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

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

Introduction Chouard CH, Fugain C, Meyer B, Chabolle F. Acta Otorhinolaryngol Belg 1985; 39(4):735-48. “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 1995. “The implantation of multi-handicapped children and adults, and particularly the prelingual children, generates special questions”.

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 %

Material and method: Patologies Usher syndrome 5 Waanderburg syndrome 5 Cerebral Palsy 4 Down syndrome 3 Autism 2 Blindness 3 CMV 3 CHARGE syndrome 1 Gregg syndrome 1 Noonan syndrome 1 Turner syndrome 1 Cogan syndrome 1 CET 2

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 30-50 dB PRE CI POST CI Cerebral Palsy 92 dB 45.35 dB Down syndrome 96.65 dB 42.5 dB Autism 115.5 dB 42.42 dB Blindness 114.16 dB 46.57 dB CHARGE 118.3 dB 38.57 dB Usher 105.46 dB 47.23 dB Gregg >120dB 30 dB Noonan 85 dB 30.71 dB Turner 75 dB 33.33 dB Cogan >120dB 45.71 dB Waanderburg 100 dB 42 dB CMV 105 dB 35 dB

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

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

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

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

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: