Cochlear implantation in children with syndromic deafness
| Autor: | Lise Henderson, Richard T. Ramsden, Iain A. Bruce, Kevin Green, Stephen J. Broomfield |
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| Rok vydání: | 2013 |
| Předmět: |
Male
medicine.medical_specialty Speech perception Adolescent Hearing loss Hearing Loss Sensorineural medicine.medical_treatment Usher syndrome Audiology CHARGE syndrome Cochlear implant otorhinolaryngologic diseases medicine Humans Abnormalities Multiple Child Pendred syndrome Retrospective Studies Waardenburg syndrome business.industry Syndrome General Medicine medicine.disease Cochlear Implantation Cochlear Implants Treatment Outcome Otorhinolaryngology Child Preschool Pediatrics Perinatology and Child Health Speech Perception Etiology Female medicine.symptom business |
| Zdroj: | International Journal of Pediatric Otorhinolaryngology. 77:1312-1316 |
| ISSN: | 0165-5876 |
| DOI: | 10.1016/j.ijporl.2013.05.022 |
| Popis: | Objective To examine the outcome of cochlear implantation in children with syndromic deafness, who are increasingly being considered for cochlear implants and who represent a unique challenge to the cochlear implant team. Method In this retrospective case series in a tertiary referral cochlear implant centre, we describe a series of 38 children with a clinical syndrome causing deafness who have undergone cochlear implantation. The outcome measures are Bench–Kowal–Bamford (BKB) speech reception score (range 0–100%) and speech perception ability using the Geers and Moog Speech Reception Score (SRS) (range from 0; no speech perception, to 6; open set recognition of words). Results The syndromes identified were Waardenburg syndrome ( n = 10), Usher syndrome ( n = 9), Pendred syndrome ( n = 7), Jervell and Lange–Nielsen syndrome ( n = 5), CHARGE syndrome ( n = 2), and 1 each of Stickler, CINCA (Chronic Infantile Neurological Cutaneous and Articular), Bartter, Down, and Donnai–Barrow syndromes. After a minimum of 19 months following implantation, BKB was measurable in 20 of 38 patients, and ranged from 46 to 100% in quiet (median 87%, mean 81%). Eighteen children (55%) achieved a SRS at level six, and a further 8 (24%) achieved level five. There was significant variation of outcome between and within syndrome groups. Conclusions Additional disabilities are frequently encountered when considering children for cochlear implantation, and may be part of a recognised syndrome. Outcome is often excellent but can be variable even within the same syndrome group, and such children are therefore assessed on an individual basis to ensure a realistic expectation. |
| Databáze: | OpenAIRE |
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