Idiopathic intracranial hypertension: Contemporary review and implications for the otolaryngologist

Laryngoscope. 2018 Jan;128(1):248-256. doi: 10.1002/lary.26581. Epub 2017 Mar 27.


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. Laryngoscope, 128:248-256, 2018.

Keywords: Idiopathic intracranial hypertension; benign intracranial hypertension; pseudotumor cerebri; pulsatile tinnitus; spontaneous cerebrospinal fluid otorrhea/rhinorrhea.

Publication types

  • Review

MeSH terms

  • Cerebrospinal Fluid Leak / complications
  • Diagnosis, Differential
  • Encephalocele / complications
  • Humans
  • Intracranial Hypertension / complications
  • Intracranial Hypertension / diagnosis*
  • Intracranial Hypertension / therapy*
  • Obesity / complications
  • Pseudotumor Cerebri / complications
  • Risk Factors