Abstract
Auditory neuropathy is a recently described disorder in which patients demonstrate hearing loss for pure tones, impaired word discrimination out of proportion to pure tone loss, absent or abnormal auditory brainstem responses, and normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. We have identified eight pediatric patients having hearing deficits that are most likely due to a neuropathy of the eighth nerve. In this study, the results of audiologic testing performed with these eight children are described. Retrospective review of audiologic findings in eight children with auditory neuropathy. Each subject was tested with pure tone and speech audiologic testing, auditory brainstem response, and click-evoked otoacoustic emissions. Results of these tests were tabulated and summarized. Pure tone audiologic testing revealed five children with upsloping sensorineural hearing loss, two with high frequency loss, and one with a mild, flat configuration. Six children demonstrated poor word discrimination scores, and the other two had fair to good word discrimination. All eight subjects had normal distortion product and transient otoacoustic emissions. All eight children demonstrated absent or marked abnormalities of brainstem auditory evoked potentials. These findings suggest that while cochlear outer hair cell function is normal, the lesion is located at the eighth nerve. Recent advances in otoacoustic emissions testing permit differentiation of neural deafness from sensory deafness. This paper describes the clinical presentation and audiologic findings in pediatric auditory neuropathy, as well as the recommended management of these patients. Otolaryngologists should be aware of this disorder and implications for its management, which differs from treatment of sensorineural hearing loss.
Highlights
A group of pediatric patients has been identified who have hearing loss and absent or severely abnormal auditory brainstem responses (ABRs), yet have normal cochlear function as measured by otoacoustic emissions ( O m s ) .Starr et al.[1] named this disorder auditory neuropathy and studied the auditory abilities of these patients
Otolaryngologists should be aware of this disorder and implications for its management, which differs from treatment of sensorineural hearing loss
Starr et al believed that a disorder occurring anywhere along the afferent auditory system from the inner hair cells to the eighth nerve cell body could account for the audiometric loss and the loss of auditory evoked potentials
Summary
A group of pediatric patients has been identified who have hearing loss and absent or severely abnormal auditory brainstem responses (ABRs), yet have normal cochlear function as measured by otoacoustic emissions ( O m s ) .Starr et al.[1] named this disorder auditory neuropathy and studied the auditory abilities of these patients. Starr et a1.2 described an 11-year-oldgirl who developed progressive hearing loss from the age of 7 years, and who had such great difficulty understanding speech that she was dependent on lip-reading. Her pure-tone audiogram progressed from mild hearing loss to bilateral, moderately severe neurosensory hearing loss with poor word discrimination scores (16% right and 8% left). Starr et al believed that a disorder occurring anywhere along the afferent auditory system from the inner hair cells to the eighth nerve cell body could account for the audiometric loss and the loss of auditory evoked potentials They thought that cochlear microphonic preservation was due to the presence of normal outer hair cell function in this child
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