Explore this issue:April 2018
What is the role of the otolaryngologist in managing idiopathic intracranial hypertension (IIH) and related disorders?
Bottom Line: The knowledge base for idiopathic intracranial hypertension has greatly expanded over the past two decades. Otolaryngologists now play a key role in the co-management of this disorder including diagnosis, initiation of timely referrals, patient counseling, and treatment of related disorders. A keen understanding of this disorder and its management may optimize outcomes in a growing number of patients.
Background: IIH is defined as an elevation in cerebrospinal fluid (CSF) pressure with normal brain parenchyma, absence of ventriculomegaly, and no identifiable cause. This disease has been previously known under other names including pseudotumor cerebri syndrome, meningeal hydrops, and benign intracranial hypertension. Patients classically present with severe headaches, papilledema, and vision loss, which may progress to blindness in advanced or rapidly progressive cases. Due to the relative rarity of IIH, a number of significant controversies continue to surround its pathophysiology and management. Otolaryngologists play an increasingly important role in managing secondary and/or comorbid conditions. They may also be the first provider to suspect the diagnosis while treating related symptomatology. Given the dearth of studies focusing on this disease in the otolaryngic literature (only one published review over the past 20 years), otolaryngologists may feel ill-equipped to identify, diagnose, or assist in managing this disorder