1. Cochlear Implantation in multi-handicapped children
ESPO 2006
Cochlear Implantation in multi-handicapped children
Jorge Alberto Rey Martínez
ENT Department
Clínica Universitaria de Navarra. Spain

2. Introduction
The prevalence of sensorineural hearing loss in children is about 1 case for 1000 born children
Is relatively frequent the association of sensorineural hearing loss with other pathologies, principally with motor, systemic and other sensorineural affectation
Vartiainen E, Kemppinen P, Karjalainen S
Int J Pediatr Otorhinolaryngol 1997;41(2):175-85

3. Introduction
Chouard CH, Fugain C, Meyer B, Chabolle F. Acta Otorhinolaryngol Belg 1985; 39(4): “The associated deficiencies (autism, palsy, blindness) are not contraindications and is frequent that increases the indication of cochlear implantation”.
Cochlear Implants in Adults and Children. National Institutes of Health. Consensus Statement “The implantation of multi-handicapped children and adults, and particularly the prelingual children, generates special questions”.

4. Material and method: Patients
CUN-2006:
In 32 children of 544 implanted patients are there another neurological or sensorineural affectation (Nucleus 22 or/and 24 system)
Average age: 8,41
P 25-75: 4
Prelingual: 72 %
Evolution time: 6m-11y
5,9 %

5. Material and method: Patologies
Usher syndrome
Waanderburg syndrome 5
Cerebral Palsy
Down syndrome
Autism
Blindness
CMV
CHARGE syndrome
Gregg syndrome
Noonan syndrome
Turner syndrome
Cogan syndrome
CET

6. Results: PTA PRE CI POST CI
In most of cases the previous audiometric levels was between 90 – 120 dB, and the post surgery levels was between dB
PRE CI POST CI
Cerebral Palsy 92 dB dB
Down syndrome dB 42.5 dB
Autism dB dB
Blindness dB dB
CHARGE dB dB
Usher dB dB
Gregg >120dB 30 dB
Noonan 85 dB dB
Turner 75 dB dB
Cogan >120dB dB
Waanderburg 100 dB 42 dB
CMV dB 35 dB

7. Results: Communication after CI
Full
Oral
Gestual
Other
Cerebral palsy
Limited in expression
Graphic system
Dawn S.
Early implantation / Intellectual coefficient 51
Autism
Progressive hearing loss
Deep autism
Blindness
1 Postlingual / both with Braille
Prelingual

8. Results: Communication after CI
Full
Oral
Gestual
Other
CHARGE S.
Cardiac, optical, intellectual, nasal, genital affectation
Limited expression
Graphic system
Usher S.
Optical affectation
Later implantation
Cytomegalovirus
Waanderburg S.
Skin, facial and optical affectation

9. Results: Communication after CI
Full
Oral
Gestual
Other
Turner S.
Cardiac, endocrine, renal affectation
Noonan S.
Intellectual, facial and skeletal, genital affectation
Gregg S.
Intellectual, optical, cranial, cardiac affectation
Cogan S
Optical affectation

10. Discussion
The auditive results are similar in this patients compared with the obtained in not multi-handicapped children. All patient uses the implant.
For the rehabilitation and programmation process of this cases was necessary to have other specialized professionals in the treatment of the associated deficiencies. This professionals was able to develop specific strategies and adapted teachings that best fit with the develop stages of the patients.
Blindness: Braille language
For the cases in witch there was not possible to get a full rehabilitation, the cochlear implant was able to improve the development and integration of this children.
Chromosome 3 monosomy: Patient has reaction with audible stimulus

11. Conclusion According to other authors:
“The additional deficiencies not excludes a patient for cochlear implantation. A personalized decision must to be evaluated based in additional diagnostic test and the experience of the implantation centre”
Lenarz T. Cochlear implants: selection criteria and shifting borders. Acta Otorhinolaryngol Belg 1998; 52(3):183-99: