Transient click-evoked otoacoustic emissions (TEOAE) and distortion-product otoacoustic emissions (DPOAE) are produced by an active biomechanical process in the cochlea, presumably related to outer hair-cell activity. Although it is generally accepted that in most cases of hearing loss with absent auditory evoked potentials neither TEOAE nor DPOAE can be found, some cases with such a constellation have been described. Here we report another four cases of children with severe to profound hearing loss where we discovered reproducible TEOAE and DPAOE, whereas auditory evoked potentials were missing. TEOAE and DPOAE recordings in these cases indicate substantially preserved outer hair-cell function independent of profound pre-sensineural hearing loss. Since the incidence of children with preserved otoacoustic emissions together with impairment of synaptic or postsynaptic function of the first neuron is not known, the unconditioned use of TEOAE nor DPOAE as a screening instrument must be seriously questioned. Secondly, in conjunction with subjective audiometry and brain-stem-evoked potentials, emission recordings is an indispensable measurement prior to cochlear implantation and use of high-power hearing aids.