Chen Xw

Psychophysical performance and Mandarin tone recognition in noise by cochlear implant users.

Objective: The present study was aimed to examine the relationship between psychophysical performance in temporal and spectral resolution and Mandarin tone recognition in noise by cochlear-implant (CI) listeners. Design: Seventeen Nucleus-24 implant users, 10 postlingually deafened and 7 prelingually deafened, participated in the experiments. A 3-interval, forced-choice procedure was used to measure gap detection and pure-tone frequency discrimination at 250 to 4,000 Hz in octave steps. A 4-alternative forced-choice procedure was used to measure Mandarin tone recognition in quiet and in noise. Signal-to-noise ratios (SNRs) varied from +10 to –10 dB. All stimuli were delivered to the clinical processor via a speaker in a sound free field. The obtained data were compared to data collected from normal-hearing control subjects, as well as cochlear-implant users who performed similar tasks using single-electrode stimulation via a research interface. Results: Postlingually-deafened CI subjects generally performed better than prelingually-deafened subjects. The average gap detection threshold was 30 ms with a range from 4 to 128 ms. The average frequency difference limen was 100 Hz with a range from 12 to 192 Hz, regardless of the standard frequency. The average tone recognition was 80% correct in quiet, which dropped to 55% at +10 dB SNR and essentially chance performance at –5 dB SNR. In comparison, the normal-hearing control subjects maintained essentially perfect performance over this SNR range. Only frequency discrimination at 1,000 Hz was significantly correlated with tone recognition in quiet but all psychophysical measures were correlated to tone recognition in noise. Conclusions: The present result suggests that the CI users can rely on either temporal or spectral cues to perform tone recognition in quiet, but need both cues for tone recognition in noise. Future CI processors need to extract and encode these acoustic cues to achieve better performance in tone perception and production.

High Prevalence of the Connexin 26 (GJB2) Mutation in Chinese Cochlear Implant Recipients

Background: The GJB2 gene, mapping to chromosome 13q12, encodes a gap junction protein, connexin 26, and is responsible for certain forms of congenital deafness, such as DFNB1 and DFNA3. Mutations of this gene are responsible for about one half of severe autosomal recessive non-syndromic deafness. Methods: To determine whether GJB2 mutations are major causes of deafness in Chinese cochlear implant recipients, we enrolled 115 cochlear implant recipients for mutation screening. Results: The results showed that 36.5% (42/115) of all cochlear implant recipients and 41% (41/100) of non-syndromic deafness patients exhibit GJB2 mutations; only 1 inner ear malformation patient was detected with GJB2 mutations. The present study found 11 different variations in the GJB2 gene. Conclusion: The 235delC mutation was the most prevalent mutation, found in 18.3% (42/230 alleles) of all cochlear implant recipients and 21.0% (42/200 alleles) of the non-syndromic deafness group. Only 0.6% of GJB2 mutations were detected in the inner ear malformation group. The novel 187G→T mutations are likely to be pathological mutations.