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.

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.

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.

OBJECTIVES/HYPOTHESIS Cochlear implantation (CI) is effective in the treatment of childhood sensorineural hearing loss and is associated with minimal surgical complications. We investigated the incidence of anesthetic complications in young patients undergoing general anesthesia for CI. STUDY DESIGN Retrospective chart review. METHODS A retrospective chart review of 123 patients younger than 18 years, who underwent CI between 2007 and 2008, was conducted for identification of intra- and postoperative anesthesia-related complications. The relation of collected variable to the complication events was analyzed using logistic regression. RESULTS Of the 123 CI procedures, eight patients had nine anesthesia-related complications, yielding a complication rate of 6.5% and included the following: postoperative wheezing/stridor (5 cases), laryngospasm (3 cases), and emesis during inhalational induction (1 case). Divided by age group, 12 patients were <12 months with one complication (8%), 18 patients were between 1 and 2 years with one complication (5.6%), 35 patients were between 2 and 5 years with one complication (3%), 39 patients were between 5 and 12 years with five complications (13%), and 19 patients were older than 12 years with no complication (0%). Logistic regression failed to identify a significant association of any collected variable(s) with the observed complications. The incidence of complications is similar to that previously reported in elderly patients (4.3%)(Pearson χ(2), P =.523). CONCLUSIONS General anesthesia is well tolerated by pediatric patients undergoing CI, even under 1 year of age. Significant perioperative complications are primarily respiratory, are usually free of long-term sequelae, and occur with an incidence similar to other reported age groups.

OBJECTIVE:: Jugular bulb (JB) abnormalities such as JB diverticulum and high-riding JBs of the temporal bone can erode into the inner ear and present with hearing loss, vestibular disturbance, and pulsatile tinnitus. Their cause and potential to progress remain to be studied. This comprehensive radiologic study investigates the postnatal development of the venous system from transverse sinus to internal jugular vein (IJV). SETTING:: Academic medical center. PATIENTS, INTERVENTION, MAIN OUTCOME MEASURE:: Measurements of the transverse and sigmoid sinus, the JB, IJV, and carotid artery were made from computed tomographic scans of the neck with intravenous contrast in infants (n = 5), children (n = 13), adults (n = 35), and the elderly (n = 15). RESULTS:: Jugular bulbs were not detected in patients younger than 2 years, enlarged in adulthood, and remained stable in the elderly. The venous system was larger in men than in women. From transverse sinus to IJV, the greatest variation in size was just proximal and distal to the JB with greater symmetry observed as blood returned to the heart. Right-sided venous dominance was most common occurring in 70% to 80% of cases. CONCLUSION:: The JB is a dynamic structure that forms after 2 years, and its size stabilizes in adulthood. The determinants in its exact position and size are multifactorial and may be related to blood flow. Improved understanding of this structure's development may help to better understand the cause of the high-riding JB and JB diverticulum, both of which may cause clinical symptoms.

OBJECTIVE In 2002, the electrode combined with positioner was identified as a significant risk factor for postimplantation meningitis and was voluntarily discontinued by the manufacturer. The ongoing risk of meningitis with passage of time with the positioner remains unknown. The objective of this study was to determine the current risk of meningitis in patients implanted with the positioner. STUDY DESIGN Data regarding the date of implantation, report of meningitis, and presence or absence of risk factors for meningitis (otitis media, inner ear malformation) were obtained from the clinical database maintained by the manufacturer. RESULTS There were 8,329 devices implanted that may have used a positioner, yielding a cumulative postimplantation time of 74,976 patient-years. The incidence of meningitis during the 3-year spans of 1999 to 2001, 2002 to 2004, 2005 to 2007, and 2008 to 2010 was 33, 40, 11, and 2, respectively; the risk of meningitis in cases per patient-years has decreased significantly from 0.0044 at its peak in 2001 to 0.0011 in 2010. Only a single case of positioner-related meningitis 96 months or longer after implantation has been reported. More than 40% of children had otitis media within 1 week of developing meningitis; less than 10% had a history of meningitis or inner ear malformation. CONCLUSION The risk of meningitis with the use of positioner is highest within the first 24 months after implantation and is frequently associated with otitis media. The risk of meningitis more than 96 months after implantation is significantly reduced. Thus, the prophylactic removal of the positioner is not recommended in patients who underwent implantation more than 8 years ago.

OBJECTIVE To characterize bacterial microbiota in middle ear, adenoid, and tonsil specimens using 16SrRNA gene-based pyrosequencing analysis. DESIGN Cross-sectional study of bacterial microbiota in middle ear, adenoid, and tonsil specimens from a pediatric patient with chronic serous otitis media. Middle ear, adenoid, and tonsil specimens from a pediatric patient were collected and underwent cell lysis and DNA isolation. Pyrosequencing was performed on the 454 Life Sciences GS FLX platform (Roche Diagnostics Corp, Branford, Connecticut). Pyrosequencing data were processed, quality-checked, and taxonomically classified to generate an abundance-based matrix. Ecological analyses were performed. SETTING Academic, tertiary referral center. MAIN OUTCOME MEASURES Comparative microbiome analysis. RESULTS We detected a total of 17 unique bacterial families, with 9, 9, and 12 bacterial families from the middle ear, tonsil, and adenoid specimens, respectively. Pseudomonadaceae dominated the middle ear microbiota at 82.7% relative abundance, whereas Streptococcaceae dominated the tonsil microbiota at 69.2%. Multiple bacteria, including Pseudomonadaceae, Streptococcaceae, Fusobacteriaceae, and Pasteurellaceae, dominated the adenoid microbiota. Overlap between the middle ear and the tonsil microbiota was minimal. In contrast, the adenoid microbiota encompassed bacteria detected from middle ear and tonsil. CONCLUSIONS Bacterial community analysis using pyrosequencing analysis revealed diverse, previously unknown bacterial communities in a set of pediatric middle ear, tonsil, and adenoid specimens. Our findings suggest that the adenoid may be a source site for both the middle ear and tonsil microbiota. An ecological framework is appropriate in comparative analysis of microbiota from nonsterile body sites.

OBJECTIVE To investigate the hypothesis that second-hand smoke (SHS) exposure is associated with sensorineural hearing loss (SNHL) in adolescents. DESIGN A complex, multistage, stratified geographic area design for collecting representative data from the noninstitutionalized US population. PARTICIPANTS Cross-sectional data from National Health and Nutrition Examination Survey (2005-2006) were available for 1533 participants 12 to 19 years of age who underwent audiometric testing, had serum cotinine levels available, and were not actively smoking. MAIN OUTCOME MEASURES SNHL was defined as an average pure-tone level greater than 15 dB for 0.5, 1, and 2 kHz (low frequency) and 3, 4, 6, and 8 kHz (high frequency). RESULTS Secondhand smoke exposure, as assessed by serum cotinine levels, was associated with elevated pure-tone hearing thresholds at 2, 3, and 4 kHz, a higher rate of unilateral low-frequency SNHL (11.8% vs 7.5%; P <.04), and a 1.83-fold increased risk of unilateral low-frequency SNHL in multivariate analyses (95% confidence interval, 1.08-3.41). The prevalence of SNHL was directly related to level of SHS exposure as reflected by serum cotinine levels. In addition, nearly 82% of adolescents with SNHL did not recognize hearing difficulties. CONCLUSIONS Secondhand smoke is associated with elevated pure-tone thresholds and an increased prevalence of low-frequency SNHL that is directly related to level of exposure, and most affected individuals are unaware of the hearing loss. Thus, adolescents exposed to SHS may need to be closely monitored for early hearing loss with periodic audiologic testing.