内耳先天性畸形102例临床分析

报道１０２例（２００耳）内耳先天性畸形的临床分析。此类患儿多为双侧重度听力损失或全聋，３／４患儿于一岁以内即被家长发现耳聋，其中４７例（４６％）患儿的母亲有妊娠早期感冒史。采用高分辨内耳ＣＴ检查，其颞骨畸形大致分为五型：①Ｍｉｃｈｅｌ型，１６耳；②Ｍｏｎｄｉｎｉ型，１５５耳；③前庭导水管扩大畸形，１６耳；④耳蜗导水管发育畸形，１２耳；⑤内耳道发育畸形，１耳。值得指出的是多数先天性内耳畸形伴有前庭或前庭导水管畸形。     

内耳先天性畸形102例临床分析

报道１０２例（２００耳）内耳先天性畸形的临床分析。此类患儿多为双侧重度听力损失或全聋，３／４患儿于一岁以内即被家长发现耳聋，其中４７例（４６％）患儿的母亲有妊娠早期感冒史。采用高分辨内耳ＣＴ检查，其颞骨畸形大致分为五型：（１）Ｍｉｃｈｅｌ型，１６耳，（２）Ｍｏｎｄｉｎｉ型，１５５耳；（３）前庭导水管扩大畸形，１６耳，（４）耳蜗导水管发育畸形，１２耳；（５）内耳道发育畸形，１耳。值得指出的是多数先天     

"井喷"在内耳畸形人工耳蜗植入术中的发生率

目的：探讨“井喷”在内耳畸形人工耳蜗植入术中的发生率，为有“井喷”现象的人工耳蜗植入术积累临床经验。方法：对680例行人工耳蜗植入术患者行术前高分辨CT检查，显示80例双耳有内耳畸形，其中20例并发内耳道底骨性缺损与内耳相通，行人工耳蜗植入术。结果：20例（25％）并发内耳道底骨性缺损与内耳相通者，术中发生了“井喷”，其中大前庭导水管畸形者5例，Mondini畸形者11例，耳蜗前庭与内耳道共同腔畸形者4例；耳蜗、前庭、外半规管共同腔和前庭、外半规管共同腔畸形者无“井喷”发生。结论：①80例内耳畸形人工耳蜗植入术患者“井喷”的发生率为25％；②内耳畸形人工耳蜗植入术中“井喷”的发生率依次为：耳蜗、前庭与内耳道共同腔畸形，Mondini畸形，大前庭导水管畸形；③单纯前庭、耳蜗、外半规管共同腔畸形及半规管畸形者，人工耳蜗植入术中未发生“井喷”；④80例内耳畸形人工耳蜗植入术中，发生“井喷”者均存在内耳道底骨缺损，与内耳形成共同开放的通道。     

内耳畸形中脑脊液搏动致镫骨底板瘘

脑脊液（ＣＳＦ）耳漏起因多见于颞骨骨折、慢性破坏性耳部疾病、经乳突颅内手术、先天性内耳畸形。前三者诊断较易，而内耳畸形中脑膜炎常是骨折缺损的首发病症。早在１７９１年Ｃａｒｌｏｍｏｎｄｉｎｉ描述了Ｍｏｎｄｉｎｉ畸形的特征为耳聋，耳蜗仅有１．５转，前庭及前庭导水管膨大。该文报告１例双侧Ｍｏｎｄｉｎｉ伴镫骨畸形致单侧ＣＳＦ耳漏的病例。患者女性，１０个月，出生后二度发生脑膜炎，双中耳腔已置通气管，有耳聋家族史。耳镜检查见左耳通气管内有大量清水样液溢出（液体性质与ＣＳＦ一致），右耳除通气管外余正常。听力测…     

半规管开窗放置链霉素对正常的膜迷路积水耳的内耳功能…

为探索临床应用氨基甙类药物治疗Ｍｅｎｉｅｒｅ病的途径和可行性，对豚鼠正常耳和人工造成膜迷路积水耳行外半规管开窗放置硫酸链霉素后的内耳功能和形态进行观察。结果表明开窗放药后正常耳耳蜗电图（ＥＣｏｃｈＧ）动作电位（ＡＰ）阈值无变化，积水耳ＡＰ阈值升高，正常耳和积水耳冰水试验眼震消失。光镜下正常耳给药后三个半规管壶腹嵴和椭圆囊斑前庭上皮严重受损，耳蜗毛细胞正常；积水耳用药后前庭上皮受损更重，部分变性、萎     

感音神经性聋患者内耳高分辨率CT检查内耳畸形的分类

目的 探讨感音神经性聋患者中通过颞骨高分辨率螺旋CT检查内耳畸形的检出率及分类.方法 回顾性分析近10年来5 398耳感音神经性聋患者的颞骨高分辨率螺旋CT资料,患者年龄2个月~68岁,平均17.8±3.3岁;其中单侧24耳,双侧5 374耳;平均听阈83.90±5.2 dB HL,按听力损失程度分为:轻度170耳,中度1 446耳,重度1 386耳,极重度2 396耳;参照Sennaroglu 2010分类标准分析各类内耳畸形的检出情况.结果 5 398耳感音神经性聋患耳中共检出1 640耳内耳畸形(30.38%,1 640/5 398),其中,耳蜗畸形53.66%(880/1 640),非耳蜗畸形46.34%(760/1 640);880耳耳蜗畸形中,Michel畸形5耳、耳蜗未发育23耳、共同腔畸形6耳、耳蜗发育不全畸形69耳(CH-I 19耳、CH-II 16耳、CH-III 34耳)、耳蜗分隔不全畸形777耳(IP-I 44耳、IP-II 703耳、IP-III 30耳);760耳非耳蜗畸形中,大前庭导水管680耳,单纯前庭、半规管、内听道畸形80耳.与大前庭水管相关畸形共1 383耳(IP-II型 703耳、大前庭水管680耳),占全部内耳畸形的84.33%(1 383/1 640).结论 本组感音神经性聋患者内耳畸形检出率较高,且以大前庭水管相关畸形多见,Sennaroglu2010分类方法有利于各类内耳畸形发病率的流行病学统计.     

Mondini内耳畸形的诊断与治疗

为总结内耳畸形的诊断与治疗特点，报告５例（６耳）Ｍｏｎｄｉｎｉ内耳畸形。４例（５耳）伴发脑脊液耳、鼻漏（其中３例伴发Ｋｌｉｐｐｅｌ－Ｆｅｉｌ综合症）经手术补漏成功，１例伴发耳硬化症。结合病理诊断及手术处理特点重点讨论：①诊断依据除听力检查外，特别强调ＣＴ为确诊提供的影像学资料；②Ｍｏｎｄｉｎｉ内耳畸形所致外淋巴瘘主要在前庭窗或其附近，镫骨底板及圆窗部位；③自发性脑脊液耳、鼻漏处理可采取耳内切口的鼓     

高分辨率CT多平面重组对半规管病变的诊断价值

目的:探讨高分辨率CT(HRCT)多平面重组对半规管病变的诊断价值,提高对半规管病变的认识。方法:对怀疑上半规管裂缺的患者3例(6耳),突发性聋患者30例(60耳),感音神经性聋患者30例(60耳)及同期进行体检无耳部疾患的患者20例(40耳)行HRCT扫描,对原始数据行多平面重组,观察正常耳各半规管的形态并与病变组相比较。结果:各半规管均表现为不同倾斜度的C形,正常耳上半规管全程斜矢状位表现为"∩",前端与前庭相通,后端与后半规管上半部形成总脚并与前庭相通;外半规管全程轴位呈""形,前后脚与前庭相通;后半规管全程斜矢状位表现为""形,下端与前庭相通,上端与上半规管形成总脚与前庭相通。半规管管腔平滑,骨质无缺损。发现上半规管裂1耳;突发性聋组前庭导水管与上半规管相通1耳,与后半规管相通2耳;感音神经性聋上半规管闭塞1耳,外半规管闭塞2耳,外半规管骨质不完整2耳,上半规管粗短2耳、外半规管与前庭融合4耳、后半规管短小2耳。结论:HRCT扫面多平面重组能全程观察各半规管,对诊断半规管病变具有重要价值,值得临床推广。     

耳蜗性耳硬化症3例报告及文献复习

目的：探讨耳蜗性耳硬化症的临床特点。方法：结合文献复习,报告3例2007年3月-2008年10月诊治的经高分辨颞骨CT确认的耳蜗性耳硬化症患者。结果：3例以反复发作性眩晕或（和）平衡障碍为首要主诉,同时有进行性听力下降病史。2例（各1耳）为单纯感音神经性聋,考虑为“纯”耳蜗性耳硬化症,其余为伴不同程度骨导下降的混合性聋。高分辨颞骨CT的特点：耳蜗、前庭、半规管及内耳道等部位可见低密度区,耳蜗区呈现“晕影”征或“双环”征,密度有不均。结论：对不能解释原因的感音神经性聋、存在前庭症状的混合性聋并呈进行性听力下降的慢性病史者应考虑耳蜗性耳硬化症的可能,CT对耳蜗性耳硬化症的临床诊断有重要价值。     

3D-FIESTA序列在先天性内耳畸形中应用

目的评估3D-FIESTA序列在先天性内耳畸形中的应用价值。方法采用3D-FIESTA序列对38例内耳畸形患者进行扫描,并进行多平面重组。结果 38例（62耳）内耳畸形中耳蜗畸形31耳,前庭畸形28耳,半规管畸形32耳,前庭水管及内淋巴囊扩大21耳,内耳道神经发育异常29耳。结论 3D-FIESTA序列可以清晰显示内耳道及内耳迷路的先天发育异常,具有较高的临床应用价值。     

Der erweiterte Ductus und Saccus endolymphaticus

Objective

Large endolymphatic duct and sac (LEDS) syndrome is the most common radiologically detectable form of inner ear malformation (IEM) associated with sensorineural hearing loss (SNHL). Nevertheless, it is relatively unknown in the German-speaking countries.

Methods

Among all patients referred from ear, nose, and throat specialists to the radiology department with clinically suspected IEM between 1994 and 2003, we identified the cases of LEDS. The retrospective study included clinical records, high-resolution computed tomography, and magnetic resonance imaging.

Results

Of 169 patients, 17 (median age 12 years; 12 females) showed LEDS. A total of 28 ears were affected. Ten patients (6%; 15 ears) had isolated LEDS, while seven patients showed additional IEM (4%; 13 ears). The most frequent of these was dysplastic vestibule (13/13 ears), followed by Mondini deformity (10/13 ears) and dilated semicircular canals (7/13 ears). Three of 13 ears revealed severe IEM of the cochlea, vestibule, and semicircular canals. No correlation could be demonstrated between the severity of morphological changes and the degree of SNHL.

Conclusion

LEDS may be an underestimated cause of SNHL. Imaging is necessary for a confident diagnosis.     

Common clinical features of children with enlarged vestibular aqueduct and Mondini dysplasia

OBJECTIVES/HYPOTHESIS: The purpose of the study was to investigate the etiological factors and the audiological data of different types of inner ear malformations, which the authors thought might be helpful in elucidating the inter-relation among malformations and shedding light on pathogenesis. STUDY DESIGN: Retrospective study from 1998 to 2002 at a tertiary care university hospital. METHODS: One hundred sixty consecutive children with a total of 302 affected ears undergoing high-resolution computed tomography of the temporal bone for sensorineural hearing loss were enrolled. The image results were correlated with causes and origins, hearing loss patterns, hearing levels, and audiogram configurations. RESULTS: Inner ear malformation was present in 114 (38%) ears. The most common malformations were enlarged vestibular aqueduct, incomplete partition of cochlea (Mondini dysplasia), large vestibule, and semicircular canal dysplasia, presenting either as isolated finding or in combination. Eighty-four (74%) ears had abnormalities confined to these four malformations; only 30 (26%) ears showed other malformations. Patients with complex of enlarged vestibular aqueduct, Mondini dysplasia, large vestibule, and semicircular canal dysplasia (EMVS complex) demonstrated a significantly higher incidence of fluctuating hearing loss (93%) and a better hearing level compared with those with other malformations. Homogeneity in audiological features among these four malformations was also disclosed. CONCLUSION: The authors identified a distinct clinical entity, the EMVS complex, which is characterized by fluctuating hearing loss and a better hearing level. The authors proposed that malformations belonging to this complex result from a common pathogenetic mechanism.     

A new classification for cochleovestibular malformations

OBJECTIVE: The report proposes a new classification system for inner ear malformations, based on radiological features of inner ear malformations reviewed in 23 patients. STUDY DESIGN: The investigation took the form of a retrospective review of computerized tomography findings relating to the temporal bone in 23 patients (13 male and 10 female patients) with inner ear malformations. The subjects were patients with profound bilateral sensorineural hearing loss who had all had high-resolution computed tomography (CT) with contiguous 1-mm-thick images obtained through the petrous bone in axial sections. METHODS: The CT results were reviewed for malformations of bony otic capsule under the following subgroups: cochlear, vestibular, semicircular canal, internal auditory canal (IAC), and vestibular and cochlear aqueduct malformations. Cochlear malformations were classified as Michel deformity, common cavity deformity, cochlear aplasia, hypoplastic cochlea, incomplete partition types I (IP-I) and II (IP-II) (Mondini deformity). Incomplete partition type I (cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance, and there is an accompanying large cystic vestibule. In IP-II (the Mondini deformity), there is a cochlea consisting of 1.5 turns (in which the middle and apical turns coalesce to form a cystic apex) accompanied by a dilated vestibule and enlarged vestibular aqueduct. RESULTS: Four patients demonstrated anomalies involving only one inner ear component. All the remaining patients had diseases or conditions affecting more than one inner ear component. Eight ears had IP-I, and 10 patients had IP-II. Ears with IP-I had large cystic vestibules, whereas the amount of dilation was minimal in patients with IP-II. The majority of the semicircular canals (67%) were normal. Semicircular canal aplasia accompanied cases of Michel deformity, cochlear hypoplasia, and common cavity. In 14 ears, the IAC had a defective fundus at the lateral end. In two ears the IAC was absent. In all seven cases of common cavity malformations, there was a bony defect at the lateral end of the IAC. In five of them the IAC was enlarged, whereas in two the IAC was narrow. All patients with IP-I had an enlarged IAC, whereas in patients with type II disease, four had a normal IAC and 10 had an enlarged IAC. All cases of IP-II had an enlarged vestibular aqueduct, whereas this finding was not present in any of the cases of IP-I. In all cases, the vestibular aqueduct findings were symmetrical on both sides (simultaneously normal or enlarged). No patient demonstrated enlargement or any other abnormalities involving the cochlear aqueduct. CONCLUSIONS: Radiological findings of congenital malformations in the present study suggested two different types of incomplete partition. Cystic cochleovestibular malformation (IP-I) and the classic Mondini deformity (IP-II). The type I malformation is less differentiated than the type II malformation. Classic Mondini deformity has three components (a cystic apex, dilated vestibule, and large vestibular aqueduct), whereas type I malformation has an empty, cystic cochlea and vestibule without an enlarged vestibular aqueduct. Mondini deformity represents a later malformation, so the amount of dysplasia is much less than in type II. Therefore, it is more accurate and useful for clinical purposes to classify these malformations (in descending order of severity) as follows: Michel deformity, cochlear aplasia, common cavity, IP-I (cystic cochleovestibular malformation), cochlear hypoplasia, and IP-II (Mondini deformity). Only in this way can these complex malformations be grouped precisely and the results of cochlear implantation compared.     

Cochlear implantation in children with congenital inner ear malformations

OBJECTIVE/HYPOTHESIS: To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. STUDY DESIGN: Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. METHODS: Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed- and open-set testing of speech perception were analyzed. RESULTS: Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited. CONCLUSIONS: Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.     

Congenital facial palsy and ipsilateral deafness: Association with maternal diabetes mellitus

Congenital facial palsy and ipsilateral deafness were found in two children of unrelated insulin-requiring diabetics. Multidirectional tomography showed hypoplasia of the internal auditory canal in each case but the cochlea, vestibule and semicircular canals were radiologically normal. The malformation in these patients must have been determined before 23 weeks of gestational age when ossification of the inner ear is complete. The association of this malformation and maternal diabetes is unlikely to be coincidental.     

The widely patent cochleovestibular communication of Edward Cock is a distinct inner ear malformation: implications for cochlear implantation

OBJECTIVES: In 1838, Edward Cock described the anatomic findings in 4 inner ears with a widely patent communication between the cochlea and the vestibule that is now frequently referred to as the "common cavity deformity" and is often confused with Michel's "otocyst deformity." Little is known about the anatomic characteristics, including the presence of neural elements in this malformation. METHODS: Light microscopy and 2-dimensional and computerized 3-dimensional reconstructions were used to determine the histopathology and spiral ganglion cell counts in 7 temporal bones with a widely patent cochleovestibular communication. RESULTS: In all 7 specimens, the cochlea, vestibule, and semicircular canals were distinguishable and a bony defect resulting in an abnormal communication of perilymphatic space between the cochlea and vestibule was present. The ductus reuniens was abnormally wide in all. The cochlear duct varied from less than 1 turn to up to 2 turns. The mean spiral ganglion cells were estimated as a percentage of age-matched normal controls at 2.3%, 16.5%, and 26.8% when the cochlea was approximately 1, 1(1/2), and 2 turns, respectively (p = .007). The cribrose area consisted of a thin membrane in 2 specimens, and Rosenthal's canal openly communicated with the cerebrospinal fluid space in 3 specimens. The stapes footplate was abnormal in all 7 specimens and consisted of a central defect bridged by a thin membrane in 4 specimens. The facial nerve was dehiscent in 5 specimens (71%) and also followed an anomalous course in 2 specimens (28%). CONCLUSIONS: The widely patent cochleovestibular communication is a distinct inner ear malformation, recognition of which may have important clinical implications. Estimates of spiral ganglion cells can be predicted from the number of cochlear turns. Although cochlear implantation is feasible in patients with this malformation, a higher risk of cerebrospinal fluid gushers, facial nerve injuries, meningitis, and poor performance would be predicted. A better understanding of the anatomy will allow more effective surgical planning and techniques and may have a significant impact in improving outcomes.     

CT of the temporal bone in the study of the inner ear structures in detection of the causes of neurosensory hypoacusis

Computed tomograms of the inner ear structures (n=175) of 150 patients (age 0-75 years) with unaffected temporal bones were studied in detail. It is shown that polyposition CT of the temporal bone is a non-invasive method of visualization of the osseous labyrinth structures: cochlea, vestibule, semicircular canals, aqueducts of the labyrinth and internal acoustic meatus which are reflected on tomograms in 100% cases irrespective of the patient age. Their age-related features are analysed. CT imaging was made in 65 patients with neurosensory hypoacusis of inherited and acquired genesis. The following causes of neurosensory hypoacusis and deafness were revealed: congenital malformation of the labyrinth of Mondini type, common cavity of the labyrinth, cochlear hypoplasia, dysplasia of the vestibule and semicircular canals, a wide aqueduct of the vestibule, stenosis of the internal acoustic meatus, bulboform enlargement of the inner acoustic meatus, neurinoma (schwannoma) of the hearing nerve, Langerhans-cell histiocytosis with affection of the labyrinth capsule, atypical cholesteatoma. The detected changes in the inner ear structures determine further treatment policy.