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Table 5. Selected Conditions That May Be Associated With
Bilateral SSNHL
Cause Other Features
Mitochondrial Disorders including
MELAS (Metabolic Encephalopathy,
Lactic Acidosis and Stroke-like episodes)
and others
Confusion, stroke like spells, elevated
lactate, MRI white matter changes; others
with variable phenotypes
Stroke Vertigo, dysarthria, facial weakness, ataxia,
nystagmus, unilateral numbness, abnormal
CT or MRI or MR angiogram of the
vertebrobasilar vasculature
Cogan's syndrome Non-syphilitic interstitial keratitis of the
cornea, hearing loss, vertigo
Neoplastic (neurofibromatosis II, bilateral
vestibular schwannomas, carcinomatous
meningitis, intravascular lymphomatosis,
others)
Abnormal brain MRI, cerebrovascular
imaging study, or CSF
Sarcoidosis Pulmonary symptoms, bilateral vestibular
loss, elevated angiotensin converting
enzyme (ACE) level, abnormal gallium
scan
Hyperviscosity syndrome Mucous membrane bleeding, neurologic
and pulmonary symptoms, associated
retinopathy
Diagnosis
(cont'd)
