Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients

Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks are usually accompanied by episodes of recurrent meningitis, hearing loss, or chronic headache. In this article, we report seven new cases of spontaneous CSF leakage. Six of these patients had conductive hearing loss and serous otitis media, and three had recurrent meningitis. Prior to a definitive diagnosis, six patients had received myringotomy tubes, which produced profuse clear otorrhea. Three patients had positive beta-2 transferrin assays. Computed tomography and magnetic resonance imaging confirmed a defect in the temporal bone tegmen. A combined transmastoid and middle fossa surgical approach with a three-layer closure was used to repair the tegmen defect. All patients had a lumbar drain placed prior to surgery. In addition to describing the seven new cases, we review the history of CSF leakage and discuss diagnostic methods, surgical findings, and our recommendations for management.     

Management of Spontaneous Cerebrospinal Fluid Otorrhea

Objectives/Hypothesis: To describe the surgical approaches and materials used to repair spontaneous cerebrospinal fluid (CSF) otorrhea of temporal bone origin. Study Design: Retrospective case review at a tertiary academic medical center. Methods: All patients presenting with spontaneous CSF otorrhea or rhinorrhea over a consecutive 8‐year period were included. Clinic charts and operative reports were reviewed to obtain the clinical presentation, examination findings, diagnostic test results, intraoperative findings, operative technique, and postoperative follow‐up. Surgical approach and materials used for repair were determined by the location of the defect(s) and surgeon preference. Results: Seventeen patients underwent 19 operations for repair of spontaneous CSF otorrhea or rhinorrhea. The mean age was 61 years and the male to female ratio was 5:12. All female patients had a body mass index (BMI) greater than 30 mg/kg². The most common presenting symptom was otorrhea after a myringotomy or placement of a tympanostomy tube. A middle fossa craniotomy was used in 17 approaches. The most common defect sites were located over the tegmen mastoideum and tegmen tympani. Multiple materials were used in most repairs including allogenic bone cement and autologous materials. One patient had persistent otorrhea after a transmastoid approach and required a middle fossa craniotomy to repair a tegmen mastoideum defect. Conclusions: Spontaneous CSF otorrhea is uncommon and often not diagnosed until a myringotomy or tympanostomy tube is placed. The middle fossa craniotomy provides the best exposure for defects involving the middle fossa floor. Both alloplastic and autologous materials are highly successful in repairing the defect(s) responsible for CSF otorrhea. No infections of the alloplastic bone cement occurred in our series.     

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.     

Bilateral spontaneous cerebrospinal fluid otorrhea

We present a rare case of bilateral cerebrospinal fluid (CSF) otorrhea via multiple bony defects in the left tegmen and a single defect with the herniated brain tissue on the right side. Initially, the patient complained of left hearing loss and fullness and was diagnosed with serous otitis media. After myringotomy, the pulsating watery discharge suggested CSF otorrhea. Five months after surgical repair of the left side, right-side CSF leakage occurred. The right side was repaired surgically, and the patient recovered without incident. From our findings and a review of the literature, we postulate that bilateral CSF otorrhea resulted mainly from the thinness of the tegmen because of well-pneumatized mastoid air cells and the weakness of the dura after chronic inflammatory changes. In case of spontaneous CSF otorrhea, the roof of tegmen should be assessed bilaterally with care using radiologic examinations so as not to overlook a subclinical condition on the contralateral side.     

Porencephalic cyst: a review of the literature and management of a rare cause of cerebrospinal fluid otorrhea.

OBJECTIVE: To discuss the first reported case of spontaneous cerebrospinal fluid (CSF) otorrhea caused by a massive CSF-containing porencephalic cyst. STUDY DESIGN: A case report and review of the literature (MEDLINE 1962-2005). SETTING: A tertiary neurotologic referral center. PATIENT: A 65-year-old woman with congenital hemiplegia presented with left-sided spontaneous CSF otorrhea of 4-month duration. An audiogram revealed a mixed hearing loss. High-resolution computed tomography revealed a thinning of the tegmen and epitympanum without an obvious defect. Magnetic resonance imaging revealed a massive porencephalic cyst essentially replacing the entire left cerebral hemisphere. INTERVENTION: A transmastoid approach with three-layered closure was used to successfully repair the sieve-like defects that were discovered in her tegmen. RESULTS: The patient remains free of drainage, and the conductive hearing loss has resolved. CONCLUSION: Spontaneous CSF otorrhea caused by a porencephalic cyst is an uncommon cause of conductive hearing loss that has never been reported before. Only a few cases of traumatic CSF otorrhea/rhinorrhea associated with a porencephalic cyst have been reported. A high level of suspicion, a beta2-transferrin assay, and appropriate radiographic imaging are required for diagnosis in adults without a history of trauma, meningitis, chronic ear disease, or previous ear surgery.     

Diagnosis and management of spontaneous cerebrospinal fluid-middle ear effusion and otorrhea

OBJECTIVES/HYPOTHESIS: Spontaneous leak of cerebrospinal fluid (CSF) into the middle ear can occur in adults without a history of temporal bone trauma or fracture, meningitis, or any obvious cause. Therefore, clues may be lacking that would alert the otolaryngologist that fluid medial to an intact eardrum, or fluid emanating from an eardrum perforation, is likely to be CSF fluid. A review of relevant medical literature reveals that herniation of the arachnoid membrane through a tegmen defect may be congenital, or CSF leak may occur when dynamic factors (i.e., brain pulsations or increases in intracranial pressure) produce a rent in the arachnoid membrane. Because tegmen defects may be multiple rather than single, identifying only one defect may not be sufficient for achieving definitive repair. Data on nine cases of spontaneous CSF leak to the ear in adult patients from four medical centers are presented and analyzed to provide collective information about a disorder that can be difficult to diagnose and manage. STUDY DESIGN: Retrospective review of nine cases of spontaneous CSF middle ear effusion/otorrhea. RESULTS: The majority of patients presented with symptoms of aural fullness and middle ear effusion. Many developed suspicious clear otorrhea only after insertion of a tympanostomy tube. Two patients had multiple defects in the tegmen and dura, and five patients had meningoencephaloceles confirmed intraoperatively. Five patients underwent combined middle cranial fossa/transmastoid repair. Materials used in repair included temporalis fascia, free muscle graft, Oxycel cotton, calvarial bone, pericranium, bone wax, and fibrin glue. CONCLUSIONS: CSF middle ear effusion/otorrhea can develop in adults without a prior history of meningitis or head trauma or any apparent proximate cause. Although presenting symptoms can be subtle, early suspicion and confirmatory imaging aid in establishing the diagnosis. Because surgical repair by way of a mastoid approach alone can be inadequate if there are multiple tegmen defects, a middle fossa approach alone, or in combination with a transmastoid approach, should be considered in most cases.     

Tegmen tympani cerebrospinal fluid leak repair

OBJECTIVE: To describe the transmastoid approach for closing cerebrospinal fluid leaks in the tegmen tympani using autologous materials (temporal muscle fascia and abdominal fat) and heterologous materials (bovine collagen), together with its advantages and disadvantages. MATERIAL AND METHODS: We present the cases of three patients who underwent closure of spontaneous cerebrospinal fluid leaks in the tegmen tympani in which we used the transmastoid approach and autologous and heterologous materials. The three patients underwent canal wall-up mastoidectomy, posterior tympanotomy, removal of the malleus head and incus and, consequently, exposure of the tegmen tympani and the cerebrospinal fluid leak. Closure was performed using temporal muscle fascia, abdominal fat and bovine collagen, fixed in place with fibrin glue. A lumbar shunt was established, as is routine. RESULTS: The cerebrospinal fluid leak was closed in all patients using the technique described, with a minimum follow-up of 14 months. CONCLUSION: The technique presented herein is an excellent option in cases of cerebrospinal fluid leakage in the tegmen tympani; it is easy to perform and results in minimal risk to patients.     

Tegmen tympani cerebrospinal fluid leak epair

Objective To describe the transmastoid approach for closing cerebrospinal fluid leaks in the tegmen tympani using autologous materials (temporal muscle fascia and abdominal fat) and heterologous materials (bovine collagen), together with its advantages and disadvantages.

Material and Methods We present the cases of three patients who underwent closure of spontaneous cerebrospinal fluid leaks in the tegmen tympani in which we used the transmastoid approach and autologous and heterologous materials. The three patients underwent canal wall-up mastoidectomy, posterior tympanotomy, removal of the malleus head and incus and, consequently, exposure of the tegmen tympani and the cerebrospinal fluid leak. Closure was performed using temporal muscle fascia, abdominal fat and bovine collagen, fixed in place with fibrin glue. A lumbar shunt was established, as is routine.

Results The cerebrospinal fluid leak was closed in all patients using the technique described, with a minimum follow-up of 14 months.

Conclusion The technique presented herein is an excellent option in cases of cerebrospinal fluid leakage in the tegmen tympani; it is easy to perform and results in minimal risk to patients.     

Middle cranial fossa approach for the repair of spontaneous cerebrospinal fluid otorrhoea using autologous bone pate

Tegmen plate defects causing cerebrospinal fluid (CSF) leaks and brain hernias have conventionally been repaired using soft tissue grafts by a transmastoid approach. A review of the literature reveals that the results of transmastoid repairs have been less than satisfactory. We present here four patients with spontaneous CSF otorrhoea who had tegmen defects repaired using the middle cranial fossa approach. An autologous bone pate slab mixed with fibrin glue with additional temporalis fascia reinforcement was used for the repairs. All four patients had uneventful postoperative periods and have had no subsequent CSF leak (follow-up range: 1-3 years). We believe that the middle cranial fossa approach is more effective than the transmastoid approach for the repair of CSF leaks and brain hernias through tegmen plate defects. An important additional benefit is hearing preservation. We also recommend the use of bone pate along with soft tissue for the repair in order to achieve a secure seal. Bone pate can effectively seal multiple defects in the tegmen plate without any risk of migration.     

Elimination of Temporal Bone Cerebrospinal Fluid Otorrhea Using Hydroxyapatite Cement

Objective This report introduces a new method to control cerebrospinal fluid (CSF) otorrhea using hydroxyapatite cement (HAC) via a transmastoid approach. This technique eliminates the need for a transmastoid or middle cranial fossa approach with soft tissue repair and prolonged hospitalization caused by lumbar drainage. Study Design Retrospective review. Methods Thirteen cases of transmastoid repairs of CSF otorrhea using HAC from August 1996 to February 1999 were reviewed. Results The CSF leak was controlled in every patient using HAC through a transmastoid approach. The reconstruction involved eight tegmen defects, three posterior fossa dural plate defects, and two congenital inner ear fistula secondary to Mondini malformation. Postoperative wound infection in one patient was the only complication that occurred. The average hospital stay was 48 hours. Follow‐up ranged from 12 to 44 months with no recurrence of CSF otorrhea. Conclusion The successful use of HAC to control CSF otorrhea through a transmastoid approach reduces patient morbidity by obviating the need for middle cranial fossa approaches, donor soft tissue sites, and spinal drainage.     

Spontaneous cerebrospinal fluid otorrhea

Spontaneous cerebrospinal fluid (CSF) otorrhea is rare. We present four new cases and an analysis of the literature. Two distinct subtypes occur. Seventy-two percent of cases are the childhood type with congenital defects of the otic capsule. Meningitis, usually pneumococcal and frequently recurrent, occurs in 92% of these cases. CSF otorrhea follows myringotomy for a presumed serous effusion. The child usually has unilateral and sometimes bilateral absence of cochlear and vestibular function and commonly exhibits a Mondini deformity. CSF usually enters the inner ear through a dural defect in the lateral aspect of the internal auditory canal and exists through the oval window. Treatment should consist of stapedectomy and packing of the vestibule with muscle or subtotal petrosectomy. Twenty-eight percent of cases of spontaneous CSF otorrhea are the adult type characterized by bony dehiscenses, most commonly of the tegmen tympani or tegmen mastoideum and less commonly of the posterior fossa plate. The meningeal defects are either meningoencephaloceles or simply holes in the dura. Therapy should consist of a mastoidectomy in conjunction with a transtemporal supralabyrinthine (middle fossa) approach if a meningoencephalocele of the tegmen is found.     

Spontaneous defects of the lateral cranial base. 1. Clinical aspects,diagnosis and therapy

Background. Spontaneous cerebrospinal fluid otorrhea (SCFO) or rhinorrhea originating from laterobasal defects is uncommon. An immediate closure of the defect is mandatory due to the risk of life-threatening meningitis. The purpose of this paper is to review the literature and to present two additional case reports with emphasis on diagnostic problems and surgical techniques. Patients and methods. Two adults have been treated for SCFO in our clinic. A 62-year-old woman was complaining of pertinent otorrhea after placement of a ventilation tube and conservative treatment of serous otitis for 1 year elsewhere. A 66-year-old male patient had been treated for acute pneumococcal meningitis. He presented with unilateral deafness and vertigo 3 months later. High-Resolution-Computer-Tomography (HRCT) revealed a unilateral defect in the female and a bilateral defect in the male patient. A transmastoidal approach was used to amputate the meningoencephalocele and modified (Mini-Craniotomy) in the second (male) patient. The defect was closed in two layers using allogenic material and fibrin glue. Results. One year after surgery both patients were free of symptoms and defect closure remained stable in HRCT and under microscopic view. Conclusions. SCFO from tegmen defects is an uncommon cause of otorrhea and even rhinorrhea. History and clinical presentation of our patients can be regarded as typical for adults with SCFO. Evaluation of history, HRCT and a high index of suspicion is a prerequisite for correct diagnosis. The approach (trans-mastoidal, transtemporal, Mini-Craniotomy) to repair leaks of the tegmen should be related to the extent of the defect.     

Atypical etiology of rhinorrhea: spontaneous bilateral temporal encephalocele

Spontaneous herniation of brain parenchyma through a dural and osseous defect in the temporal bone is a rare entity and a bilateral form is even more infrequent. It usually presents as an intermittent but persistent otorrhea. Manifestation as nose cerebrospinal fluid (CSF) leak is very uncommon. Our objective is presenting this unusual case report of a spontaneous bilateral encephalocele with a bilateral tegmen tympani defect.     

自发性脑脊液耳漏的研究进展

自发性脑脊液耳漏是一类无明显颞骨创伤史、手术史、感染、肿瘤等原因的一类脑脊液耳漏。病理生理学目前尚不清楚,主要公认的有“先天性缺陷论”和“蛛网膜颗粒论”两种形成学说。成人和儿童漏口部位存在一定差异,常见于乳突天盖、鼓室盖、底板周围、窦脑膜角、内听道底、后颅窝等。该病临床表现不特异,不及时治疗严重者引起脑膜炎危及生命。行颞骨HRCT、葡萄糖定量及核素脑池显影等检查确诊后,应积极行手术治疗。     

Cerebrospinal fluid otorrhea at myringotomy. A meningocele through a defect in the tegmen

Spontaneous cerebrospinal fistulae can simulate secretory otitis media or be discovered at myringotomy, but the diagnosis is frequently made after one or more episodes of meningitis. Congenital perilabyrinthine fistulae are extremely rare, and unlike translabyrinthine fistulae there is usually no sensorineural hearing loss. This is the first reported case of a congenital meningocele through the tegmen in a child. An 11-year-old boy presented with signs and symptoms which mimicked serous otitis media. A cerebrospinal fistula was produced at myringotomy and this was closed surgically via an endaural approach. A CT scan defined a defect in the tegmen tympani anterior to the superior semicircular canal.     

Spontaneous cerebrospinal fluid otorrhoea presenting as otitis externa

A case of spontaneous cerebrospinal fluid (CSF) otorrhoea in a 59-year-old lady is reported. The patient was initially diagnosed and treated for chronic otitis externa. Following the referral by her GP, it was found that her symptom of otorrhoea was secondary to cerebrospinal fluid leak with middle ear encephalocoele. The CSF leak was repaired using a transmastoid approach with fascia, bone paté and fibrin glue. In this paper, we describe an unusual case of spontaneous CSF otorrhoea presenting as otitis externa. We review the pathophysiology of spontaneous CSF otorrhoea, and discuss the pitfalls in the diagnosis of CSF otorrhoea.     

Temporal breach management in chronic otitis media

In a retrospective study performed at the Otolaryngology, Head and Neck Surgery Department, Pitié-Salpêtrière Hospital, Paris from 1991 to March 2007, we determined surgical procedures for the treatment of tegmen breaches in chronic otitis media. Forty-two cases were examined: 76% corresponded to chronic otitis media with cholesteatoma, and 24% to chronic otitis media without cholesteatoma. Twenty-eight cases were operated using a combined approach, eight cases using a single suprapetrous approach, and six cases using a transmastoid approach. A total of 33% of the cases showed a meningocele or a meningoencephalocele treated through either a combined or a suprapetrous approach. No recurrence or neural/meningeal infectious involvement was found after a mean time of 43 months in the 36 long-term follow-up cases operated through the combined or suprapetrous approaches. Two cases included in the study were a loss to follow-up. Three of the former cases had already been operated for supracentimetric fissure using lower approach. Two out of the six patients operated using lower approach presented post surgery cerebrospinal fluid otorrhea. Combined or suprapetrous approaches seem to be best adapted to the treatment of supracentimetric or recurrent tegmen breaches, as well as to the precise examination and repair of meningeal lesions. Treatment for tegmen breach can be achieved in a single intervention, even when there is an ongoing infection of the middle ear. The mastoid approach should be used only for infracentimetric defects when there is no neural/meningeal lesion.     

Spontaneous encephalocele of the temporal bone

Postsurgical and posttraumatic encephaloceles of the tegmen tympani and tegmen mastoideum are well-recognized occurrences. Less frequently recognized, however, is the spontaneous occurrence of an encephalocele associated with cerebrospinal fluid otorrhea or rhinorrhea. This cerebral herniation into the middle ear and the mastoid cavity in adults is associated with a loss of both bony and dural support, and surgical management must deal with both the cerebrospinal fluid leak and herniation of the brain.     

Relationship between obesity, obstructive sleep apnea, and spontaneous cerebrospinal fluid otorrhea

OBJECTIVES/HYPOTHESIS: To investigate the relationship between obesity, obstructive sleep apnea (OSA), and spontaneous cerebrospinal fluid (CSF) otorrhea. STUDY DESIGN: Retrospective review of patients presenting with the diagnosis of CSF otorrhea during a 15-year period to a tertiary care medical center. METHODS: Patient records were reviewed and the following data were extracted: age, sex, height, weight, past medical/surgical history, laterality of otorrhea, findings at operation, and method of repair. Otorrhea was considered spontaneous in the absence of trauma, otologic surgery, infection, and neoplasm. RESULTS: Of the 29 adult patients identified, 14 patients presented with CSF otorrhea that was spontaneous in nature. The average body mass index among these patients was 35.2 +/- 8, which was higher than the average in the nonspontaneous group, 28.5 +/- 5 (P = .01). Diagnoses of OSA were also more common in the spontaneous group (n = 4) compared with the nonspontaneous group (n = 0). One case of bilateral spontaneous CSF leaks was recorded. The majority of patients were repaired with hydroxyapatite cement through a transmastoid approach. There were no recurrences. CONCLUSIONS: In this series, patients with spontaneous CSF otorrhea were more likely to be severely and morbidly obese than were patients with nonspontaneous otorrhea. OSA was also more common in these patients. The findings from this study support an association between obesity and spontaneous CSF leaks. Patients presenting with spontaneous CSF otorrhea should therefore be screened for OSA and signs of increased intracranial pressure.