Faculty Bibliography

Objective Jugular bulb abnormalities (JBA), including high-riding jugular bulb (HRJB) and jugular bulb diverticulum (JBD), can erode into the inner ear. In this study, the authors investigate the prevalence and consequences of JBA and their erosion into inner ear structures using temporal bone histopathology and computed tomography (CT). Study Design Cross-sectional study of temporal bone histopathology and radiology. Setting Academic medical center. Subjects and Methods In total, 1579 temporal bone specimens and 100 CT of the temporal bones (200 ears) were examined for JBA and any associated dehiscence of inner ear structures. Temporal bone specimens were examined for histological consequences of inner ear erosion. Jugular bulb dimensions were measured on axial CT scans and compared across groups. Accompanying demographic and clinical information were reviewed. Results High jugular bulbs were noted in 8.2% (130/1579) of temporal bone specimens and in 8.5% (17/200) of temporal bone CT. The prevalence of JBA increases during the first 4 decades of life and stabilizes thereafter. High-riding jugular bulbs eroded inner ear structures such as the vestibular aqueduct, vertical facial nerve, or posterior semicircular canal in 2.8% (44/1579) of cases histologically and 1.5% (3/200) radiologically. In most, jugular bulb-mediated inner ear dehiscence was clinically and radiologically silent. Conclusion Jugular bulb abnormalities are common. They are present in 10% to 15% individuals and are primarily acquired by the fourth decade of life. In 1% to 3% of cases, the HRJB erodes into the inner ear and most frequently involves the vestibular aqueduct.

OBJECTIVE: Jugular bulb abnormalities (JBA), such as jugular bulb diverticula (JBD) or large jugular bulbs, rarely present in the middle ear. We review a large series of temporal bone histopathologic specimens to determine their prevalence and present a series of cases of JB abnormalities involving the middle ear (JBME) that shed light on the probable mechanism for their development. PATIENTS: 1,579 unique temporal bone specimens and individuals with radiographically-diagnosed JBME. INTERVENTION: Histopathologic and clinical review of temporal bone specimens and patient presentations, radiographic findings, treatments and outcomes. MAIN OUTCOME MEASURE: Shared characteristics of JBME. RESULTS: There were 17 cases of JBME in 1,579 temporal bone (1.1%), of which, 15 involved the inferior mesotympanum below the level of the round window membrane (RWM), whereas 2 encroached upon the RWM or ossicles. In addition, 4 clinical cases of large JBME extending above RWM were encountered; these occurred in both sexes with ages spanning from young to old (7-66 yr). They presented with conductive hearing loss (n = 3), ear canal mass (n = 1), and intraoperative bleeding (n = 1). Radiologically, they had multiple diverticula of the JB on the side with JBME, with 1 patient demonstrating growth on serial imaging studies. All patients who underwent additional imaging had marked hypoplastic contralateral transverse sinus. CONCLUSION: JBME abnormalities are rare, present across age groups, and may demonstrate serial growth over time. They are usually associated with multiple other diverticula within the same JB. Our clinical series suggests that JBME's development and uniquely aggressive behavior results from contralateral transverse sinus outflow obstruction.

OBJECTIVE: To describe and characterize diplopia resulting from skew deviation after cerebellopontine angle (CPA) surgery and labyrinthectomy. PATIENTS AND INTERVENTIONS: Retrospective case series of 4 patients who developed vertical diplopia from skew deviation after resection of tumors in the CPA or labyrinthectomy MAIN OUTCOME MEASURE: Complete neuro-opthalmologic examination including opticokinetic testing, confrontational visual field assessment, color plate, pupillary reflex, slit lamp examination, and head tilt test. RESULTS: Four patients with residual hearing preoperatively developed skew deviation immediately after surgical intervention, including translabyrinthine (n = 1) and retrosigmoid (n = 2) approaches to the CPA and labyrinthectomy (n = 1). Neuro-ophthalmologic examination demonstrated intact extraocular movements, and 2- to 14-mm prism diopter hypertropia on both primary gaze and head tilt testing. In all cases, skew deviation resolved spontaneously with normalization of the neuro-ophthalmologic examination within 10 weeks. CONCLUSION: Patients undergoing CPA surgery or labyrinthectomy can develop postoperative diplopia due to skew deviation as a consequence of acute vestibular deafferentation. Patients with significant hearing preoperatively, a probable marker for residual vestibular function, may be especially at risk for developing skew deviation postoperatively.

OBJECTIVES/HYPOTHESIS: CHARGE (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and/or deafness) syndrome is a genetic disorder with prominent otolaryngologic features including choanal atresia and inner ear malformations. Recent experience with venous malformations during cochlear implant surgery prompted this study to define the spectrum of venous abnormalities in CHARGE and their surgical implications in otology. STUDY DESIGN: Retrospective review of medical and radiologic records from databases of patients with CHARGE syndrome from three tertiary care academic medical centers. METHODS: Eighteen patients with CHARGE for whom temporal bone CT scans were available were included in the review. RESULTS: Venous anomalies of the temporal bone were present in 10 of 18 (56%) patients. The most common were large emissary veins (n = 5). In two of these cases, these veins were associated with an ipsilateral a hypoplastic sigmoid sinus or jugular foramen. Other abnormalities included an aberrant petrosal sinus, venous lakes in proximity to the lateral venous sinus, condylar canal veins, and jugular bulb abnormalities, including a high riding bulb obscuring the round window niche and a dehiscent jugular bulb. In four of six patients undergoing cochlear implantation, the course of the aberrant vessel necessitated a change in the surgical approach, either during mastoidectomy or placement of the cochleostomy. CONCLUSIONS: Temporal bone venous abnormalities are a common feature in CHARGE syndrome. The pattern of venous abnormality suggests that there is a failure of the sigmoid sinus/jugular bulb to fully develop, resulting in persistence of emissary veins. Recognition of these abnormal venous structures during otologic surgery is critical to avoiding potentially catastrophic bleeding. Laryngoscope, 2012.

OBJECTIVE: There have been 283 cases of meningitis after cochlear implantation reported since 2002 to the CDC Database Manufacturer and User Facility Device Experience, resulting in 30 fatalities. This is the latest of a number of reports to track the continuing problem of meningitis occurring in cochlear implant users, mainly children. RESULTS: Although a number of these patients had received a device with a 2-piece electrode, the withdrawal of that particular device has not prevented further cases from occurring. There are many potential etiologic factors potentially involved in postimplantation bacterial meningitis; although some can be managed or eliminated, vaccination against the most prevalent causal organism, the streptococcus pneumoniae, seems to be a powerful tool against this uncommon but serious complication of cochlear implantation. CONCLUSION: Although up to 20 new cases of post-cochlear implantation meningitis continue to occur annually worldwide, considering the number of new devices implanted each year, it is expected that the incidence of this complication seems to be diminishing

OBJECTIVE: To further define the spectrum of clinical presentation and explore the histologic sequelae of jugular bulb abnormalities (JBAs). DESIGN: Retrospective review. SETTING: Academic medical center. PATIENTS: Thirty patients with radiologic evidence of inner ear dehiscence by JBA. MAIN OUTCOME MEASURE: Thirty patients with radiologic inner ear dehiscence by JBA and 1579 temporal bone specimens were evaluated for consequences from JBA. RESULTS: We found that JBA-associated inner ear dehiscence could be identified on computed tomography of the temporal bone but not on magnetic resonance imaging scan. Jugular bulb abnormalities eroded the vestibular aqueduct most often (in 25 patients), followed by the facial nerve (5 patients) and the posterior semicircular canal (4 patients). Half of the patients (15) were asymptomatic. Results from vestibular evoked myogenic potential (VEMP) tests were positive in 8 of 12 patients with inner ear dehiscence. Histologically, only 2 of 41 temporal bones with dehiscence of the vestibular aqueduct demonstrated endolymphatic hydrops. CONCLUSIONS: Jugular bulb abnormalities can erode into the vestibular aqueduct, facial nerve, and the posterior semicircular canal. While symptoms may include pulsatile tinnitus, vertigo, or conductive hearing loss, in contrast to earlier reports, half of the patients were asymptomatic. Dehiscence of vestibular aqueduct rarely leads to clinical or histologic hydrops. The VEMP testing was useful in confirming the presence of inner ear dehiscence due to JBAs. Because the natural history of JBAs is unknown, these patients should be followed closely to evaluate for progression of the JBA or development of symptoms