Auditory and language skills development after cochlear ... · Auditory and language skills...

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Auditory and language skills development after cochlear implantation in children with

multiple disabilities

Medhat Yousef, M.D., Ph.D.Consultant Audiovestibular medicine

King Abdullah Ear Specialist Center (KAESC)King Saud University

KSACI2019 MIAMI, USA

Background According to the 2018 report of WHO, hearing loss is

estimated to be the fourth leading cause of disability

globally that affects millions of people around the world.

It has been estimated that approximately 40-50% of

children with hearing loss have an additional medical or

developmental disability.

Background

• Physical disabilities• Developmental delay• Cerebral palsy • Attention deficit hyperactivity

• Learning disabilities• Visual impairment• Autism spectrum disorders• Mental retardation

Background Recently the candidacy criteria for pediatric cochlear

implantation are getting more flexible to include some hard of

hearing children with other disabilities and complex needs.

Although some studies investigated the quality of life issues in

cochlear-implanted children with other disabilities, few data are

available regarding the rehabilitation outcomes in terms of

auditory and verbal communication skills of those children.

Objectives

To investigate the outcomes of CI children with different co-

disabilities through using the Meaningful Auditory Integration

Scale (MAIS) and the Meaningful Use of Speech Scale

(MUSS) as outcome measurement tools.

Material & MethodsStudy Population

The study population comprised 2 groups:

1. The case group: 25 hearing-impaired children with co-disability who received cochlear implantation at our center.

2. The control group: Age and gender-matched controlgroup of 25 cochlear-implanted children without co-disabilities.

Material & MethodsStudy PopulationInclusion criteria for subjects in both groups:

1. All children should have pre-lingual severe to profoundsensorineural hearing loss and used a hearing aid for aperiod ranged between 3 to 6 months before surgery.

2. All children had used the CI and underwent rehabilitationfor at least 12 months using auditory-verbal therapy.

3. Documented diagnosis of at least one disability in additionto deafness.

Material & MethodsAuditory Skills Outcomes

Meaningful Auditory Integration Scale (MAIS)

• A 10-questions tool in which parents are asked to reportthe behavior and reactions of their children in using thecochlear implant device on throughout the day.

• Its maximum score is 40 points is obtainable under thescoring system.

• There are three divisions in scoring MAIS includingreliance on using the device, alerting to sounds, andderiving meaning from sound.

Material & MethodsLanguage and Speech Outcomes

Meaningful Use of Speech Scale (MUSS)

• A 10-item questionnaire in which the parents ratespeech abilities of their children on a scale from 0 to4 (highest).

• There are 3 divisions in scoring MUSS including thesound generation, the communication capability,and communication skills .

ResultsThe disability group was subdivided according to the type of the disability into four subgroups:

1. Usher syndrome (N = 6).

2. Global Developmental Delay (GDD) (N = 6).

3. Autism Spectrum Disorder (ASD) (N = 9).

4. Attention Deficit Hyperactive Disorder (ADHD) (N = 4).

ResultsCommunication Mode

Group Behavioral Oral Total

Usher 2 4 6ADHD 1 3 4GDD 4 2 6ASD 8 1 9

Control 1 24 25

Post-implantation, 15 out of 25 children in the case group made communication progress.

I. Auditory Skills Outcomes

Groups Aided threshold MAISMean SD P Mean SD P

Usher (N=6)Control (N=25)

33.54 28.00

10.53 4.77

0.77 38.540.00

3.670.01

Developmental delay (N=9)

Control (N=25)

39.5828.00

15.50 4.77

0.11 23.8340.00

14.740.01

Autism (N=6)Control (N=25)

49.86 28.00

13.23 4.77

0.0001 18.8840.00

15.430.01

ADHD (N=4)Control (N=25)

33.68 28.00

1.02 4.77

0.85 24.7540.00

10.400.01

Patients (N=25)Control (N=25)

40.89 28.00

3.58 4.77

0.0001 25.72 40.00

14.15 0.01

0.0001

I. Auditory Skills Outcomes: There was a statistically significant difference between the patients and control groups

33.54 28.00

10.53 4.77

0.77 38.540.00

3.670.01

Control (N=25)

39.5828.00

15.50 4.77

0.11 23.8340.00

14.740.01

49.86 28.00

13.23 4.77

0.0001 18.8840.00

15.430.01

33.68 28.00

1.02 4.77

0.85 24.7540.00

10.400.01

40.89 28.00

3.58 4.77

0.0001 25.72 40.00

14.15 0.01

0.0001

I. Auditory Skills Outcomes:Usher syndrome subgroup has obtained the best comparable auditory skills level as the control group.

33.54 28.00

10.53 4.77

0.77 38.540.00

3.670.01

Control (N=25)

39.5828.00

15.50 4.77

0.11 23.8340.00

14.740.01

49.86 28.00

13.23 4.77

0.0001 18.8840.00

15.430.01

33.68 28.00

1.02 4.77

0.85 24.7540.00

10.400.01

40.89 28.00

3.58 4.77

0.0001 25.72 40.00

14.15 0.01

0.0001

I. Auditory Skills Outcomes:The autism spectrum disorder subgroup had the worst MAIS score.

33.54 28.00

10.53 4.77

0.77 38.540.00

3.670.01

Control (N=25)

39.5828.00

15.50 4.77

0.11 23.8340.00

14.740.01

49.86 28.00

13.23 4.77

0.0001 18.8840.00

15.430.01

33.68 28.00

1.02 4.77

0.85 24.7540.00

10.400.01

40.89 28.00

3.58 4.77

0.0001 25.72 40.00

14.15 0.01

0.0001

II. Language and speech outcomes

Groups MUSSMean SD P

25.8329.88

11.037.61

Developmental delay (N=9)Control (N=25)

12.529.88

10.117.61

7.3329.88

7.567.61

0.0001

10.529.88

4.437.61

13.52 29.88

11.05 7.61 0.0001

II. Language and speech outcomesA significantly statistical difference was obtained between the patients and control groups

25.8329.88

11.037.61

12.529.88

10.117.61

7.3329.88

7.567.61

0.0001

10.529.88

4.437.61

13.52 29.88

11.05 7.61 0.0001

II. Language and speech outcomes: There was no statistical difference between the Usher syndrome subgroup and the control group

25.8329.88

11.037.61

12.529.88

10.117.61

7.3329.88

7.567.61

0.0001

10.529.88

4.437.61

13.52 29.88

11.05 7.61 0.0001

Summary & ConclusionHearing-impaired children with co-disabilities can demonstrate somebenefits from receiving CI including not only the auditory skills but alsothe communicative and language acquisition skills.

However, those benefits are limited and cannot be compared to the outcomes obtained in hearing-impaired children without additional disabilities.

Cochlear implantation decision for those group of children shouldbe cautiously considered and studied on an individual basis

according to the potential excepted benefits for each case.

AcknowledgementTO all KAESC team specially:

Dr. Tamer Mesallam.

Miss. Ayna Almosaad

Thank YouanY QuesTion?

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