caa a22 4500 388085363 CHVBK 20180307125228.0 cr unu---uuuuu 161130e199911 xx s 000 0 eng 10.1017/S0022215100145840 doi S0022215100145840 pii (NATIONALLICENCE)cambridge-10.1017/S0022215100145840 Surviving cochlear function in the presence of auditory nerve agenesis [Elektronische Daten] This case reports electrophysiological evidence for cochlear function in a child with radiological evidence of bilateral auditory nerve agenesis or severe hypoplasia. The diagnosis of auditory nerve agenesis was supported by a bilateral atresia of internal auditory canals on computed tomography (CT) scan and magnetic resonance imaging (MRI) absent auditory brainstem responses and absent behavioural responses to sound. Despite the apparent absence of an auditory nerve or spiral ganglion, electrocochleography revealed surviving cochlear function at 70-80 db HL and an abnormal electrocochleographic waveform. This case demonstrates that cochlear function may develop without afferent, or efferent innervation. It also emphasizes that cochlear function may occur in the presence of profound deafness. Copyright © JLO (1984) Limited 1999 Evoked potentials, auditory nationallicence Deafness nationallicence Acoustic nerve nationallicence Acoustic nerve diseases nationallicence O'Leary S. J. Departments of Otolaryngology, The Royal Victorian Eye and Ear Hospital, East Melbourne 3002, Australia. Gibson W. P. Departments of Otolaryngology, University of Sydney, Sydney, Australia. The Journal of Laryngology & Otology Cambridge University Press 113/11(1999-11), 1008-1010 0022-2151 113:11<1008 1999 113 JLO https://doi.org/10.1017/S0022215100145840 text/html Onlinezugriff via DOI 1 other jats NATIONALLICENCE 856 40 https://doi.org/10.1017/S0022215100145840 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- O'Leary S. J. Departments of Otolaryngology, The Royal Victorian Eye and Ear Hospital, East Melbourne 3002, Australia NATIONALLICENCE 700 1- Gibson W. P. Departments of Otolaryngology, University of Sydney, Sydney, Australia NATIONALLICENCE 773 0- The Journal of Laryngology & Otology Cambridge University Press 113/11(1999-11), 1008-1010 0022-2151 113:11<1008 1999 113 JLO CC0 http://creativecommons.org/publicdomain/zero/1.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-cambridge