Audiometric outcomes following surgery for spontaneous temporal bone cerebrospinal fluid leaks - A single institution study.

PURPOSE: To assess the audiometric outcomes following surgical repair of spontaneous temporal bone cerebrospinal fluid otorrhea and compare different surgical approaches. MATERIALS AND METHODS: Retrospective review of adults (≥18 years old) who underwent repair of spontaneous CSF leak between 2011 and 2022. Audiometric outcomes were compared across the three surgical groups: transmastoid, middle cranial fossa and combined. RESULTS: Thirty-nine patients (40 ears) met the inclusion criteria (71.8 % females; mean age 59.77 +/- 12.4). Forty-two percent underwent transmastoid, 12.5 % middle cranial fossa and 45 % transmastoid-middle cranial fossa. Four patients (10 %) had recurrence, 3 in the transmastoid group and 1 in the combined approach. The mean change in air-bone gap (ABG) for all patients (postoperative-preoperative) was -7.4 (paired t-test, p-value = 0.0003). The postoperative ABG was closed in 28 (70 %) ears (postoperative ABG ≤ 15). The mean change in pure tone average (PTA) for all patients (postoperative-preoperative) was -4.1 (paired t-test, p-value = 0.13). The mean change in word recognition scores (WRS) for all patients (postoperative-preoperative) was -3 (paired t-test, p-value = 0.35). On multivariable analysis (controlling for site and reconstruction material), there was no significant difference in ABG, PTA and WRS change between surgical groups. CONCLUSIONS: Transmastoid, middle cranial fossa and combined approaches are all effective in treatment of spontaneous CSF leaks and all showed mean decrease in post-operative ABG. Transmastoid approach showed the greatest decrease in ABG and PTA (although middle cranial fossa approach shows the greatest decrease, when excluding profound hearing loss in a patient with superior canal dehiscence). Further studies comparing audiometric outcomes are needed.

Brainstem herniation into the internal acoustic canal secondary to hydrocephalus in context of spontaneous cerebrospinal fluid otorrhea: report of a novel entity.

INTRODUCTION AND CLINICAL PRESENTATION: The authors report a case of a 5-year-old boy presenting with vision loss, right-sided hearing loss, and facial paralysis secondary to hydrocephalus causing brainstem herniation into the internal auditory canal (IAC) following cerebrospinal fluid (CSF) otorrhea. MANAGEMENT AND OUTCOME: After placement of a ventriculo-peritoneal shunt (VP shunt), the vision and facial palsy improved whilst hearing loss persisted. Imaging demonstrated partial reduction of the herniated brainstem and resolution of hydrocephalus. To our knowledge, this is the first case reported of brainstem herniation into the internal auditory canal.

[A Case Report of Spontaneous Cerebrospinal Fluid Otorrhea].

Spontaneous cerebrospinal fluid(CSF)otorrhea is less common than CSF leakage caused by trauma, and rarely occurs in adults. We report an adult case of CSF otorrhea. A 71-year-old woman with no traumatic or otologic history was hospitalized due to bacterial meningitis. After hospitalization, CSF leakage started suddenly from the left external ear canal. A high resolution CT scan with intrathecal administration of contrast material revealed CSF leakage in the left ear canal and multiple bone erosions in both the tegmen mastoideum and the posterior fossa aspect of the petrous bone. We performed closure and surgery via the middle fossa approach. We identified a bone defect in the tegmen mastoideum but could not detect any obvious abnormality in the dura mater. We placed both a pericranial flap and a free abdominal fat on the middle base of the skull as sealing materials. There was no recurrence of CSF otorrhea following surgery. In this surgery, the use of a multilayered closure technique is very important to avoid the recurrence of CSF leakage.

Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report.

PURPOSE: Mondini dysplasia is a rare congenital inner ear malformation that presents with abnormal cochlear development with accompanied vestibular dilation and vestibular aqueduct enlargement. This dysfunctional anatomy provides the potential for sensorineural hearing deficits, cerebrospinal fluid leaks, and severe cases of recurrent meningitis. METHODS: We present the case of a child with Mondini dysplasia who presented with unilateral hearing loss and cerebrospinal fluid (CSF) otorrhea that was surgically repaired through a combined middle fossa/transmeatal middle ear approach to alleviate any recurrence of infection and cerebrospinal fluid otorrhea. RESULTS: Postoperatively, the patient remained neurologically stable without any further CSF leakage. CSF cultures revealed a Pseudomonas aeruginosa infection, a rare occurrence within the context of Mondini dysplasia. Retrograde bacterial spread from the external ear canal into the CSF space has been theorized as the possible pathogenesis of the resulting meningitis. The patient was successfully treated with intravenous antibiotics without any neurologic complications. CONCLUSIONS: Although Mondini dysplasia is a rare malformation, the life-threatening sequelae of meningitis that can result from the dysfunctional anatomy makes it a condition that requires elevated clinical vigilance, especially when considering children with hearing loss associated with recurrent meningitis, otorrhea, or rhinorrhea.

Lateral petrosectomy with obliteration cavity for spontaneous cerebrospinal otorrhea in children.

PURPOSE: The most common causative factors of CSF otorrhea in children are injuries and congenital abnormalities of the temporal bone. Spontaneous CSF leak as a consequence of congenital temporal bone defects may result in recurrent meningitis. Diagnosis and management of such an entity are particularly difficult in early childhood. MATERIALS AND METHODS: The aim of this study was to investigate clinical features and to discuss possible methods of treatment of spontaneous CSF otorrhea in children. RESULTS: Severe unilateral sensorineural hearing loss or total deafness was found in children with CSF otorrhea. CT and MRI of the temporal bones revealed dehiscences in the walls of the tympanic cavity and defects of the inner ear, which were confirmed intraoperatively. Lateral petrosectomy and closure of the fistula with muscle tissue and fat obliteration cavity were performed. The children remain free of otorrhea and recurrences of meningitis. CONCLUSION: The diagnosis of spontaneous otorrhea in children is based on the severe unilateral sensorineural hearing loss and presence of CSF in the middle ear cavity. It may be successfully treated by means of lateral petrosectomy with obliteration of the vestibule with muscle tissue and tympanic cavity with fat tissue.

[Clinical diagnosis and treatment analysis of spontaneous cerebrospinal fluid otorrhoea].

Objective:To investigate the clinical features, imaging findings, surgical methods， diagnostic and treatment experience of spontaneous cerebrospinal fluid otorrhoea. Methods:The clinical data of 11 patients with spontaneous cerebrospinal fluid otorrhoea treated surgically at our hospital from May 2018 to May 2023 were retrospectively analyzed. The medical data included medical history, imaging data, leak location, surgical repair method, treatment effect and postoperative follow-up. Results:Among the 11 surgical patients, 4 patients were initially diagnosed with secretory otitis media, 1 was initially diagnosed with purulent otitis media, and 5 patients had a history of meningitis or presented because meningitis as the initial diagnosis. There were 2 cases of cerebrospinal fluid leakage repaired through the ear canal pathway and 9 cases of cerebrospinal fluid leakage repaired through the mastoid pathway. During the operation, leaks were located in the stapes floor plate in 4 cases, sinus meningeal angle in 1 case, posterior cranial fossa combined with middle cranial fossa in 1 case, middle cranial fossa in 4 cases, and labyrinthine segment of the internal auditory canal and facial nerve canal in 1 case. Ten patient was successfully repaired, and another patient developed intracranial hypertension after surgery, with symptoms alleviated by a lateral ventriculoperitoneal shunt. Postoperative follow-up ranged from 6 months to 4 years, and there was no CSF otorrhoea and meningitis recurrence. Conclusion:The incidence of spontaneous cerebrospinal fluid otorrhea is low, the clinical symptoms are atypical, and the rate of delayed diagnosis or missed diagnosis and misdiagnosis is high. Surgery is currently the preferred treatment for spontaneous cerebrospinal fluid otorrhoea, and satisfactory results are usually achieved; During diagnosis and treatment, it is crucial to be vigilant for intracranial hypertension to prevent serious complications and irreversible damage.

Challenges in the Management of Symptomatic Fallopian Canal Meningoceles: A Multicenter Case Series and Literature Review.

OBJECTIVE: To describe the presentations, the diagnosis, our treatment approaches, and the outcomes for 11 patients with fallopian canal meningocele (FCM). STUDY DESIGN MULTICENTER: Retrospective case series. SETTING: Tertiary referral centers. PATIENTS: Patients (N = 11) with radiographically or intraoperatively identified, symptomatic FCM. INTERVENTIONS: Surgical repair of cerebrospinal fluid (CSF) leak and meningocele versus observation. MAIN OUTCOME MEASURES: Presentation (including symptoms, radiographic imaging, and comorbidities), management (including surgical approach, technique for packing, use of lumbar drain), clinical outcomes (control of CSF leak, meningitis, facial nerve function), and revision surgery. RESULTS: Patients presented with spontaneous CSF leak (n = 7), conductive (N = 11) and sensorineural hearing loss (n = 3), nonpositional intermittent vertigo (n = 3), headaches (n = 4), and recurrent meningitis (n = 1). Risk factors in our series included obesity (n = 4), Chiari 1 malformation (n = 1), and head trauma (n = 2). Noncontrast computed tomography of the temporal bone and magnetic resonance imaging were positive for FCM in 10 patients. Eight patients were managed surgically via a transmastoid approach (n = 4), combined transmastoid and middle fossa (N = 3), or middle fossa alone (n = 1); three were managed conservatively with observation. Postoperative complications included worsened facial nerve palsy (n = 1), recurrent meningitis (n = 1), and persistent CSF leak that necessitated revision (n = 1). CONCLUSIONS: Facial nerve meningoceles are rare with variable presentation, often including CSF otorrhea. Management can be challenging and guided by symptomatology and comorbidities. Risk factors for FCM include obesity and head trauma, and Chiari 1 malformation may present with nonspecific otologic symptoms, in some cases, meningitis and facial palsy. Layered surgical repair leads to high rates of success; however, this may be complicated by worsening facial palsy.

[Postoperative complications after surgery for vestibular schwannoma via the MCF approach]. / Postoperative Komplikationen nach transtemporaler Resektion von Akustikusneurinomen.

BACKGROUND: The aim of this study was to analyze complications after surgery for vestibular schwannoma (VS) via the middle cranial fossa (MCF) approach. PATIENTS AND METHODS: 112 consecutive patients have been operated on via MCF approach in our department between October 2005 and March 2011. Postoperative complications have been registered during hospital stay as well as during outpatient follow-up. RESULTS: 25 complications occurred in 20 patients, most common was a CSF leak (12%). In 9 cases leakage stopped after conservative management. 3 patients needed lumbar drainage and 1 patient had to undergo revision-surgery. 6 patients suffered from hemorrhage: 2 cerebellar bleedings, 1 subdural hematoma, 2 hematomas of the scalp and 1 bleeding of the sutured wound. All bleedings except the case with subdural hematoma could be managed conservatively. Furthermore, one case of meningitis and one transient ischemic attack were observed. 3 patients suffered from a deep venous thrombosis which led to a lethal pulmonary embolism in one case. CONCLUSIONS: Surgery for VS via the MCF approach is an established method with satisfying functional results and a low -complication rate. CSF leak is a common complication (12%) that can be managed conservatively in most of the cases. Severe complications that may lead to revision surgery are rare (2%). Persisting sequels of these complications occur in less than 1% of the cases.

[A case report: a repeated cerebrospinal fluid otorrhea].

Cerebrospinal fluid (CSF) otorrhea, leakage of CSF through the ear structures, may occur from a traumatic or operative defect in the skull, tumor, cholesteatoma, or congenital anomalies. A case of repeated CSF otorrhea is uncommon. In this report, we presented a case of a repeated CSF otorrhea which occurred a decade after the first middle ear surgery for chronic otitis media. The first CSF leakage, which might have been due to bone defects in the tegmen at the first middle ear sutgery, was surgically repaired using a transmastoid approach. However, CSF leakage with a meningoencephalocele occurred again 8 years after our first surgery for the CSF and the fistula was repaired using a transmiddle cranial fossa approach. Although 2 years have passed since the surgery, the CSF leakage has not recurred.

[Imaging evaluation of cerebrospinal fluid otorrhea associated with inner ear malformation in children].

Objective: To explore the imaging evaluation of cerebrospinal fluid (CSF) otorrhea associated with inner ear malformation (IEM) in children. Methods: The clinical data of 28 children with CSF otorrhea associated with IEM confirmed by surgical exploration in Beijing Children's Hospital, from Nov, 2016 to Jan, 2021, were analyzed retrospectively,including 16 boys and 12 girls, aged from 8-month to 15-year and 8-month old, with a median age of 4-year old. The shapes of stapes were observed during the exploration surgery, and the imaging features of temporal bone high resolution CT(HRCT) and inner ear MRI pre- and post-operation were analyzed. Results: In 28 children with CSF otorrhea, 89.3%(25/28) had stapes footplates defect during exploration. Preoperative CT showed indirect signs such as IEM, tympanic membrane bulging, soft tissue in the tympanum and mastoid cavity. IEM included four kinds: incomplete partition type I (IP-Ⅰ), common cavity (CC), incomplete partition type Ⅱ (IP-Ⅱ), and cochlear aplasia (CA); 100%(28/28) presented with vestibule dilation; 85.7%(24/28) with a defect in the lamina cribrosa of the internal auditory canal. The direct diagnostic sign of CSF otorrrhea could be seen in 73.9%(17/23) pre-operative MRI: two T2-weighted hyperintense signals between vestibule and middle ear cavity were connected by slightly lower or mixed intense T2-weighted signals, and obvious in the coronal-plane; 100%(23/23) hyperintense T2-weighted signals in the tympanum connected with those in the Eustachian tube.In post-operative CT, the soft tissues in the tympanum and mastoid cavity decreased or disappeared as early as one week. In post-operative MRI, the hyperintense T2-weighted signals of tympanum and mastoid decreased or disappeared in 3 days to 1 month,soft tissues tamponade with moderate intense T2-weighted signal were seen in the vestibule in 1-4 months. Conclusions: IP-Ⅰ, CC, IP-Ⅱ and CA with dilated vestibule can lead to CSF otorrhea. Combined with special medical history, T2-weighted signal of inner ear MRI can provide diagnostic basie for most children with IEM and CSF otorrhea.HRCT and MRI of inner ear can also be used to evaluate the effect of surgery.

[Diagnosis and treatment of frontobasal cerebrospinal fluid fistulas]. / Diagnostik und Therapie von frontobasalen Liquorfisteln.

Independent of its origin a frontobasal cerebrospinal fluid (CSF) fistula is characterized by an open connection between the intracranial space with the outside world (usually the nose and paranasal sinuses). It leads to loss of CSF and is associated with a significant risk of developing meningitis for the patient during further life. Precise knowledge of possible aetiologies, clinical symptoms, current diagnostic options and surgical strategies are essential for the detection and successful management of frontobasal CSF fi fistulas. This article summarizes aetiology and clinical signs of frontobasal CSF fistulas. Further, laboratory tests to analyse fluid samples for cerebrospinal fluid as well as radiological and endoscopic investigation methods aiming to localize a CSF-fistula are presented. Finally, surgical techniques to close a frontobasal CSF fistula are explained.

[Two cases of spontaneous cerebrospinal fluid (CSF) otorrhea with meningitis].

We report on two cases of spontaneous CSF otorrhea, which were considered to have been caused by enlarged arachnoid granulation with bone erosion of the posterior fossa. Both cases visited us complaining of severe headache, due to bacterial meningitis. In the first patient, a 68-year-old male, a high resolution CT scan showed a bony defect in the posterior fossa plate in the right temporal bone, where CSF leakage was confirmed during the operation. In the second patient, a 54-year-old female, a bony defect was located in the posterior fossa in the left temporal bone. In both cases, the bony defects were repaired by occlusion with the pedicled temporal muscles after the meningitis had been treated. CSF otorrhea disappeared after the surgery, and has not recurred during the postoperative observation period of 1 to 3 years.

Treatment of tegmen dehiscence using a middle fossa approach and autologous temporalis fascia graft: Outcomes from a single center.

INTRODUCTION: Although bony defects of the tegmen surface are relatively common, the majority of dehiscences are asymptomatic. For those who experience symptoms, there is a wide spectrum of relatively benign manifestations such as hearing loss and otorrhea to potentially more serious but rare sequelae such as epilepsy and meningitis. Surgical management of tegmen dehiscences (TDs) can help prevent these symptoms. In this manuscript, we present one of the largest reported single team experiences of using a temporal craniotomy with middle cranial fossa approach and temporalis fascia graft in the treatment of tegmen defects. METHODS: We retrospectively reviewed every case of a TD surgically repaired by the same neurosurgeon/otolaryngologist team at Loyola University Medical Center from May 2015 to January 2022. In our chart review, we identified 44 patients with 48 cases of tegmen defect repair. We analyzed patient characteristics, operative details, and postoperative outcomes. RESULTS: 44 patients met inclusion criteria for the presence of TD (mean age 55 years, 55% male, and average body mass index 35.6). 89% of these patients had no clear etiology for the dehiscence. Commonly reported symptoms were hearing loss (89%) and CSF otorrhea (82%). The least reported presenting signs and symptoms were seizures (5%) and meningitis (2%). Most defects were repaired with both temporalis fascial and calvarial bone grafts (63%), while a minority were treated with temporalis fascia only (33%), temporalis fascia with muscle (2%), or fascia lata (2%). Every patient in our sample experienced resolution of CSF otorrhea after tegmen repair and 81% of the sample reported subjective hearing improvements after surgery. 6% of our sample had post-operative infections and 8% of patients underwent repeat unilateral surgery for a surgical complication. CONCLUSION: Craniotomy for middle fossa approach using autologous temporalis fascial grafts is a safe and effective method for the treatment of TD. These procedures should be performed by experienced and multidisciplinary teams.

Spontaneous osteodural defects of the temporal bone: diagnosis and management of 12 cases.

OBJECTIVES: The objectives of the study were to describe the clinical presentation, diagnostic process, surgical treatment, and outcome of patients with spontaneous dural defect of temporal bone and to explore the possible contribution of idiopathic intracranial hypertension. METHODS: Medical records of consecutive patients with spontaneous defects of the temporal bone were reviewed. Clinical presentation, diagnostic process, exploration of benign intracranial hypertension, surgical management, and outcome of patients are presented. RESULTS: Six of the 12 patients presenting with spontaneous cerebrospinal fluid otorrhea were women. Ages ranged from 38 to 76 years. Seven patients presented with meningitis. The location and the extent of the occurring defect were detected by computed tomography in all cases. Radiologic signs of empty sella syndrome, indicator of benign intracranial hypertension, were revealed in 3 cases using magnetic resonance imaging. Six tegmen defects were repaired using a middle fossa approach without recurrence. Four patients received the combined approach. All patients had complete resolution of the cerebrospinal fluid leak, although 2 cases developed adverse effects attributable to surgical procedure. CONCLUSION: The diagnosis of spontaneous cerebrospinal fluid otorrhea requires clinical suspicion in the setting of meningitis and persistent serous otitis media. High-resolution computed tomography can confirm the diagnosis. The authors' findings advocate the multilayered closure technique through a middle fossa approach.

Gorham-Stout disease and cerebrospinal fluid otorrhea.

OBJECTIVE AND IMPORTANCE: Gorham-Stout disease is a rare entity characterized by vascular proliferation causing local destruction of bone tissue. Owing to its low incidence and variable clinical presentation, the diagnosis requires a high degree of awareness by the clinician. CLINICAL PRESENTATION: We present the case of a 2-year-old boy diagnosed of Gorham-Stout syndrome with involvement of the temporal bone and secondary cerebrospinal fluid (CSF) leakage. INTERVENTION: Because of the CSF leakage, the patient required two surgical interventions. The second intervention included mastectomy and placement of a patch and a lumbar drainage device during 50 days, after which the leakage ceased. CONCLUSION: Gorham-Stout disease is a rare condition that can affect the skull base and even present with CSF leakage.

[Adult spontaneous cerebrospinal fluid otorrhea. Report of two cases and review of the literature]. / Otolicuorrea espontánea en el adulto. Presentación de dos casos y revisión de la literatura.

INTRODUCTION: Spontaneous cerebrospinal fluid otorrhea is a relatively rare entity and can be easily missed in adults. Every adult older than 50 years with a negative history of otologic disease who has recurrent serous otitis media should be evaluated for this pathology. Meningitis is the most serious complication, so there is no doubt that the condition needs immediate attention and correction. OBJECTIVE: We present two patients who were diagnosed with spontaneous CSF otorrhea and make a review of what is reported about this topic. CONCLUSION: Surgical repair is mandatory to seal these nontraumatic CSF leaks. There are two main surgical approaches, the middle fossa craniotomy and the transmastoid approach. A multilayered closure technique in which autologous and artificial materials are combined is considered to result in the highest rate of success.

Audiometric Outcomes Following Transmastoid and Middle Cranial Fossa Approaches for Repair of Cerebrospinal Fluid Otorrhea.

OBJECTIVE: Describe audiometric outcomes following transmastoid and middle cranial fossa (MCF) approaches for repair of cerebrospinal fluid (CSF) otorhinorrhea. STUDY DESIGN: Retrospective case series. SETTING: Tertiary skull base referral center. PATIENTS: Adult patients presenting with CSF otorhinorrhea undergoing operative repair between January 2009 and July 2019. INTERVENTION: Transmastoid repair, MCF repair, or a combined approach. MAIN OUTCOME MEASURES: Primary outcome measures included preoperative and postoperative four-frequency pure-tone average (PTA), air-bone gap (ABG) and word recognition score. Secondary outcomes included success of repair, recurrence of CSF leak, and length of stay. RESULTS: Twenty-nine patients underwent 32 operations (mean age 52 yr, 75.9% female). Twenty (62.5%) patients underwent transmastoid repair, while 8 (25%) underwent an MCF approach. Patients had significant postoperative improvement in both PTA (34.8 dB preop vs. 24.5 dB postop, p = 0.003) and ABG (20.2 dB preop vs. 8.6 dB postop, p  = 0.0001). CSF leak recurred in 3 patients (9.4%) over 17-month follow-up. Compared to MCF or combined approaches, transmastoid repair was associated with greater improvement in PTA (15.6 vs. 3.0 dB, p = 0.001) and shorter length of stay (0.3 vs. 1.2 days, p = 0.005). On subset analysis, patients with spontaneous CSF leaks, a single skull base defect, or meningoencephaloceles demonstrated significant audiometric improvements. CONCLUSIONS: The transmastoid approach for repair of CSF otorhinorrhea is effective, safe, and can be done on an outpatient basis. Patients with spontaneous CSF leaks, a single skull base defect, and associated encephaloceles may have better audiometric outcomes.

Fetal Bovine Collagen Grafts for Repair of Tegmen Defects and Encephaloceles Via Middle Cranial Fossa Approach.

To evaluate the use of commercially available allogenic dural graft materials made of fetal bovine collagen, we present an analysis of our case series with use of autologous and allogenic graft materials. Patients who underwent surgical repair of a tegmen tympani defect associated with ipsilateral conductive hearing loss and cerebrospinal fluid (CSF) otorrhea using a middle cranial fossa (MCF) approach from 2004 to 2018 at Loyola University Medical Center were included. Resolution of CSF otorrhea, audiologic outcomes, facial nerve preservation, and surgical complications was analyzed. Thirty-three patients with an average age of 55.3 years (range: 21-78, standard deviation [SD]: 12.9) and body mass index of 34.4 (range: 22-51, SD: 7.4) underwent an MCF repair of a tegmen and dural defect. All patients presented with CSF otorrhea and conductive hearing loss ipsilateral to the defect. Repairs were made with combinations of allograft and autograft in 17 cases, allograft only in 15 cases, and autograft only in 5 cases. Improvement in hearing was noted in 33 cases, and resolution of CSF otorrhea was noted in 36 cases; one patient required repeat surgery which resolved CSF otorrhea. Three patients had minor complications; all these were in the autograft group. The MCF approach coupled with the use of fetal bovine collagen grafts is a safe and viable method to repair tegmen tympani and associated dural defects with salutary outcomes and low morbidity.

[Surgical management of 27 cases with temporal bone cerebrospinal fluid leakage].

Objective:To study the clinical manifestations and surgical treatment of temporal bone cerebrospinal fluid leakage. Methods:The clinical data of twenty seven cases with temporal bone cerebrospinal fluid leakage were analyzed retrospectively. Different surgical procedures were adopted according to the location of the leak and the hearing status of the affected ear.If the leakage location was clear before surgery, direct repair was performed via mastoid path or middle cranial fossa path with or without mastoid abdominal fat packing.For patients with unclear leak, large lesion or intractable CEREBROspinal fluid leakage of temporal bone, subtotal petrosal resection and abdominal fat packing were performed.Subtotal temporal bone resection and abdominal fat packing were performed for patients with cochlear involvement. Results:In this study, cases of temporal bone CSF leakage including 13 cases of inner ear malformation; 5 cases secondary to head trauma or previous surgery,5 cases of idiopathic intracranial hypertension or meningocele, 2 cases of langerhans histiocytosis in the temporal bone and 2 cases of inner ear inflammation. High resolution CT （HRCT） of temporal bone showed bone defect of inner auditory canal with vestibular communication in 13 patients with inner ear malformation.The temporal bone HRCT of the remaining 14 patients showed bone defects in the middle cranial fossa or posterior cranial fossa, while MRI of the temporal bone showed meningeal continuity interruption with cerebrospinal fluid inflow into the temporal bone or meningoencephalocele in 12 patients. The 26 patients were followed up from 6 months to 6 years. 1 patient lost follow-up.Meningitis recurred in only 1 patient with inner ear malformation, and subtotal resection of rock bone plus abdominal fat packing was performed.Postoperative hearing was preserved or improved in 7 patients.None of the patients had serious complications, and only 1 patient developed HB Grade Ⅱ facial paralysis after vestibular obliteration, and the facial paralysis recovered within one week. Conclusion:Temporal bone cerebrospinal fluid leakage is relatively rare. Surgical intervention should be taken as early as possible when conservative treatment is failure. Preoperative HRCT and MRI examination are necessary for the localization of the leakage, and individualized surgical approaches can be adopted according to the location of the leakage and the features of the lesion.