Imaging evaluation of cerebrospinal fluid otorrhea associated with inner ear malformation in children / 中华耳鼻咽喉头颈外科杂志

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.

Application of modified enlarged translabyrinthine approach in the removal of vestibular schwannoma / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To describe and analyze the surgical outcomes of modified enlarged translabyrinthine approach(ETLA), which is combined with middle ear eradication and blind sac technique.</p><p><b>METHODS</b>A retrospective study was conducted on 28 patients who underwent surgery for vestibular schwannoma (VS) via modified ETLA from Jan. 2001 to Dec. 2012. The sizes of tumors were 25-52 mm[(38.2 ± 9.1) mm (X(-) ± s)]. Main outcomes measures included the rate of total removal, the rate of cerebral spinal fluid (CSF) leakage after surgery, complications, and intraoperative anatomical preservation of facial nerve and facial nerve function.</p><p><b>RESULTS</b>Among the 28 patients who underwent modified ETLA, the rate of total removal was 100%. There was no death after surgery via modified ETLA. The rate of CSF leakage after surgery was 3.5% (1/28). The rate of intraoperative anatomical preservation of facial nerve was 89.3% (25/28).Short-term and long-term good facial nerve function rates were 25.0% (7/28) and 42.9% (12/28), respectively.</p><p><b>CONCLUSIONS</b>Modified ETLA is suitable for VS>3 cm with extension to the anterior region of cerebellopontine angle, VS involve with labyrinth part of facial nerve, high jugular bulb, proposing sigmoid sinus and highly pneumatized temporal bone. This approach can provide a wide surgical field and well prevention of CSF leakage.</p>

Clinical characteristics of 42 cases of spontaneous intracranial hypotension with cerebrospinal leakage / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To review the clinical characteristics of spontaneous intracranial hypotension (SIH) with cerebrospinal fluid (CSF) leakage.</p><p><b>METHODS</b>Clinical data of 42 SIH patients with cerebrospinal leakage, whose diagnosis met the criteria of the International Headache Classification, were retrospectively reviewed. The patients were divided into short (n=27) and long (n=15) course groups. The clinical data and imaging features were compared between two groups.</p><p><b>RESULTS</b>Thirty-nine patients (92.9%) had orthostatic headache. Compared with the short course group, the frequency of headache were significantly lower in patients with long disease duration (80% vs 100%, P =0.040); the ratio of high CSF opening pressure (>=60.0 mm H2O), the average CSF opening pressure, and the frequencies of subdural hematoma were higher in long course group than those in short course group [60.0% vs 20.8%, (64.7±42.1) vs (40.0±33.8)mm H2O, and 50.0% vs 11.6%; P=0.019, 0.038 and 0.018, respectively]. Forty-two patients underwent CT myelography;definite focal CSF leakage sites were found in all patients and multiple sites of CSF leakage in 38 patients.</p><p><b>CONCLUSION</b>All SIH do not necessarily show the typical clinical manifestations, and cranial MRI and CT myelography are helpful in the diagnosis. Because of higher risk of subdural hemorrhage, patients with long disease duration require active intervention.</p>

Repair of Spontaneous Cerebrospinal Fluid Otorrhea from Defect of Middle Cranial Fossa

Spontaneous cerebrospinal fluid (CSF) otorrhea is defined as CSF otorrhea where there are no identifiable causes including previous trauma, surgery, infection, neoplasm or congenital anomaly. The condition is rare. The origin of CSF leak is commonly a defect in the tegmen of the middle cranial fossa. The pathophysiology of spontaneous CSF otorrhea is unclear. Two theories of the etiology of bony defects of the temporal bone are the congenital bony defect theory and arachnoid granulation theory. The authors experienced a case of a 49-year-old female patient admitted with the complaint of persistent right ear fullness. Computed tomography revealed a large defect of the middle fossa and suspicious CSF otorrhea through the defect of tegmen tympani. Repair was successful with multiple bone chips using the transmastoid approach. The postoperative course was good and there has been no recurrence of the CSF leakage.

Cerebrospinal Fluid Otorrhea Caused by Arachnoid Granulation

Cerebrospinal fluid (CSF) leakage otorrhea may be congenital or can be caused by trauma, surgery, cholesteatoma, and tumors. Spontaneous CSF leakage through the middle ear without a secondary cause is a relatively rare disease. The pathophysiology of CSF otorrhea has not been clear yet. However, there are two theories of the pathophysiology of spontaneous CSF otorrhea have been studied in the medical field: one based on the congenital defect; the other about the arachnoid granulation which causes bone erosion. Herein, we examine and report a case of CSF otorrhea caused by arachnoid granulation. Literatures pertaining to the disorder will be reviewed and characteristics of the disorder also will be discussed.

Cerebrospinal Fluid Otorrhea Caused by Arachnoid Granulation

Cerebrospinal fluid (CSF) leakage otorrhea may be congenital or can be caused by trauma, surgery, cholesteatoma, and tumors. Spontaneous CSF leakage through the middle ear without a secondary cause is a relatively rare disease. The pathophysiology of CSF otorrhea has not been clear yet. However, there are two theories of the pathophysiology of spontaneous CSF otorrhea have been studied in the medical field: one based on the congenital defect; the other about the arachnoid granulation which causes bone erosion. Herein, we examine and report a case of CSF otorrhea caused by arachnoid granulation. Literatures pertaining to the disorder will be reviewed and characteristics of the disorder also will be discussed.

Traumatic acute brain herniation through the ear in a child: concealed compound fracture

A seven-year-old girl presented to Sultan Qaboos University Hospital, Oman, with a history of having been hit by a motor vehicle. After this, she had right-sided cerebrospinal fluid otorrhoea, and a week later, brain matter extruded through the right ear. A computed tomography scan of the brain demonstrated a tegmen fracture communicating with the external auditory canal. There was no hearing or facial nerve impairment and an otoscopic examination showed an intact tympanic membrane. She underwent a transcranial repair of the middle cranial fossa base, which revealed a wide dural and bony defect of the tegmen with herniation of the temporal lobe. Repair was made with an intradural patch of artificial dura. The rarity of this type of presentation of temporal bone fracture and its management are discussed

Surgical Treatment of Spontaneous Meningocele of the Temporal Bone Causing a Pulsatile Tinnitus / 대한이비인후과학회지

A meningocele is a protrusion of meninges through a defect in the skull base. It is a rare but potentially life-threatening condition requiring surgery. It can present with conductive hearing loss, persistent middle ear effusion, cerebrospinal fluid otorrhea, recurrent meningitis, and epilepsy. We report a patient who presented with a three-year history of intermittent pulsatile tinnitus in her left ear. She had clear middle ear effusion and radiological evaluations suggesting an attic cholesteatoma. She underwent a tympanomastoidectomy and we discovered a meningocele in the epitympanum and a bony defect of the tegmen tympani. We easily restored the meningocele using a microelevator and bolstered the bone with temporalis fascia, conchal cartilage and musculoperiosteal flap. There was no evidence of cerebrospinal fluid leakage and the patient's tinnitus disappeared six months after the operation.

Relato de caso: otoliquorreia espontânea em adulto / Case study spontaneous otoliquorrhea in adult

Introdução: As fístulas liquóricas otológicas espontâneas (FLOEs) são entidades raras. Geralmente manifestam-se em crianças com meningite e perda sensorioneural profunda e em adultos com quadro de otite média com efusão. Objetivo: Descrever o quadro clínico, o diagnóstico e o tratamento cirúrgico de uma paciente com FLOE. Relato do Caso: Paciente do sexo feminino, 57 anos, com história de hipoacusia, plenitude aural e tinnitus à direita há 10 anos. Após colocação de tubo de ventilação à direita em outro serviço, iniciou otorreia líquida, transparente e constante em grande quantidade. O diagnóstico foi realizado através da cisternocintilografia, sugestiva de fístula liquórica; e da cisternotomografia que mostrou área de deiscência óssea em região de tegmen tympani à direita, velamento parcial de células mastóideas e de orelha média à direita, além concentração do meio de contraste no espaço sub-aracnoídeo à direita menor em relação ao lado esquerdo. Através de via transmastoídea foi localizada fístula liquórica e meningoencefalocele na região de tegmen tympani à direita. O fechamento da fístula foi realizado através do uso de retalho de músculo temporal, cola de fibrina e Surgicel®. Comentários Finais: Em adultos com história clínica sugestiva de otite média com efusão recorrente, a hipótese diagnóstica de fístula liquórica deve ser levantada. A investigação deve prosseguir com exames de imagem, destacando-se a cisternotomografia. O tratamento cirúrgico nesta paciente, através da técnica transmastoídea, se revelou eficaz a curto e a longo prazo...

Introduction: The spontaneous otological CSF leaks are rare entities. Usually they manifest themselves in children with meningitis and deep sensorineural hearing loss and in adults with middle otitis and effusion clinical picture. Objective: To describe the clinical picture, diagnosis and surgical treatment of a patient with CSF otological. Case Report: A female sex patient, 57 years old, with hearing loss, aural fullness and right ear tinnitus case history 10 years ago. After insertion of tympanostomy tubes on the right in another service, it began liquid, transparent and constant otorrhea in large quantities. The diagnosis was performed by suggestive cisternoscintigraphy of CSF leak, and by cisternography that showed that the area of bone dehiscence in the tegmen tympani region to the right, partial opacification of mastoid cells and middle ear to the right, besides concentration of contrast medium in sub-arachnoid space to the lower right compared to the left side. Through transmastoid via was located the CSF leak and meningoencephalocele in the region of the right tegmen tympani. The closure of the fistula was accomplished through the use of the temporal muscle flap, fibrin glue and Surgicel®. Final Comments: In adults with symptoms suggestive of recurrent otitis media with effusion, the diagnosis of CSF fistula should be raised. The research should proceed with imaging, highlighting the CTC. Surgical treatment in this patient using the transmastoid technique has proved effective in the short and long term...

High-resolution CT findings of inner ear malformations with cerebrospinal fluid leakage / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To describe the HRCT appearances of congenital inner ear malformations with cerebrospinal fluid leakage.@*METHOD@#Conducted a retrospective case review. The subjects comprised 11 patients who were decisively confirmed by HRCT and exploratory tympanotomy. The result of temporal bone computed tomography scans (HRCT) were analyzed.@*RESULT@#Inner ear malformations were classified into 4 types according to HRCT findings. There were 4 patients with cochlear aplasia, 2 patients with common cavity anomaly, 2 patients with IP-I anomaly, and 3 patients with IP- II anomaly. Eight patients had defects in the lamina cribrosa of the internal auditory canal. The development and aeration in the mastoid of involved ears was normal. Soft tissues were seen near the oval window in all involved ears.@*CONCLUSION@#HRCT image has an important value in the diagnosis and treatment of inner ear malformations with cerebrospinal fluid leakage.

Current imaging of cerebrospinal fluid leaks / Imágenes médicas actuales de pérdidas de fluido cerebroespinal

A CSF leak results from a defect in the dura and skull base. Due to the risk of potentially devastating central nervous system infection, it is an important entity. Timely diagnosis of CSF leaks is crucial. Laboratory testing of nasal or aural fluid drainage for the presence of the protein Beta(β)-2 transferrin plays a key role in establishing the presence of a CSF leak. Such assays are not always available, making imaging pivotal in the diagnosis of this entity. The development of minimally invasive endoscopic repair further underscores the importance of imaging as precise anatomic localization is important for repair. In this article, we review the literature and make suggestions for the appropriate radiological investigation ofpatients with suspected CSF leaks.

Las pérdidas de fluido cerebroespinal (FCE) son el resultado de un defecto en la dura y la base craneal. Debido a que tales pérdidas presentan el riesgo de infecciones capaces de devastar potencialmente el sistema nervioso central, las mismas constituyen una entidad importante. El diagnóstico a tiempo de pérdidas del FCE es crucial. Las pruebas de laboratorio realizadas al drenaje del fluido de la nariz y los oídos a fin de detectar la proteína Beta (β) 2 transferrina, desempeñan un papel clave a la hora de determinar la presencia de pérdida de FCE. Estos ensayos no se hallan siempre a disposición, con lo cual la imagen médica se hace fundamental para el diagnóstico de esta entidad. El desarrollo de reparaciones endoscópicas mínimamente invasivas subraya aún más la importancia de la imagen médica, por cuanto se requiere una localización anatómica precisa para realizar la reparación. En el presente artículo, se revisa la literatura y se hacen sugerencias a fin de lograr una investigación radiológica adecuada de los pacientes con sospecha de pérdida de FCE.

A Case Report of Brain Herniation into the Middle Ear Mimicking as Secondary Cholesteatoma Following Temporal Bone Fracture / 대한이비인후과학회지

Brain herniation into the middle ear cavity is a rare entity that occurs mostly as a complication of otologic surgery. Other causes include a congenital skull base defect, infection, trauma, neoplasm and irradiation. It gives rise to cerebrospinal fluid otorrhea, progressive hearing loss, tinnitus, meningitis and other neurologic symptoms. Such patients tend to be misdiagnosed as having chronic otitis media and are often treated for long periods with inadequate conservative therapy. We report a case of brain herniation into the middle ear following traumatic temporal bone fracture, which was treated surgically via a transmastoid approach.

A Case of Profuse Cerebrospinal Fluid Otorrhea with Common Cavity Cochlea in Adult / 대한이비인후과학회지

Cerebrospinal fluid (CSF) otorrhea with inner ear anomaly and common cavity anomaly in adults is very rare, especially as a first symptom except hearing loss. Surgical intervention is the primary treatment for profuse CSF leaks by cochlear anomaly. It can be treated when CSF leaks are encountered after cochlear implant surgery and are controlled with temporalis muscle plugs, fascia and fibrin glue. We should advise patients with congenital inner ear anomaly about sudden cerebrospinal fluid leaks or recurrent meningitis. We report a case of cerebrospinal fluid otorrhea with common cavity cochlea in an adult with a review of literature.

Cerebellar Hernia in a Patient with Chronic Otorrhea / 대한이비인후과학회지

Brain hernia into the middle ear cavity is quite a rare entity which is often caused by chronic otitis media with cholesteatoma and surgical complications. Other unusual factors such as extension of the intracranial meningioma, primary temporal bone meningioma and aberrant arachnoid granulation can also cause hernia of brain tissue. Brain hernia can be diagnosed through temporal bone computed tomography (TBCT) and brain magnetic resonance image (MRI), which show the location of bony defect and similar signal intensity between the herniated tissue and brain. We present a 50-year old female patient, who suffered from otorrhea 8 years ago; otorrhear disappeared 4 years ago and rhinorrhea 1 years ago. She had not have any history of chronic otitis media and otologic surgery. The herniated cerebellum was diagnosed through the TBCT and brain MRI. She was successfully cured after cerebellar reduction operation without any postoperative complications.

Manejo de fístulas de LCR de la lámela lateral de la lámina cribosa / Handling of CSF fistulae of the lateral lamella of the cribiform plate

Introducción: Las fístulas de líquido cefalorraquídeo (LCR) hacia la región nasosinusal, se presentan por la aparición de una brecha en la estructura osteo-meníngea que constituye el piso anterior de la base del cráneo. El diagnóstico debe ser corroborado con el apoyo de laboratorio e Imágenes, que permite disminuir los márgenes de error. El avance de las técnicas endoscópicas nos ha familiarizado con la base del cráneo y nos ha llevado a tener una participación junto a los neurocirujanos en el tratamiento de patologías de esta zona. La base anterior craneal contiene los sulcus olfatorios, en cuyo fondo encontramos orificios para los filetes olfatorios, constituyéndose en una zona ósea débil. Su cara lateral, la lámela lateral de la lámina cribosa, se articula con el hueso frontal. Esta unión habitualmente muestra zonas de baja densidad ósea a veces normal pero ante la sospecha de fístula espontánea de LCR, deben ser examinadas minuciosamente sin descartar otras cavidades paranasales y los oídos. Objetivo: Mostrar la técnica de reparación de la fístula de la lámela lateral por cirugía endonasal endoscópica. Material y método: Se utilizó una misma técnica quirúrgica en tres diferentes pacientes. Se describe la técnica en detalle y luego se hace un análisis de la fisiopatología, diagnóstico y tratamiento de esta patología en relación a la presentación de tres casos. Resultados: El seguimiento posquirúrgico de los casos presentados fue de entre 12 y 18 meses, no observando recidiva en ninguno de ellos.

The clinical manifestation and management of Mondini dysplasia / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To improve the possibility of diagnosis of the Mondini dysplasia.@*METHOD@#The clinical manifestation and the examination of CT and MRI and surgical treatment of the Mondini dysplasia were discussed.@*RESULT@#Because Mondini dysplasia with cerebrospinal fluid leak would occur recurrent meningitis, the patients were often difficult to be diagnosed. Especially. if the defect was unilateral, it was frequently unrecognized.@*CONCLUSION@#The patients with recurrently unclear cerebrospinal fluid leak and meningitis would be suspected. The diagnosis of the disease is based on the examination of the temporal bone CT and MRI. To the patients with cerebrospinal fluid leak, a transtympanic closure is one of very effective management.

Management of operative complications in acoustic neuroma surgery / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To explore the common complications related to acoustic neuromas and to search methods for preventing from them.</p><p><b>METHODS</b>One hundred and five patients with acoustic neuromas underwent 110 operations with the retrosigmoid approach, middle cranial fossa approach and labyrinth approach. All cases were followed up more than 1 month after surgery.</p><p><b>RESULTS</b>Of 110 cases, the most frequent complication was hearing disability which occurred in 95 cases (95/110, 86.4%). and the facial paralysis was in 63.6% (70/110) after one month. Other complications were cerebrospinal fluid fistulas (CSF, 12.7%, 14/110) , intracranial hematoma (5.5%, 6/110), cranial nerve palsies (4.5%, 5/110), meningitis (3.6%, 4/110), tetraparesis (3.6%, 4/110), balance disturbance (1.8%, 2/110), hemiparalysis and anepia (0.9%, 1/110). Effective stopping bleeding during operation and controlling blood pressure after operation, as well as keeping effective sedation in 24 hours after operation were the important ways to prevent from intracranial hematoma The haemorrhage often accrued in 48 hours post-operation. CSF in this series was another common complication in acoustic neuroma surgery. Ten cases with CSF subcutaneous retro-auricular had been successfully controlled by conservative treatment. Of 4 cases with rhinorrhea CSF, 3 of them were required surgical management, another one got self-cure by bed rest. The ventricular drainage pro-operation was the most important procedure for drawdown the hypsi-cranium pressure.</p><p><b>CONCLUSIONS</b>The key factors to avoid the complications include mastering the anatomy of different surgical approach, how much of the tumor size, surgical experience and preoperative evaluation of patients' imaging information and other clinical data.</p>

A Case of Congenital Inner Ear Anomaly Combined with Cerebrospinal Leak Using Hydroxyapatite Cement for Treatment / 대한이비인후과학회지

Cerebrospinal fluid otorrhea is a rare disease entity, especially when brought on by spontaneous causes. The site of leakage should be identified to treat the disorder effectively. Various localizing modality was suggested in the literatures. Intrathecal gadolinium-enhanced magnetic resonance imaging identifies the small leakage site easily and correctly. Hydroxyapatite cement is a calcium phosphate-based material that forms dense paste mixed with water or sodium phosphate solution. Hydroxyapatite cement is remarkable for its excellent biocompatability in interacting with the native bone to osseointergrate with the surrounding bone. We report on our experience of successfully treating a patient with the cerebrospinal fluid leak from the congenital inner ear anomaly using the hydroxyapatite cement.

Evaluation of MR cisternography in diagnosis of cerebrospinal fluid fistula.

PURPOSE: To study the clinical usefulness and sensitivity of MR cisternography as noninvasive study in the diagnosis of CSF fistula in patients with clinical diagnosis CSF rhinorrhea or otorrhea. METHOD: Thirty-five patients with clinically diagnosed CSF leakage were examined for site of dural tear with MR cisternography with additional plain high-resolution CT in some cases from Jan. 1999 to Dec.2002. The MR imaging technique was performed as a heavily T2 weighted fast spin echo study with fat suppression in axial, coronal and sagittal projections. Criteria for positive results were demonstrable fistular tract connecting subarachnoid space to paranasal sinus/petrous bone, and/or dural discontinuity, and/or bone defect with pneumocephalus, and/or presence of brain herniation. Eighteen of the patients subsequently had exploratory surgery for fistula. Sensitivity analysis of the surgical results was compared with the findings at MR cisternography. RESULT: MR cisternography showed significant correlation with surgical findings with sensitivity of 89%. Additional high resolution CT were complete agreement with site of fistular tract. The pathogenesis of CSF leakage was related to trauma (86%). The commonly found dural/bony defect and location of connecting fislular tract were cribriform plate and ethmoid sinus of 58%, 55% respectively. CONCLUSION: In the presence of clinically diagnosed CSF leakage, the combination of MR cisternography and plain high-resolution CT are highly accurate in locating the site and extent of CSF fistula and should be considered a viable noninvasive alternative to CT cisternography and Tc-99m-DTPA cisternography.