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.

Cerebrospinal Fluid Gusher in Cochlear Implantation and Its Association with Inner-Ear Malformations.

BACKGROUND: It is aimed to investigate the incidence of cerebrospinal fluid gusher in cochlear implantation and the association between cerebrospinal fluid gusher and inner-ear malformations in adult and pediatric patients. METHODS: A retrospective case review of 1025 primary cochlear implantation procedures was performed. Patients with inner-ear malformation or cerebrospinal fluid gusher during primary cochlear implantation were included and divided into 2 groups according to age: pediatric and adult groups. RESULTS: The incidence of inner-ear malformation was 4.19% (17/405) and 7.6% (47/620) in the adult and pediatric groups, respectively. There was a significant difference in the incidence of inner-ear malformation in the pediatric group. The incidence of cerebrospinal fluid gusher was 0.9% (4/405) and 4.1% (26/620) in the adult and pediatric groups, respectively. There was a significant difference in the incidence of gusher between the adult and pediatric groups. CONCLUSION: The incidence of a cerebrospinal fluid gusher is higher in the pediatric group, compared to adults due to a higher rate of inner-ear malformation. Inner-ear malformation poses a risk factor for cerebrospinal fluid gusher.

[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.

The necessity and effect of prophylactic quinolone ear drops after ventilation tube insertion for otitis media with effusion.

BACKGROUND: Otitis media with effusion (OME) is a condition where non-infective fluid builds up in the middle ear. Long-term OME can cause damage to the middle ear and hearing impairment. Ventilation tube insertion (VTI) is an efficient procedure to drain persistent OME. However, the effect of prophylactic ear drops after VTI remains controversial because no infection is present. This study investigated the need for and effect of quinolone ear drops in patients with OME after VTI. METHODS: Between July 2018 and July 2021, 272 patients (436 ears with OME) who underwent VTI were enrolled. Prophylactic quinolone ear drops (ofloxacin) were used in 271 OME ears and not used in 165. The clinical findings and effect of the ear drops were assessed. RESULTS: The group with postoperative ofloxacin had less postoperative otorrhea (p < 0.001). In univariate analysis, age ≥ 13 years (odds ratio [OR] = 1.499, 95% confidence interval [CI]: 1.003-2.238, p = 0.046) was significantly associated with recovery to normal middle ear functioning (type A on postoperative tympanometry). No adenoid hypertrophy (OR = 1.692, 95% CI: 1.108-2.585, p = 0.014) and no postoperative otorrhea (OR = 2.816, 95% CI: 1.869-4.237, p < 0.001) were significant independent factors associated with middle ear recovery in both univariate and multivariate analysis. After VTI, 65% of tympanic membranes in the group with postoperative ofloxacin recovered to normal, while in 67% of tympanic membranes in the group without ofloxacin scarring remained. CONCLUSIONS: Patients who received prophylactic postoperative ofloxacin had less postoperative otorrhea. No adenoid hypertrophy and no postoperative otorrhea were significant independent predictors of middle ear recovery to normal function in both univariate analysis and multivariate analysis. However, prophylactic ofloxacin was not an independent predictor of normal middle ear functioning after VTI. After VTI, most OME patients who had used ofloxacin postoperatively had eardrums that were in better condition than those of patients who had not used ofloxacin. In this study, we confirmed the advantages and limitations of OME after VTI with prophylactic ofloxacin, thus providing clinicians with some guidance regarding the decision to administer prophylactic ofloxacin.

[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.

[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.

[Persistent otorrhoea caused by cerebrospinal fluid leakage]. / Chronisch loopoor door liquorlekkage.

Persistent otorrhoea is a common issue for both children and adults, which can be caused by leakage of cerebrospinal fluid from the lateral skull base. Bacterial superinfection of the chronically humid middle ear, arising from continuous cerebrospinal fluid leakage, may contribute to an atypical clinical presentation. That is, otogenous cerebrospinal fluid leakage may mimic serious otitis media with concomitant conductive hearing loss, leading to a serious diagnostic delay in some patients. On the basis of three cases with cerebrospinal fluid leakage, resulting in persistent otorrhoea, we underline the importance of its timely diagnosis and treatment.

[A case of spontaneous middle cranial fossa cerebrospinal fluid leak presenting as recurrent bacterial meningitis].

A 19-year-old man with a history of Chiari type I malformation was admitted to our hospital two times within a 2-month period because of bacterial meningitis. Cerebrospinal fluid (CSF) analysis showed neutrophilic pleocytosis and hypoglycorrhachia. During the second admission, we became aware of hearing loss on the right since age 15 years. High-resolution temporal bone CT showed soft tissue opacification of the right epitympanum and external auditory canal. Tissue biopsy resulted in CSF otorrhea and pneumocephalus. CT cisternography revealed a temporal bone CSF leak. Brain MRI showed a dural defect localized to the anterior petrous apex. Using a combined middle cranial fossa-transmastoid approach, the dural defect and associated arachnoid granulations were located along the superior side of the greater petrosal nerve and repaired. A CSF leak without underlying pathology, such as trauma, surgery, or congenital abnormality, is defined as spontaneous. Spontaneous CSF leak should be considered as a cause of recurrent bacterial meningitis even when CSF otorrhea and fluid behind the tympanic membrane are clinically absent.

Otitis media prone children with cystic fibrosis: A new normal.

OBJECTIVES: To determine if children with cystic fibrosis (CF) who are otitis media prone and treated with tympanostomy tube placement (TTP) follow the natural course of non-CF children regarding the incidence of tympanostomy tube otorrhea (TTO) (21-34%). METHODS: All CF patients seen at a large tertiary pediatric hospital were retrospectively reviewed from 2010 to 2019. A total of 483 patients were identified and seventeen met the inclusion criteria and were included in the analysis. Data collected included demographics, CF diagnosis history including date of diagnosis and genotype, TTP notes, and otorrhea found in otolaryngology clinic and pediatrician clinic notes for up to 18 months post-TTP. RESULTS: CF was diagnosed at a median age of 13 days (0 days to 6 years). In terms of surgical frequency, 14/17 (82.4%) patients had one TTP, 2/17 (11.8%) had two TTPs, and 1/17 (5.9%) had five TTPs. The median (range) age at first TTP was 2 years (3 months to 13 years). After the first TTP, TTO occurred in 5 (29.4%) patients at 3 months, 6 (35.3%) at 6 and 9 months, and 7 (41.2%) at 12 and 18 months at median (range) = 1 (0-5) otolaryngology appointments and median (range) = 0 (0-8) pediatrician appointments. CONCLUSION: To our knowledge this is the first study to report that CF children are more likely to be severely affected with recurrent acute otitis media (RAOM), to require TTP, and to exhibit a natural history of TTO commensurate with the non-CF population.

Spontaneous Congenital Perilabyrinthine Cerebrospinal Fluid Fistulas.

OBJECTIVES: To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae. METHODS: A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl's fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl's fissures. RESULTS: An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl's fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases. CONCLUSIONS: Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.

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.

Post-traumatic orbitorrhea: An underestimated life-threatening complication following anterior skull base fractures.

Orbitorrhea is defined as a leak of cerebrospinal fluid from a cranio-orbital fistula. It is usually related to anterior skull base trauma. Orbitorrhea is an exceptional and life-threatening condition which should be promptly managed. We herein report the case of a right post-traumatic orbitorrhea following anterior skull base trauma. Conservative treatment was initially attempted. At 6 weeks, recurrence was noted, and the patient underwent neurosurgical management. A few months later, a secondary upper lid retraction was diagnosed and treated by full-thickness skin graft with favourable outcome. To our knowledge, fewer than 30 cases have been previously reported. Ophthalmologists should be aware of this life-threatening condition which could be underestimated.

The Medially-Invasive Cholesteatoma: An Aggressive Subtype of a Common Pathology.

OBJECTIVE: Describe the outcomes of treatment for patients with cholesteatomas that are medially invasive to the otic capsule, petrous apex, and/or skull base. STUDY DESIGN: Retrospective case series. SETTING: Two tertiary care academic centers. PATIENTS: Patients surgically managed for medially-invasive cholesteatoma at two tertiary care institutions from 2001 to 2017. INTERVENTIONS: Surgical management of medially-invasive cholesteatomas. MAIN OUTCOME MEASURES: The presenting symptoms, imaging, pre- and post-operative clinical course, and complications were reviewed. RESULTS: Seven patients were identified. All patients had pre-operative radiographic evidence of invasive cholesteatoma with erosion into the otic capsule beyond just a lateral semicircular canal fistula. Five patients had a complex otologic history with multiple surgeries for recurrent cholesteatoma including three with prior canal wall down mastoidectomy surgeries. Average age at the time of surgery was 41.3 years (range 20-83). Two patients underwent a hearing preservation approach to the skull base while all others underwent a surgical approach based on the extent of the lesion. Facial nerve function was maintained at the pre-operative level in all but one patient. No patient developed cholesteatoma recurrence. CONCLUSIONS: The medially-invasive cholesteatoma demonstrates an aggressive, endophytic growth pattern, invading into the otic capsule or through the perilabyrinthine air cells to the petrous apex. Surgical resection remains the best treatment option for medially-invasive cholesteatoma. When CSF leak is a concern, a subtotal petrosectomy with closure of the ear is often necessary.

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.