A kindred is described with progressive autosomal dominant vestibulo-cochlear dysfunction resulting in instability in the dark, head movement dependent oscillopsia and hearing loss. The first symptoms appeared in the 4th decade and progressed to vestibular areflexia, presumably in the 5th decade and to almost total deafness in the 6th-7th decade of life. The history was negative for other neurological, otological or infectious diseases, or the use of neuro-ototoxic drugs. The affected subjects showed remarkable compensation for the loss of vestibular function.