Deafness mice are profoundly deaf from birth as a result of genetically determined cochlear dysfunction. Evoked potentials in response to direct electrical stimulation of the cochlear nerve can readily be recorded in the inferior colliculus of deafness mice, and such responses are larger in amplitude than those in control mice. These observations indicate that at least some central connections become functional in the deafness central auditory pathway in the absence of peripheral stimulation, and are relevant to the general problem of restoring function by direct nerve stimulation in the profoundly deaf.