Mechanisms of hearing loss in neurofibromatosis type 2.
Mechanisms of hearing loss in neurofibromatosis type 2.
复制标题
2型神经纤维瘤病中听力丧失的机制。
DOI：
10.1371/journal.pone.0046132
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发表时间：
2012
期刊：
影响因子：
3.7
通讯作者：
Lonser RR
中科院分区：
文献类型：
--
作者：
Asthagiri AR;Vasquez RA;Butman JA;Wu T;Morgan K;Brewer CC;King K;Zalewski C;Kim HJ;Lonser RR
2型神经纤维瘤病（NF 2）患者发生双侧耳蜗前庭神经鞘瘤（CVS），导致大多数人双耳耳聋。听力损失以不可预测的方式发生，其潜在机制尚不清楚。为了深入了解NF 2听力损失的病理生理学基础，我们对NF 2患者未经治疗的耳部进行了前瞻性横断面研究。纳入了前瞻性自然史研究中的100例连续NF 2患者。分析了未接受过治疗耳的临床和听力测定数据。除了标准的MR成像序列，利用高分辨率FLAIR序列确定内淋巴细胞蛋白含量的变化，通过检查3D T2序列确定耳蜗孔阻塞的存在，并通过延迟对比后FLAIR序列确定内淋巴积水。分析了56例连续NF 2患者（年龄30±16岁）的89只耳朵，其中84只未治疗CVS。34耳（38%）有不同程度的听力损失。通过FLAIR MR成像，在70只（75%）耳朵中发现了升高的内胆红素蛋白，并且与听力损失的存在密切相关（32/34只听力损失耳朵; 94%）（Fisher精确检验; P = 0.005）。 在正常耳和听力损失耳中，血清内谷胱甘肽蛋白升高与CVS相关的耳蜗孔阻塞的存在相关（67耳中有64耳蛋白升高; 96%）（Fisher精确检验; P<0.0001）。无CVS的耳（5耳）中未发现血清内谷胱甘肽蛋白升高。虽然较大的肿瘤大小与听力损失相关（P = 0.006），但16只听力损失耳（47%）的CVS小于0.5 cm 3，其中14只耳（88%）伴有耳蜗孔阻塞和蛋白质升高。 这些发现与一个模型一致，在该模型中，听力损失的发展是由于耳蜗孔阻塞和内谷氨酰胺蛋白的积累。基于磁共振成像的内谷胱甘肽蛋白升高的识别可能有助于识别有听力损失风险的耳朵。
Patients with neurofibromatosis type 2 (NF2) develop bilateral cochleovestibular schwannomas (CVSs) that cause binaural deafness in most individuals. Hearing loss occurs in an unpredictable manner and the underlying mechanisms are not known. To gain insight into the pathophysiologic basis for hearing loss in NF2, we performed a prospective cross-sectional study of untreated ears in NF2 patients. One hundred consecutive NF2 patients in a prospective natural history study were included. Clinical and audiometric data were analyzed for treatment naïve ears. In addition to standard MR-imaging sequences, alterations in intralabyrinthine protein content were determined utilizing high resolution FLAIR, the presence of cochlear aperture obstruction was determined by examining 3D T2 sequences, and endolymphatic hydrops was identified on delayed post-contrast FLAIR sequences. Eighty-nine ears harboring 84 untreated CVSs in 56 consecutive NF2 patients (age 30±16 years) were analyzed. Thirty-four (38%) ears had varying degrees of hearing loss. Elevated intralabyrinthine protein was identified in 70 (75%) ears by FLAIR MR-imaging and was strongly associated with the presence of hearing loss (32/34 hearing loss ears; 94%)(Fisher's exact test; P = .005). Elevated intralabyrinthine protein was associated with the presence of CVS-associated cochlear aperture obstruction (64 of 67 ears with elevated protein; 96%)(Fisher's exact test; P<0.0001) in both normal and hearing loss ears. Elevated intralabyrinthine protein was not identified in ears without CVS (5 ears). While larger tumor size was associated with hearing loss (P = 0.006), 16 hearing loss ears (47%) harbored CVSs less than 0.5 cm3, including 14 ears (88%) with block of the cochlear aperture and elevated protein. These findings are consistent with a model in which hearing loss develops as a result of cochlear aperture obstruction and accumulation of intralabyrinthine protein. MRI based identification of elevated intralabyrinthine protein may help identify the ear at-risk for developing hearing loss.
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影响因子：
5
作者：
Melhem, ER;Jara, H;Eustace, S
通讯作者：
Eustace, S
影响因子：
120.7
作者：
Butman, John A.;Kim, H. Jeffrey;Lonser, Russell R.
通讯作者：
Lonser, Russell R.
影响因子：
2.1
作者：
Fisher, Laurel M.;Doherty, Joni K.;Slattery, William H.
通讯作者：
Slattery, William H.
影响因子：
4
作者：
EVANS, DGR;HUSON, SM;HARRIS, R
通讯作者：
HARRIS, R
DOI：
10.1097/mao.0b013e318248ee02
发表时间：
2012-04
期刊：
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
影响因子：
--
作者：
Roosli C;Linthicum FH Jr;Cureoglu S;Merchant SN
通讯作者：
Merchant SN