Charles Bonnet plus syndrome: apropos of a case

Charles Bonnet syndrome plus is an exceedingly rare variant of this disorder. The variant has been described in patients with sight impairment and severe hypoacusis, and is usually characterized by complex visual and auditory--musical--hallucinations that the patients recognize as unreal.

Case report.

A 75-year-old woman diagnosed with Usher syndrome presented with visual acuity of light perception in both eyes, which did not improve with the use of a pinhole occluder. She also had coptosis in right ear and severe hypoacusis in left ear, confirmed through audiometry. Audiometric tests were normal once the implant and the hearing aid were connected. The patient was referred to the Neuro-Ophthalmology Unit after recounting experiencing complex visual hallucinations, as well as auditory (musical) ones at night after disconnecting the hearing aid. She described the latter as a nightly occurrence of hearing "cabaret music." Nevertheless, she was aware of reality and of her sensory impairments. The patient was diagnosed at the interdisciplinary Neuro-Ophthalmology Unit, and began pharmacologic treatment with clear improvement.

Knowledge of Charles Bonnet syndrome and in particular of Charles Bonnet syndrome plus--due to its infrequency--on the part of ophthalmologists is fundamental to adequately diagnose and treat this rare disorder.