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Observation
November 21, 2018

Secondary Endolymphatic Hydrops Associated With Spontaneous Intracranial Hypotension

Author Affiliations
  • 1Department of Otolaryngology–Head and Neck Surgery, Kansai Rosai Hospital, Amagasaki, Hyogo, Japan
  • 2Department of Otolaryngology–Head and Neck Surgery, Osaka University, Graduate School of Medicine, Osaka, Japan
JAMA Otolaryngol Head Neck Surg. Published online November 21, 2018. doi:10.1001/jamaoto.2018.3073

Spontaneous intracranial hypotension (SIH), which is caused by cerebrospinal fluid (CSF) leakage, is a rare pathology with annual incidence at 5 per 100 000 of the population.1 Patients with SIH report orthostatic headache, dizziness, hearing disturbance, nausea and vomiting, cervical pain, and other symptoms. Ménière disease (MD) is a common inner ear disease characterized by recurrent vertigo, fluctuating hearing loss and tinnitus, and histopathologically, is associated with endolymphatic hydrops (EH). The key symptom of SIH is orthostatic headache, but SIH can be misdiagnosed as MD owing to similarities in clinical presentation. The cochleovestibular symptoms in patients with SIH are speculated to be related to EH in connection with CSF leakage.2,3 However, to our knowledge, no previous SIH patient with EH has been reported.

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