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.

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.

Successful Onyx embolization of a giant glomus jugulare: case report and review of nonsurgical treatment options.

BACKGROUND: Otorrhagia is an uncommon but severe symptom of patients with large glomus jugulare tumors that erode through the tympanic membrane. In this case report we describe the use of transarterial embolization for long-term palliative management of otorrhagia in a patient with an unresectable glomus jugulare tumor. CASE DESCRIPTION: A 53-year-old woman presented with intermittent otorrhagia 10 years after subtotal resection of a glomus jugulare tumor. Follow-up magnetic resonance imaging showed progressive enlargement of the tumor with significant extension into the posterior fossa. Resection was thought to be impractical; therefore, transarterial embolization was offered as a palliative measure to help reduce the frequency and severity of bleeding episodes. RESULTS: Long-term control of otorrhagia was achieved after three rounds of intra-arterial embolization. In round one, the tumor was embolized from multiple external carotid artery feeding branches by the use of polyvinyl alcohol particles. In round two, the internal carotid artery was sacrificed by embolizing the cavernous and petrous segments with coils. In round three, persistent feeders from the cervical internal carotid artery were embolized with Onyx. In 6 years of clinical follow-up, the patient has had no otorrhagia or new neurological deficits. Serial magnetic resonance imaging revealed there has been no significant interval tumor growth. CONCLUSION: Long-term control of otorrhagia from glomus jugulare tumors can be safely achieved by intravascular embolization with Onyx.

The role of obesity in spontaneous temporal bone encephaloceles and CSF leak.

OBJECTIVE: Temporal bone encephaloceles and cerebrospinal fluid (CSF) otorrhea can lead to life-threatening sequelae such as meningitis and cerebritis. Obesity has been associated with spontaneous CSF leaks. We wished to determine if there were differences in the body mass index (BMI) of patients with spontaneous temporal bone encephaloceles/CSF leaks and nonspontaneous temporal bone encephaloceles/CSF leaks to help determine if obesity may be associated with their development. STUDY DESIGN: Single institution retrospective comparison. METHODS: Demographic and clinical information was obtained from 20 patients treated for surgically confirmed temporal bone dehiscence leading to encephalocele and/or CSF otorrhea. Statistical analysis was performed on patients with spontaneous and nonspontaneous encephaloceles/CSF leaks to determine if there were differences in the degree of obesity between these groups. RESULTS: Temporal bone dehiscence was spontaneous in 11 patients, related to chronic infection in 4 patients, posttraumatic in 3 patients, postsurgical in 1 patient, and because of massive hydrocephalus in a final patient. The average BMI for patients with spontaneous encephalocele was 33.4 kg/m. The average BMI for patients with nonspontaneous encephaloceles was 27.0 kg/m. This difference was statistically significant (p = 0.02). CONCLUSION: Although several previous studies have demonstrated an association of obesity with spontaneous encephaloceles or CSF leaks, this study is one of the first to demonstrate a statistically significant increase in BMI for patients with spontaneous encephaloceles and CSF leaks as compared with patients developing them for other reasons. This suggests a potential etiologic role of obesity in the development of spontaneous encephaloceles and CSF leaks. LEVEL OF EVIDENCE: 4.

Oxymetazoline ototoxicity in a chinchilla animal model.

OBJECTIVE: To investigate possible ototoxic effects of a one-time application of oxymetazoline drops in a chinchilla animal model with tympanostomy tubes. Study Design. A prospective, controlled animal study. SETTING: The Research Institute of the Montreal's Children Hospital, McGill University Health Centre. SUBJECTS AND METHODS: Ventilation tubes were inserted in both ears of 12 animals. One ear was randomly assigned to receive oxymetazoline drops (0.5 mL). The contralateral ear did not receive any drops, serving as a control ear. OUTCOME MEASURES: Distortion product otoacoustic emissions were measured bilaterally for a wide range of frequencies (between 1 and 16 kHz) before and 1 day after the application of oxymetazoline in the experimental ears. Two months later, the animals were sacrificed and all cochleae were dissected out and processed for scanning electron microscopy. RESULTS: In this established chinchilla animal model, the measured distortion product otoacoustic emission amplitudes and the morphological appearance on scanning electron microscopy were similar for both control and experimental ears. CONCLUSION: Oxymetazoline did not cause ototoxicity in a chinchilla animal model 2 months after a single application via a tympanostomy tube.

Arachnoid granulations of the temporal bone: a histologic study of dural and osseous penetration.

HYPOTHESIS: Arachnoid granulations (AG) are more prevalent along the middle fossa surface of the temporal bone, where they produce larger bony defects than those occurring on the posterior surface. BACKGROUND: Dural and bony defects formed by AGs are proposed to lead to spontaneous meningoencephaloceles and cerebrospinal fluid otorrhea. They most commonly occur at the tegmen and in individuals older than 40 years. METHODS: Vertically sectioned temporal bones were evaluated using light microscopy to determine AG histology, distribution, and morphometry and to determine the prevalence of AG penetration in the donor population. RESULTS: AGs were observed to penetrate the dura mater and make direct contact with cortical surfaces in 12.7% of donors in the Johns Hopkins Temporal Bone Collection. AGs occurred at middle fossa sites 13% more frequently than at posterior fossa sites. At middle fossa sites AGs produced significantly larger bony openings and were more likely to be associated with herniating brain tissue. Donors with AGs were significantly older, and all were in the late 30s or older. CONCLUSION: Erosion of the temporal bone by AGs is not a rare occurrence in the population and becomes increasingly prevalent with age. It is estimated that 14 in 1,000 donors were at greatest risk of eventual cerebrospinal fluid leakage at the tegmen. The age and anatomic distribution described in this study strengthens the notion that AG penetration plays a role in the pathophysiology of spontaneous cerebrospinal fluid leaks and meningoencephaloceles of the temporal bone.

Stereoscopic virtual reality presurgical planning for cerebrospinal otorrhea.

We present a complicated case of spontaneous cerebrospinal otorrhea, which had not been cured despite undergoing 5 surgical interventions in the past. The disability to identify the location of the fistula was the main crux of the past failures. On this occasion, stereoscopic virtual reality presurgical planning was applied to identify the exact location of the fistula and a surgical simulation was performed, and was later confirmed during the actual operation. Interactive manipulation in a stereoscopic virtual environment makes the decision making process easier in the treatment of cerebrospinal otorrhea.

Diagnosis and management of otologic cerebrospinal fluid leak.

This article categorizes cerebrospinal fluid leaks as traumatic or nontraumatic in origin. It explains the physiology of the milieu of cerebrospinal fluid that surrounds the brain and spinal cord. It then discusses the detection, assessment, causes, clinical presentation, and management related to clinical pathologies.

Surgical considerations in cochlear implantation in children and adults: a review of 342 cases in Vienna.

CONCLUSIONS: Our data represent the experience of the largest cochlear implant program in Austria. In conclusion, cochlear implantation is a safe procedure, associated with a low rate of intra- and postoperative complications. Nevertheless, patients should be informed about possible problems and especially about the risk of a reoperation due to device failure. OBJECTIVES: To evaluate the cause of deafness, the intraoperative findings and the complication rate for all cochlear implant operations performed consecutively between 1994 and 2003 at Vienna General Hospital. MATERIAL AND METHODS: Including all surgeries for bilateral implantation and revision, a series of 342 operations performed on 164 adults (age range 14.5-81 years; mean age 50.79 years) and 128 children (age range 0.75-14 years; mean age 5.00 years) was retrospectively analyzed. RESULTS: The etiology of deafness was predominantly congenital or progressive (66.89%). The routine mastoidectomy approach was chosen in 300 patients (87.72%) and the suprameatal approach in 42 (12.28%). Intraoperatively, 4 children (2.53%) had a cerebrospinal fluid fistula and 35 patients (10.23%) showed cochlear ossification. Three adults (1.63%) and two children (1.27%) had facial nerves with an aberrant course. The overall complication rate was 12.2%, the rate of major complications was 4.97% and the rate of minor complications was 4.09%. There were no cases of either postoperative meningitis or facial nerve palsy. Both flap necrosis and electrode dislocation occurred in one adult patient (0.54%), but in none of the children. Formation of cholesteatoma was found in one adult (0.54%) and one child (0.63%). The rate of device failure was 7.07% for adults and 13.92% for children.

Mucoepidermoid carcinoma arising from the eustachian tube and middle ear.

We report a case of mucoepidermoid carcinoma (MEC) originating from the eustachian tube and middle ear. A 31-year-old male who presented with otorrhoea and methicillin-resistant Staphylococcus aureus (MRSA) in the right ear was admitted to hospital due to cerebral infarction and deep vein thrombosis. After recovery, biopsies from a granulomatous mass found in the middle ear during operation for chronic otitis media revealed intermediate-grade MEC and a nasopharyngeal mass identified after surgery also revealed the same result. He received combined radiation therapy and chemotherapy and no residual or recurrent tumour was detected after two years of follow-up.

[Cerebro-spinal fluid otorrhea and a spontaneous defect of the tegmen tympani or antri. A report of 3 cases . Rôle of arachnoid granulations]. / Otorrhée cérébro-spinale et brèche spontanée du tegmen tympani ou antri. A propos de 3 cas. Rôle des granulations arachnoïdiennes.

UNLABELLED: Less than 150 cases of cerebro-spinal fluid leak with spontaneous defect of the roof of the temporal bone have been described in the international literature. THE AIM OF THIS WORK: Is to define this pathology, to describe the clinical features, to suggest a diagnostic strategy, and to clarify the treatment method and the hypotheses on causation. MATERIALS AND METHODS: This is a retrospective study of 3 cases. RESULTS: At the first medical examination, the most common clinical feature is serous otitis media or otorrhea after myringotomy. Rhinorrhea is rarely pointed out by the patients but exists in our 3 observations. The diagnosis of cerebro-spinal fluid leak with spontaneous defect of the roof of the temporal bone needs; cerebro-spinal fluid leakage, absence of an otologic history or cranial trauma and a bony defect on CT scan. CT scan with millimeter slices is able to show the location and the size of the bony defect(s) of the roof of the temporal bone and often shows partial or total opacity of the middle ear cavities. MRI is able to show if this opacity exists in conjunction with meningeal hernia or cerebro-meningeal hernia. Surgical repair consists of placing an autologous graft over the bony defect by the middle fossa approach. The origin of a spontaneous defect of the temporal bone is discussed. We study the hypothesis in which arachnoïd granulations could be responsible for a temporal bone defect.

[Dural bone defects and encephalocele associated with chronic otitis media or its surgery]. / Kronik otit veya cerrahisi sonrasinda olusan dural kemik defektleri ve ensefalosel.

OBJECTIVES: We investigated the incidence of dural bone defects and encephalocele following radical mastoidectomy or open cavity tympanoplasty. PATIENTS AND METHODS: We retrospectively evaluated 190 operations of 182 patients (97 males, 85 females; mean age 30.6 years; range 9 to 70 years) who underwent radical mastoidectomy (n=89) or open cavity tympanoplasty (n=101). Follow-up examinations included otomicroscopy. Patients with documented dural bone defects on examination or operation records were further evaluated by axial and coronal computed tomography scans of the temporal bone. Those with suspected encephalocele were studied by magnetic resonance imaging. RESULTS: Dural bone defects were detected in 17 patients perioperatively. Of these, 14 defects were associated with chronic otitis media. Three iatrogenic bone defects were induced during obliteration of lateral sinus thrombosis. The mastoid tegmen was the most common site of defects (41%) and the most common etiologic factor was cholesteatoma (84%). Encephalocele was detected in one patient in whom radical mastoidectomy with lateral sinus obliteration was performed due to lateral sinus thrombophlebitis. Encephalomalasia was found nearby the defect area in two patients. CONCLUSION: Dural bone defects associated with chronic otitis media are not rare, the most common cause being cholesteatoma. Repair of dural lacerations requires watertight closure to prevent the occurrence of encephalocele.

Skull base trauma: diagnosis and management.

The singular anatomical relationship of the base of the skull is responsible for the particular problems that may arise after injury. Extensive dural laceration and severe neurovascular damage may accompany skull base injuries. Trauma to the anterior skull base is frequently related to the paranasal sinuses, and trauma to the middle and the posterior skull base usually affects the petrous bone. Injury to the anterior fossa including the paranasal sinuses may produce CSF leakage, damage the olfactory nerves, optic nerves, and orbita contents. Fractures may affect the carotid canal, injure the internal carotid artery and result in carotid-cavernous fistula. Trauma to the petrous bone may cause facial palsy and deafness, and CSF leakage with otorrhoea or paradoxal rhinoliquorrhoea. Trauma to the posterior fossa may lacerate the major venous sinuses, and affect the cranio-cervical stability. Each one of these injuries will need a particular strategy. Decision making for management as a whole must consider all aspects, including the fact that these injuries frequently involve polytraumatized patients. Decisions regarding the timing of surgery and the sequence of the surgical procedures must be made with great care. Modern surgical techniques and recent technologies including functional preservation of the olfactory nerves in frontobasal trauma, visual evoked potentials, assisted optic nerve decompression, facial nerve reconstruction, interventional technique for intravascular repair of vascular injuries, and recent developments in cochlea implants and brain stem implants, all contributed significantly to improve outcome and enhance the quality of life of patients. This article reviews basic principles of management of skull base trauma stressing the role of these advanced techniques.