Auditory neuropathy in childhood

• Published in: The Laryngoscope 1998-09, Vol.108 (9), p.1374-1377
• Main Authors: Doyle, Karen Jo, Sininger, Yvonne, Starr, Arnold
• Format: Article
• Language: English
• Subjects: Adolescent; Audiometry, Pure-Tone - methods; Biological and medical sciences; Child; Child, Preschool; Cochlea - physiopathology; Evoked Potentials, Auditory, Brain Stem; Female; Hearing Loss, Sensorineural - diagnosis; Hearing Loss, Sensorineural - etiology; Hearing Loss, Sensorineural - physiopathology; Humans; Investigative techniques, diagnostic techniques (general aspects); Male; Medical sciences; Otorhinolaryngology functional investigation (larynx, voice, audiometry, vestibular function, equilibration...); Retrospective Studies; Severity of Illness Index; Speech Discrimination Tests
• ISSN: 0023-852X; 1531-4995
• DOI: 10.1097/00005537-199809000-00022

Objectives: 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. Study Design: Retrospective review of audiologic findings in eight children with auditory neuropathy. Methods. 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. Results: 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. Conclusions: 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. Key Words: Auditory neuropathy, childhood, hearing loss, auditory brainstem response, evoked otoacoustic emissions.