There are significant challenges to restoring binaural hearing to children who have been deaf from an early age. The uncoordinated and poor temporal information available from cochlear implants distorts perception of interaural timing differences normally important for sound localization and listening in noise. Moreover, binaural development can be compromised by bilateral and unilateral auditory deprivation. Here, we studied perception of both interaural level and timing differences in 79 children/adolescents using bilateral cochlear implants and 16 peers with normal hearing. They were asked on which side of their head they heard unilaterally or bilaterally presented click- or electrical pulse- trains. Interaural level cues were identified by most participants including adolescents with long periods of unilateral cochlear implant use and little bilateral implant experience. Interaural timing cues were not detected by new bilateral adolescent users, consistent with previous evidence. Evidence of binaural timing detection was, for the first time, found in children who had much longer implant experience but it was marked by poorer than normal sensitivity and abnormally strong dependence on current level differences between implants. In addition, children with prior unilateral implant use showed a higher proportion of responses to their first implanted sides than children implanted simultaneously. These data indicate that there are functional repercussions of developing binaural hearing through bilateral cochlear implants, particularly when provided sequentially; nonetheless, children have an opportunity to use these devices to hear better in noise and gain spatial hearing.

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Complete agenesis of the bony labyrinth, first described by Michel, represents the most severe form of inner ear defect. A search of the literature yielded only one report of this rare anomaly, affecting two siblings. Three familial cases of bilateral inner ear aplasia are reported here, and the probable inheritance pattern of this condition is discussed.

We confirmed that the ESR threshold is a good measure of comfortably loud levels in adolescents with cochlear implants and their normal-hearing peers. Adolescents using CIs show normal-like rates of loudness growth on average, despite having highly variable dynamic ranges of hearing. Individual rates of loudness growth in the upper dynamic range in CI users can be predicted by the rate of amplitude growth of the ECAP. Thus, the rate of neural recruitment with increasing CI current is important for loudness perception in pre/perilingually deaf listeners and should be considered when programming their CIs.