外淋巴瘘——仅只发生于圆窗和卵圆窗吗?

无论是闭合性或开放性头外伤后突然出现耳蜗和前庭症状,很可能是发生了外淋巴瘘,但中耳探查未必能显示病变。此外,某些证实有外淋巴瘘的患者,尽管作了修补术而症状仍无改善,则可能是由于未发现存在于两窗以外的瘘管。作者在1984年收治的682例头外伤患者中,11例(1.4%)有突发性耳蜗和前庭症状,因拟诊为外淋巴瘘而手术,结果10例证实有瘘管:8例瘘管在圆窗或卵圆窗,或两窗都有瘘管,其中2例圆窗瘘者同时有外半规管瘘管;另2例则仅在外半规管有瘘管。以上发现瘘管的病例经修复后眩晕     

圆窗反射与卵圆窗瘘

圆窗和卵圆窗外淋巴瘘是突聋的常见原因,其发生率比以往报道要高得多,如不及时行鼓室探查术,就会失去对圆窗或卵圆窗瘘的及时诊断和治疗时机,使患者发展为永久性耳聋。本文作者报道6例与感染有关的卵圆窗瘘引起圆窗反射消失的病例,并分析了术中所见。 6例病人选自作者观察的11例卵圆窗镫骨     

卵圆窗缺如处理的研讨

１９８３年３月～１９９１年６月为严重传导性耳聋患者施行中耳探查术中发现卵圆窗缺如１０例（１２耳），遂行前庭开窗术和听骨链重建术。对术中发现面神经水平段下垂或移位遮住卵圆窗龛，或前庭开窗术后听力下降再次新窗骨性封闭者行水平半规管开窗术。前庭开窗（１０耳）术后语言区频率平均提高１０～３０ｄＢ以上者７耳，４例手术后３个月听力下降，其中２耳行内耳开窗术；内耳开窗（４耳）术后语言区频率平均提高２０～４５ｄＢ。介绍了手术处理经验。     

卵圆窗缺如处理的研讨

１９８３年３月～１９９１年６月为严重传导性耳聋患者施行中耳探查术中发现卵圆窗缺如１０例（１２耳），遂行前庭开窗术和听骨链重建术，对术中发现面神经水平段下垂或移位遮住卵圆窗龛，或前庭开窗术后听力下降再次新窗骨性封闭行水平半规管开窗术，前庭开窗（１０耳）术后语言区频率平均提高１０～３０ｄＢ以上者７耳，４例手术后３个月听力下降，其中２耳行内耳开窗术，内耳开窗（４耳）术后语言区频率平均提高２０～４５ｄＢ。     

外伤性圆窗及卵圆窗外淋巴瘘

外伤性圆窗和卵圆窗外淋巴瘘乃严重疾患,因它可引起迷路炎和脑膜炎,损伤膜迷路后则可产生混合性或感觉神经性聋。综合文献,导致外淋巴漏的因素可归纳为:(1)圆窗膜或卵圆窗足板韧带先天性薄弱(Althaus,1977);(2)左耳蜗小管较右耳者宽大,因而更易发生外淋巴漏(Goodhill等,1973);(3)一些成年人的蜗小管呈婴儿型,增加了向外爆破途径的外淋巴漏发生(Goodhill,1971);(4)圆窗膜缺乏深龛保护,易发生向内爆破的外淋巴漏(Pullen,1972;     

透明质酸—链霉素圆窗灌注术抗致残性眩晕的效用

为探索一种既消除眩晕又保存听力的治疗致残性眩晕的药物性迷路切除，应用透明质酸－链霉素（ＨＡ－ＳＭ）圆窗灌注术治疗６例梅尼埃病（Ｍｅｎｉｅｒｅ病，ＭＤ）和４例迟发性内淋巴积水（ＤＥＨ）。结果：９例眩晕控制，８例听力不变或提高，７例耳鸣耳闭减轻或消失，前庭功能检测示眼震时间明显缩短（Ｐ〈０．００１）。结论：ＨＡ－ＳＭ圆窗灌注术可选择性次全或完全不前庭功能，消除眩晕，保存听力，前庭功能代偿快，手术安全有     

伴有卵圆窗瘘的耳穿透伤

耳穿透伤常引起外耳道损伤和鼓膜穿孔,发生听骨链损伤和外淋巴瘘的机率很少。报告2例耳穿透伤患者都主诉患耳听力下降,头部运动后出现轻微的眩晕。检查发现鼓膜穿孔,听力学检查提示传导性聋。无自发性眼震,瘘管试验阴性。分别于伤后24和48小时在局麻下行中耳探查术。均发现槌砧关节脱位,砧骨长脚接触面神经管,卵圆窗开放,镫骨深压入前庭。因此认为常规进行的外淋巴瘘试验能使人     

圆窗破裂

本文报告了1975～1979年内有外伤或用力病史而突发感觉神经性聋8例,均经鼓室探查证实为圆窗破裂,其中3例卵圆窗及圆窗均有破裂。窗破裂处分别用耳垂脂肪、筋膜、静脉瓣,有的加用明胶海绵修补。作者们提出对怀疑有圆窗膜破裂的病例可绝对卧床并用镇静剂三、四天,每日测听,当测听结果无改进时则是手术探查之指征。手术探查所见:8例圆窗膜破裂病例中,5例显示有异常浅的,面向外的圆窗龛。1例为潜水员,潜水110英尺深上升时发病,术中见圆窗龛处空虚无膜,轻探证实为广泛开放,鼓阶几乎干涸。因此作者们告诫术中不可在圆窗龛处吸引,因可能造成中阶膜或螺旋器的损伤,可用无耳毒性的棉花小拭子或泡沫海绵轻轻吸净圆窗龛的血和外淋巴液。     

圆窗膜的解剖结构、生理功能及临床意义

圆窗膜是位于中耳与内耳之间的唯一的膜性结构，被称之为第二鼓膜。圆窗膜由三层膜结构组成：外层为中耳粘骨膜延续的上皮细胞，中间层为胶原纤维和弹力纤维，内层为被覆鼓阶的细胞层延续，其在维持正常听力中起着重要的作用。圆窗膜破裂后对听力会产生一定的影响。尽管圆窗膜具有三层结构，其仍具有半透膜性质，临床治疗中利用这一特性而采用鼓室内给药治疗内耳疾病。     

先天性双耳镫骨及卵圆窗缺如的诊断和治疗

目的　一家有 2例 (兄妹 )双耳镫骨及卵圆窗缺如 ,对其诊断和治疗进行研讨。方法　通过病史、听力学检查、高分辨CT扫描及 /或手术探查 ,确定诊断 ,采用改良Lempert水平半规管开窗术治疗 2例各一耳。结果　兄语言频率气导听力提高 47dB ,听阈为 2 5dB ;妹提高 52dB ,听阈为 2 8dB ,分别随访 8年及 1年余 ,听力稳定。结论　双耳先天性镫骨和卵圆窗缺如可能与遗传有关 ,骨导尚好 ,骨导曲线有Carhart切迹是诊断此病的重要指征 ,改良Lempert水平半规管开窗术是治疗此病的安全而有效的方法之一。     

Transcanal endoscopic ear surgery for perilymphatic fistula after electric acoustic stimulation

Transcanal endoscopic ear surgery (TEES) will become a very useful therapeutic option. A perilymphatic fistula (PLF) is defined as sudden sensorineural hearing loss and/or vertigo caused by leakage of the perilymph through a fistula from the oval window and/or round window. We report a case of PLF after electric acoustic stimulation (EAS), a kind of cochlear implant, successfully treated by TEES. A 38-year-old man presented to our hospital with vertigo and hearing loss (HL). His vertigo was induced by Valsalva maneuvers. Eight months ago, he underwent EAS for his right ear for congenital sensorineural HL. Although he maintained his hearing level after EAS, his pure tone audiogram this time showed deterioration of hearing at low frequencies in his right ear. A diagnosis of right PLF was made. After confirming the non-effectiveness of oral prednisolone treatment, PLF repair surgery to patch the oval and round windows by TEES was performed. His vertigo did not recur after the surgery. To the best of our knowledge, this is the first report of PLF repair surgery by TEES without a microscope. The wide-field view of the middle ear by TEES was useful to prevent electrode damage in a PLF patient with a cochlear implant.     

Traumatic perilymphatic fistulas in children: etiology, diagnosis and management

Post-traumatic perilymphatic fistulas have been described following ear and temporal bone injury, particularly in the setting of temporal bone fractures. However, indications for exploratory surgery in cases of trauma without temporal bone fracture are vague and not well described. We describe three children who presented with symptoms suggestive of perilymphatic fistula (PLF) without an associated temporal bone fracture: two with penetrating tympanic membrane injuries and one with blunt temporal bone trauma. All had symptoms of hearing loss and vestibular disturbance. Two of the children cooperated with ear-specific audiologic assessment, which demonstrated sensorineural hearing loss (SNHL) on the traumatized side. The third child showed audiometric evidence of a SNHL on the injured side, but due to his age, the degree of severity of the SNHL was unable to be appropriately addressed prior to the patient being surgically managed. All three children underwent exploratory surgery and were found to have bony defects in the region of the oval window. All were repaired with fascial grafts to the oval and round windows with complete resolution of vestibular symptoms. However, two of the three patients with documented post-operative audiograms suffered from persistent SNHL on the injured side. We conclude that exploratory middle ear surgery is indicated in patients suffering from blunt or penetrating temporal bone or middle ear trauma who demonstrate persistent vestibular symptoms, sensorineural hearing loss or radiographic evidence of oval window pathology. As this is a limited number of patients, a larger series may be warranted to study the actual incidence of post-traumatic PLF in the child with persistent hearing loss and vertigo after head or ear trauma.     

Labyrinthine window rupture with round window predominance: a long-term review of 32 cases

A retrospective series is presented of 32 cases of spontaneous labyrinthine window rupture proven at tympanotomy. There was no prior history of stapedectomy or other middle ear surgery. Seventy-eight per cent of cases involved the round window, in contrast to previous series on the subject. The pattern of hearing loss and vestibular symptoms varied widely and followed different antecedent factors, the commonest of which was head injury (46.9%). Including cases misdiagnosed initially, 92.9% of patients with vestibular symptoms experienced improvement following surgery, but only 20.7% of patients had improved hearing; these figures changed with long-term follow-up, which has been rarely reported previously. Seven patients, the majority oval window ruptures, required re-exploration, mainly for vertigo, and prolonged follow-up is required.     

When to suspect a perilymphatic fistula?

INTRODUCTION: The diagnosis of perilymphatic fistula (PLF) is difficult since no single clinical situation gives the diagnosis for sure. The goal of this study is to clarify the clinical situations where you must suspect a PLF. METHODS: Retrospective study of 20 patients that had an exploratory tympanotomy with a PLF confirmed peroperatively. An analysis of the symptoms, signs and complementary exams was done. The surgical findings and the postoperative evolution were noted. RESULTS: 100% of patients reported a hearing loss, 80% vertigo, 70% a tinnitus and 35% equilibrium problems. Every patient had an etiological event to explain the PLF (trauma 85%), stapedotomy (10%), other ear surgeries. Five patients had a positive fistula or Vasalva test. All patients except one had an hearing loss on the audiogram (sensorineural, mixte or conductive). 50% had a CT scan, 70% of which were abnormal. A VNG was done on 3 patients. The sites of the PLF were as follows: 90% oval window, 5% round window and 5% both windows. The hearing got better or was stabilised in 95% of patients after the operation. 64% saw an improvement of their tinnitus and 87% of their vertigo. CONCLUSION: The diagnosis of PLF is difficult and a high index of suspicion is mandatory. One must look for an etiologic situation to explain the PLF. The audiogram is almost always modified, a mixte hearing loss being common due to the high incidence of ossicular trauma associated with PLF. The clinical clinical situations where you must suspect a PLF were identified as follows: An old trauma, a recent trauma, a history of otologic surgery particularly on the stapes and a preexisting hearing loss that aggravates. A diagnosis scale to evaluate the risk of PLF, based on clinical situations, physical exam and complementary exams was done to help the clinician in the evaluation of PLF.     

Exploratory tympanotomy revisited.

The history of exploratory tympanotomy is somewhat obscure. Methods and findings of exploratory tympanotomy were described and assessed for unexplained conductive and occasional sensorineural hearing losses. Of 316 recent cases positive findings leading to diagnosis and therapy occurred in all 250 cases of conductive hearing losses and in 43 of 63 cases of sensorineural hearing losses. In decreasing order of occurrence findings were sequelae of otitis media, otosclerosis, oval and round window changes including perilymph leakage, congenital and traumatic fixation and disarticulation of ossicles. These observations and indications are discussed.     

Can low frequency sound stimulation during posturography help diagnosing possible perilymphatic fistula in patients with sensorineural hearing loss and/or vertigo?

Perilymphatic fistula (PLF) is often difficult to diagnose because of the similar symptomatology, such as vertigo, tinnitus and hearing loss, which is found in several inner ear diseases. We attempted to correlate a positive result of low frequency sound (LFS) stimulation tests in posturography with the presence or absence of a PLF confirmed by transtympanic endoscopy in 209 patients with various inner ear diseases (Meniere's disease ( n =128), vestibulopathy ( n =41), cochleopathy ( n =28) and sudden deafness ( n =12). LFS provoked unsteadiness in posturography without PLF in 24 patients with Meniere's disease, in 5 patients with vestibulopathy, in 3 patients with cochleopathy and in 2 patients with sudden deafness. In one patient, tympanoscopy revealed fistula in the round window membrane that was covered with a fibrinous layer. In four cases there was abnormal light reflex in the round window but without PLF. In eight cases, Hennebert's sign was present with nystagmus, without PLF. We conclude that pathological responses to the LFS test in posturography can also be encountered in other inner ear diseases without PLF.     

Positional audiometry in the diagnosis of perilymphatic fistula.

Fifty-eight cases with a presumptive clinical diagnosis of perilymphatic fistula (PLF) are described with the results of a positional audiometric test designed to detect the presence of air in the cochlea. All patients underwent tympanotomy and observations of the middle ear are recorded together with the results of treatment. A definite leak was found in 33 cases and none in 25, but grafting of the round and oval window was performed in all but 10 cases. Pure-tone audiometry was performed before and after a 30-minute period of positioning the patient horizontally with the affected ear uppermost. A change in audiometric thresholds was noted in the group where a presumptive diagnosis of PLF was made, including some of those not found to have leaks at operation. However these changes were not observed in the positional tests of a group of 22 patients with hearing losses attributable to other causes. Also, an abnormal air-bone gap was noted in the PLF group compared with the other group. Although the original two-frequency criteria of earlier studies applied to the positional test did not predict the operative findings (leak or no leak), new data on frequency specific changes are presented. It is possible that fistulas at the oval window may be associated with positional threshold change at 500 Hz, and those at the round window with changes at 8 kHz.     

Spontaneous and traumatic perilymph fistulas

Clinicians have been aware of the problem of post-stapedectomy peri-lymph fistulas for some time. The existence of non-surgical oval and round window fistulas has been known and was first described in detail by Fee in 1968. This paper concerns a small series of patients with spontaneous and traumatic perilymph fistulas. Five oval window fistulas and one round window fistula are reported. Clinical features, audiometric, radiographic and vestibular findings are discussed. The etiology of traumatic and spontaneous fistulas is not well understood, but seems to bear a relationship to sudden increases in intracran-ial pressure transmitted to the inner ear through the cochlear aqueduct. Middle ear pressure changes, as seen in acoustic or barotrauma, may also cause these leaks. Indications for surgery and techniques of perilymph fistula identification and repair are discussed in the paper. Surgical correction led to relief of vertigo in 80 percent of patients in this series, and significant hearing improvements were seen in 50 percent of the patients. In evaluating patients with sudden sensori-neural hearing loss, or persistent vestibular symptoms following head or ear trauma, the otologist should keep in mind the possibility of a perilymph fistula and actively investigate these patients. Evidence presented in this paper and in the literature suggests that identification and correction of spontaneous and traumatic perilymph fistulas can lead to resolution of vestibular symptoms and improved hearing in a significant number of patients with these lesions.     

Role of transtympanic endoscopy of the middle ear in the diagnosis of perilymphatic fistula in patients with sensorineural hearing loss or vertigo

BACKGROUND: The diagnosis of perilymphatic fistula (PLF) is often difficult, and therefore the condition can be overlooked. Tympanoscopy presents an alternative procedure for visualising the middle ear anatomy, and it may help to diagnose PLF. AIM: The aim of this study was to evaluate the use of middle ear endoscopy in establishing the diagnosis of PLF and in defining its incidence in patients with sensorineural hearing loss and/or vertigo and tinnitus. SUBJECTS AND METHODS: Two hundred and sixty-five patients (22-80 years of age, mean 48 years) were prospectively and consecutively referred for middle ear examination with tympanoscopy. Tympanoscopy was performed using endoscopes with visual angles of 5 and 25 degrees and an outer diameter of 1.7 mm. The round window niche (with its secondary membrane), the oval window with a stapes superstructure, a part of the facial recess and the area in the fissula ante fenestram were examined and video-recorded. RESULTS: For 1 patient, tympanoscopy revealed fistula in the round window membrane that was covered with a fibrinous layer. In 4 cases abnormal mucosal shining appeared in the round window, but no PLF was present. In 7 cases the tympanic cavity could not be visualised because of the adhesive tympanic membrane, abnormal anatomy or the prominent exostoses of the external ear canal. In 6 cases a postendoscopic middle ear infection was found. No permanent tympanic membrane perforation occurred in any of the patients in this study. CONCLUSIONS: Tympanoscopy is a rapid examination tool with which to verify certain areas of the middle ear anatomy, but it is of limited value for ruling out the presence of PLF.     

The role of explorative tympanotomy in patients with sudden sensorineural hearing loss with and without perilymphatic fistula

Purpose

The purpose of this study was to describe the role of explorative tympanotomy in patients with Profound Sudden Sensorineural Hearing Loss (SSNHL) without clinical evidence of perilymphatic or labyrinthine fistula and to compare intraoperative findings with the postoperative hearing outcome.