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.
Male ; Female ; Child ; Humans ; Aged ; Infant ; Child, Preschool ; Cerebrospinal Fluid Otorrhea/surgery* ; Retrospective Studies ; Vestibule, Labyrinth ; Temporal Bone ; Ear, Middle

Cerebrospinal Fluid Otorrhea/surgery* ; Child ; Humans ; Temporal Bone

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.
Adolescent ; Adult ; Cerebrospinal Fluid Otorrhea/therapy* ; Child ; Child, Preschool ; Ear, Inner/abnormalities* ; Humans ; Infant ; Middle Aged ; Retrospective Studies ; Young Adult

OBJECTIVETo review the clinical characteristics of spontaneous intracranial hypotension (SIH) with cerebrospinal fluid (CSF) leakage.

METHODSClinical 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.

RESULTSThirty-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.

CONCLUSIONAll 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.

Adolescent ; Adult ; Aged ; Cerebrospinal Fluid Otorrhea ; complications ; Cerebrospinal Fluid Rhinorrhea ; complications ; Female ; Humans ; Intracranial Hypotension ; diagnosis ; etiology ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

Cerebrospinal Fluid Otorrhea ; surgery ; Craniotomy ; Encephalocele ; Humans ; Mastoid ; surgery

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.
Arachnoid ; Cerebrospinal Fluid ; Cerebrospinal Fluid Otorrhea* ; Cranial Fossa, Middle* ; Ear ; Female ; Humans ; Middle Aged ; Recurrence ; Temporal Bone

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.
Arachnoid ; Cerebrospinal Fluid Otorrhea ; Cholesteatoma ; Ear, Middle ; Granulation Tissue ; Rare Diseases

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.
Arachnoid ; Cerebrospinal Fluid Otorrhea ; Cholesteatoma ; Ear, Middle ; Granulation Tissue ; Rare Diseases

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.
Cartilage ; Cerebrospinal Fluid Otorrhea ; Cholesteatoma ; Ear ; Epilepsy ; Fascia ; Hearing Loss, Conductive ; Humans ; Meninges ; Meningitis ; Meningocele ; Otitis Media with Effusion ; Skull Base ; Temporal Bone ; Tinnitus

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.
Adolescent ; Adult ; Cerebrospinal Fluid Otorrhea ; diagnostic imaging ; Child ; Child, Preschool ; Ear, Inner ; diagnostic imaging ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Tomography, X-Ray Computed ; methods ; Young Adult