Idiopathic intracranial hypertension associated with polycystic ovarian syndrome, sensorineural hearing loss, and elevated inflammatory markers that lead to bilateral blindness: A case report with literature review

Sara Zarei; Setareh Kamali; William Narinyan; Farnoush Nasouri; Sara Hassani; Abdul Mahmoud Ibrahim; Rojeen Zarei; Sadiq Altamimi

doi:10.25259/SNI_670_2023

Idiopathic intracranial hypertension associated with polycystic ovarian syndrome, sensorineural hearing loss, and elevated inflammatory markers that lead to bilateral blindness: A case report with literature review

Surg Neurol Int. 2023 Nov 17:14:399. doi: 10.25259/SNI_670_2023. eCollection 2023.

Authors

Sara Zarei¹, Setareh Kamali², William Narinyan², Farnoush Nasouri³, Sara Hassani⁴, Abdul Mahmoud Ibrahim¹, Rojeen Zarei⁵, Sadiq Altamimi¹

Affiliations

¹ Department of Neurology, The Neurology Group, Pomona, United States.
² Department of Medicine, Western University of Health Sciences, Pomona, United States.
³ Department of Biology, University of California, Irvine, California, United States.
⁴ Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States.
⁵ Department of Biology, University of Arizona, Tucson, Arizona, United States.

Abstract

Background: Pseudotumor cerebri (PTC) or idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure without hydrocephalus or mass lesion, with normal cerebrospinal fluid (CSF) studies and neuroimaging. The exact cause remains uncertain, but potential mechanisms include increased CSF production, impaired CSF absorption, cerebral edema, and abnormal cerebral venous pressure gradients. Patients may present with various accompanying symptoms such as unilateral or bilateral visual obscuration, pulsatile tinnitus, back pain, dizziness, neck pain, blurred vision, cognitive difficulties, radicular pain, and typically intermittent horizontal diplopia.

Case description: We report a case of a 32-year-old female who initially presented with chronic headaches and oligomenorrhea, which resulted in the diagnosis of polycystic ovary syndrome (PCOS) a few years before the initial diagnosis of PTC. Despite receiving maximum medical treatment and undergoing optic nerve sheath fenestration, the patient experienced complete bilateral vision loss. Nearly 5 years later, the patient sought care at our outpatient neurology clinic, presenting with symptoms including tinnitus, left-sided hearing loss, and joint pain with elevated inflammatory markers and headaches. The focus of this research was to discuss the pathophysiology of each of these comorbidities.

Conclusion: This case report aims to explore the pathophysiological relationships between PTC and concurrent comorbidities, including PCOS, sensorineural hearing loss, empty sella (ES) syndrome, and elevated inflammatory markers. Remarkably, no other PTC case with this unique constellation of concurrent comorbidities have been reported in existing medical literature. The case report underscores the critical importance of early diagnosis of IIH and prompt medical intervention, particularly in patients with PCOS experiencing chronic headaches.

Keywords: Bilateral blindness; Empty sella syndrome; Idiopathic intracranial hypertension; Polycystic ovary syndrome; Pseudotumor cerebri; Sensorineural hearing loss.

Publication types

Case Reports