Analysis of the Clinical and Audiological Features of the Patients with Auditory Neuropathy Spectrum Disorder
GU Xi
YE Shengnan
ZHANG Rong
LIN Youhui
LIN Shaolian
SHU Bo
Abstract：Objective to investigate and analyze the clinical and audiological features of the patients with auditory neu-ropathy spectrum disorder (ANSD). Methods The clinical data and audiological results of 14 ANSD patients (22 ears) were analyzed. Acoustic immittance, auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) were performed on all the 14 patients. 9 patients were examined by pure tone audiometry while 6 patients were examined by auditory steady-state response (ASSR). 5 patients accepted temporal bone computed tomography scan and 9 patients were test-ed by magnetic resonance imaging of the inner ear. Results There were 8 patients with bilateral ANSD and 6 patients with unilateral ANSD. 7 ears (50%) with ascending type curve, 2 ears (14.29%) with saddle type curve, 1 ear (7.14%) with flat type curve, 1 ear (7.14%) with descending type curve and 3 ears (21.43%) with total deafness were found in the audiograms of 9 pa-tients (14 ears) who were examined by pure tone audiometry. Mean threshold of low frequency (250Hz、500Hz) was higher than those of medium frequency (1kHz、2kHz) and high frequency (4kHz、8kHz). The results of acoustic immittance presented type A tympanogram and absence of acoustic stapedius reflex in all the patients. ABR was not induced in 11 patients (6 unilat-eral ANSD, total 16 ears) and other 3 patients (6 ears) had severe abnormalities in the ABR tests. The DPOAE results were normal in all the 22 ears of ANSD. The ASSR results of 6 patients (9 ears) indicated profound deafness of the tested ears. Conclusion There was obvious individual difference of the clinical manifestation in the patients of ANSD and the reasons are still unclear. For the patients with sensorineural hearing loss who are suspected to be ANSD, we should perform comprehen-sive audiological tests and for the patients with definite diagnosis of ANSD, we should do the regular follow-up.
Keywords：Auditory neuropathy spectrum disorderAuditory brainstem responseDistortion product otoacoustic emis-sionAuditory steady-state response
Publication Date：2015-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 262-266 )