Annals of Otology,Rhinology & Laryngology

To examine the expression of hypoxia-inducible factor-1α (HIF-1α) and its related molecules (cellular repressor of E1A-stimulated genes [CREG], osteopontin [OPN], proto-oncogene tyrosine-protein kinase Src [c-Src], and vascular endothelial growth factor [VEGF]) in juvenile nasopharyngeal angiofibroma (JNA) and explore the correlation between clinical prognosis and HIF-1α expression.

The study performed a retrospective review of the clinical records of patients with JNA treated between 2003 and 2007. Specimens were analyzed by immunohistochemistry for HIF-1α, CREG, OPN, c-Src, and VEGF expression, and microvessel density (MVD) was assessed by tissue microarray. The correlation between expression levels and clinicopathological features including age, tumor stage, intraoperative blood loss, and recurrence was analyzed.

HIF-1α, CREG, OPN, c-Src, and VEGF were upregulated in endothelial cells (ECs) of patients with JNA, and strong correlations in the expression of these molecules were observed. HIF-1α expression was higher in young patients (P = .032) and in recurrent cases (P = .01). Survival analysis showed that low HIF-1α levels in ECs predicted longer time to recurrence (log rank test P = .006). Receiver operating characteristic curve analysis showed that HIF-1α was a prognostic factor for recurrence (area under the curve = 0.690, P = .019). No correlation was found between the expression of molecules and Radkowski stage or intraoperative blood loss.

In cases of JNA treated surgically, HIF-1α expression in ECs is a useful prognostic factor for tumor recurrence.

Evaluate usage trends of neural response telemetry (NRT) in cochlear implant centers across the nation and assess reported benefits of intraoperative NRT for pediatric cochlear implant recipients.

Survey.

All US cochlear implant centers (n = 110).

A 15-question multiple-choice survey was distributed electronically to all centers. The survey captured demographic information of all centers, practice patterns surrounding the use of NRT, and the extent to which intraoperative NRT is of benefit.

Thirty-two invited participants (29%) completed the survey. A majority of participants reported practicing in an academic center (66%), followed by a hospital setting (19%) and private practice (16%). Seventy-two percent of survey participants reported using NRT for pediatric cochlear implant recipients. Sixty-three percent felt it improved the ability to program at initial activation, and 50% of participants felt that NRT improves satisfaction at initial activation.

This study suggests that a majority of surgeons use intraoperative NRT for pediatric cochlear implantation as an additional measure to ensure appropriate electrode placement and improve device activation. Larger studies are needed to better establish the relationship between intraoperative NRT and postoperative outcomes and justify the additional costs associated with intraoperative NRT.

To evaluate the potential origins via magnetic resonance imaging and the relevant hearing recovery course of pediatric sudden sensorineural hearing loss.

We retrospectively analyzed data of 25 pediatric patients from our center with sudden sensorineural hearing loss from January 2011 to December 2016. All individuals were closely followed up at baseline and 1 and 6 months.

Magnetic resonance imaging identified presumed causes in 9 cases, 5 of which showed intralabyrinthine hyperintensity, suggesting presumptive intralabyrinthine hemorrhage. The remaining 20 patients showed no hyperintensity. Restoration of hearing and speech discrimination abilities were noted in these 25 children at 6 months versus the initial levels (74.2 ± 22.6 vs 93.5 ± 20.5 dB, p = .000, and 45.8 ± 36.0 vs. 18.3 ± 22.1%, p = .004, respectively). The prognosis of the individuals with intralabyrinthine hemorrhage were superior in terms of frequency and hearing threshold at 6 months compared with that of the no-hemorrhage participants. Word recognition scores improved in either studied group.

The potential recovery of hearing in children raises concerns about very early surgical intervention within the first 6 months. Rational imaging and sequential audiometric evaluation to monitor the progression of recovery may be beneficial.

Expansion sphincter pharyngoplasty (ESP) is a surgical option for patients with obstructive sleep apnea (OSA). Upper airway stimulation (UAS) is an alternative that has shown success in initial outcomes studies. We compare outcomes of a cohort of patients undergoing UAS to ESP.

We compared demographic and polysomnographic data of the UAS to ESP cohorts. We also calculated the proportion of patients achieving surgical success.

The ESP cohort consisted of 33 patients. The mean preoperative Apnea-Hypopnea Index (AHI), O₂ nadir, Epworth Sleepiness Scale (ESS), and BMI were 36.47 ± 20.01, 82.63 ± 5.37, 10.69 ± 4.42, and 29.6 ± 4.49, which improved to 13.47 ± 18.74, 84.84 ± 5.48, 7.00 ± 5.81, and 29.92 ± 4.59 postoperatively. There was a 63.64% success rate. The UAS cohort consisted of 75 patients. The mean preoperative AHI, O₂ nadir, ESS, and BMI were 36.76 ± 20.72, 80.24 ± 8.43, 11.18 ± 4.16, and 29.50 ± 3.96, which improved to 7.25 ± 11.19, 88.71 ± 3.25, 5.36 ± 3.35, and 29.36 ± 3.68 postoperatively. The success rate was 86.67%. We found a significant difference in gender, age, preoperative AHI, postoperative AHI, postoperative O₂ nadir, surgical success, and patients reaching an AHI less than 10 and 5.

Upper airway stimulation is a new surgical option for select patients with OSA showing comparable or improved outcomes to a cohort of patients undergoing ESP.

To characterize the frequency of airway anomalies in patients with 22q11.2 deletion syndrome (22q11DS).

Retrospective review of patients with 22q11DS who had undergone microlaryngoscopy/bronchoscopy (MLB) for aerodigestive symptoms at a tertiary care children’s hospital from 2011 to 2016.

Thirty patients underwent an MLB due to the following indications: aspiration (11), stridor (10), chronic respiratory failure due to ventilator dependence (8), and difficult intubation (1). Median age at MLB was 6.5 months (range, 0.25-32 months). Forty airway anomalies were identified in 20 (66%) patients. Laryngomalacia (10), tracheomalacia (8), and bronchomalcia (8) were the most common intraoperative findings, followed by laryngeal cleft (5), anterior glottic web (5), subglottic stenosis (3), and subglottic cysts (1). Synchronous airway anomalies were common and identified in 11 (55%) of the patients who had identified anomalies on MLB. Nineteen of the 20 patients required operative intervention due to the anomalies identified.

Structural airway abnormalities are common in children with 22q11DS undergoing MLB, and synchronous anomalies can frequently exist. Providers caring for children with 22q11DS should be vigilant about airway evaluation when aerodigestive symptoms are present.

To report treatment of benign paroxysmal positional vertigo (BPPV) in patients unable to undergo traditional canalith repositioning maneuvers (CRMs) using a particle repositioning chair (PRC).

A retrospective chart review was conducted at a single high-volume otology practice of patients diagnosed with BPPV from 2007 to 2017 with immobility prohibiting use of traditional CRMs. Patients were diagnosed and treated using a PRC, and outcome measures including resolution, recurrence, and number of treatment visits were recorded.

A total of 34 patients meeting criteria were identified, 24 of whom had cervical spine disease and 10 of whom had other prohibitive immobility. Symptoms were present for between 5 days and 11 years at presentation, with mean and median of 552 and 90 days, respectively. Symptoms resolved in 68% of patients and recurred in 13% of those patients. Most patients required 1 treatment visit.

Successful treatment of patients with BPPV and concomitant immobility prohibiting traditional CRMs is reported using the PRC. Benign paroxysmal positional vertigo in the setting of immobility is an indication for treatment with a PRC if available.

Summarize the effectiveness of intraoperative cryoanalgesia in the management of postoperative pain among patients undergoing palatine tonsillectomy.

A systematic review of PubMED, MEDLINE, EMBASE, Google Scholar, and Cochrane trial registries was performed through January 2017 using the PRISMA standards. We included English-language randomized controlled trials evaluating patients of all age groups with benign pathology who underwent tonsillectomy with cryoanalgesia versus without.

Three limited quality randomized controlled trials involving 153 participants (age range, 1-60 years) were included. Cryoanalgesia was performed with a cryotherapy probe (−56°C) in 1 trial and ice-water cooling (4°C to 10°C) in 2. In the 3 trials reviewed, patients who received cryoanalgesia reported 21.38%, 28.33%, and 31.53% less average relative postoperative pain than controls on the visual analog scale. Review of secondary outcomes suggested no significant difference in time to resume normal diet (2 studies) or postoperative bleeding (2 studies) between the 2 groups. Cryoanalgesia allowed patients to return to work 4 days earlier than controls in 1 study. Two studies reported a trend toward less postoperative analgesia use among the treatment group; however, no statistical conclusions could be drawn.

The available evidence suggests that patients undergoing tonsillectomy with cryoanalgesia experience less average postoperative pain without additional complications.

To compare the diagnostic performance of the Hum Test against the Weber Test using pure tone audiometry (PTA) as the “gold standard” comparator.

29 participants with normal hearing of ages 18 to 35 without any history of hearing abnormalities or otologic conditions were enrolled. Subjects underwent three tests (Hum Test, Weber Test, and PTA) across two conditions: with an ear plug in one ear (side randomized) and without ear plugs.

When examining the ability of the Hum Test to detect simulated conductive hearing loss (CHL), the test had a sensitivity of 89.7% and specificity of 100% with high pitched humming and 93.1% and 100%, respectively, with low pitched humming. The Weber Test had a sensitivity and specificity of 96.6% and 100%, respectively. McNemar’s test demonstrated agreement between the Hum Test, performed with either high pitched (P = .32) or low pitched (P = .56) humming, and the Weber Test. Receiver operating characteristic (ROC) curves for the Hum Test (both high and low pitched) and Weber test were compared and demonstrated no statistically significant difference.

The Hum Test is comparable to the Weber Test with regards to its sensitivity, specificity, and diagnostic accuracy in assessing new onset unilateral CHL in previously normal hearing subjects.

To present a novel pediatric airway anomaly encountered on direct laryngoscopy and bronchoscopy, further characterized with computed tomography imaging of the chest.

Case report and literature review.

Review of the clinical presentation, operative findings, and imaging findings of an infant who presented with respiratory distress in the setting of multiple congenital abnormalities. A brief literature review supplements the case presentation.

While multiple tracheobronchial anomalies have long been reported, this is the first report of a double or false carina: a system of symmetric paired upper and lower bronchi connected by a central conduit. Operative evaluation was limited given the narrow lumen, and postoperative imaging aided in further characterization of the pediatric airway.

This is the first report of a novel tracheobronchial anomaly presenting as a double carina. Tracheobronchial anomalies are diverse and should be considered when performing operative airway evaluations. Imaging can be a helpful adjunct with anatomic characterization.

Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased.

Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature.

The progressive nature of FHL disorder may cause bilateral, low-frequency, irreversible sensorineural hearing loss. This type of hearing loss should be considered among the long-term sequelea presenting with FHL5.

We recommend audiological evaluation at initial FHL5 diagnosis to assess for hearing functions. Follow-up in audiology should be part of the long-term monitoring of patients with FHL5 as hearing loss could develop long after diagnosis.