Hallermann-Streiff syndrome, also known as oculomandibulofacial syndrome, is a rare congenital disorder affecting growth and cranial, dental, ocular, pilocutaneous, and mental development. In addition to routine audiologic testing in patients with this syndrome, high-resolution computed tomography of the temporal bones should be performed in those with documented or suspected sensorineural hearing loss. Cochlear implantation may be considered, as in other children with syndromic hearing loss and certain inner ear abnormalities, if the appropriate audiologic, psychosocial, and medical criteria are met. The current case report radiographically and clinically characterizes inner ear dysplasia in an 8-year-old patient with Hallermann-Streiff syndrome. High-resolution computed tomography of the temporal bones revealed a hypoplastic bony island between the vestibule and horizontal semicircular canals, as well as incomplete bony coverage of the posterior semicircular canal crura bilaterally. To our knowledge, this is the first report of a pediatric patient demonstrating bilateral posterior semicircular canal dehiscence.
Perform an updated meta-analysis investigating differences in complication rates, procedure times, and costs between percutaneous dilational tracheotomy (PDT), operating room surgical tracheotomy (ST), and bedside surgical tracheotomy (BST).Meta-analysis using the Mantel-Haenszel fixed effects model. Prospective and randomized trials comparing ST with PDT from 1999 to present were identified through two independent searches. Outcome measures analyzed included mortality, early complications, late complications, procedure times, and cost.Fourteen studies were identified (1,273 patients) that satisfied the search criteria. Analysis of randomized studies demonstrated significantly more minor early complications with PDT compared with ST (odds ratio [OR], 1.6; 95% confidence interval [CI], 1.01-2.66). Randomized studies comparing PDT with BST demonstrated significantly more early complications with PDT (OR, 4.66; 95% CI, 1.46-14.91). There was no significant difference in late serious complications in studies that randomized patients to ST or PDT (OR, 1.39; 95% CI, 0-16,000). PDT is significantly faster to perform than ST (OR, 0.51; 95% CI, 95% 0.49-0.53). BST and PDT have similar costs, and both are less expensive than ST.Although significantly faster than ST, PDT has more early complications compared with open tracheotomy in the operating room or at the bedside. The long-term complications of the two techniques appear comparable but have not been thoroughly investigated. These findings suggest that a team approach between surgeons and critical care specialists is essential to select the appropriate tracheotomy technique for a given patient.
Objective: Determine treatment outcomes of stereotactic radiosurgery (SRS) for glomus jugulare tumors (GJT), focusing on three-dimensional volume change and symptoms before and after SRS, as well as complications related to SRS. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Thirty-eight patients treated with SRS between 2000 and 2015. Intervention: SRS treatment of GJT. Main Outcome Measures: The tumor volumes on pre- and posttreatment imaging were compared utilizing the Leskell GammaPlan treatment plan software to assess tumor progression. Pre- and posttreatment symptoms, Fisch classification, and complications were recorded. Results: The mean radiographic follow-up was 39.1 months. The mean dose-to-tumor margin was 13.2 Gy. The mean tumor size at treatment was 5.8 and 5.2 cm 3 at last follow-up. Thirty-three patients had follow-up imaging suitable for analysis. When defining both 10 and 15% tumor size increases as significant, 27 (82%) and 29 (88%) tumors decreased in size or remained stable, respectively. For the seven tumors with documented pre-SRS growth, treatment success was 86%. The mean marginal dose for treatment success and failure were 13.2 and 13.7 Gy, respectively. Patients receiving a higher margin dose had a greater risk of tumor progression ( p = 0.0277). Fisch classification did not impact tumor progression rate. Initial tumor volume had no significance on tumor response to SRS. Conclusions: SRS is an effective treatment option for GJT. Both initial tumor volume and Fisch classification did not impact tumor progression. There were no significant patient or lesion characteristics that distinguished treatment success and/or failure.
Recent studies suggest that radiofrequency ablation (RFA) is a treatment option for patients with obstructive sleep apnea (OSA). This study investigates the effectiveness of RFA in treating OSA using a critical literature review and meta-analysis.Critical literature review and meta-analysis.Two independent searches of PubMed 1966-present were performed to identify publications pertaining to RFA and OSA. Effectiveness of the procedure was measured by comparing the Epworth Sleepiness Scale (ESS), respiratory disturbance index (RDI), and lowest O2 saturation before and after tissue ablation.Sixteen studies met the inclusion criteria. The study found a 31% reduction in short-term ESS (odds ratios (OR) 0.69, 95% confidence interval (CI) 0.63-0.75), which was maintained beyond 12 months (OR 0.68, 95% CI 0.43-0.73). Likewise, RFA resulted in a 31% reduction in short term (<12 month) (OR 0.69, 95% CI 0.61-0.77) and 45% reduction (OR 0.55, 95% C.I. 0.45-0.72) in long-term (>24 month) RDI levels. Short-term results of the lowest O2 saturations failed to demonstrate improvement (OR 1.03, 95% CI 0.88-1.20).RFA seems to be a clinically effective tool that reduces ESS scores and RDI levels in patients with OSA syndrome. The procedure should be considered a valid treatment option for patients who refuse or are unable to tolerate continuous positive airway pressure.
To determine the most common indications for revision congenital aural atresia (CAA) surgery and the postoperative healing and hearing outcomes of revision surgery.Retrospective case review.Tertiary care academic otologic practice.Patients undergoing revision surgery for CAA.Revision surgery for CAA.Indications for revision atresiaplasty, time to revision surgery, postoperative external auditory canal (EAC) patency, incidence of chronic drainage and/or infection, and postoperative speech reception thresholds (SRTs), and air-bone gaps.Indications for 75 ears (69 patients) undergoing 107 revision operations for CAA included 58% for EAC stenosis, 19% for chronic drainage and/or infection, and 20% for conductive hearing loss (CHL) alone. Fifty ears (67%) required a single revision. Twenty-five ears (33%) required more than 1 revision. With follow-up longer than 3 months (mean, 41 mo), 69% of ears revised for EAC stenosis achieved a patent canal (29% required >1 revision); 75% of ears revised for chronic drainage and/or infection (mean follow-up, 53 mo) realized a dry canal (22% required >1 revision). For all revision surgeries with adequate follow-up (n = 80), the mean postoperative short-term SRT of 24 dB HL was a significant improvement from the mean preoperative SRT of 39 dB HL (p < 0.01, paired t test).EAC stenosis is the most common indication for revision atresiaplasty. Despite the challenges of revision surgery, improvement in canal patency, epithelialization, and hearing utcomes can be achieved.
