Cognitive delay as a risk factor for late presentation of vestibular schwannoma

The majority of patients with vestibular schwannoma (VS) initially experience audiovestibular dysfunction prompting medical evaluation. Over the past several years, the authors have noticed a significant disparity in clinical presentation among patients with preexisting cognitive impairment compared with the general population. The objective of the current study is to review the clinical presentation of 5 consecutive patients with baseline cognitive delay who were ultimately diagnosed with VS.

Retrospective review.

Two tertiary academic referral centers.

Consecutive subjects with baseline cognitive impairment who were evaluated for VS between 2010 and 2013.

Presenting symptoms and tumor characteristics.

Five adult subjects were identified. The most common reasons for seeking medical care included worsening ataxia and headaches, despite the fact that all patients had profound ipsilateral hearing loss. Additionally, 2 patients endorsed facial numbness, and 2 had partial facial paresis. MRI revealed a median preoperative tumor size of 4.7 cm (4.3-5.7 cm), and all patients had varying degrees of hydrocephalus and peritumoral vasogenic edema.

Preexisting cognitive impairment is a risk factor for late presentation of VS. Patients with normal cognitive function most commonly seek medical care after unilateral hearing loss and tinnitus. In contrast, individuals with poor cognitive capacity may be unable to acknowledge such symptoms or communicate their presence. In such patients, the diagnosis of VS is elusive, and clinicians should maintain a low threshold for imaging to avoid further delays in diagnosis and treatment.