A rare brain developmental anomaly in a patient with Usher's syndrome.

We report a rare brain developmental anomaly in Usher's syndrome. We present a 43-year-old male with visual disturbance, hearing loss, and headache. Retinitis pigmentosa and sensorineural hearing loss were determined and he was diagnosed with Usher's syndrome according to the clinical findings. Magnetic resonance imaging showed an arachnoid cyst on the left temporal lobe, cavum septum pellucidum et vergae. Uneventful cataract surgery was performed in both eyes. He was suggested to be followed up periodically for the arachnoid cyst and to use a hearing device. Although auditory and visual disturbances are the typical findings of this syndrome, it may affect other parts of the central nervous system as well. Morphological abnormalities of central nervous system and related disorders can be seen in patients with Usher's syndrome.