Patients affected by MPSs require the vigilant attention of the otolaryngologist, as sleep apnea and upper airway obstruction are common complications. Successful bone marrow engraftment may alter the natural history of airway disease and result in substantial improvement in symptomatic airway disease in children with MPSs.

Vestibular schwannomas (VS) are benign tumors arising from the Schwann cells of cranial nerve VIII. Historically the prevailing therapy for patients with VS has been microsurgical resection. More recently, stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy have gained acceptance as effective alternatives. Although the side effect profile and rates of tumor control appear to be favorable for SRS, there is a subset of radioresistant tumors that continue to progress despite properly administered radiation treatment. In this review, the authors summarize what is known about the mechanism of radioresistance in VS at the clinical and molecular level. An improved understanding of the radiobiological behavior of VS may help guide appropriate patient selection for SRS and potentially aid in the design of novel therapies to treat radioresistant tumors.

Vestibular schwannomas (VSs) are benign tumors arising from the sheath of cranial nerve VIII. The pathogenesis underlying most familial and sporadic VSs has been linked to a mutation in a single gene, the neurofibromin 2 (NF2) gene located on chromosome 22, band q11-13.1. In this review, the authors summarized what is known about the epidemiology of NF2 mutations and patients with VSs. The authors also discuss the function of the NF2 gene product, merlin, and describe the known and hypothetical effects of genetic mutations that lead to merlin dysfunction on a broad variety of cellular and histological end points. A better understanding of the molecular pathobiology of VSs may lead to novel therapeutics to augment current modalities of treatment while minimizing morbidity.

INTRODUCTION Nasal valve insufficiency is distinct from other anatomic causes of nasal obstruction. Functional rhinoplasty refers to the collective techniques used to reconstruct the lateral nasal wall, typically achieved with the use of spreader and alar grafts. Patients undergoing functional rhinoplasty can also have aesthetic desires and goals achievable with combined aesthetic-functional rhinoplasty. OBJECTIVE To evaluate the improvement in nasal obstruction symptoms after cartilage graft reconstruction in patients with nasal valve insufficiency and to compare the postoperative improvement between patients undergoing functional and aesthetic-functional rhinoplasty. DESIGN, SETTING, AND PARTICIPANTS In this prospective multicenter cohort study, 12 participating surgeons enrolled 79 consecutive patients diagnosed as having nasal valve insufficiency as the primary cause of nasal obstruction. Patients underwent functional or aesthetic-functional rhinoplasty. The study collected demographic, clinical, and surgical data from March 2006 to September 2008. Nasal symptoms were evaluated using a validated, disease-specific, quality-of-life instrument. The Nasal Obstruction Symptom Evaluation (NOSE) scale was administered to participants at baseline and 3, 6, and 12 months postoperatively. Follow-up was completed on September 2008, and data were analyzed in September 2009 and in September 2013. MAIN OUTCOMES AND MEASURES Changes in NOSE scale score (range, 0 to 100, with a higher score indicating greater severity of obstruction) at 3 months between the functional and aesthetic-functional groups. RESULTS Of the 79 patients, 31 underwent functional and 48 underwent aesthetic-functional rhinoplasty. Overall, an improvement was found in nasal breathing, with a mean decrease of 48.6 points (95% CI, 41.9-55.2; P < .001) at the 3-month assessment compared with the preoperative baseline NOSE scale score (mean [SD], 67.1 [19.7]). The improvement in nasal breathing was similar whether patients underwent functional or aesthetic-functional rhinoplasty (mean [95% CI] NOSE scale score change, 51.4 [42.1-60.7] and 46.6 [37.1-56.1], respectively; P = .49). CONCLUSIONS AND RELEVANCE Nasal valve reconstruction with spreader and alar grafts is effective in treating patients with nasal valve insufficiency. Combining aesthetic interventions with functional rhinoplasty did not seem to affect the magnitude of improvement in nasal breathing outcome. LEVEL OF EVIDENCE 2.