A semiclassical theory relating the molecular resonance Rayleigh scattering intensity to the extinction coefficient of an electronic transition is presented. For isotropic molecules, the theory is shown to be equivalent to classical fluctuation theory. The semiclassical theory is then extended to include anisotropic polarizabilities. Depolarized scattering intensities and depolarization ratios are calculated for a model system, and the general features of previous experimental data are explained. Inclusion of inhomogeneous broadening in the theory predicts increased scattering intensities and decreased vibrational resolution of the scattering profile. Depolarized Rayleigh linewidths and intensity profiles are presented for a series of diphenylpolyenes, several coumarin laser dyes, and 3,4-dinitrophenol in its neutral and ionic forms. The ideal molecular properties and experimental conditions for the use of resonance enhanced dynamic Rayleigh scattering are deduced.

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We have used animal models of long term neonatal cochlear hearing loss to study developmental plasticity of the central auditory pathways. Newborn chinchilla pups and feline kittens were treated with the ototoxic drug amikacin, so as to induce basal lesions in the cochlea. At maturity these animals were used in single unit electrophysiological mapping studies, in which the cochleotopic organization of primary auditory cortex (of the cat) and the inferior colliculus of the midbrain (in the chinchilla) were mapped. We have observed, both in the midbrain and auditory cortex, massive reorganization of frequency representation. Most striking were the presence of large monotonic regions (i.e. large areas in which all neurons have similar tuning properties). Cochlear lesions which involve inner hair cells clearly modify the normal development of cochleotopic representation in the midbrain and cortical regions. We suggest that similar abnormal patterns of frequency representation will exist in human subjects with long term neonatal hearing loss.

In the present study, anatomical projections from the medial geniculate body (MGB) to primary auditory cortex (AI) were investigated in normal adult cats and in animals that were neonatally deafened with the ototoxic drug amikacin. Cochleotopic/tonotopic maps in AI (based on neural response characteristic frequency) were obtained with microelectrode recording techniques, and single or multiple injections of retrograde tracers (horseradish peroxidase and fluorescent dyes) were introduced into AI. The AI maps of the amikacin-treated cats had an abnormal cochleotopic organization, such that deprived cortical areas exhibited an expanded representation of intact regions of the damaged cochlea. However, retrograde tracer injections into different regions of AI produced a normal pattern of labeling in the ventral division of the medial geniculate body (MGBv). In both experimental and control animals, the main mass of labeled thalamic cells was found in the MGBv. Different isofrequency contours in AI receive input from different portions of the MGBv. Thus, cell arrays labeled by anterior AI injections were situated medially in MGBv, and injections into posterior AI labeled MGBv more laterally. Furthermore, the deafened cats did not develop a more divergent thalamocortical projection compared with normal control animals, indicating that an abnormal spread of the thalamocortical afferents across the frequency domain in AI (anterior-posterior axis) is not responsible for the altered cochleotopic map in these neonatally deafened animals. The relatively normal thalamocortical projection pattern suggests that, after neonatal cochlear lesions, the major reorganization of cochleotopic maps occurs at subthalamic levels.

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Autosomal dominant sensorineural hearing loss (ADSNHL) is extremely genetically heterogeneous, making it difficult to molecularly diagnose. We identified a multiplex (n ¼ 28 affected) family from the genetic isolate of Newfoundland, Canada with variable SNHL and used a targeted sequencing approach based on population-specific alleles in WFS1, TMPRSS3 and PCDH15; recurrent mutations in GJB2 and GJB6; and frequently mutated exons of KCNQ4, COCH and TECTA. We identified a novel, in-frame deletion (c.806_808delCCT: p.S269del) in the voltage-gated potassium channel KCNQ4 (DFNA2), which in silico modeling predicts to disrupt multimerization of KCNQ4 subunits. Surprisingly, 10/23 deaf relatives are non-carriers of p.S269del. Further molecular characterization of the DFNA2 locus in deletion carriers ruled out the possibility of a pathogenic mutation other than p.S269del at the DFNA2A/B locus and linkage analysis showed significant linkage to DFNA2 (maximum LOD ¼ 3.3). Further support of genetic heterogeneity in family 2071 was revealed by comparisons of audio profiles between p.S269del carriers and non-carriers suggesting additional and as yet unknown etiologies. We discuss the serious implications that genetic heterogeneity, in this case observed within a single family, has on molecular diagnostics and genetic counseling.

This study reviews the hearing results in 80 consecutive patients who underwent complete removal of histologically proven acoustic neuromas by use of the suboccipital approach. Of these, 56 patients had successful monitoring of cochlear compound action potentials; 20 were not monitored because their surgery predated monitoring; and 4 had unsuccessful monitoring. A significant difference was found in hearing preservation rates between the group in whom compound action potential monitoring was performed and those in whom monitoring was either unavailable or failed (p = 0.02). Overall, 38% (30 of 80) had preserved hearing. There were 51 patients in whom the click threshold for the cochlear compound action potential was measured during surgery. Twenty-one patients had a threshold shift of 20 dB or less, 15 (71%) of these retained serviceable hearing (speech reception threshold < or = 50 dB; speech discrimination score > or = 60%). Of 12 patients in whom the threshold shift was 30 to 60 dB, none had serviceable hearing after surgery. The click threshold shift was predictive of a significant postoperative hearing change (p < 0.001).

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