Abstract
Auditory neuropathy spectrum disorder (ANSD) is characterized by impairment of the auditory nerve associated with preservation of outer hair cell function. ObjectiveTo establish the prevalence of ANSD in subjects with sensorineural hearing loss (SNHL). MethodThis retrospective study was carried out between 2010 and 2012 and included the charts of 2,292 individuals with SNHL. Data from otolaryngological and audiological examinations based on pure-tone and speech audiometry, impedance tests, otoacoustic emissions (OAEs), and brainstem auditory evoked potentials (BAEPs) were collected. Inclusion criteria: presence of OAEs and/or cochlear microphonic (CM); absent or altered BAEPs, and normal MRI scans of the brain. ResultsTwenty-seven (1.2%) of the 2,292 subjects with SNHL had ANSD (37% males; 63% females). Mild SNHL was seen in 29.6% of the individuals with ANSD; 55.5% had moderate SNHL; 7.4% had severe SNHL; and 7.5% had profound SNHL. In terms of age, 14.8% were aged between zero and 20 years, 44.1% were 41 to 60 years old, and 7.4% were above the age of 60. ConclusionANSD was seen in 1.2% of the individuals with SNHL included in this study.
Highlights
auditory neuropathy spectrum disorder (ANSD) was seen in 1.2% of the individuals with sensorineural hearing loss (SNHL) included in this study
The term auditory neuropathy has been used to describe diseases affecting children and adults characterized by normal outer hair cell function and anomalous or absent auditory nerve function; it has been described as a set of auditory disorders that combine otoacoustic emissions (OAEs) and/or cochlear microphonics (CM) with absent or desynchronous waves generated in brainstem auditory evoked potentials (BAEPs)
It was realized that the term auditory neuropathy describes an array of disorders that may range from auditory dyssynchrony to auditory nerve neuropathy[3]
Summary
The term auditory neuropathy has been used to describe diseases affecting children and adults characterized by normal outer hair cell function and anomalous or absent auditory nerve function; it has been described as a set of auditory disorders that combine otoacoustic emissions (OAEs) and/or cochlear microphonics (CM) with absent or desynchronous waves generated in brainstem auditory evoked potentials (BAEPs). The change in nomenclature was due to a study carried out in 2002 in which it was revealed that approximately half the children with auditory neuropathy had the same speech detection skills as children with sensorineural hearing loss, in addition to auditory brainstem responses, while the other half had unsatisfactory results in speech detection tests and no auditory brainstem responses. Individuals with auditory neuropathy spectrum disorder (ANSD) may present mild to severe, unilateral or bilateral hearing loss associated with disproportional impairment of speech discrimination in relation to hearing loss[4]. Subjects with ANSD can often hear, but fail to discriminate words due to the loss of neural synchrony between the fibers of the vestibulocochlear nerve, severely impairing temporal auditory processing and speech discrimination[6]
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