| Main clinical features
|
characterized by postlingual, progressive hearing loss, variable vestibular dysfunction, and onset of retinitis pigmentosa symptoms, including nyctalopia, constriction of the visual fields, and loss of central visual acuity, usually by the second decade of life
delayed gross motor development, hyperactive patellar tendon reflexes, mild truncal ataxia, and a wide-based gait, but upper limb coordination and reflexes, peripheral nerve function, strength, tone, and intelligence were normal |