Cochlear Implant (CI) users typically perform poorly on musical tasks, especially those based on pitch ranking and melody recognition. It was hypothesized that CI users would demonstrate deterioration in performance for a pitch ranking and a melody recognition task presented with iterated rippled noise (IRN) in comparison to pure tones (PT). In Addition, it was hypothesized that normal hearing (NH) listeners would show fewer differences in performance between IRN and PT for these two tasks. In this study, the ability of CI users and NH subjects to rank pitches and to identify melodies created with IRN and PT was assessed in free field in a sound-isolated room. CI subjects scored significantly above chance level with PT stimuli in both tasks. With IRN stimuli their performance was around chance level. NH subjects scored significantly above chance level in both tasks and with all stimuli. NH subjects performed significantly better than CI subjects in both tasks. These results illustrate the difficulties of CI subjects to rank pitches and to identify melodies.

There is considerable interest in whether sensory deficiency is associated with the development of Alzheimers disease (AD). Notably, the relationship between hearing impairment and AD is of high relevance but still poorly understood. In this study, we found early-onset hearing loss in two AD mouse models, 3xTgAD and 3xTgAD/Polβ+/-. The 3xTgAD/Polβ+/- mouse is DNA repair deficient and has more humanized AD features than the 3xTgAD. Both AD mouse models showed increased auditory brainstem response (ABR) thresholds between 16 and 32 kHz at 4 weeks of age, much earlier than any AD cognitive and behavioral changes. The ABR thresholds were significantly higher in 3xTgAD/Polβ+/- mice than in 3xTgAD mice at 16 kHz, and distortion product otoacoustic emission signals were reduced, indicating that DNA damage may be a factor underlying early hearing impairment in AD. Poly ADP-ribosylation and protein expression levels of DNA damage markers increased significantly in the cochlea of the AD mice but not in the adjacent auditory cortex. Phosphoglycerate mutase 2 levels and the number of synaptic ribbons in the presynaptic zones of inner hair cells were decreased in the cochlea of the AD mice. Furthermore, the activity of sirtuin 3 was downregulated in the cochlea of these mice, indicative of impaired mitochondrial function. Taken together, these findings provide new insights into potential mechanisms for hearing dysfunction in AD and suggest that DNA damage in the cochlea might contribute to the development of early hearing loss in AD.