Sunwoo Woongsang, Lee Won-Wook, Choi Byung Yoon
Department of Otorhinolaryngology-Head & Neck Surgery, Gachon University Gil Medical Center, Incheon, Republic of Korea.
Department of Otorhinolaryngology-Head & Neck Surgery, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.
Int J Pediatr Otorhinolaryngol. 2018 Sep;112:176-181. doi: 10.1016/j.ijporl.2018.07.013. Epub 2018 Jul 10.
To clarify the common radiographic findings of audiologically documented prelingual single-sided deafness (SSD) and identify the prevalence of cochlear nerve deficiency (CND) in SSD infants referred from the newborn hearing screening program.
Between March 2012 and March 2017, the records of all infants referred to our otology clinic after undergoing newborn hearing screening program were retrospectively reviewed. Twenty-four consecutive well infants without risk factors who had a confirmed diagnosis of prelingual SSD under the age of 1 year and who underwent internal auditory canal (IAC) magnetic resonance imaging (MRI) were included. The sizes of cochlear nerve (CN), IAC, and cochlear nerve canal (CNC) were measured on MRI. The presence of CND was visually determined by comparing the CN size to the ipsilateral facial nerve (FN) in the affected side via an oblique sagittal view of IAC MRI and defined when CN was absent or smaller than FN.
CND was seen in all 24 deaf ears (100%) on MRI. There was one with incomplete partition type I, and another with combined cochleovestibular nerve absence. Twenty-four subjects demonstrated either an absent (20/24, 83.3%) or small (4/24, 16.7%) CN. When the absent and small CN groups were compared, the former group had a higher prevalence of narrow CNC and narrow IAC. Of the 20 infants without identifiable CN on the affected side, 17 (85%) had narrow IAC and 17 (85%) had narrow CNC. In the 20 ears with absent CN, only one had both normal-sized IAC and CNC.
The contribution of CND to prelingual SSD in Korean infants reached 100%, according to IAC MRI alone.
明确经听力学证实的语前单侧重度耳聋（SSD）的常见影像学表现，并确定新生儿听力筛查项目转诊的SSD婴儿中蜗神经缺如（CND）的患病率。
回顾性分析2012年3月至2017年3月间所有经新生儿听力筛查项目后转诊至我院耳科门诊的婴儿记录。纳入24例连续的无危险因素的健康婴儿，这些婴儿年龄在1岁以下，确诊为语前SSD，并接受了内耳道（IAC）磁共振成像（MRI）检查。在MRI上测量蜗神经（CN）、IAC和蜗神经管（CNC）的大小。通过IAC MRI的斜矢状位视图，将患侧CN大小与同侧面神经（FN）进行比较，以肉眼确定CND的存在，当CN缺如或小于FN时定义为CND。
MRI显示所有24只患耳（100%）均存在CND。其中1例为不完全分隔I型，另1例为蜗神经和前庭神经联合缺如。24例受试者中，CN缺如者20例（83.3%），CN细小者4例（16.7%）。将CN缺如组和CN细小组进行比较，前者蜗神经管狭窄和IAC狭窄的患病率更高。在患侧未发现可识别CN的20例婴儿中，17例（85%）IAC狭窄，17例（85%）蜗神经管狭窄。在20只CN缺如的耳朵中，只有1只IAC和蜗神经管大小正常。
仅根据IAC MRI，韩国婴儿中CND对语前SSD的影响达100%。
