[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.

[Surgical methods of cerebrospinal fluid otorhinorrhea due to inner ear malformation in children].

Objective:To discuss the application of two surgical methods, trans-mastoid approach and trans-canal endoscopic approach, in the treatment of CerebroSpinal Fluid（CSF） otorhinorrhea due to inner ear malformation（IEM） in children. Methods:Children with CSF otorhinorrhea due to IEM, from July 2015 to Jan 2021, in ENT department, Beijing Children's Hospital were retrospectively analyzed, and the clinical data, surgical methods, complications, recurrence and follow-up were recorded. Results:30 children with CSF otorhinorrhea due to IEM were included. Half of them had a history of otorhinorrhea, and 53.3% of them were diagnosed as cerebrospinal fluid（CSF） otorrhea at the first onset Unilateral and bilateral IEM were 22 cases and 8 cases respectively. All unilateral IEM children had secondary infections such as meningitis or/and pneumonia, and 9.1% had cochlear implantation（CI） due to deafness after meningitis. 87.5% bilateral IEM children had CI due to profound/severe hearing loss. 96.7% CSF otorhinorrhea were unilateral, there was no relationship between CSF otorhinorrhea and the malformation side or the time of CI surgery. Trans-mastoid approach was used in 14 cases, usually involving CI or other operations; and endoscopic approach in 16 cases. The age of trans-mastoid group was younger than that of endoscopic group. No recurrence and complications occured in the trans-mastoid group. In the endoscopic group, 2 cases recurred; 3 cases had complications（2 with temporary facial paralysis and 1 with labyrinth and intracranial pneumatosis）. Except one case lost, the others were followed up for 1.2-6.7 years, and no recurrence of pneumonia, meningitis and CSF otorhinorrhea happened. Conclusion:Unilateral IEM usually leads to meningitis or/and pneumonia, and bilateral IEM requires CI. Both trans-mastoid and trans-canal endoscopic approach were effective for CSF otorrhea repair. Trans-mastoid approach is suitable for those who need mastoid exploration, CI or combined with other operations. Trans-canal endoscopic approach was choosed when fistula was confined to the tympanum and ear canal diameter was wide enough.

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.

[Progress in diagnosis and treatment of cerebrospinal fluid otorrhea caused by inner ear malformation].

Cerebrospinal fluid otorrhea caused by inner ear malformation is rare, and its clinical manifestations are atypical. Therefore, it can easy be misdiagnosed or missed. Recurrent meningitis caused by inner ear malformation can lead to serious complications. This article reviews the classification of inner ear malformation, the etiology the common fistula locations, clinical features, imaging features, surgical approaches, postoperative complications and influencing factors of surgical efficacy of cerebrospinal fluid otorrhea due to inner ear malformation.

Why did we encounter gusher in a stapes surgery case? Was it enlarged medial aperture of the cochlear aqueduct?

BACKGROUND AND PURPOSE: Preoperative prediction of cerebrospinal fluid (CSF) gusher is important for stapes surgery. According to the current opinion settled among otologists and radiologists, the issues of whether enlarged cochlear aqueduct might be a cause of CSF gusher in stapes surgery and which segment of the aqueduct should be taken into account to diagnose enlarged cochlear aqueduct in computerized tomography (CT) are controversial. The case we encountered led us to hypothesize that enlarged cochlear aqueduct might cause CSF gusher in stapes surgery and that shape and diameter of medial aperture of the cochlear aqueduct are important in this prediction. METHODS AND RESULTS: Enlarged medial aperture of the cochlear aqueduct with a shape differed from that of the other side was retrospectively diagnosed in thin-slice CT in a patient who had been undergone middle ear and stapes surgery for conductive hearing loss. This finding went unnoticed in preoperative CT. In the small fenestra stapedotomy operation, CSF gusher occurred through opening in the ill-defined, fixed and thickened stapes footplate. A piece of temporalis fascia and reshaped incus were appropriately placed which stopped the gusher. Re-evaluation of preoperatively taken CT showed that anterior-posterior and superior-inferior diameters of the medial aperture were 11.7 mm and 2.87 mm in CSF gusher side versus 2.95 mm and 1.88 mm on the other side, respectively. Its shape in gusher side differed from that of the other side. CONCLUSION: This report is the first to show video-documented CSF gusher in a patient with enlarged medial aperture of the cochlear aqueduct. It appears to be plausible to propose that these findings have to change the otologists' and radiologists' perspective to the cochlear aqueduct. It can be deduced that difference in shapes of the medial aperture in both sides might be an indicator of potential CSF gusher.

Diagnosis and management of pediatric cerebrospinal fluid leakage secondary to inner ear malformations: A report of 13 cases.

OBJECTIVES: Inner ear malformations (IEM) with cerebrospinal fluid (CSF) leakage in children is a rare condition, nevertheless, it may lead to meningitis. Early diagnosis and treatment are crucial. The aims of the study were to summarize the clinical characteristic of pediatric CSF leakage secondary to IEM, and to recommend transcanal endoscopic ear surgery (TEES) as an effective surgical technique for the treatment of CSF leakage with IEM in children. METHODS: This was a retrospective study. Thirteen children and fourteen ear surgery were included. Demographics, detail history, laboratory data, Audio test, and imageological examination results were recorded. All the pediatric patients underwent TEES. RESULTS: Most (92.31%) of the children presented with a history of rhinorrhea. 69.23% (9/13) of the children had suffered from meningitis, and the other had presented with respiratory tract infections. The follow-up duration ranged from 0.75 years to 5.29 years. Transcanal endoscopic repair of CSF leakage secondary to IEM was the first surgery with a success rate of 92.86% (13 out of 14 cases). A fistula could be found in the stapes footplate in all pediatric patients. CONCLUSION: Even if there has been no history of meningitis, the diagnosis of CSF leakage in children suffering from unilateral rhinorrhea and recurrent respiratory tract infection is considered. Auditory brainstem response (ABR) and Temporal bone computed tomography (CT) examinations are suggested to identify IEM. The TEES procedure is recommended in our study as the first choice that repairs CSF leakage secondary to IEM.

Simultaneous repair of cerebrospinal fluid otorrhea and cochlear implantation in two patients with recurrent meningitis and severe inner ear malformation.

OBJECTIVES: To investigate the outcomes of simultaneous cochlear implantation and repair of a cerebrospinal fluid (CSF) leak in two patients with inner ear malformations following meningitis. METHODS: In this retrospective study and review of the literature, two patients with recurrent meningitis and severe inner ear malformation underwent cochlear implantation via the round window, and repair of CSF otorrhea via a transmastoid lateral semicircular canal approach. Both patients were treated with antibiotics for 7 days after the surgery. RESULTS: Neither patient has exhibited evidence of CSF otorrhea 1 year after surgical repair. Categorical Auditory Performance (CAP) scores and the Speech Intelligibility Ratings (SIRs) were obtained before and 1 year after surgery: the scores were 0 versus 4 and 0 versus 2, respectively. Vestibular function tests of both patients were reviewed and were normal. CONCLUSION: Simultaneous repair of CSF otorrhea and cochlear implantation in patients with recurrent meningitis and severe inner ear malformation can be regarded as safe and effective.

Temporal bone meningoencephaloceles and cerebrospinal fluid leaks: experience in a tertiary care hospital.

OBJECTIVE: To recount experience with cerebrospinal fluid otorrhoea and temporal bone meningoencephalocele repair in a tertiary care hospital. METHOD: A retrospective review was conducted of 16 cerebrospinal fluid otorrhoea and meningoencephalic herniation patients managed surgically from 1991 to 2016. RESULTS: Aetiology was: congenital (n = 3), post-traumatic (n = 2), spontaneous (n = 1) or post-mastoidectomy (n = 10). Surgical repair was undertaken by combined middle cranial fossa and transmastoid approach in 3 patients, transmastoid approach in 2, oval window plugging in 1, and subtotal petrosectomy with middle-ear obliteration in 10. All patients had successful long-term outcomes, except one, who experienced recurrence after primary stage oval window plugging, but has been recurrence-free after second-stage subtotal petrosectomy with middle-ear obliteration. CONCLUSION: Dural injury or exposure in mastoidectomy may lead to cerebrospinal fluid otorrhoea or meningoencephalic herniation years later. Congenital, spontaneous and traumatic temporal bone defects may present similarly. Middle cranial fossa dural repair, transmastoid multilayer closure and subtotal petrosectomy with middle-ear obliteration were successful procedures. Subtotal petrosectomy with middle-ear obliteration offers advantages over middle cranial fossa dural repair alone; soft tissue closure is more robust and is preferred in situations where hearing preservation is not a priority.

Cerebrospinal fluid otorrhea secondary to congenital inner ear dysplasia: diagnosis and management of 18 cases.

OBJECTIVE: To describe the characteristics of the clinical presentation, diagnosis, surgical methods, and outcomes of patients with otogenic cerebrospinal fluid (CSF) leakage secondary to congenital inner ear dysplasia. METHODS: A retrospective review was performed of 18 patients with otogenic CSF leakage secondary to inner ear dysplasia who underwent surgery in our group from 2007 to 2017 and had a follow-up of at least 4 months. The average length of follow-up was three years. The characteristics of the clinical presentations of all patients, such as self-reported symptoms, radiographic findings, surgical approaches and methods of repair, position of the leakage during surgery, and postoperative course, including the success rate of surgery, are presented. RESULTS: The patients presented mostly with typical symptoms of meningitis, severe hearing impairment, and CSF otorrhea or rhinorrhea. All 18 patients had at least one previous episode of meningitis accompanied by a severe hearing impairment. The preoperative audiograms of 17 patients showed profound sensorineural hearing loss, and one patient had conductive hearing loss. Twelve patients presented with an initial onset of otorrhea, and two had accompanying rhinorrhea. Six patients complained of rhinorrhea, two of whom were misdiagnosed with CSF rhinorrhea and underwent transnasal endoscopy at another hospital. High-resolution computed tomography (HRCT) images can reveal developments in the inner ear, such as expansion of a vestibular cyst, unclear structure of the semicircular canal or cochlea, or signs of effusion in the middle ear or mastoid, which strongly suggest the possibility of CSF otorrhea. The children in the study suffered more severe dysplasia than adults. All 18 patients had CSF leakage identified during surgery. The most common defect sites were in the stapes footplates (55.6%), and 38.9% of patients had a leak around the oval window. One patient had a return of CSF otorrhea during the postoperative period, which did not re-occur following a second repair. CONCLUSIONS: CSF otorrhea due to congenital inner ear dysplasia is more severe in children than in adults. The most common symptoms were meningitis, hearing impairment, and CSF otorrhea or rhinorrhea. HRCT has high diagnostic accuracy for this disease. The most common fistula site was around the oval window, including the stapes footplates and the annular ligament.

Analysis of Audiometric Outcomes following Combined Middle Cranial Fossa/Transmastoid Approaches for Spontaneous Cerebrospinal Fluid Otorrhea.

Outcome Objectives To (1) explore audiometric outcomes following use of a combined transmastoid/middle cranial fossa (TM-MCF) approach in the treatment of spontaneous cerebrospinal fluid (CSF) otorrhea and (2) determine the influence of dehiscence location and reconstructive methodology on audiometric outcomes. Study Design Case series with chart review. Setting Tertiary care center. Subjects and Methods Adults with spontaneous CSF otorrhea were reviewed from 2007 to 2016 if they underwent combined TM-MCF. Definitive audiometric evaluations were performed at least 3 months postoperatively. The primary outcomes measures were pre- to postoperative changes in pure-tone average (PTA) and air-bone gap (ABG). Two subset analyses were performed for audiometric outcomes comparisons: first, patients with skull base dehiscences anterior to the ossicular chain were compared with those with posterior dehiscences. Second, patients undergoing skull base resurfacing plus abdominal fat graft mastoid obliteration (AFGMO) were compared with those undergoing resurfacing alone. Results A total of 28 patients and 31 ears were reviewed. There was 1 recurrent leak (3.5%). The cohort demonstrated significant improvement in mean postoperative ABG ( P = .008) but not PTA. On subset analysis, ears with posterior dehiscences demonstrated significant improvements in PTA ( P = .03) and ABG ( P = .05), while ears with anterior dehiscences did not. In addition, ears undergoing resurfacing plus AFGMO achieved significant improvements on all parameters ( P = .01). Only 3 of 15 ears undergoing resurfacing plus AFGMO experienced worsened postoperative hearing. Conclusion Use of the combined TM-MCF approach for treating spontaneous CSF otorrhea achieved good audiometric outcomes. Patients with skull base dehiscences posterior to the ossicles and those undergoing skull base resurfacing plus AFGMO achieved the most favorable results.

[Idiopathic intracranial hypertension and spontaneous cerebrospinal fluid fistula. Usefulness of intracranial pressure monitoring]. / Hipertensión intracraneal idiopática y fístula espontánea de líquido cefalorraquídeo. Utilidad de la monitorización de la presión intracraneal.

Spontaneous cerebrospinal fluid (CSF) fistulas are rather common in daily practice. The aim of the surgical treatment is closure of the leak, but recurrences are quite frequent. The association between spontaneous CSF fistulas and idiopathic intracranial hypertension (IIH) is not uncommon, and this is probably the cause of the low rate of success of the surgical treatment. Symptoms of IIH associated with spontaneous CSF fistula are atypical, and diagnosis is often missed. Continuous intracranial pressure monitoring is very useful in the diagnosis of chronic IIH and in patients with spontaneous CSF fistula, as it helps in making decisions on the treatment of these patients.

[Diagnosis and management of spontaneous cerebral spinal fluid otorrhea].

Spontaneous cerebral spinal fluidotorrhea (SCSFO) is a type of CSF otorrhea without obvious causes including previous trauma, surgery, infection or neoplasm. The etiology of SCSFO remains unclear, the diagnosis can be overlooked because of the untypical clinical features. In this paper, we reviewed etiology, clinical features, diagnosis and therapy of SCSFO from recent relative literatures.

[Analysis of the diagnosis and treatment of cerebrospinal fluid otorrhea].

Objective:To analyze the etiology and clinical symptoms and to investigate the therapeutic strategies of cerebrospinal fluid otorrhea. Method:A retrospective analysis of 37 cases of patients with cerebrospinal fluid otorrhea.The clinical symptoms, auxiliary examination, intraoperative findings, surgical methods and postoperative follow-up were analyzed. Result:In 37 cases, 35 patients underwent the plugging surgery once and cured, 1 patient with inner ear malformation underwent another operation and cured, 1 patient didn't have the operation. No cerebrospinal fluid leakage or meningitis recurrence was reported by the followed up from 1 months to 7 years after operation. Conclusion:Surgical repair is an effective method to treat the cerebrospinal fluid otorrhea. It is significant to take appropriate surgical approach to expose and to find the leak, according to the etiological factor and imaging examination.

CSF otorhinorrhea in a child with inner ear dysplasia: diagnosis with T2-weighted and intrathecal contrast-enhanced MR cisternography.

During the follow-up of recurrent pneumonia in a 9-month-old girl, rhinorrhea with discharge of a positional and intermittent nature was discovered. Radiological assessment was requested to detect any skull base openings and cerebrospinal fluid (CSF) leakage. T2-weighted MR cisternography showed bilateral inner ear dysplasia, communication of the internal auditory canal with the vestibule, and effusion in the right middle ear. Intrathecal contrast-enhanced MR cisternography revealed a CSF fistula from the right internal auditory canal to the Eustachian tube. The patient was operated upon on the right side, and the presence of a CSF leak near the oval window was confirmed. No adverse effects were seen during the short-term and long-term follow-up. Diagnosing this case required special attention, careful examination, and relevant investigations to find the site of CSF leakage in this patient with bilateral inner ear dysplasia.

Unruptured translabyrinthine meningocele without CSF otorrhea.

Labyrinthine meningocele can be classified into translabyrinthine and perilabyrinthine type. We describe a case of rare unruptured translabyrinthine meningocele (TLM). It is rare to encounter an unruptured TLM because it is usually diagnosed after rupture as a labyrinthine fistula, cerebral spinal fluid otorrhea, and subsequent meningitis. We provide for the first time an intraoperative photo and video of a case of an unruptured TLM that developed through an inner ear malformation in a single-side deaf child, which was preoperatively misdiagnosed as congenital cholesteatoma in preoperative temporal bone computed tomography. TLM without CSF otorrhea in an unruptured state merit attention because of its importance during the workup of congenital cholesteatoma or cochlear implantation in spite of its rarity of reports.

ICP, BMI, surgical repair, and CSF diversion in patients presenting with spontaneous CSF otorrhea.

OBJECTIVE: To assess intracranial pressure (ICP), body mass index (BMI), surgical repair, and cerebrospinal fluid (CSF) diversion in patients presenting with spontaneous CSF otorrhea. STUDY DESIGN: Retrospective series review. SETTING: Tertiary referral center. PATIENTS: Thirty-two patients were treated surgically from 2004 to 2013 for spontaneous CSF otorrhea by the principal investigators. Patients with a history of chronic ear disease and cholesteatoma, previous mastoid surgery, head trauma, or iatrogenic injury were excluded. Average age was 56 years. Twenty-two patients (69%) were female. INTERVENTION(S): Middle fossa repair, transmastoid repair, lumbar puncture, V-P shunt, L-P shunt, and magnetic resonance imaging. MAIN OUTCOME MEASURE(S): Patients underwent middle fossa or transmastoid repair of tegmen defects. Intracranial pressures were determined with lumbar puncture at time of surgical repair or shortly after surgery. CSF diversion procedures were performed in patients who were found to have elevated ICP, which was not controlled medically, presented with recurrent leak or had ICP of 25 cm or greater of H2O. Preoperative BMI was calculated. RESULTS: Thirty-two patients underwent 37 operations. Average BMI was 35.0 kg/m2 (median, 34.7; range, 18.7-53.2 kg/m2). There were 21 repairs on the left and 16 on the right. The majority underwent a middle fossa craniotomy for repair (27/32). Two patients had bilateral repairs. Three patients (8%) underwent revision surgery, of which, 2 had untreated intracranial hypertension (ICP 24.5 and 24 cm H2O). ICP measurements were available for 29 patients. The mean ICP was 23.4 cm H2O (median, 24; range, 13-36 cm H20). Twenty-two patients (69%) had ICP of 20 cm or greater of H20; of those, 13 had an ICP of 25 cm or greater of H20. Seventeen patients (53%) underwent CSF diversion procedures. CONCLUSION: Our findings of elevated ICP and BMI in patients presenting with spontaneous CSF otorrhea are consistent with previous reports in the literature. The percentage of patients that underwent CSF diversion procedures was high at 53% and represents an aggressive stance in managing elevated ICP in a population that may be at risk for subsequent leaks.

Spontaneous cerebrospinal fluid otorrhea and rhinorrhea in idiopathic intracranial hypertension patients.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leakage may occur in patients with normal or increased intracranial pressure (ICP). We describe herein spontaneous CSF leakage as a result of chronic increased ICP in 4 patients with idiopathic intracranial hypertension (IIH). Although rhinorrhea previously has been described in IIH patients, to our knowledge this is the first report of otorrhea in these patients. METHODS: Four patients with spontaneous CSF leakage were examined between 2001 and 2011; 3 presented with rhinorrhea and 1 with otorrhea. Clinical settings and manifestations were analyzed. RESULTS: All patients were found to have IIH. Three had been diagnosed with IIH several years earlier and had been noncompliant with their medical treatment, whereas in 1 patient, CSF rhinorrhea was the presenting symptom of IIH. CONCLUSION: CSF leak is a rare complication in IIH patients. We have shown that rhinorrhea can be the presenting sign in these patients and that rhinorrhea and otorrhea can be a late sign of the disease.