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OBJECTIVE. This project aims to prospectively and objectively assess otolaryngological manifestations and quality of life of children with primary ciliary dyskinesia (PCD) and compare these findings with healthy pediatric controls. STUDY DESIGN. Cross-sectional. SETTING. Two high-volume pediatric PCD specialty centers. METHODS. Standardized clinical assessment; Sino-Nasal Outcome Test 22 (SNOT-22); Hearing Environment and Reflection Quality of Life (HEAR-QL); Reflux Symptom Index (RSI); standardized physical examination of the sinonasal, laryngeal, and otological systems; and investigations including pure-tone audiograms (PTAs) and sinonasal cultures were collected. RESULTS. Forty-seven children with PCD and 25 control participants were recruited. Children with PCD had more upper airway symptoms than healthy children. They had significantly higher scores in both SNOT-22 and RSI, indicating worse sinonasal and reflux symptoms, with worse quality of life on the HEAR-QL index compared to healthy children (P < .05). Fifty-two percent of children with PCD-related hearing loss were not aware of their hearing deficit that was present on audiological assessment, and only 23% of children who had ventilation tubes had chronic otorrhea, most of which was easily controlled with ototopic drops. Furthermore, although all children with PCD had chronic rhinosinusitis, only 36% of them were using topical nasal treatment. The most common bacteria cultured from the middle meatus were Staphylococcus aureus in 11 of 47 (23%), followed by Streptococcus pneumoniae in 10 of 47 (21%). CONCLUSION. This multisite cohort highlights the importance of otolaryngology involvement in the management of children with PCD. More rigorous otolaryngological management may lead to reductions in overall morbidity and improve quality of life for children with PCD.

Background Concha bullosa mucocele is a rare diagnosis that presents as a nasal mass. It impinges on surrounding structures and can easily be mistaken for a neoplasm. Objective The objective of this study was to shed light on this rare entity and report its diagnostic features and treatment outcomes. Methods A case series conducted in a tertiary health care center. Demographic data, clinical presentation, imaging, cultures, and treatments were recorded. Operative video illustration and key images were obtained. A review of the literature was also performed. Results A total of five cases were reviewed, four of which were concha bullosa mucoceles and one was a mucopyocele. Three of the patients had some form of previous nasal trauma. Headache and nasal obstruction were the most common symptoms with a nasal mass finding on physical examination. Computed tomography was used in all the patients, and magnetic resonance imaging was used in four of the five patients. Four patients had coexistent chronic rhinosinusitis, and three had positive bacterial cultures. All these patients were treated endoscopically either with middle turbinate marsupialization or subtotal resection. No recurrence has been noted thus far. Conclusion Concha bullosa mucocele is a rare diagnosis. Imaging characteristics are helpful in considering the diagnosis, although surgical intervention is often necessary to confirm the diagnosis and treat concha bullosa mucocele.

Allergen instillation in anaesthetized vs. awake animals results in increased distribution of allergen in the lung. Halothane is a more potent bronchodilator of the small airways than isoflurane. As small airways contribute to asthma pathogenesis, we questioned whether intranasal challenge under halothane vs. isoflurane anesthesia would lead to an increase in allergen deposition in the lung periphery and, consequently, an enhanced allergic response. C57Bl/6 mice were sensitized twice and repeatedly challenged with ovalbumin (OA) under halothane or isoflurane anesthesia. After OA-challenge, in vivo lung function was measured and BAL performed. Peribronchial and peripheral inflammation, cytokine mRNA production and collagen deposition were assessed. Airway hyperresponsiveness, BAL eosinophilia, peripheral lung inflammation, IL-5 mRNA production and collagen deposition were significantly increased in halothane OA-challenged compared to isoflurane OA-challenged mice. Airway challenge induced a higher level of airway hyperresponsiveness, inflammation and remodeling under halothane than isoflurane anesthesia in a murine model of asthma. These differences may be due to increased allergen deposition in the small airways.

Decorin (Dcn) is an extracellular matrix proteoglycan, which affects airway mechanics, airway-parenchymal interdependence, airway smooth muscle proliferation and apoptosis, and transforming growth factor-β bioavailability. As Dcn deposition is differentially altered in asthma, we questioned whether Dcn deficiency would impact the development of allergen-induced asthma in a mouse model. Dcn−/− and Dcn+/+ mice (C57Bl/6) were sensitized with ovalbumin (OA) and challenged intranasally 3 days/wk × 3 wk. After OA challenge, mice were anesthetized, and respiratory mechanics measured under baseline conditions and after delivery of increasing concentrations of methacholine aerosol. Complex impedance was partitioned into airway resistance and tissue elastance and damping. Bronchoalveolar lavage was performed. Lungs were excised, and tissue sections evaluated for inflammatory cell influx, α-smooth muscle actin, collagen, biglycan, and Dcn deposition. Changes in TH-2 cytokine mRNA and protein were also measured. Airway resistance was increased in OA-challenged Dcn+/+ mice only ( P < 0.05), whereas tissue elastance and damping were increased in both OA-challenged Dcn+/+ and Dcn−/−, but more so in Dcn+/+ mice ( P < 0.001). Inflammation and collagen staining within the airway wall were increased with OA in Dcn+/+ only ( P < 0.001 and P < 0.01, respectively, vs. saline). IL-5 and IL-13 mRNA were increased in lung tissue of OA-challenged Dcn+/+ mice. Dcn deficiency resulted in more modest OA-induced hyperresponsiveness, evident at the level of the central airways and distal lung. Differences in physiology were accompanied by differences in inflammation and remodeling. These findings may be, in part, due to the well-described ability of Dcn to bind transforming growth factor-β and render it less bioavailable.

Objectives To identify whether measurement of the prosthesis length is mandatory in patients requiring otosclerosis surgeries and to assess their postoperative audiometric outcomes. In addition, evaluation of prosthesis length used in revision compared to primary stapedectomy was carried out. Study Design Case series with chart review. Methods Chart review of 393 patients undergoing primary (321) versus revision stapedectomy (72) was performed in a tertiary referral center. The indication for surgery was the presence or persistence/recurrence of an air-bone gap (ABG) greater than 20 dB. Air and bone conduction thresholds (ACT and BCT, respectively), ABG as well as pure tone averages (PTAs) were determined for all patients, and the results were compared preoperatively and postoperatively. Results Prosthesis length used ranged from 3.0 to 6.0 mm without differences between primary and revision groups. Of the revision surgeries, 62.5% were stapedectomies versus stapedotomies (P < 0.001). Patients showed significant decrease in speech discrimination score, with increased air and bone conduction thresholds as well as mean ABG and PTA before the revision surgeries as a first procedure failure. Prosthesis length changes occurred in 73.5% of the cases, with an average absolute change of 0.55 mm. Prosthesis length did not affect postoperative audiometric results between primary and revision groups, in all surgeries combined. When grouping stapes surgery into accurately versus inaccurately measured incus-footplate distance, significant differences were observed in prosthesis length employed (P < 0.01). Hearing outcomes were also better in the group in which an accurately measured prosthesis was chosen, as opposed to “standard-length” prosthesis. Conclusions This study corroborates postoperative success rates of revision surgeries, which show smaller improvements in hearing compared to a primary intervention. Accurate intra-operative measurement of prosthesis length was correlated with better audiometric results postoperatively.

Varying concentrations of lipopolysaccharide (LPS) in ovalbumin (OVA) may influence the airway response to allergic sensitization and challenge. We assessed the contribution of LPS to allergic airway inflammatory responses following challenge with LPS-rich and LPS-free commercial OVA. BALB/c mice were sensitized with LPS-rich OVA and alum and then underwent challenge with the same OVA (10 µg intranasally) or an LPS-free OVA. Following challenge, bronchoalveolar lavage (BAL), airway responsiveness to methacholine and the lung regulatory T cell population (Treg) were assessed. Both OVA preparations induced BAL eosinophilia but LPS-rich OVA also evoked BAL neutrophilia. LPS-free OVA increased interleukin (IL)-2, IL-4 and IL-5 whereas LPS-rich OVA additionally increased IL-1β, IL-12, IFN-γ, TNF-α and KC. Both OVA-challenged groups developed airway hyperresponsiveness. TLR4-deficient mice challenged with either OVA preparation showed eosinophilia but not neutrophilia and had increased IL-5. Only LPS-rich OVA challenged mice had increased lung Tregs and LPS-rich OVA also induced in vitro Treg differentiation. LPS-rich OVA also induced a Th1 cytokine response in human peripheral blood mononuclear cells. We conclude that LPS-rich OVA evokes mixed Th1, Th2 and innate immune responses through the TLR-4 pathway, whereas LPS-free OVA evokes only a Th2 response. Contaminating LPS is not required for induction of airway hyperresponsiveness but amplifies the Th2 inflammatory response and is a critical mediator of the neutrophil, Th1 and T regulatory cell responses to OVA.