title: Auditory perception in auditory neuropathy: clinical similarity with auditory verbal agnosia.
abstract: The precise features of auditory perception in patients with auditory neuropathy have not been well described. In the present study, we examined auditory perception in a patient with auditory neuropathy. The patient was a right-handed 7-year-old boy. His chief complaint was delayed speech and suspected of verbal learning disability. He could talk, read and repeat rather fluently but could not understand fully what was asked. V-IQ, P-IQ and F-IQ of Wechsler Scale for Children III-R were 53, 118 and 81, respectively. Pure tone audiogram was completely normal. His speech discrimination ability was very poor. He could identify environmental sounds with visual matching. He could differentiate intensity difference but not time difference. This phenomenon was reported in patients with hemispheric symptoms. These clinical features are very similar to verbal auditory agnosia. ABR showed no response at 90dBnHL alternating clicks and tone bursts. Click evoked and distortion product otoacoustic emissions (OAE) were normal. Electrocochleogram was also normal. Motor and sensory nerve conduction velocity was completely normal. Pa of MLR and N1 of SVR were present. His diagnosis should be "pure type" of auditory neuropathy or auditory nerve disease. Importance of both ABR and OAE examination should be widely announced and auditory neuropathy must be campaigned stressed to be clinical entity among personnel who take care of children with speech delay.
authors.affiliation: Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry (NCNP), 1-7-3, Kohnodai, Ichikawa, Chiba 272-0827, Japan. kaga@ncnp-k.go.jp
authors.forename: Makiko
authors.initials: M
authors.lastname: Kaga
authors.forename: Kaori
authors.initials: K
authors.lastname: Kon
authors.forename: Akira
authors.initials: A
authors.lastname: Uno
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Horiguchi
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Yoneyama
authors.forename: Masumi
authors.initials: M
authors.lastname: Inagaki
mesh_terms: D000377:Agnosia / Q000175:diagnosis / Q000503:physiopathology / Q000523:psychology; D001307:Auditory Perception; D002648:Child; D003056:Cochlear Nerve; D003937:Diagnosis, Differential; D016057:Evoked Potentials, Auditory, Brain Stem; D006309:Hearing; D006801:Humans; D008297:Male; D009420:Nervous System / Q000503:physiopathology; D009483:Neuropsychological Tests; D017084:Otoacoustic Emissions, Spontaneous; D010469:Perceptual Distortion; D011584:Psychology / Q000379:methods; D013067:Speech Perception; D000160:Vestibulocochlear Nerve Diseases / Q000175:diagnosis / Q000503:physiopathology* / Q000523:psychology*