Auditory neuropathy: a challenge for diagnosis and treatment
F. Declau , A. Boudewyns , J. Van den Ende and P. van de Heyning 1 1 2
Department of Otorhinolaryngology, Head and Neck Surgery, and Communication Disorders; Department of Medical Genetics, University of Antwerp, Antwerp, Belgium
Hearing loss; neuropathy; genetics; electrophysiology; hearing aids; cochlear implants
65 - 79
Auditory neuropathy: a challenge for diagnosis and treatment. In current terminology, auditory neuropathy spectrum disorder (ANSD) is a disease involving the disruption of the temporal coding of acoustic signals in auditory nerve fibres, resulting in the impairment of auditory perceptions that rely on temporal cues. There is debate about almost every aspect of the disorder, including aetiology, lesion sites, and the terminology used to describe it. ANSD is a hetero- geneous disease despite similar audiological findings. The absence of an auditory brainstem response (ABR) and the presence of otoacoustic emissions (OAE) suggest an ANSD profile. However, to determine the exact anatomical site of the disorder, more in-depth audiological and electrophysiological tests must be combined with imaging, genetics and neurologicalexaminations.Greaterdiagnosticspecificityisthereforeneededtoprovidethesepatientswithmoreadequate treatment.
Suppl. 21, 2013

Auditory neuropathy: a challenge for diagnosis and treatment