Deterioration of hearing function in mice with neural crest defect.

Cochlear potentials were recorded in black-eyed white-mutant mice (strains W/Wv and S1/S1d) during the first months of postnatal life. In these animals melanocytes from the neural crest do not reach the stria vascularis. Hearing thresholds indicated by the compound action potential of the auditory nerve progressively increased. Hearing loss in comparison with the control CBA mice amounted 40 dB six weeks after birth, at eight months practically all mutants were deaf. Endocochlear potential was near zero already in the majority of 6 week-old animals. Abnormalities were observed also in intracellular potentials of the stria vascularis cells which were recorded in vitro and dye-marked. While in marginal cells of CBA mice positive potentials of about 10 mV were recorded, potentials of marginal cells in mutants were near zero. Basal cell potentials were negative in CBA mice (approximately -25 mV) and positive in young mutants (approximately +12 mV). However, in mutants older than 3 months the intracellular potentials of basal cells changed to negativity (approximately -10 mV). The results demonstrate gradual deterioration of hearing function observed in both W/Wv and S1/S1d mutants during first months of the postnatal life. In addition to the dysfunction of stria vascularis, where intermediate cells are missing, also the function of receptors progressively deteriorates.