Cochlear implantation in children with syndromic deafness.

[pendred syndrome]

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 .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 ).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 .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 .

Diseases
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Diseases presenting "open set recognition" symptom

pendred syndrome

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