4. Laryngoscope, 124:1204-1208, 2014.

No abstract

Objectives: 1) Review controversies pertaining to the pathophysiology, diagnosis, and treatment of idiopathic intracranial hypertension. 2) Discuss the evolving role of otolaryngologists in managing this disease and related disorders.Data Sources: Primary literature review, Centers for Disease Control and Prevention website, International Classification of Headache Disorders, Second Edition.Methods: A comprehensive review of the primary literature was performed from 1990 to 2016 utilizing keywords idiopathic intracranial hypertension, pseudotumor cerebri, benign intracranial hypertension, spontaneous cerebrospinal fluid leak, and encephalocele. Articles were included at the discretion of the authors based on novel and/or historical contributions to the literature.Results: The incidence of idiopathic intracranial hypertension is increasing along with the obesity epidemic. Undiagnosed patients may present to otolaryngologists with pulsatile tinnitus, dizziness, sleep apnea, and spontaneous cerebrospinal fluid leaks. Although diagnosis is predicated upon imaging findings and lumbar puncture, radiographic signs including empty sella, optic nerve dilation, and globe flattening may suggest the diagnosis. The most effective intervention is weight loss combined with acetazolamide. Surgery is reserved for severe or refractory symptoms and can be highly morbid. Otolaryngologists are increasingly responsible for managing a number of secondary disorders including cerebrospinal fluid rhinorrhea and otorrhea. Failure to manage intracranial hypertension may lead to adverse surgical outcomes.Conclusions: The knowledge base for idiopathic intracranial hypertension has greatly expanded over the past 25 years. This disease is associated with a number of conditions directly relevant to otolaryngologists. A keen understanding of this disorder and its management may optimize outcomes in a growing number of patients.

Patients diagnosed with neurofibromatosis type 2 (NF2) are extremely likely to develop meningiomas, in addition to vestibular schwannomas. Meningiomas are a common primary brain tumor; many NF2 patients suffer from multiple meningiomas. In NF2, patients have mutations in the NF2 gene, specifically with loss of function in a tumor-suppressor protein that has a number of synonymous names, including: Merlin, Neurofibromin 2, and schwannomin. Merlin is a 70 kDa protein that has 10 different isoforms. The Hippo Tumor Suppressor pathway is regulated upstream by Merlin. This pathway is critical in regulating cell proliferation and apoptosis, characteristics that are important for tumor progression. Mutations of the NF2 gene are strongly associated with NF2 diagnosis, leading to benign proliferative conditions such as vestibular schwannomas and meningiomas. Unfortunately, even though these tumors are benign, they are associated with significant morbidity and the potential for early mortality. In this review, we aim to encompass meningiomas and vestibular schwannomas as they pertain to NF2 by assessing molecular genetics, common tumor types, and tumor pathogenesis.

Patients with SSCD demonstrated higher BMIs, higher rates of OSA, and were more likely to have accompanying tegmental defects. These results may support a possible causality between increased intracranial pressure and the formation of superior semicircular canal dehiscence.