Morlet Thierry, Pazuniak Markian, O'Reilly Robert C, Kandula Vinay, Choudhary Arabinda K
*Auditory Physiology and Psychoacoustics Research Laboratory, Center for Pediatric Auditory Speech Sciences, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware †Department of Psychiatry, Sidney Kimmel Medical College, Albert Einstein Medical Center, Philadelphia, Pennsylvania ‡Department of Otolaryngology §Department of Radiology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
Otol Neurotol. 2017 Mar;38(3):429-440. doi: 10.1097/MAO.0000000000001308.
To investigate the intracranial abnormalities present in children with cochlear nerve deficiency (CND), including abnormalities of other cranial nerves, and to describe their auditory abilities.
The prevalence of CND has increased with the development of high resolution magnetic resonance imaging (MRI). There are varying degrees of CND from true aplasia to hypoplasia. The etiology of CND remains unclear and it may be associated with intracranial abnormalities in some instances. CND needs to be identified as early as possible to ensure prompt and adequate management of hearing loss since hearing aids and cochlear implants may not be an option.
A retrospective chart review of 56 ears of pediatric patients with CND was conducted between August 2006 and November 2014 at a tertiary care pediatric hospital.
27.6% of children had cochlear abnormalities and 48.9.8% had concomitant vestibular anomalies. Five patients had absent or abnormal facial nerves and two patients had aplastic bilateral olfactory nerves. In the 27 ears with an absent nerve that were functionally tested, eight (29.6%) had partial hearing which indicates the presence of an extremely small nerve whose size is below the limits of spatial resolution of the MRI.
MRI is becoming the initial imaging choice for children with sensorineural hearing loss to identify CND and other brain anomalies. Concomitant vestibular and cochlear abnormalities were observed in respectively half and one-third of the pediatric patients with CND. The incidence of vestibular malformation suggests that balance testing should be recommended for most if not all CND patients.
研究耳蜗神经缺如（CND）患儿存在的颅内异常，包括其他颅神经异常，并描述其听觉能力。
随着高分辨率磁共振成像（MRI）技术的发展，CND的患病率有所增加。从真正的发育不全到发育不良，CND存在不同程度。CND的病因尚不清楚，在某些情况下可能与颅内异常有关。由于助听器和人工耳蜗可能不是选择，因此需要尽早识别CND，以确保对听力损失进行及时和充分的管理。
2006年8月至2014年11月期间，在一家三级儿科医院对56例患有CND的儿科患者的耳朵进行了回顾性病历审查。
27.6%的儿童存在耳蜗异常，48.9%存在前庭异常。5例患者面神经缺如或异常，2例患者双侧嗅神经发育不全。在27只神经缺如且经过功能测试的耳朵中，8只（29.6%）有部分听力，这表明存在一条极其细小的神经，其大小低于MRI空间分辨率的极限。
MRI正成为感音神经性听力损失患儿识别CND和其他脑异常的首选影像学检查方法。分别有一半和三分之一的CND患儿观察到前庭和耳蜗异常。前庭畸形的发生率表明，对于大多数（如果不是全部）CND患者，应建议进行平衡测试。
