearing that’s the problem”(parent quote, 2014)

Learning outcome• Multilingualism is common, especially in children with hearing

impairment

• High level of comorbidity within the group of children with HI–the etiological background explains some of the variation

• LENA – a new screening tool for assessment and for intervention actions

Multilingualism vs. monolingualism

Which is the norm?

Multilingualism - definition

”A child is considered to be bilingual if she/he lives in a non-monolingual environment and is regularly exposed to at least two languages”

Salameh, 2003

Comorbidity - defintion

Coexistence of two or more diseases in the same patient

e.g. Diabetes and heart disease

Feinstein, 1970

Ex: Jervell Lange Nielsen syndrome: Hearing loss, Heart Disease, Balance Disorder

Ex: Congenital CMV infection: Deafness + microcephaly + liver disease + mental retardation + visual disorder + language Impairment (not explained by the deafness)

A changing population of children with hearing impairment

Infants come to the clinic with parents – preventive and family-centered actions are needed

Increase of multi-lingual cohorts globally – demands cultural knowledge, new assessment tools and intervention options (preferable preventive)

High degree of comorbidity within mono- and multi-lingual cohorts –demands a multidisciplinary team approach

Increased socio-economical differences in society

Are all multilingual individuals similar?

Born in another country (immigrant)

Second- or third generation

Speaks two, three or more languages

Arrival age: 0-6, 7-11 or 12-16 years of age

Language level of parents and level of daily exposure

Confounding factors in the research domain of children with HI

Heterogeneous, small population

”Children with HI” = different subgroups including an increasing number of children with multilingual background

Uni- or bilateral CI, use of bimodal approach

Fulltime use of hearing aids?

Nordic situation: small sample spread over a large geographical area

Different type of language stimulation and habilitation options

Clinical research – biased?

Theoretical research – limited knowledge of the clinical reality and complexity?

Hearing impairment and multilingualism

• Common – why?

• Few studies, often small samples

• No evidence-based intervention policy

Etiologi - orsakACMG statement (2002) Genetics Evaluation Guidelines for the Etiologic Diagnosis ofCongenital hearing Loss, May/June, vol:4, no:3 Genetics in Medicine

(Karolinska Univ.Hospital 2000-15)Cause of deafness (n=355)

Multilingual children n=106 (30 %)

Monolingual childrenn=249 (70 %)

Unknown N= 51 (48 %) N= 99 (40 %)

Genetic & Acquired N= 55 N= 150

Genetic deafness

Connexin 26Usher’s syndromeJervell-Lange Nielsen’s syndromeNoonan syndromeCharge syndromeUnspecified hereditaryZellweger syndromeX-linked malformationPendred’s syndromeMöbius syndromeLVAS (malformation)Waardenburg syndromeAuditory N.PKUInterstelarUnspecified malformationUnspecified syndrome

Acquired deafness

Congenital CMV infectionMeningitisOtoxicityPerinatalTraumaRubella

N= 46 (84 % of 55)

n= 6 (11 %)n= 4 (9 %)n= 1 (2 %)n= 0 (0 %)n= 1 (2 %)n= 17 (36 %)n= 0 (0 %)n= 3 (7 %)n= 0 (0 %)n= 1 (2 %)n= 0 (0 %)n= 0 (0 %)n= 1 (2 %)n= 0 (0 %)n= 0 (0 %)n= 10 (23 %)n= 2 (5 %)

N= 9 (16 %)

n= 3 (5 % of 55)n= 2n= 1n= 1n= 1n= 1

N= 104 (60 % of 150)

n=18 (7%)n= 8 (3 %)n= 5 (2 %)n= 2 (1 %)n= 1 (0,5 %)n= 28 (11 %)n= 1 (0,5 %)n= 7 (3 %)n= 4 (2 %)n=0 (0%)n= 2 (1 %)n= 4 (2 %)n= 0 (0 %)n= 1 (0,5 %)n= 1 (0,5 %)n= 17 (7 %)n= 5 (2 %)

N= 46 (40 %)

n= 30 (20 % of 150)n= 11n= 1 n= 6 n= 0n= 0

Preliminary results from the retrospective study(Children who are followed-up at the clinic)

Children implanted with CI at Karolinska Univ.sjh; 2000-15

79 % had twoparents who speak another language

than Swedish in the multilingual group

Arabic and tigrinia the most common languages (Middle East and North Africa)

Prevalence – children with CI in Norway and Sweden

32,14%ofchildrenimplantedbetween2000-15(Karolinskauniv.sjh,Sweden)(Löfkvistetal.,inpreparation)

30,9%ofallNorwegianchildren(Amundsenetal.,2016)

Prevalence of children with hearing loss who use hearing aids with multilingual background?

Thomas et al., 2008

Purpose: determine if exposure to a second (heart) language impacts the ability of children with CI to develop english skills

Participants: Matched pairs: mono- or bilingual children with CI with similar age at implantation, SES, educational setting (mainly AVT) and cochlear anatomy (n=24)

Material: different language tests (PPVT etc), longitudinal design

Results: no statistical significant differences between groups over time

Conclusion: multilingual children can learn multiple spoken languages and parents do not need to avoid using a minority language with their child who has a CI

Teschendorf et al., 2011

Objective: 1. To investigate whether exposure in a second language in bilingual families

influences the German skills after cochlear implantation2. To explore how many children with immigration background who are bilingual

Method: Retrospective study from a CI center: 93 children in total (53 bilingual and 41 monolingual)

Results: Children from bilingual homes had worse results in all performed speechtests compared to controls, German was indicated as their main language. Only a few children had higher competence in the second (heart) language

Conclusion: Advanced language skills including learning a second language is possible for children with CI – but that is the exception rater than the rule.Special support is needed in this subgroup.

Aragon & Yoshinaga-Itano, 2013

Purpose: to investigate language environment in children with HI in Spanish-speaking homes and in comparison to English-speaking children with HI and two other age- and language-matched control groups (with NH)

Method: LENA recordings in the homes, coaching of parents

Results: despite lower average level of maternal education, children from Spanish-speaking homes with HI showed similar levels as age-matched English-speaking NH controls – and both had higher medians when compared to the Spanish-speaking group with NH

Conclusion: it is important with intervention not only in homes of multilingual children with HI – but also in homes of multilingual children with NH and lower SES

Age at identification of comorbidity

A hearing impairment can delay the identification and diagnosis of other deficits or diagnoses that is not related to hearing (however hearing loss is blamed)

Autism-spectrum disorder is diagnosed around one year later in children with hearing loss*

A neurodevelopmental disorder/diagnosis for a child with multilingual background can delay the identification of a hearing impairment (the delayed language development is blamed on multilingual development)

*Mandell et al Pediatrics 2005:116:1480-1486

Case example:

“Cured from ADHD” after changing school !?!

Ex Syndroms with hearing loss

• Eye (Usher, Alström, Waardenburg, CHARGE)• Kidney (Aport, Branchio oto-renal)• Heart (Jervell-Lange Nielsen, Downs s.)• Brain (Auditive neuropathy, mitokondriella)• Thyroid (Pendred’s syndrom)

Syndroms with hearing and visual impairment

• 50 % of all children (at 7 years) with hearing loss also have visual impairment

• 20 % of normal hearing children

• Vision must be checked regularly

Usher syndrome and deafness

• Bilateral CIs as early as possible• Typically no cognitive deficits • Good language outcome with CI • Some receive CI as adults• Environmental sounds important to pay attention

to and learn to identify• Sign language – tactile sign language

Symptomatic 10 %

1 or more deficits at birthSNHL at birth

Asymptomatic 90 %

Pass OAE-screeningProgressive SNHL

Congenital Cytomegalovirus (cCMV) infection

Karltorp, E., Löfkvist, U., Lewensohn-Fuchs. I., Lindström, K., Eriksson Westblad, M., Teär Fahnehjelm, K., Verecchia, L., Engman, M-L. (2014). Impaired balance and neurodevelopmental disabilities among children with congenital cytomegalovirus infection, Acta Paediatrica. 1-9.

Aim

•To evaluate the potential comorbid conditions among children with HI resulting from cCMV infection

•To compare with children with HI caused by GJB2(Connexin 26) mutations

Clinical results

• Neonatal symptoms: 6/26 (0)

• Head issues: 23/26 (0)

• Age of walking: 19 months (12 months)

Results

• Low vestibular function: 10/11 (0)

• Feeding problems: 46 % (0 %)

• Visual disability: 20 % (0 %)

• MRI findings (white matter)

Neurological disabilities in deaf children with cCMV infection and CI (n=26)

• Autism spectrum disorder = 4

• ADHD = 2 (and 2 under investigation)

• Language impairment = 2

• Oral motor disorder = 4

• Poor executive functioning compared to controls

General implications for intervention and treatment for children with cCMV infection

² BROAD Early Intervention program – prevention Look for Red flags

² Regular hearing assessments in children with cCMV(progressive HL) - TEAM approach important

² Include cognitive tests in follow-up assessment

² PREVENTION ACTIONS

Intervention actions besides hearing technology

”We don’t sing because she is deaf”

”We let her sit in front of the TV all the time”

(Parental quotes 2002, 2014)

1 – 3 – 6 (Joint Committee on Infant Hearing, Policy Statement, 2007)

Spoken language understanding (Reynell-III)

5 subgroups with different ages at 1st CI (n=115)Group 1: <9 months Group 2: 9-11 months Group 3: 12-17 months Group 4: 18-23 months Group 5: 24-29 months

Group1,N=19

0

1

2

3

4

5

6

7

8

0 1 2 3 4 5 6 7 8

Chronologicalage[years]

Developm

entalage[years]

Group2,N=29

0

1

2

3

4

5

6

7

8

0 1 2 3 4 5 6 7 8

Chronologicalage[years]

Developm

entalage[years]

Group3,N=28

0

1

2

3

4

5

6

7

8

0 1 2 3 4 5 6 7 8

Chronologicalage[years]

Developm

entalage[years]

Group4,N=22

0

1

2

3

4

5

6

7

8

0 1 2 3 4 5 6 7 8

Chronologicalage[years]

Developm

entalage[years]

Group5,N=17

0

1

2

3

4

5

6

7

8

0 1 2 3 4 5 6 7 8

Chronologicalage[years]

Developm

entalage[years]

Meanslopeingroups

0

1

2

3

4

5

6

7

8

0 1 2 3 4 5 6 7 8

Chronologicalage[years]

Developm

entalage[years]

Reg1Reg2

Reg3

Reg4

Reg5

Karltorp, E., Eklöf, M., Freijd, A., Östlund, E., Asp, F., Smeds, H., Hellström, S. & Löfkvist, U.Cochlear implantation before nine months of age is beneficial for the outcome of spoken language – a longitudinal study (in preparation)

Auditory habilitation in Scandinavia 2017

• Higher expectations in a child’s spoken language development

• Most parents choose spoken language(s)

• Deaf parents choose CI for their child (bilingual approach)

• Early AV habilitation options and education of parents

• More awareness of special needs for children with additional disabilities

– not always early diagnosis of LI, ADHD, ASD – especially not when the child also is multilingual

• Difficulties to reach some families – due to SES/sociocultural factors?

Environmental factors that influence development

Quantity & Quality of language use (Hart & Risely, 1995, Van Dam et al, 2012)

Parental sensitivity to child initiatives (Wieslader & Fernald, 2013)

SES level factors (Aragon & Yoshinaga-Itano, 2012)

Parental stress level (Ostberg, 1998; Hintermaier, 2006)

Socio-economical factors affecting outcome

• Everything is paid for (CI/HA, habilitation etc)

• Parents get financial support from the government

• No national protocol for early intervention services - Intervention services unequally distributed

• Most Swedish parents have high education degree – except for some groupsof immigrants with low SES

• Some parents with multicultural background and low SES do not attendindividual AV practice – for unclear reasons

How much language stimulation is needed?

Longitudinal study of language and development in 42 children with NH living in Kansas City, USA (Hart & Risley, 1995)

30 million word gap – due to SES level factors

Qualitative language parameters were also important for positive language outcome (less directive behavior and more interactive behavior from parents)

There was also a correlation between size of vocabulary at age three and follow-up results of language and IQ at 9 years

LENA – pedometer for words and communication

Language Environmental Analysis (LENA)§ Adult word count (AWC) (key female & key male)§ Child vocalisation (CV) (key child)§ Conversational Turn count (CTC)

§ Percentage of televison or radio§ Percentage of silence§ Percentage of noise§ Percentage of meaningful language ” or ”distant language”

§ Developmental snapshot – parent questionnaire

Ref: www.Lenafoundation.org

Visual feedback of LENA results in combination with coaching/parent education programs promotes change in communicative behavior of adults, regardless of SES (Aragon & Yoshinaga-Itano, 2012; Leffel & Suskind, 2013)

38 Föreläsningens namn

Automated Vocal Analysis of Children With Hearing Loss and Their Typical and Atypical Peers (n=273)

Van Dam et al (2015)

Conclusions: broad similarity between children with hearing loss and typically developing children, although children with hearing loss show some delay in their production of speech

Words make a difference

Evaluation of early listening- and spoken language environment in Swedish infants, toddlers and young children with HI or NH

(1) Swedish LENA validation study for children with NH and typical development aged 0-48 months

(2) Longitudinal cohort study and clinical implementation of the LENA method for all children with HI followed at Karolinska University Hospital, aged 0-48 months , including 30 % with multilingual background

(3) Prevention Education Program for Parents (PEPP) – a pilot study (3 months + follow-up) in a sample of children, aged 0-2, with HI and their families immediately from the time of diagnosis

A Norwegian pilot study has started at UiO this fall

Early Hearing Detection and Intervention “1-3-6 months”? (Joint Committee on Infant Hearing, 2007)

Subjects 1month(OAE)

3months(HL

confirmed)

6months(1st fittingofHAorCI)

Family-centeredIntervention

CI(n=22) 19children(lackofinfoabout2,1CMV)

4months(Md)

(1-20)

5months(Md)

(1.5-22)

5months(Md)

(4-10)HA(n=12) 12children 3months

(Md)

(1.5-8)

5months(Md)

(3-9)

7months(Md)

(4-11)

Age at recording (mean): 19 (NH), 24 (CI), 20 (HA) months (2-48)

PILOT STUDY (N=68)

Preliminary Swedish LENA results (n=66)

(Mean, min-max)

Percentage/day NH (n=34) CI (n=20) HA (n=12)Silence (%) 46 (21-73) 37 (9-69) 40 (19-56)TV/Ipad (%) 3 (1-7) 5 (1-13) 7 (1-32)Meaningful (%) 23 (16-36) 27 (16-45) 23 (13-30)Distant (%) 24 (8-46) 28 (9-49) 27 (11-51)Noise (%) 4 (1-6) 3 (1-8) 3 (1-6)

Two children with suspected language impairment excluded

Preliminary Swedish LENA pilot study results (n=66)

(Mean, min-max)

Number/day NH (n=34) CI (n=20) HA (n=12)Childvocalisations

2988 (289-6257)

2866 (817-7381)

1944 (1118-3396)

Adult words 17977(10023-36241)

20441(10013-33512)

21073(14847-33512)

Conversationalturns

833(124-2142)

766(304-1806)

609(401-876)

Two children with suspected language impairment excluded

How can we evaluate AND perhaps affect the variation of outcome in individuals with HI and special needs by using LENA?

Child A: multilingual backgroundand mild-moderate HLSlow progress, low SES Daycare and home recording

Child B: with multilingual background, suspected mental retardation and/or language impairment, malformed cochleaHigh school level (SES)Day care and home recordingIpad at dinner time

Child B: After home-based intervention actions and some adjustment at Daycare – still difficulties for the child to interact verbally or with signs with other childrenNo Ipad at dinner time

Possible impact of the Words make a difference-project

Generallybetterunderstandingoftheenvironmentalfactorsthatareinvolvedinearlyspokenlanguageacquisitionanditspossibleinfluenceonunexplainedvariabilityinoutcome

Newknowledgeofparentinvolvementeffects,notonlyinchildrenwithHI,butalsointhetypicalpopulationofyoungchildrenwithNH

TheresultsmightultimatelyhaveapositiveimpactonthelivesofmanychildrenwithHIandtheirfamilies,irrespectivelyofwhichkindofhearingtechnologytheyuseandlanguage- orsocio-economicalbackgroundtheyhaveandidentificationofearlymarkersofcomorbidconditions

Theresultsfromthevalidationstudywillalsolaythefoundationforfuturestudiesinotherclinicalgroups

In summaryComorbidity is common in society, and especially in multilingual subgroups with hearing impairment

Comorbidity is challenging for researchers, clinicians and families

Narrow focus on diagnosing and treating single diseases should be replaced by a more holistic team approach

Ulrika.lofkvist@isp.uio.no