听神经病患者听力转归与病程的相关性研究
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摘要
目的本研究旨在探索听神经病患者听力转归与病程的相关性,为临床咨询提供理论依据。方法本研究对1995年7月至2017年12月之间连续就诊的听神经病患者进行回顾性分析。纯音听阈变化与病程的相关性由线性回归模型评估并进行曲线拟合。结果听神经病患者性别差异不显著,男性占50.9%(198/389)。起病年龄0-40岁,以3岁以内和青春期前后发病最为常见。92.5%(360/389)为双侧AN。初次测听结果以上升型听力曲线最常见,占50.6%(379/749),其次为不规则型(24.3%),全聋型和平坦型占比接近(分别为10.9%和8.8%),下降型仅为4.8%,尚有0.5%为正常听力。轻度及以下听力损失最多(39.3%),其次为中度听损(32.7%),重度和极重度听损各占13.9%和14.2%。经过随访,患者的听力转归呈现较大差异,56.0%(51/94)出现听力恶化,23.1%(23/94)听力保持稳定,20.9%(20/94)得到改善。进行曲线拟合后发现,病程与AN患者听阈并非简单的直线相关性,转折点出现在病程3-4年时,在此之前AN患者听阈维持相对稳定,在此之后则表现为逐渐变差的整体趋势。结论听神经病患者可以表现为各种不同的听力曲线和听力损失程度,纯音听阈的转归有较大个体差异。随着病程延长,整体趋向于听力逐渐恶化。
Objective This study aimed to study the correlation between pure tone hearing threshold and time duration in patients with auditory neuropathy(AN) to facilitate clinical consultation. Methods Dara from AN patients seen consecutively at the ENT clinic of General Hospital of the People's Liberation Army from July 1995 to December 2017 were retrospectively analyzed. The correlation between pure tone threshold and time duration was assessed by linear regression model and curve fitting. Results Gender differences were not significant in these AN patients, with males accounting for 50.9%(198/389). Ages of symptom onset were between 0-40 years old, mostly affecting children younger than 3 years and adolescents. In 92.5%(360/389) of the patients, hearing was bilaterally affected. The most common audiogram configuration was the ascending type, accounting for 50.6%(379/749), followed by the irregular type(24.3%).The proportion of cophosis and flat type was comparable(10.9% and 8.8%, respectively). The descending type was found in only 4.8% of these patients, while 0.5% showed normal hearing. Mild hearing loss or normal hearing was most common and seen in 39.3% of the patients, followed by moderate hearing loss(32.7%), while severe and profound hearing loss were seen in 13.9% and 14.2% of the patients, respectively. During follow-up, large variability was seen in the change of pure tone hearing. Hearing threshold deteriorated in 56.0%(51/94), remained unchanged in 23.1%(23/94),and even improved in 20.9%(20/94) of the patients. In addition, we found that the correlation between time duration and hearing threshold was not simply linear in AN patients. Curve fitting showed that the turning point was at the third or fourth year. Before that, hearing thresholds remained relatively stable, and then the overall trend was gradually worsening. Conclusion AN Patients show various audiogram types and hearing severities. There is a large individual difference in the change of pure tone threshold over time. As the disease progresses, the overall trend of hearing is gradually deteriorating.
Objective This study aimed to study the correlation between pure tone hearing threshold and time duration in patients with auditory neuropathy(AN) to facilitate clinical consultation. Methods Dara from AN patients seen consecutively at the ENT clinic of General Hospital of the People's Liberation Army from July 1995 to December 2017 were retrospectively analyzed. The correlation between pure tone threshold and time duration was assessed by linear regression model and curve fitting. Results Gender differences were not significant in these AN patients, with males accounting for 50.9%(198/389). Ages of symptom onset were between 0-40 years old, mostly affecting children younger than 3 years and adolescents. In 92.5%(360/389) of the patients, hearing was bilaterally affected. The most common audiogram configuration was the ascending type, accounting for 50.6%(379/749), followed by the irregular type(24.3%).The proportion of cophosis and flat type was comparable(10.9% and 8.8%, respectively). The descending type was found in only 4.8% of these patients, while 0.5% showed normal hearing. Mild hearing loss or normal hearing was most common and seen in 39.3% of the patients, followed by moderate hearing loss(32.7%), while severe and profound hearing loss were seen in 13.9% and 14.2% of the patients, respectively. During follow-up, large variability was seen in the change of pure tone hearing. Hearing threshold deteriorated in 56.0%(51/94), remained unchanged in 23.1%(23/94),and even improved in 20.9%(20/94) of the patients. In addition, we found that the correlation between time duration and hearing threshold was not simply linear in AN patients. Curve fitting showed that the turning point was at the third or fourth year. Before that, hearing thresholds remained relatively stable, and then the overall trend was gradually worsening. Conclusion AN Patients show various audiogram types and hearing severities. There is a large individual difference in the change of pure tone threshold over time. As the disease progresses, the overall trend of hearing is gradually deteriorating.
引文
1 Berlin CI,Hood LJ,Morlet T,et al.Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony(Auditory Neuropathy Spectrum Disorder).Int J Audiol,2010,49(1):30-43
2 Kumar AU,Jayaram M.Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony.Int J Audiol,2006,45(6):360-366
3 Sininger YS,Oba S.Patients with auditory neuropathy:who are they and what they can hear?2001,In:Sininger YS,Starr A(eds)Auditory neuropathy:a new perspective on hearing disorders.Singular-Thomson Learning,Sandiago,pp 15-35
4 Starr A,Sininger YS,Pratt H.The varieties of auditory neuropathy.J Basic Clin Physiol Pharmacol,2000,11(3):215-230
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.Chinese Journal of Otology,2015(2):234-238.
7 Hood L.Auditory neuropathy/auditory dys-synchrony:new insights.Hear J,2002,55(2):10-18
8 Shivashankar N,Satishchandra P,Shashikala HR,et al.Primary auditory neuropathy-an enigma.Acta Neurol Scand,2003,108(2):130-135
9 Hood L.Auditory neuropathy:what is it and what can we do about it?Hear J,1998,51(8):10-18
10 Hood L,Berlin C,Morlet T,et al.Considerations in the clinical evaluation of auditory neuropathy/auditory dys-synchrony.Semin Hear,2002,23(3):201-208
11 Moser T,Starr A.Auditory neuropathy--neural and synaptic mechanisms.Nature Reviews Neurology,2016,12(3):135-149
12 Delmaghani,S.et al.Mutations in the gene encoding pejvakin,a newly identified protein of the afferent auditory pathway,cause DFNB59 auditory neuropathy.Nat.Genet,2006,38:770-778
13 Borck,G.et al.High frequency of autosomal-recessive DFNB59hearing loss in an isolated Arab population in Israel.Clin.Genet,2012,82(3):271-276
14 Ebermann,I.et al.Truncating mutation of the DFNB59 gene causes cochlear hearing impairment and central vestibular dysfunction.Hum.Mutat,2007,28(6):571-577
15 Hashemzadeh Chaleshtori,M.et al.Novel mutations in the pejvakin gene are associated with autosomal recessive non-syndromic hearing loss in Iranian families.Clin.Genet,2007,72(3):261-263
16 Collin,R.W.J.et al.Involvement of DFNB59 mutations in autosomal recessive nonsyndromic hearing impairment.Hum.Mutat,2007,28(7):718-723
2 Kumar AU,Jayaram M.Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony.Int J Audiol,2006,45(6):360-366
3 Sininger YS,Oba S.Patients with auditory neuropathy:who are they and what they can hear?2001,In:Sininger YS,Starr A(eds)Auditory neuropathy:a new perspective on hearing disorders.Singular-Thomson Learning,Sandiago,pp 15-35
4 Starr A,Sininger YS,Pratt H.The varieties of auditory neuropathy.J Basic Clin Physiol Pharmacol,2000,11(3):215-230
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.Chinese Journal of Otology,2015(2):234-238.
7 Hood L.Auditory neuropathy/auditory dys-synchrony:new insights.Hear J,2002,55(2):10-18
8 Shivashankar N,Satishchandra P,Shashikala HR,et al.Primary auditory neuropathy-an enigma.Acta Neurol Scand,2003,108(2):130-135
9 Hood L.Auditory neuropathy:what is it and what can we do about it?Hear J,1998,51(8):10-18
10 Hood L,Berlin C,Morlet T,et al.Considerations in the clinical evaluation of auditory neuropathy/auditory dys-synchrony.Semin Hear,2002,23(3):201-208
11 Moser T,Starr A.Auditory neuropathy--neural and synaptic mechanisms.Nature Reviews Neurology,2016,12(3):135-149
12 Delmaghani,S.et al.Mutations in the gene encoding pejvakin,a newly identified protein of the afferent auditory pathway,cause DFNB59 auditory neuropathy.Nat.Genet,2006,38:770-778
13 Borck,G.et al.High frequency of autosomal-recessive DFNB59hearing loss in an isolated Arab population in Israel.Clin.Genet,2012,82(3):271-276
14 Ebermann,I.et al.Truncating mutation of the DFNB59 gene causes cochlear hearing impairment and central vestibular dysfunction.Hum.Mutat,2007,28(6):571-577
15 Hashemzadeh Chaleshtori,M.et al.Novel mutations in the pejvakin gene are associated with autosomal recessive non-syndromic hearing loss in Iranian families.Clin.Genet,2007,72(3):261-263
16 Collin,R.W.J.et al.Involvement of DFNB59 mutations in autosomal recessive nonsyndromic hearing impairment.Hum.Mutat,2007,28(7):718-723