Auditory dyssynchrony or auditory neuropathy: understanding the pathophysiology and exploring methods of treatment

To review the literature on auditory dyssynchrony (AD) or neuropathy which is characterized by absent auditory brainstem responses, normal otoacoustic emissions, and word discrimination disproportional to the pure tone audiogram.

Several papers attempt to estimate the prevalence of AD that seems to range between 0.23 and 2% in infants "at risk" for hearing impairment and it is considered to be responsible for approximately 8% of newly diagnosed cases of hearing loss in children per year. The prevalence gets even higher, reaching 10% in children with permanent hearing loss. It seems that AD is not a single disease but a spectrum of pathologies that affect the auditory pathway at the level of the inner hair cells, the synapses, or the auditory nerve. Many predisposing or contribution factors have been reported, including prematurity, hyperbilirubinemia, hypoxia, low-birth weight, immunological conditions, infectious diseases, etc. Before proposing any method of management, parents should be informed about the variation among patients and the changes that may appear in some children's audition over time. Sigh language, speech and language therapy, hearing-aids, and cochlear implantation have been used in the treatment of AD. In general, AD is rather refractory to conventional amplification and cochlear implantation has been found an effective method of management in young implanted children with severe/profound deafness and AD. However, the situation is more complicated and challenging in milder hearing losses.

The selected and proposed 30 related articles published in the International Journal of Pediatric Otorhinolaryngology aim to stimulate the related research in order to further explore the etiology, pathophysiology, and management of AD.