Spontaneous labyrinthine hemorrhage in sickle cell disease

We report the clinical and MR imaging findings in two African-American patients with manifestations of sickle cell disease affecting the inner ear. Both suffered sudden-onset sensorineural hearing loss and vestibular symptoms, and both had high labyrinthine signal on T1-weighted MR images attributed to labyrinthine hemorrhage. Follow-up studies of the first patient revealed a decrease in abnormal vestibular signal. Careful attention to the labyrinth on T1-weighted MR images can reveal vestibulocochlear clinical findings in sickle cell patients, with important implications for management and prognosis.