Usher syndrome is a heterogeneous autosomal recessive disorder characterized by dual sensory impairment, that is, profound congenital hearing impairment and progressive visual loss due to retinal degeneration. Retinitis pigmentosa is essential for the diagnosis of Usher syndrome.1 Two types established by Charles Usher are forms which can be divided clinically.1 Patients with Usher syndrome type I have severe congenital hearing impairment, vestibular dysfunction, and retinal degeneration since the first decade. Others with type II have moderate hearing impairment, preserved vestibular function, and relative late onset retinitis pigmentosa. Gene mutations in several locations (1q, 3q, 5q, 10q, 11q, 21q etc.) associated with Usher syndrome2 have been reported. However, most genetic researches emphasize sensory impairment. There are only a limited number of reports with descriptions about the psychotic features of Usher syndrome, in which queer behaviors, irritability, anxiety, depression, Capgras syndrome, schizophrenia-like symptoms, function deterioration course, and withdrawing from work have been found.3–5 Even though these patients are deaf, auditory hallucinations are suspected. Nevertheless, the etiology of its psychiatric manifestation is still unknown and rarely discussed by professionals. Until now, only one report, which found central nervous system changes in a case of Usher’s syndrome with schizophrenia-like mental disorder, was Asian people.6 Herein, the authors report that variable congenital hearing impairment and progressive visual loss occur in five of seven family members and two of them meet the diagnostic criteria of Usher syndrome and present with psychotic symptoms.