Diagnosis and treatment of a severe psychotic illness in a man with dual severe sensory impairments caused by the presence of Usher syndrome

The present paper reports the case history of a 50-year-old man born with U sher syndrome, who developed a psychotic illness later in life. to illustra te the specific diagnostic problems, and the value of direct observation an d a detailed assessment of communication. The subject had had a significant hearing impairment since birth, problems with balance and developed retini tis pigmentosa, leaving him with progressively limited vision in adult life . A pattern of bizarre and aggressive behaviour, and a disintegration in hi s ability to communicate using signs developed over 3 months. An initial di agnosis of depression was made, but it later became clearer that the subjec t had developed a psychotic illness. This condition responded well to a com bination of antidepressant and antipsychotic medication. The possible assoc iation between Usher syndrome and psychotic illness is also discussed.