[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.

[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.

Fallopian Canal Meningocele with Spontaneous Cerebrospinal Fluid Otorrhea: Case Report and Systematic Review of the Literature.

OBJECTIVE: To present a case of spontaneous cerebrospinal fluid (CSF) otorrhea from a fallopian canal meningocele involving the geniculate fossa and review all cases of fallopian canal CSF leak reported in the literature with discussion of management and outcomes. METHODS: A 53-year-old woman with history of morbid obesity and hypertension presented to a tertiary care referral center with unilateral high-volume CSF otorrhea. High-resolution temporal bone computed tomography demonstrated significant dilatation of the geniculate fossa. Rates of postoperative facial paralysis and refractory CSF leak were reported for the present case and prior cases reported in the literature. RESULTS: Locations of fallopian canal dehiscence, surgical approaches, techniques for packing dehiscence, rates of postoperative facial paralysis and CSF leak, and revision procedures were reported for the present case and 14 cases in the literature. The present case involved dehiscence of the geniculate fossa that was approached via combined transmastoid-middle cranial fossa exploration with facial nerve monitoring. The area of dehiscence was carefully packed with temporalis fascia, muscle, and artificial dural substitute overlay to repair the CSF leak without injuring the facial nerve. Postoperatively, no facial weakness was noted; however, right-sided high-volume CSF otorrhea persisted. After discussing treatment options, the patient underwent subtotal petrosectomy and blind-sac closure of the external auditory canal the following day. This successfully resolved the CSF leak without causing facial nerve weakness. CONCLUSIONS: Fallopian canal meningocele is an exceedingly rare cause of CSF otorrhea. Successful repair requires precise packing of the dilated facial canal to occlude the leak without injuring the facial nerve. For refractory CSF leak, subtotal petrosectomy and closure of the external auditory canal warrants consideration.

Cerebrospinal fluid rhinorrhea and otorrhea: A multimodality imaging approach.

Cerebrospinal fluid (CSF) leaks are extracranial egress of CSF into the adjacent paranasal sinus or tympanomastoid cavity due to an osteodural defect involving skull base. It can be due to a multitude of causes including accidental or iatrogenic trauma, congenital malformations and spontaneous leaks. Accurate localization of the site of the leak, underlying causes and appropriate therapy is necessary to avoid associated complications. In this paper relevant anatomy, clinical diagnosis, imaging modalities and associated findings are discussed along with a brief mention about management.

Are Patients with Spontaneous CSF Otorrhea and Superior Canal Dehiscence Congenitally Predisposed to Their Disorders?

Objectives (1) Compare lateral skull base (LSB) height/thickness in patients with spontaneous cerebrospinal fluid otorrhea (CSF), superior canal dehiscence (SCD), acoustic neuromas (AN), and otosclerosis (OTO). (2) Perform correlations between age, body mass index (BMI), sex, and LSB height/thickness. Study Design Case series with chart review. Setting Tertiary referral center. Subjects and Methods Patients with CSF, SCD, AN, and OTO diagnosed from 2006 to 2016 were included if they had high-definition temporal bone computed tomography (CT) and absence of trauma, radiation, chronic ear disease, and/or congenital anomaly. CT-based measurements included LSB height/thickness and pneumatization rates overlaying the external auditory canal (EAC), tegmen tympani (TgT), perigeniculate region (PG), and internal auditory canal (IAC). LSB height/thickness, age, sex, and BMI were statistically correlated. In total, 256 patients and 493 ears (109 CSF, 115 SCD, 269 AN/OTO) were measured. Results Patients with CSF had significantly higher BMIs than the other groups ( P < .001). Patients with CSF and SCD had similar radiographic LSB phenotypes at most measured locations. Both groups exhibited a significantly lower LSB height compared to the AN and OTO groups (mean, 3.9-4.2 mm vs 4.9-5.6 mm; P < .001). Patients with CSF and SCD also demonstrated significantly lower pneumatization rates, as low as 17% to 23% overlaying the PG and IAC ( P < .001). There were no statistically significant correlations found between age, sex, BMI, and LSB height/thickness at any measurement location in any group. Conclusions Patients with CSF and SCD exhibit similar radiographic LSB phenotypes. Age, sex, and BMI do not significantly correlate with LSB height/thickness. These data support the theory that CSF and SCD arise via similar congenital pathoetiologic mechanisms.

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.

Endoscopic Middle Cranial Fossa Reconstruction with a Subtemporal Keyhole.

BACKGROUND: Skull base reconstruction is an essential technique for repairing cerebrospinal fluid (CSF) leakage. A reliable method for middle cranial fossa (MCF) reconstruction with minimal invasiveness has not been reported. An initial case of endoscopic MCF reconstruction with a subtemporal keyhole is described. CASE DESCRIPTION: A 57-year-old man developed severe meningitis and was diagnosed with spontaneous CSF leakage from bone defects on the tegmen tympani. Endoscopic MCF reconstruction with a subtemporal keyhole was carried out. Three skin incisions, including 1 subtemporal incision for a subtemporal keyhole and 2 temporal line incisions on the superior temporal line, were made, and a 0-degree endoscope was introduced into the subcutaneous space. The deep temporal fascia (DTF) was bluntly dissected and separated from the superficial temporal fascia and the temporal muscle, and the DTF was incised to shape a pedicled flap under endoscopic view. Blood supply to the pedicled DTF flap was confirmed with indocyanine green angiography. A subtemporal keyhole was then made, and a 30-degree endoscope was used to explore the extradural space of the MCF floor, visualizing the bone defects on the tegmen tympani. The vascularized DTF flap passed easily through the subtemporal keyhole and adequately overlaid the bone defects. The patient's postoperative course was uneventful, and the CSF leakage disappeared without mastication problems. CONCLUSIONS: This purely endoscopic technique using a vascularized DTF flap provided reliable MCF reconstruction through a subtemporal keyhole. This technique is also expected to be applicable for MCF reconstruction after subtemporal keyhole surgery for skull base tumors.

Imaging of Cerebrospinal Fluid Rhinorrhea and Otorrhea.

As the obesity epidemic grows in the United States, rhinorrhea and otorrhea from spontaneous cerebrospinal fluid (CSF) leaks secondary to untreated idiopathic intracranial hypertension are increasing in prevalence. CSF rhinorrhea and otorrhea should also be carefully evaluated in posttraumatic and postsurgical settings, because untreated CSF leaks often have serious consequences. The work-up, diagnosis, and characterization of a CSF leak can be complex, often requiring a multimodality approach to optimize surgical planning. This article reviews the causes of CSF leaks, describes the methodology used to work up a suspected leak, and discusses the challenges of making an accurate diagnosis.

Middle cranial fossa approach for the repair of spontaneous cerebrospinal fluid leaks to the middle ear. / Abordaje por fosa media para el cierre de fístulas de líquido cefalorraquídeo espontáneas a oído medio.

INTRODUCTION: Spontaneous cerebrospinal fluid (CSF) leaks to the middle ear due to tegmen tympani defects can result in hearing loss or hypoacusis and predispose to meningitis as well as other neurological complications. Surgical repair of the defect can be performed through a middle cranial fossa (MCF) approach or a transmastoid approach. MATERIAL AND METHODS: We conducted a retrospective study of the patients in our Department due to a spontaneous CSF leak to the middle ear treated using a MCF approach during a 6-year period (2009-2014). RESULTS: Thirteen patients with spontaneous CSF leak to the middle ear were treated with this approach. The primary and first symptom in all of them was conductive hearing loss. In all cases, the defect or defects were closed in a multilayer manner using muscle, temporalis fascia and cortical bone. Minimum follow-up in this series was 14 months, with successful closure in all but one patient (who required reintervention). We found no intra- or postoperative complications due to the craniotomy, and the audiometry improved and normalised in all cases except for the failed case. CONCLUSIONS: The MCF approach with a multilayer closure of the defect is an effective technique for repairing spontaneous CSF leaks to the middle ear and for restoring hearing in these patients.

[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.

Lateral Skull Base Attenuation in Superior Semicircular Canal Dehiscence and Spontaneous Cerebrospinal Fluid Otorrhea.

OBJECTIVES: (1) To quantitatively assess the lateral skull base thickness in patients with superior semicircular canal dehiscense (SSCD) using a standardized and validated radiographic measure and to compare it with that of a population with spontaneous cerebrospinal fluid otorrhea (CSFO). (2) To analyze demographic and clinical factors associated with skull base thickness in the SSCD group. STUDY DESIGN: Case series with chart review. SETTING: Tertiary neurotologic referral center. SUBJECTS AND METHODS: Based on computed tomography imaging of the tegmen, mean skull base thickness was calculated for 16 patients with radiographic and clinical SSCD. Similar measures were performed in 4 comparison groups consisting of adults with spontaneous CSFO (n = 33), as well as 3 control groups recruited from our adult cochlear implant database: 30 obese controls (body mass index [BMI] >30 kg/m(2)), 11 overweight controls (BMI, 25-30 kg/m(2)), and 20 normal weight controls (BMI <25 kg/m(2)). RESULTS: The SSCD group had a significantly lower mean BMI (28.6 kg/m(2)) than the spontaneous CSFO group (37.7 kg/m(2); P = .0007). The mean skull base thickness of SSCD patients was 17% thinner than that of the CSFO group, 31% thinner vs obese controls, 49% thinner vs overweight controls, and 45% thinner vs normal weight controls. These differences were all statistically significant (P < .05). CONCLUSION: Patients with SSCD have a marked thinning of the lateral skull base, more so than patients with spontaneous CSF otorrhea and control groups with different BMIs. Skull base attenuation in SSCD patients did not correlate with BMI.

Association between Lateral Skull Base Thickness and Surgical Outcomes in Spontaneous CSF Otorrhea.

OBJECTIVES: (1) Correlate skull base thickness with perioperative outcomes for spontaneous cerebrospinal fluid (CSF) otorrhea. (2) Augment perioperative counseling of patients with abnormally thin skull bases. STUDY DESIGN: Case series with chart review. SETTING: Tertiary center. Patients with spontaneous CSF otorrhea have thin skull bases. This is associated with obesity and/or idiopathic intracranial hypertension. The influence of skull base thinning on perioperative outcomes is unknown. SUBJECTS AND METHODS: A retrospective review was conducted from 2004 to 2014. Forty-eight cases of spontaneous CSF otorrhea met the inclusion criteria of primary surgery by the senior authors: preoperative dedicated temporal bone computed tomography, absence of other leak etiologies, and follow-up >6 months. Patients were stratified into thin (<0.9 mm) and thick (>0.9 mm) groups based on computed tomography measures of their tegmen. Primary outcomes measures were as follows: postoperative meningitis, recurrent leak, second site leak (contralateral ear/anterior fossa), and permanent shunt placement. Hearing outcomes were not assessed in this study. RESULTS: Thirty and 15 patients composed the thin and thick groups, respectively. Both the incidence (P < .0001) and the rate (P = .005) of adverse outcomes were significantly higher in the thin group. Only 2 patients in the thick group experienced an adverse outcome. Eleven patients underwent multiple procedures for spontaneous leaks. The recurrence rate was 14.5%. All but 1 recurrence occurred in the thin group. CONCLUSIONS: An abnormally thin tegmen was significantly associated with adverse perioperative outcomes in cases of spontaneous CSF otorrhea. A thick skull base and the presence of an encephalocele may be protective against recurrence. The effect of untreated intracranial hypertension on the results is unknown.

Internal auditory canal meningocele-perilabyrinthine/translabyrinthine fistula: Case report and imaging.

The case of a 17-year-old patient with progressive unilateral sensorineural hearing loss and temporal bone malformations concerning for internal auditory canal meningocele with translabyrinthine/perilabyrinthine cerebrospinal fluid fistula is presented with associated computed tomography and magnetic resonance imaging. As the second reported case of an unruptured internal auditory canal meningocele with translabyrinthine/perilabyrinthine fistula, the case presents several clinically relevant points for otologists, neurotologists, and neuroradiologists. Although rare, it is an additional entity to consider as a cause of unilateral sensorineural hearing loss and may pose a risk for developing meningitis and possible "gushing" of cerebrospinal fluid should surgical intervention be attempted. Laryngoscope, 126:1931-1934, 2016.

Novel radiographic measurement algorithm demonstrating a link between obesity and lateral skull base attenuation.

OBJECTIVES: (1) To describe a validated algorithm for measuring tegmen thickness on computed tomography scans. (2) To compare the tegmen thickness in 3 groups: patients with spontaneous cerebrospinal fluid (CSF) leaks, obese controls, and nonobese controls. STUDY DESIGN: Retrospective review. SETTING: Patients with spontaneous CSF otorrhea often have highly attenuated tegmen plates. This is associated with obesity and/or idiopathic intracranial hypertension (IIH). No evidence exists, however, that objectively links obesity and/or IIH with skull base attenuation. SUBJECTS AND METHODS: This was a retrospective review from 2004 to the present. Patients with spontaneous CSF otorrhea and matched obese (body mass index [BMI] >30 kg/m(2)) and nonobese (BMI <30 kg/m(2)) controls were selected. Tegmen thickness was measured radiographically. Interrater validity was assessed. RESULTS: Ninety-eight patients were measured: 37 in the CSF group (BMI, 36.6 kg/m(2)), 30 in the obese group (BMI, 34.6 kg/m(2)), and 31 in the nonobese group (BMI, 24.2 kg/m(2)). The CSF group had a significantly thinner tegmen compared to both the obese control (P < .01) and nonobese control (P = .0004) groups. Obese controls had a thinner tegmen than nonobese controls (P < .00001). A significant inverse correlation was detected between skull base thickness and BMI. Signs/symptoms of IIH were most commonly found in the CSF group. Good to very good strength of agreement was detected for measures between raters. CONCLUSION: This is the first study to (1) quantify lateral skull base thickness and (2) significantly correlate obesity with lateral skull base attenuation. Patients who are obese with spontaneous CSF leaks have greater attenuation of their skull base than matched obese controls. This finding supports theories that an additional process, possibly congenital, has a pathoetiological role in skull base dehiscence.

Control of cerebrospinal fluid otorrhea via C-arm-guided reduction of the zygomatic arch as a part of the temporal bone: interdisciplinary approach to an unusual craniomaxillofacial fracture.

Maxillofacial fractures are often associated with blunt head injuries, of which skull base trauma is a common component. However, most oral and maxillofacial surgeons do not provide definitive management of temporal bone fractures involving the skull base and their sequelae. Persistent cerebrospinal fluid (CSF) leakage that is refractory to conservative measures usually requires surgical closure to decrease the risk of meningitis. In general, reduction of the displaced fragment of the skull base in temporal bone fractures is not considered a priority. We describe an unusual case of craniomaxillofacial injury exhibiting CSF otorrhea because of a temporal bone fracture with a fragment that included the zygomatic arch. The persistent traumatic leakage was stopped after C-arm-guided reduction of the depressed zygomatic arch. This technique facilitated minimal and only necessary manipulation, without overcorrection, thereby avoiding additional damage to the surrounding tissues. The present case illustrates the definitive contribution of therapeutic measures based on maxillofacial surgery as part of an interdisciplinary approach to the management of the complications of severe head injuries; more invasive neurosurgery was thus avoided.

Use of the translabyrinthine approach to repair congenital spontaneous cerebrospinal fluid leakage in five Chinese patients with Mondini dysplasia.

OBJECTIVES: To introduce a more effective surgical technique for the repair of spontaneous incurable cerebrospinal fluid (CSF) leakage (CSF otorrhea). METHODS: The cases of five Chinese patients with Mondini dysplasia and CSF leakage were reviewed. All patients presented with rhinorrhea or otorrhea from childhood and recurrent meningitis attacks. CSF leakage was repaired surgically using the translabyrinthine approach. RESULTS: Facial paralysis occurred in the first two patients, but was cured 2 months later. The other three patients recovered well and had no facial paralysis or complication. CSF leakage did not recur in any patient after follow-up periods of 0.5-3 years. CONCLUSION: Mondini dysplasia with CSF leakage is rare. Adequate diagnosis and treatment are required to avoid life-threatening complications, such as meningitis. We recommend the translabyrinthine approach as an alternative surgical technique that reduces recurrence.

Cerebrospinal fluid leak of the fallopian canal.

Spontaneous cerebrospinal fluid (CSF) leaks from the fallopian canal are extremely rare, as only a few cases have been reported in the world literature. We describe a case of spontaneous CSF otorrhea through an enlarged geniculate fallopian canal. The patient was a 45-year-old woman who presented with a history of CSF rhinorrhea and otorrhea from the right ear. Myringotomy and tube insertion revealed CSF otorrhea. Contrast-enhanced computed tomography revealed that the geniculate fossa was smoothly enlarged (demonstrating remodeling of bone). A middle fossa craniotomy with temporal bone exploration was performed. Intraoperative inspection detected the presence of a fistula secondary to a lateral extension of the subarachnoid space through the labyrinthine segments of the fallopian canal. We discuss the management of this unusual finding, which involves sealing the fistula while preserving facial nerve function.