Otolaryngologic Clinics of North America

Herein we briefly describe the translabyrinthine approach to vestibular schwannoma resection as well as a focused literature review as to the best candidates, technical recommendations, and key outcomes with respect to other approaches.

Microsurgical removal of acoustic neuroma has advanced tremendously; however, complications still occur. Facial nerve injury is the most common detrimental complication and should take precedence over gross tumor removal in cases where there is an unfavorable tumor-facial nerve interface. Cerebrospinal fluid leakage can occur even with meticulous closure techniques and is generally treatable with either lumbar-subarachnoid drainage or revision wound closure. Meningitis is a serious complication that requires a high index of suspicion in the postoperative period...

Monitoring the cochlear nerve during vestibular schwannoma (VS) microsurgery depends on the hearing status and surgical approach. Traditional hearing preservation VS microsurgery relies on acoustically driven auditory brainstem response (ABR) and cochlear nerve action potential. Both modalities have advantages and disadvantages that need to be understood for proper implementation. When hearing is lost or the approach violates the otic capsule, electrically evoked monitoring methods may be used. Evoked ABR (eABR) is feasible and safe but may be limited by artifact...

The current management of vestibular schwannomas (VS) includes observation, microsurgery (MS), and stereotactic radiosurgery (SRS) or radiotherapy, and treatment failures may occur with any primary modality. SRS is most often used for microsurgical failures, as it carries a low risk of adverse events. Salvage MS following previous MS is reserved for specific cases and can present certain surgical challenges. Irradiation failures can be managed with both salvage MS and repeat SRS. This article is intended to review an approach to the failure of primary interventions for VS, with a focus on the time interval between modalities, rates of tumor control, functional outcomes, and possible complications...

Neurofibromatosis type 2 (NF2) is an autosomal dominant syndrome caused by a mutation in the NF2 suppressor gene and is characterized by the development of multiple benign tumors throughout the central nervous system. Bilateral vestibular schwannomas (VSs) are pathognomonic for NF2 and are associated with progressive hearing loss and eventual deafness in most patients. This review presents current management options for NF-2-associated VSs.

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Granulomatous and vasculitic diseases of the airway may be part of more widespread systemic disease but can occur in isolation. They may present to the ear, nose, and throat (ENT) surgeon initially with vague symptoms that mimic more common chronic inflammatory unified airway conditions, such as rhinitis, chronic rhinosinusitis, and asthma. Early diagnosis is associated with better long-term outcomes, so a high index of suspicion is required. Bloody nasal discharge and crusting are highly suspicious for granulomatous disease, which should also be considered in atypical or recalcitrant disease...

The unified airway is in constant exposure to environmental factors that promote inflammation and disease. Environmental allergens, including house dust mites, pets, pollens, and molds, are strongly linked with development and exacerbation of upper airway disease with nonlinear dose-dependent relationships. Occupational triggers, including combat exposures and construction workers, are associated with the development of both upper and lower airway disease resulting in a "healthy worker effect" where many people leave jobs due to significant morbidity...

Although unified airway disease (UAD) may have heritable components, genetic changes involving coexistent chronic rhinosinusitis (CRS) are not well understood. Genetic predisposition is stronger in patients with CRS with nasal polyps compared with those without nasal polyps (CRSsNP). Genetic factors account for 25% to 80% of asthma risk and 90% of allergic rhinitis risk but risk contributions are not well described for CRS. Susceptibility genes identified in coexistent CRS-asthma relate to innate and adaptive immunity, cytokine signaling, tissue remodeling, arachidonic acid metabolism, and other proinflammatory pathways...

Unified airway disease describes the shared epidemiologic and pathophysiologic relationship among the chronic inflammatory diseases of the upper and lower airways including allergic rhinitis, chronic rhinosinusitis, asthma, and chronic otitis media. This concept proposes that these diseases are manifestations of a single inflammatory process and require an integrated diagnostic and therapeutic approach to achieve global disease control. Future directions to further establish this entity should focus on pathophysiology, diagnostic markers, flora microbes with particular emphasis on fungi, the role of type 3 inflammation, and targeted therapeutics including biologics, JAK inhibitors, and synthetic peptides...

Support for the unified airway hypothesis is embedded in similarities in upper and lower airway structure, function, and cellular/extracellular compositions. The impact of endoscopic sinus surgery (ESS) on the unified airway is influenced by multiple factors including the underlying upper and lower airway condition(s) present and severity of pathology. Beyond improvements in subjective and objective CRS outcomes, ESS also improves clinical asthma outcomes and measures of asthma control. Emerging evidence suggests that early ESS may mitigate the risk of developing asthma in CRS patients without asthma...

The concept of a unified airway posits that pathology affects the respiratory tract in a continuum and that disease in one part of the respiratory tract may be associated with or directly or indirectly affect the function of a different part. Transcriptomic analysis has shown 91% homology between the genes expressed in the upper and the lower airway. Approaching inflammatory airway disorders using the unified airway hypothesis allows for a better clarification of disease process and provides a detailed and a high-level overview of dysfunction...

Upper airway cough syndrome (UACS), formerly known as postnasal drip syndrome, is one of the most common causes of chronic cough. UACS, asthma, and gastroesophageal reflux make up 90% of the cause of chronic cough. UACS is a clinical diagnosis of exclusion with no diagnostic testing or objective findings. UACS can be present with or without associated rhinitis and chronic rhinosinusitis. Treatment includes dual therapy with H1 receptor antihistamines and decongestants. Diagnosis is confirmed when therapeutic intervention results in symptom resolution...

Primary Ig deficiencies are a heterogeneous group of disorders with widespread implications for the unified airway. Manifestations can vary greatly, with some patients being asymptomatic, whereas others suffering from acute and chronic life-threatening pathologic conditions of the upper and lower airways. Although the diagnosis of PIDs can be complex, the onus of early diagnosis and initiation of treatment will often fall on the shoulders of the otolaryngologist.

The unified airway concept is a framework for the understanding and management of the upper and lower airways as one integrated physiologic unit. The sinonasal and bronchopulmonary systems have an interdependent physiologic function, and inflammatory conditions that impact one system tend to impact the other similarly. The application of the unified airway concept in the pediatric population is not well described. This study identifies and characterizes the common manifestations of the pediatric unified airway, including pediatric chronic rhinosinusitis, adenoid disease, asthma, cystic fibrosis, and primary ciliary dyskinesia...

Concurrent chronic rhinosinusitis with nasal polyps (CRSwNP) in the upper airway, and asthma in the lower airway, often have a shared underlying pathophysiology, namely type 2 inflammation; hence, the term "unified airway disease." The combination of CRSwNP and asthma is associated with uncontrolled disease. The range of treatment of CRSwNP includes intranasal corticosteroids, nasal saline irrigation, oral corticosteroids, antibiotics, and biologics. A combined clinical algorithm for the management of the upper and lower airways in type 2 inflammation will be beneficial, especially for patients with uncontrolled disease who may benefit from biologics...

It is evident that sex and gender differences impact pathophysiology, disease burden, and treatment outcomes for a variety of systems and major illnesses including those affecting the unified airway. Important male-female differences in unified airway disease are driven by various intrinsic and extrinsic mechanisms including anatomic, morphometric, hormonal, genetic (and epigenetic), environmental, psycho-social, and comorbidity-related factors. This review highlights current knowledge of how patient sex influences epidemiology, diagnosis, treatment, and outcomes for diseases affecting the unified airway...

The "unified airway" describes the concept that inflammation affects upper and lower airways by similar mechanisms; this often manifests as rhinitis, rhinosinusitis, and/or nasal polyposis in the upper airway with associated asthma or bronchial inflammation in the lower airways. The relationships between eosinophilic diseases of the upper and lower airways are highlighted by examining their prevalence and treatment regimens along with the synergistic effects of treatment on upper and lower airway symptoms. It is important to recognize the interrelatedness of upper and lower airway eosinophilic disease to assess and manage patients accurately and holistically...

Upper and lower airways diseases are very common, with population prevalence of 10% to 40%. The conditions are usually interlinked and referred to as "unified airway disease" or "the united airways." Especially in phenotypes with more severe disease, type 2 immunologic endotype is often noted. Comorbid upper and lower airway diseases are usually caused by similar underlying immunologic response. Any patient with rhinitis or rhinosinusitis should have their lower respiratory tract evaluated. A multidisciplinary approach in the diagnosis and treatment of airway disease is advised, especially, for more severe phenotypes...