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目的:研究单侧听神经病(UAN)的听力学特点,以提高对UAN的认识。方法:回顾性分析11例UAN患者的听力学资料,并与双侧AN及正常组进行对照研究。结果:AN患者340例,其中UAN11例,占AN患者3.2%。左耳5例,右耳6例;男4例,女7例,男女之比为1.00∶1.75;年龄5～35岁,平均20岁;病程平均3.1年。患者均诉单侧听力减退,听不清说话6例,伴耳鸣4耳,无眩晕发作。纯音听力图下降型5耳(45.4%),低频上升型4耳(36.4%),平坦型1耳(9.1%),盆型1耳(9.1%);听力损失极重度聋6耳(54.5%),中重度聋3耳(27.3%),中度聋2耳(18.2%)。纯音听阈各频率均值UAN组明显高于双侧AN组,健侧听力图正常。言语识别率患耳差,健耳正常。声导抗患侧声反射消失,健侧引出,1例双耳同侧及交叉部分频率引出。听性脑干反应(ABR)患耳自波Ⅰ起均未引出(>100dBnHL),健耳正常引出。畸变产物耳声发射(DPOAE)引出双侧均正常或基本正常,同正常对照组相比,DP-gram幅值和SNR值在全频(0.5～8.0kHz)降低(P<0.05),以中、高频显著(P<0.01),而低频较双侧AN组明显降低(P<0.01)。4例UAN健耳的对侧声抑制DP-gram幅值下降,患耳的对侧声抑制效应减弱或消失,甚至在5kHz幅值增加较健耳及正常组显著(P<0.05)。7例行眼震电图(ENG)检查,患耳半规管功能减退及麻痹各1例;5例行前庭诱发性肌源性电位(VEMP)检查,双侧未引出1例,引出4例,其中患耳幅值降低2例。结论:UAN的听力学特点与双侧AN基本相同,但UAN以下降型听力图和极重度聋较多见,DPOAE幅值与双侧AN比较全频降低,健耳对侧声抑制明显,患耳对侧声抑制效应减弱或消失。需注意与一般单侧感音神经性聋相鉴别。DPOAE及ABR对诊断、鉴别诊断具重要意义。
Objective: To study the audiological features of unilateral auditory neuropathy (UAN) to improve the understanding of UAN. Methods: The audiological data of 11 UAN patients were retrospectively analyzed and compared with the bilateral AN and normal controls. Results: AN patients 340 cases, including 11 cases of UAN, accounting for 3.2% of AN patients. Left ear in 5 cases, right ear in 6 cases; 4 males and 7 females, the ratio of male to female was 1.00: 1.75. The patients were 5 to 35 years old with an average of 20 years old. The average duration of disease was 3.1 years. Patients were complained of unilateral hearing loss, can not hear the speech in 6 cases, with tinnitus 4 ears, no vertigo attack. (45.4%), 4 (36.4%) with low frequency, 1 (9.1%) with flat ear, and 9.1% with flat ear (54.5% ), Moderate to severe deafness 3 ears (27.3%), moderate deafness 2 ears (18.2%). Pure tone threshold of the mean UAN group was significantly higher than the bilateral AN group, normal contralateral hearing map. Speech recognition rate of ear problems, healthy ears normal. Acoustic impedance of the affected side of the acoustic reflex disappeared, contralateral lead, a case of both ears and the intersection of cross-part of the frequency of leads. Auditory brainstem response (ABR) of the ear from the wave Ⅰ were not induced (> 100dBnHL), the normal ear leads. Distortion product otoacoustic emissions (DPOAE) were normal or almost normal at both sides. Compared with the normal control group, DP-gram amplitude and SNR decreased at full frequency (0.5 ~ 8.0kHz) (P <0.05) , High frequency significantly (P <0.01), while low frequency than bilateral AN group was significantly lower (P <0.01). The amplitude of contralateral acoustic suppression DP-gram decreased in 4 UAN ears, and contralateral acoustic suppression decreased or disappeared even in the 5 kHz amplitude compared with the normal ear and normal group (P <0.05). 7 cases underwent routine electronystagmography (ENG) examination, 1 case of semicircular canal hypofunction and paralysis were observed in 1 case, 5 cases were examined by vestibular evoked myogenic potential (VEMP), 1 case was not induced on both sides and 4 cases were induced, Decreased ear value in 2 cases. Conclusions: The audiological characteristics of UAN are basically the same as that of bilateral AN, but the descending audiogram and extreme deafness of UAN are more common. The amplitude of DPOAE is lower than that of bilateral AN, Ear contralateral acoustic suppression effect weakened or disappeared. Need to pay attention to the general unilateral sensorineural deafness differential. DPOAE and ABR diagnosis, differential diagnosis of great significance.