Objective The benefits of cochlear implantation for children with developmental delays (DD) are often unclear. We compared cognition, adaptive behavior, familial stress, and communication in children with and without DD. Study Design Retrospective review Setting Two tertiary care pediatric hospitals Patients 204 children who underwent cochlear implantation assessed before and >1 year after implantation Main Outcome Measures The Mullen Scales of Early Learning (MSEL), Vineland Adaptive Behavior Scales (VABS), Parental Stress Index (PSI), and Preschool Language Scale (PLS). Results We developed a specific definition of DD for hearing-impaired children based upon DSM-IV criteria for mental retardation; 60 children met the criteria for DD and 144 children did not. Prior to implantation, multiple linear regression demonstrated that children with DD had lower scores in every domain of the MSEL and VABS (p<0.05) but no differences in any domains of the PSI and PLS (p>0.1) compared to children without DD. After implantation, children without DD demonstrated significant improvements in intelligence as measured by the MSEL, age-appropriate improvements in adaptive behavior as evaluated by the VABS, and their familial stress levels were not increased after cochlear implantation. In contrast, children with DD underwent implantation at a later age and demonstrated less comprehensive developmental improvements after cochlear implantation and higher stress levels. However, when the age differences were taken into account using multiple linear regression analyses, the differences between two cohorts were reduced. Conclusions These data indicate that our definition of DD is a reliable method of stratifying deaf children. While children with DD have a normal developmental rate of adaptive behavior after cochlear implantation, their developmental rate of intelligence is lower and they have higher stress levels than children without DD. However, our data suggest that if children with DD could be implanted as early as children without DD, their intelligence and stress outcomes would be improved.

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This paper proposes two relatively common "syndromes" seen in children with perilymph fistulas using illustrative case reports. One, a 2 1/2-year-old child with bilaterally symmetric progressive sensorineural hearing loss, was found to have bilateral oval and round window fistulas. The other was a child who presented originally with an unexplained unilateral sensorineural loss. It was only when hearing deteriorated in the opposite ear several years later that a perilymph fistula was suspected and confirmed by tympanotomy. The clinical presentations and other details of these cases diverge widely, but may represent two emerging syndromes of perilymph fistulas in children: 1. a congenital or hereditary predisposition, and the other, 2. sudden hearing loss in the "good" ear of children with an unexplained sensorineural hearing loss in the opposite ear.