Clinical relationship between auditory neuropathy and nervous system diseases

Objective: To explore the clinical relationship between auditory neuropathy (AN) and nervous system diseases.

Methods: A total of 134 AN patients who were treated in our hospital from December 2011 to April 2016 were selected. Then 120 cases (240 ears) with complete data of pure tone audiometry and acoustic immittance test were selected as an AN1 group, which was compared with 30 patients (49 ears) with general sensorineural hearing loss (SHL) in regard to the results of pure tone audiometry and acoustic immittance test. On the other hand, 79 cases (158 ears) of the 134 patients with complete data of DP otoacoustic emission test were selected as an AN2 group, which was compared with 30 normal subjects (60 ears) regarding the results of DP otoacoustic emission test.

Results: Increases in the pure-tone hearing threshold by air conduction of AN1 group significantly exceeded those of SHL group at 0.125 and 0.25 kHz (low frequency) (P<0.05). The former group had significantly lower values at 1.0, 2.0 kHz (moderate frequency) and 4.0, 8.0 kHz (high frequency) (P<0.05). Of 134 patients, 14 (19 ears) had evoked V wave upon auditory brainstem response, whereas no waves after I wave were evoked in other tested ears. Distortion product (DP) otoacoustic emissions could all be evoked. AN2 group had significantly higher amplitudes of DP-gram than those of normal control group at 0.5 and 0.7 kHz (low frequency) (P<0.05). Except for three cases of unsteady walking and 10 of dizziness, others did not suffer from typical symptoms of vertigo attack. As to caloric test-induced electronystagmograms, there were 30 bilaterally normal cases (75.0%), one case of left-side semicircular canal paresis (25%) and nine cases of bilateral semicircular canal paresis (22.5%). Four patients with other nervous system diseases were complicated with AN. Other nervous system disorders included three cases of optic nerve atrophy and 7 of lower limb nerve damage.

Conclusion: According to characteristic hearing dysfunction, AN may occur in the afferent pathway of acoustic nerve, probably accompanied by the pathological changes of efferent nerve in the olivocochlear system inside the brainstem.