The development of language and communication may play an important role in the emergence of behavioral problems in young children, but they are rarely included in predictive models of behavioral development. In this study, cross-sectional relationships between language, attention, and behavior problems were examined using parent report, videotaped observations, and performance measures in a sample of 116 severely and profoundly deaf and 69 normally hearing children ages 1.5 to 5 years. Secondary analyses were performed on data collected as part of the Childhood Development After Cochlear Implantation Study, funded by the National Institutes of Health. Hearing-impaired children showed more language, attention, and behavioral difficulties, and spent less time communicating with their parents than normally hearing children. Structural equation modeling indicated there were significant relationships between language, attention, and child behavior problems. Language was associated with behavior problems both directly and indirectly through effects on attention. Amount of parent–child communication was not related to behavior problems.

This report shows that children with ANSD who receive CIs are a heterogeneous group with a wide variety of impairments. Although many of these children may ultimately benefit from implantation, some will not, presumably because of a lack of electrical-induced neural synchronization, the detrimental effects of their other associated conditions, or a combination of factors. When preoperative magnetic resonance imaging reveals central nervous system pathology, this portends a poor prognosis for the development of open-set speech perception, particularly when CND is evident. These results also show that electrical-evoked intracochlear compound action potential testing may help identify those children who will develop good open-set speech perception. Instead of recommending CI for all children with electrophysiologic evidence of ANSD, the stepwise management procedure described herein allows for the identification of children who may benefit from amplification, those who are appropriate candidates for cochlear implantation, and those who, because of bilateral CND, may not be appropriate candidates for either intervention.

Children with cochlear nerve deficiency can present with electrophysiologic evidence of AN. These children frequently refer on newborn screening examinations that use ABR-based testing methods. Similar to other causes of AN, diagnostic ABR testing will show a CM with absent neural responses. Given that 9 (18%) of 51 children with available MRI and electrophysiologic characteristics of AN in our program have been identified as having cochlear nerve deficiency makes this a relatively common diagnosis. These findings suggest that MRI is indicated for all children diagnosed with AN. Moreover, electrophysiologic evidence of unilateral AN in association with a profound hearing loss should make the clinician highly suspicious for this problem. Although children with cochlear nerve deficiency who have a small nerve may benefit from cochlear implantation or amplification, these interventions are obviously contraindicated in children with completely absent cochlear nerves.

The findings of this study suggest that CN deficiency is not an uncommon cause of congenital hearing loss. The findings that most ears with CN deficiency had normal IAC morphology and that two ears with small IACs had CNs present indicate that IAC morphology is an unreliable surrogate marker of CN integrity. On the basis of these findings, we think that high-resolution MRI, rather than CT imaging, should be performed in all cases of pediatric hearing loss, especially in those cases where profound hearing loss has been documented. For ears with small IACs, the resolution of MRI currently remains limiting. In these cases, the determination of CN status frequently requires a variety of anatomic (CT and MRI) and functional tests (auditory brainstem response, otoacoustic emissions, behavioral audiometry, and physical examination).

Children with IP-EVA malformations have an excellent prognosis for developing open-set speech perception and using oral communication modes following CI. On the contrary, children with severe malformations or CND may have elevated charge requirements for attaining sound detection alone. These children's prognosis for obtaining open-set speech understanding, using exclusive oral communication, and participating in mainstream education is more limited. These findings have important implications for considering alternative forms of intervention such as auditory brainstem implantation and/or supplementation with visually based communication strategies.