伴耳鸣听神经病患者的随访研究
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摘要
目的分析伴耳鸣听神经病患者(auditory neuropathy, AN)的耳鸣及听力学特征,总结伴耳鸣AN患者的临床听力学转归情况并分析其耳鸣特征。方法对2001年9月至2015年11月间确诊为AN并伴有耳鸣的19例(37耳)患者,随访期1-14年。对比首诊及复诊的听力学特征,并分析患者的耳鸣特征。检测项目包括:声导抗测试、纯音测听、言语识别率测试(SDS)、听性脑干反应测试(ABR)、畸变产物耳声发射测试(DPOAE)、耳鸣问卷、耳鸣评估。结果伴耳鸣AN患者耳鸣及听力学特点:①纯音测听平均听阈复诊与首诊相比0.5-4kHz平均听阈未见明显变化(P>0.05),首诊为40.45±16.21dB HL,复诊为42.43±17.52 dB HL。低频0.25-1kHz平均听阈复诊较首诊未见明显变化(P>0.05),首诊为44.58±16.35dB HL,复诊为46.88±16.59 dB HL。4、8kHz此2个频率纯音阈值复诊较首诊升高(P<0.05);②镫骨肌反射引出率首诊与复诊均为5.4%;③言语识别率复诊与首诊相比未见明显变化(P>0.05),首诊为39.00±23.76%,复诊为37.20±21.67%。言语识别率与平均听阈之间无显著相关性(P>0.05),与250Hz、500Hz、1000Hz频率处的听阈呈显著相关(P<0.05),与余频率无显著相关性;④首诊患者的ABR有1耳引出反应,阈值异常升高。复诊所有患者均未引出反应;⑤首诊患者的DPOAE均正常或大部分正常引出,复诊全频引出率降低;⑥听力损失程度与耳鸣严重程度无明显相关性;⑦患者耳鸣程度多集中在轻中度,耳鸣频率以中低频为主。结论随着病情发展:①伴耳鸣AN患者4、8kHz频率处纯音阈值升高,DPOAE引出率降低,ABR引出率降低。患者应定期复诊了解听力变化,以便及时采取干预措施;②单侧AN患者可能发展成为双侧AN,单侧AN患者应关注健耳听力发展情况;③患者耳鸣程度多集中在轻中度,对生活影响较小,但感冒、疲劳、环境噪音等会使其耳鸣加重。
Objective To report audiological characteristics of patients with auditory neuropathy(AN) and tinnitus for gaining a better understanding of prognosis of hearing in patients with AN as well as their tinnitus. Methods A total of 19 patients(37 ears) with AN and tinnitus were enrolled, all of whom had subsequent visits during 14 years after the first visit. Acoustic immitance, pure tone audiometry, speech-discrimination scores(SDS), auditory brainstem responses(ABR), distortion product otoacoustic emissions(DPOAE) and tinnitus assessments were performed on these patients.Results There were no significant changes in pure tone average(PTA) over 0.5-4 kHz(40.45±16.21 dB HL vs 42.43±17.52 dB HL, P>0.05), or over 0.25-1 k Hz(44.58±16.35 dB HL vs 46.88±16.59 dB HL, P>0.05), although significant changes were seen at 4 and 8 kHz(P<0.05). The rate of positive acoustic reflex(AR) was 5.4% at both the initial and follow up visits. There were no significant changes in SDS(39.00 ± 23.76% vs 37.20 ± 21.67%, P>0.05). SDS overall showed no correlation with hearing threshold, except for thresholds at 0.25, 0.5 and 1 kHz. One ear showed ABRs at the initial visit, although with severely abnormal morphology and high thresholds. ABRs were absent in all ears at subsequent visits. Patients had normal or near normal DPOAEs at the initial visit, but the rate of positive DPOAEs decreased at the subsequent visit. There were no correlations between severities of hearing loss and tinnitus. Tinnitus in these patients was mostly mild or moderate with low or mid-range pitches. Conclusion Over time, PTA over 0.25-1 kHz,DPOAEs and ABRs will deteriorate in patients with auditory neuropathy and tinnitus, warranting monitoring in these patients at regular intervals for appropriate management and prompt actions. Unilateral AN may developed to involve both ears, and patients with unilateral AN should be instructed to pay attention to their good ears. Tinnitus in these patients is of mostly mild or moderate severity, with little impact on their quality of life. Tinnitus however can get worse if the patient catch a cold, is tired or exposed to noise.
Objective To report audiological characteristics of patients with auditory neuropathy(AN) and tinnitus for gaining a better understanding of prognosis of hearing in patients with AN as well as their tinnitus. Methods A total of 19 patients(37 ears) with AN and tinnitus were enrolled, all of whom had subsequent visits during 14 years after the first visit. Acoustic immitance, pure tone audiometry, speech-discrimination scores(SDS), auditory brainstem responses(ABR), distortion product otoacoustic emissions(DPOAE) and tinnitus assessments were performed on these patients.Results There were no significant changes in pure tone average(PTA) over 0.5-4 kHz(40.45±16.21 dB HL vs 42.43±17.52 dB HL, P>0.05), or over 0.25-1 k Hz(44.58±16.35 dB HL vs 46.88±16.59 dB HL, P>0.05), although significant changes were seen at 4 and 8 kHz(P<0.05). The rate of positive acoustic reflex(AR) was 5.4% at both the initial and follow up visits. There were no significant changes in SDS(39.00 ± 23.76% vs 37.20 ± 21.67%, P>0.05). SDS overall showed no correlation with hearing threshold, except for thresholds at 0.25, 0.5 and 1 kHz. One ear showed ABRs at the initial visit, although with severely abnormal morphology and high thresholds. ABRs were absent in all ears at subsequent visits. Patients had normal or near normal DPOAEs at the initial visit, but the rate of positive DPOAEs decreased at the subsequent visit. There were no correlations between severities of hearing loss and tinnitus. Tinnitus in these patients was mostly mild or moderate with low or mid-range pitches. Conclusion Over time, PTA over 0.25-1 kHz,DPOAEs and ABRs will deteriorate in patients with auditory neuropathy and tinnitus, warranting monitoring in these patients at regular intervals for appropriate management and prompt actions. Unilateral AN may developed to involve both ears, and patients with unilateral AN should be instructed to pay attention to their good ears. Tinnitus in these patients is of mostly mild or moderate severity, with little impact on their quality of life. Tinnitus however can get worse if the patient catch a cold, is tired or exposed to noise.
引文
1 John S,Graham S,Chris B,et al.Guidelines for cochlear microphonic testing.CM Testing Guidance v2.0.September 2011 NEW-BORN HEARING SCREENING ASSESSMENT 10.
2齐悦,兰兰,史伟,等.225例听神经病患者的耳鸣发生特点及临床特征分析[J].中华耳科学杂志,2013,11(1):46-49.Qi Y,Lan L,Shi W,et al.Analysis of clinical features and tinnitus characteristics in 225 patients with auditory neuropathy[J].Chinese Journal of Otology,2013,11(1):46-49.
3 David J.耳鸣及其美国的诊治指导方案[J].中国听力语言康复科学杂志,2005,3(1):58-61.David J.Tinnitus and recommendation for diagnosis and the treatment[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2005,3(1):58-61.
4王洪阳,齐悦,关静,等.听神经病谱系障碍患者的耳鸣特征分析[J].临床耳鼻咽喉头颈外科杂志.2015,29(8):712-715.Wang HY,Qi Y,Guan J,et al.Analysis of characteristics of tinnitus in patients with auditory neuropathy spectrum disorder[J].JClin Otorhinolaryngol Head Neck Surg(China),2015,29(8):712-715.
5 Chandan H S,Prabhu P.Audiological changes over time in adolescents and young adults with auditory neuropathy spectrum disorder.European Archives of Oto-Rhino-Laryngology,2015,272(7):1801-1807.
6谢林怡,兰兰,王大勇,等.听神经病谱系障碍患者听力学特征随访研究[J].中华耳科学杂志,2015(2):234-238.Xie LY,Lan L,Wang DY,et al.The follow-up study on audiological characteristics of auditory neuropathy Spectrum Disorder[J].Chinese Journal of Otology,2015,13(2):234-238.
7 Report of the informal working group on prevention of deafness and hearing impairing program planning WHO.Geneva,1991.WHO/PD H/91.1.
8 With adaptations from report of the first informal consultation on future organization development for the prevention of deafness and hearing impairment,World Health Organization.Geneva,1997,WHO/PD H/97.3.
9 Newman CW,Jacobson GP,Spitzer JB.Development of the tinnitus handicap inventory[J].Arch Otolaryngol Head Neck Surg,1996,122:143-148.
10石秋兰,卜行宽,王俊国,等.耳鸣致残量表中文版的研译与临床应用[J].南京医科大学学报,2007,27(5),476-479.Shi QL,Bu XK,Wang JG,et al.Translation and application of tinnitus handicap inventory in Chinese version[J].Acta Universitatis Medicinalis Nanjing,2007,27(5),476-479.
11王剑.听神经病随访观察及致病基因筛查研究[D].陕西:第四军医大学,2011.Wang J.Auditory neuropathy patients’follow-up study and related pathogenetic genes sequencing[D].Shanxi:The Fourth Military Medical University,2011.
12 Shivashankar N,Satishchandra P,Shashikala HR,et al.Primary auditory neuropathy-an enigma.Acta Neurol Scand,2003,108(2):130-135.
13 Hood L.Auditory neuropathy:what is it and what can we do about it?Hear J,1998,51(8):10-18.
14 Kraus N,Bradlow A R,Cheatham M A,et al.Consequences of neural asynchrony:a case of auditory neuropathy.J Assoc Res Otolaryngol,2000,1(1):33-45.0
15 Rance G,Cone-Wesson B,Wunderlich J,et al.Speech perception and cortical event related potentials in children with auditory neuropathy.Ear Hear,2002,23(3):239-253.
16董瑞娟,王硕,刘冬鑫,等.听神经病谱系障碍患者噪声下言语识别能力研究[J].中华耳科学杂志,2015,13(4):604-607.Dong RJ,Wang S,Liu DX,et al.Speech perception in noise in adults with auditory neuropathy spectrum disorders[J].Chinese Journal of Otology,2015,13(4):604-607.
17 Rance G,Mckay C,Grayden D.Perceptual characterization of children with auditory neuropathy.Ear Hear,2004,25(1):34-46.
18 Rance G,Cone-Wesson B,Wunderlich J,et al.Speech perception and cortical event related potentials in children with auditory neuropathy.Ear Hear,2002,23(3):239-253.
19 Kumar U A,Jayaram M M.Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony.Int J Audiol,2006,45(6):360-366.
20 Narne V K.Temporal processing and speech perception in noise by listeners with auditory neuropathy.PLoS One,2013,8(2):e55995.
21 Joseph A,Nora B,Yehudith R,et al.Multiple auditory steady-state responses in children and adults with normal hearing,sensorineural hearing loss,or auditory neuropathy.Ann Otol Rhinol Laryngol,2006.115(4):268-76.
22 Lorenzi C,Gilbert G,Carn H,et al.Speech perception problems of the hearing impaired reflect inability to use temporal fine structure.Proc Natl Acad Sci U S A,2006,103(49):18866-18869.
23闻雨婷,李东英,冀飞,等.听神经病患者纯音听力和耳声发射表现特征分析[J].听力学及言语疾病杂志,2007,15:98-100.Wen YT,Li DY,Ji F,et al.The contradiction between pure tone threshold and distortion product otoacoustic emission of auditory neuropathy patients[J].Journal of Audiology and Speech Pathology,2007,15:98-100.
24王秋菊,赵亚丽,兰兰,等.新生儿聋病基因筛查实施方案与策略研究[J].中华耳鼻咽喉头颈外科杂志.2007,11:809-813.Wang QJ,Zhao YL,Lan L,et al.Studies of the strategy for newborn gene screening.Chin J Otorhinolaryngol Head Neck Surg.2007,11:809-813.
25 Savastano M.Tinnitus with or without hearing loss:are its characteristics different?[J].Eur Arch Otorhinolaryngol,2008,265:1295-300.
26陈秀兰,秦兆冰.性别、年龄、听力损失程度与耳鸣严重程度的关系探讨[J].听力学及言语疾病杂志.2012,20(1):26-28.Chen XL,Qin ZB.The impact of gender,age and hearing loss on the THI scores of patients with tinnitus[J].Journal of Audiology and Speech Pathology.2012,20(1):26-28.
2齐悦,兰兰,史伟,等.225例听神经病患者的耳鸣发生特点及临床特征分析[J].中华耳科学杂志,2013,11(1):46-49.Qi Y,Lan L,Shi W,et al.Analysis of clinical features and tinnitus characteristics in 225 patients with auditory neuropathy[J].Chinese Journal of Otology,2013,11(1):46-49.
3 David J.耳鸣及其美国的诊治指导方案[J].中国听力语言康复科学杂志,2005,3(1):58-61.David J.Tinnitus and recommendation for diagnosis and the treatment[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2005,3(1):58-61.
4王洪阳,齐悦,关静,等.听神经病谱系障碍患者的耳鸣特征分析[J].临床耳鼻咽喉头颈外科杂志.2015,29(8):712-715.Wang HY,Qi Y,Guan J,et al.Analysis of characteristics of tinnitus in patients with auditory neuropathy spectrum disorder[J].JClin Otorhinolaryngol Head Neck Surg(China),2015,29(8):712-715.
5 Chandan H S,Prabhu P.Audiological changes over time in adolescents and young adults with auditory neuropathy spectrum disorder.European Archives of Oto-Rhino-Laryngology,2015,272(7):1801-1807.
6谢林怡,兰兰,王大勇,等.听神经病谱系障碍患者听力学特征随访研究[J].中华耳科学杂志,2015(2):234-238.Xie LY,Lan L,Wang DY,et al.The follow-up study on audiological characteristics of auditory neuropathy Spectrum Disorder[J].Chinese Journal of Otology,2015,13(2):234-238.
7 Report of the informal working group on prevention of deafness and hearing impairing program planning WHO.Geneva,1991.WHO/PD H/91.1.
8 With adaptations from report of the first informal consultation on future organization development for the prevention of deafness and hearing impairment,World Health Organization.Geneva,1997,WHO/PD H/97.3.
9 Newman CW,Jacobson GP,Spitzer JB.Development of the tinnitus handicap inventory[J].Arch Otolaryngol Head Neck Surg,1996,122:143-148.
10石秋兰,卜行宽,王俊国,等.耳鸣致残量表中文版的研译与临床应用[J].南京医科大学学报,2007,27(5),476-479.Shi QL,Bu XK,Wang JG,et al.Translation and application of tinnitus handicap inventory in Chinese version[J].Acta Universitatis Medicinalis Nanjing,2007,27(5),476-479.
11王剑.听神经病随访观察及致病基因筛查研究[D].陕西:第四军医大学,2011.Wang J.Auditory neuropathy patients’follow-up study and related pathogenetic genes sequencing[D].Shanxi:The Fourth Military Medical University,2011.
12 Shivashankar N,Satishchandra P,Shashikala HR,et al.Primary auditory neuropathy-an enigma.Acta Neurol Scand,2003,108(2):130-135.
13 Hood L.Auditory neuropathy:what is it and what can we do about it?Hear J,1998,51(8):10-18.
14 Kraus N,Bradlow A R,Cheatham M A,et al.Consequences of neural asynchrony:a case of auditory neuropathy.J Assoc Res Otolaryngol,2000,1(1):33-45.0
15 Rance G,Cone-Wesson B,Wunderlich J,et al.Speech perception and cortical event related potentials in children with auditory neuropathy.Ear Hear,2002,23(3):239-253.
16董瑞娟,王硕,刘冬鑫,等.听神经病谱系障碍患者噪声下言语识别能力研究[J].中华耳科学杂志,2015,13(4):604-607.Dong RJ,Wang S,Liu DX,et al.Speech perception in noise in adults with auditory neuropathy spectrum disorders[J].Chinese Journal of Otology,2015,13(4):604-607.
17 Rance G,Mckay C,Grayden D.Perceptual characterization of children with auditory neuropathy.Ear Hear,2004,25(1):34-46.
18 Rance G,Cone-Wesson B,Wunderlich J,et al.Speech perception and cortical event related potentials in children with auditory neuropathy.Ear Hear,2002,23(3):239-253.
19 Kumar U A,Jayaram M M.Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony.Int J Audiol,2006,45(6):360-366.
20 Narne V K.Temporal processing and speech perception in noise by listeners with auditory neuropathy.PLoS One,2013,8(2):e55995.
21 Joseph A,Nora B,Yehudith R,et al.Multiple auditory steady-state responses in children and adults with normal hearing,sensorineural hearing loss,or auditory neuropathy.Ann Otol Rhinol Laryngol,2006.115(4):268-76.
22 Lorenzi C,Gilbert G,Carn H,et al.Speech perception problems of the hearing impaired reflect inability to use temporal fine structure.Proc Natl Acad Sci U S A,2006,103(49):18866-18869.
23闻雨婷,李东英,冀飞,等.听神经病患者纯音听力和耳声发射表现特征分析[J].听力学及言语疾病杂志,2007,15:98-100.Wen YT,Li DY,Ji F,et al.The contradiction between pure tone threshold and distortion product otoacoustic emission of auditory neuropathy patients[J].Journal of Audiology and Speech Pathology,2007,15:98-100.
24王秋菊,赵亚丽,兰兰,等.新生儿聋病基因筛查实施方案与策略研究[J].中华耳鼻咽喉头颈外科杂志.2007,11:809-813.Wang QJ,Zhao YL,Lan L,et al.Studies of the strategy for newborn gene screening.Chin J Otorhinolaryngol Head Neck Surg.2007,11:809-813.
25 Savastano M.Tinnitus with or without hearing loss:are its characteristics different?[J].Eur Arch Otorhinolaryngol,2008,265:1295-300.
26陈秀兰,秦兆冰.性别、年龄、听力损失程度与耳鸣严重程度的关系探讨[J].听力学及言语疾病杂志.2012,20(1):26-28.Chen XL,Qin ZB.The impact of gender,age and hearing loss on the THI scores of patients with tinnitus[J].Journal of Audiology and Speech Pathology.2012,20(1):26-28.