Deafness in nmf329 mice.

(A) Schematic diagram of a cochlear turn. The scala vestibuli and scala tympani are filled with perilymph (yellow), whereas the scala media is filled with endolymph. The thick black line indicates the tight junction barrier, which insulates the endolymphatic compartment and both sides of the stria vascularis (SV). The organ of Corti contains one row of inner hair cells (light blue), three rows of outer hair cells (dark blue), three rows of Deiters' supporting cells (green), and two rows of pillar cells (P) that form the tunnel of Corti (T). The paracellular spaces in the organ of Corti are filled with basolateral fluid (yellow shading), and this fluid communicates with the perilymph through the basilar membrane (BM). RM indicates the Reissner's membrane. (B) Representative ABR waveforms for nmf329/+ mice and nmf329/nmf329 mice at P28. Broadband click stimuli were applied at the indicated intensities, in dB-SPL. Bold and underlined numbers indicate the auditory thresholds. (C) Mean ABR thresholds (±SEM) to broadband click stimuli and pure-tone sounds in wild-type (+/+), nmf329/+, and nmf329/nmf329 mice at P28. Mice with ABR thresholds higher than the maximum SPL (120 dB or 100 dB, depending on the frequency) were not included in the statistical analysis, and are listed separately (bottom row). The number of mice used for the generation of ABR data is shown in parenthesis.