The Radiological Characteristics of Cochlear Nerve in Patients with Auditory Neuropathy Spectrum Disorder

ZHANG Jiao; ZHANG Qiujing; QI Yue; LAN Lan; YU Lan; LI Na; WANG Dayong; WANG Qiuju

doi:10.3969/j.issn.1672-2922.2015.02.011

Abstract：Objective To identify the radiological characteristics of cochlear nerve in patients with auditory neuropa-thy spectrum disorder(ANSD). Methods We enrolled 19 patients with auditory neuropathy spectrum disorder (ANSD) in this study who had undergone cochlear nerve high-resolution magnetic resonance imaging (HRMRI). All the above subjects were studied by history collection, physical examination and audiological examination, including pure tone audiometry, acoustic impedance, ABR, DPOAE, speech perception testing, et al. This study analyzed the radiological characteristics of cochlear nerve in patients with ANSD, and also analyzed the audiometric features of cochlear nerve deficiency patients with ANSD. Results In this study, the 19 ANSD patients showed a high occurrence of cochlear nerve deficiency (21.05%, 4/19). Within the 4 patients (8 ears)having small cochlear nerves, one patient had movement disorders, one patient had optic nerve atrophy, and two patients had family histories of hearing loss. These 4 patients presented different configuration and degree of hearing loss. Two patients presented flat hearing curve with moderate hearing loss at all frequencies, another one showed low frequency ascending pattern with moderate to severe hearing loss,while and the other displayed high frequency sloping curve with moderate to severe hearing loss. This study also found that patients with cochlear nerve deficiency had bad speech discrimination scores, with 7 out of 8ears showed speech discrimination scores less than 40%. Conclusion HRMRI should be the initial imaging study of choice for children with ANSD to evaluate the radiological characteristics of cochlear nerve, and to analyse the possible site of lesion and one of the etiology of ANSD.