Santarelli et al. reveal that hearing impairments in patients carrying OPA1 missense mutations are the result of disordered synchrony in auditory nerve fibre activity owing to degeneration of terminal dendrites. Cochlear implantation improves speech perception and synchronous activation of auditory pathways in these patients by bypassing the lesion site.

This study evaluated the noise level inside the incubators in a neonatal intensive care unit and identified its sources in order to attempt to reduce it. Although noise is not a proven risk factor as far as the sensory integrity of newborns is concerned, it is certainly an important cause of stress to them and a source of serious and dangerous changes in their behavioral and physiologic states. Noise recorded inside the incubators had two components. The first was background noise from the incubator motors, which varied from 74.2 to 79.9 dB, and was similar to environmental noise. The second source was impulsive events beyond 80 dB. These events were the result of voluntary and involuntary contact with the incubators' Plexiglas surface or to the abrupt opening and closing of their access ports. Considering its decibel levels and frequency, this latter component is undoubtedly an important source of stress to newborns. Moreover, these data reveal the need to train health care personnel on how to reduce such noise by taking more care in the handling of infants.

Exposure to high-intensity noise produced in PICU causes evident behavioural and physiological effects (EMG). This is a field of study that could have important repercussions, given the medium- and long-term effects of repeated noise stimulation.

The measurement of auditory brainstem evoked responses and middle latency evoked responses may improve the evaluation of diabetic neuropathy. Twenty diabetic patients were studied (12 males, 8 females), aged 21 to 63 years with normal hearing, together with 20 age- and sex-matched normal subjects (10 males, 10 females). Auditory brainstem evoked responses were induced by rarefaction clicks of 0.1 ms at a repetition rate of 21.1 CPS and an intensity of 75 dB hearing level. Middle latency evoked responses were induced with clicks of 0.1 ms, a repetition rate of 7.7 CPS and an intensity of 75 dB hearing level. Diagnostic criteria were: a I-V interval latency shift greater than 2SD of the control group for the auditory brainstem evoked response test or the interval difference of wave V greater than 0.2 ms. Middle latency evoked response was diagnostic if the latency of the Pa component was greater than 2SD of normals. Twenty-five per cent of subjects had retrocochlear impairment (absence of I wave) even in the absence of symptoms. The combined technique of auditory brainstem evoked response and middle latency evoked response improved the detection rate of central nervous system dysfunction. Auditory brainstem response is important for detecting desynchronization of the auditory response, whereas middle latency evoked response detects abnormalities in the more rostral regions of the central nervous system. In conclusion, there is a role for auditory brainstem evoked response and middle latency evoked response in the global assessment of diabetic neuropathy.