The gap junction protein connexin30 (Cx30) is expressed in a variety of tissues that include epithelial and mesenchymal structures of the inner ear. We generated Cx30 (Gjb6) deficient mice by deletion of the Cx30 coding region. Homozygous mutants (Cx30((-/-))) were born at the expected Mendelian frequency, developed normally and were fertile. However, they exhibit a severe constitutive hearing impairment. From the age of hearing onset, these mice lack the electrical potential difference between the endolymphatic and perilymphatic compartments of the cochlea, i.e. the endocochlear potential, which plays a key role in the high sensitivity of the mammalian auditory organ. In addition, after postnatal day 18, the cochlear sensory epithelium starts to degenerate by cell apoptosis. This degeneration process is likely to account for the concomitant decrease of the endolymphatic potassium concentration and the aggravation of the hearing loss in adult Cx30((-/-)) mice. The Cx30 ((-/-)) phenotype thus reveals the critical role of Cx30 both in generating the endocochlear potential and for survival of the auditory hair cells after the onset of hearing. The Cx30 deficient mice may represent a valuable model to study the mechanism of the hearing loss in human patients carrying a homozygous deletion of the CX30 gene (del Castillo et al., 2002, New Engl. J. Med., 346, 243-249).

Four adult bilateral cochlear implant users, with good open-set sentence recognition, were tested with three different sound coding strategies for binaural speech unmasking and their ability to localize 100 and 500 Hz click trains in noise. Two of the strategies tested were envelope-based strategies that are clinically widely used. The third was a research strategy that additionally preserved fine-timing cues at low frequencies. Speech reception thresholds were determined in diotic noise for diotic and interaurally time-delayed speech using direct audio input to a bilateral research processor. Localization in noise was assessed in the free field. Overall results, for both speech and localization tests, were similar with all three strategies. None provided a binaural speech unmasking advantage due to the application of 700 micros interaural time delay to the speech signal, and localization results showed similar response patterns across strategies that were well accounted for by the use of broadband interaural level cues. The data from both experiments combined indicate that, in contrast to normal hearing, timing cues available from natural head-width delays do not offer binaural advantages with present methods of electrical stimulation, even when fine-timing cues are explicitly coded.