Speech recognition performance was measured in normal-hearing and cochlear-implant listeners with maskers consisting of either steady-state speech-spectrum-shaped noise or a competing sentence. Target sentences from a male talker were presented in the presence of one of three competing talkers (same male, different male, or female) or speech-spectrum-shaped noise generated from this talker at several target-to-masker ratios. For the normal-hearing listeners, target-masker combinations were processed through a noise-excited vocoder designed to simulate a cochlear implant. With unprocessed stimuli, a normal-hearing control group maintained high levels of intelligibility down to target-to-masker ratios as low as 0 dB and showed a release from masking, producing better performance with single-talker maskers than with steady-state noise. In contrast, no masking release was observed in either implant or normal-hearing subjects listening through an implant simulation. The performance of the simulation and implant groups did not improve when the single-talker masker was a different talker compared to the same talker as the target speech, as was found in the normal-hearing control. These results are interpreted as evidence for a significant role of informational masking and modulation interference in cochlear implant speech recognition with fluctuating maskers. This informational masking may originate from increased target-masker similarity when spectral resolution is reduced.

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As the most successful neural prosthesis, cochlear implants have provided partial hearing to more than 120,000 persons worldwide; half of which being pediatric users who are able to develop nearly normal language. Biomedical engineers have played a central role in the design, integration and evaluation of the cochlear implant system, but the overall success is a result of collaborative work with physiologists, psychologists, physicians, educators, and entrepreneurs. This review presents broad yet in-depth academic and industrial perspectives on the underlying research and ongoing development of cochlear implants. The introduction accounts for major events and advances in cochlear implants, including dynamic interplays among engineers, scientists, physicians, and policy makers. The review takes a system approach to address critical issues from design and specifications to integration and evaluation. First, the cochlear implant system design and specifications are laid out. Second, the design goals, principles, and methods of the subsystem components are identified from the external speech processor and radio frequency transmission link to the internal receiver, stimulator and electrode arrays. Third, system integration and functional evaluation are presented with respect to safety, reliability, and challenges facing the present and future cochlear implant designers and users. Finally, issues beyond cochlear implants are discussed to address treatment options for the entire spectrum of hearing impairment as well as to use the cochlear implant as a model to design and evaluate other similar neural prostheses such as vestibular and retinal implants.

We studied a family with hereditary sensory motor neuropathy and deafness accompanying a missense mutation in the MPZ gene. Pathological examination of the cochlea in one of the family members revealed marked loss of auditory ganglion cells and central and peripheral auditory nerve fibres within the cochlea. The inner hair cells were of normal number with preserved morphology. The outer hair cells were normal in number except for a 30% reduction in just the apical turn. Examination of the sural nerve and the auditory nerve adjacent to the brainstem showed marked loss of fibres with evidence of incomplete remyelination of some of the remaining fibres. Studies of auditory function in surviving family members using electrophysiological and psychoacoustic methods provided evidence that the hearing deficits in this form of auditory neuropathy were probably related to a decrease of auditory nerve input accompanying axonal disease. Altered synchrony of discharge of the remaining fibres was a possible additional contributing factor.

Cochlear-implant users perform far below normal-hearing subjects in background noise. Speech recognition with varying numbers of competing female, male, and child talkers was evaluated in normal-hearing subjects, cochlear-implant users, and normal-hearing subjects utilizing an eight-channel sine-carrier cochlear-implant simulation. Target sentences were spoken by a male. Normal-hearing subjects obtained considerably better speech reception thresholds than cochlear-implant subjects; the largest discrepancy was 24 dB with a female masker. Evaluation of one implant subject with normal hearing in the contralateral ear suggested that this difference is not caused by age-related disparities between the subject groups. Normal-hearing subjects showed a significant advantage with fewer competing talkers, obtaining release from masking with up to three talker maskers. Cochlear-implant and simulation subjects showed little such effect, although there was a substantial difference between the implant and simulation results with talker maskers. All three groups benefited from a voice pitch difference between target and masker, with the female talker providing significantly less masking than the male. Child talkers produced more masking than expected, given their fundamental frequency, syllabic rate, and temporal modulation characteristics. Neither a simulation nor testing in steady-state noise predicts the difficulties cochlear-implant users experience in real-life noisy situations.