Selective B-wave reduction with congenital cataract in neurofibromatosis-2

Objective: To electroretinographically evaluate a case of neurofibromatosis-2 (NF-2). Design: Case report and literature review. Participant: A 29-year-old man diagnosed with NF-2. Main Outcome Measures: Electroretinography (ERG), ophthalmic biomicroscopy, and ultrasound examination results were reviewed. Results: The ERG of one eye showed selective reduction of b-wave amplitudes. A characteristic posterior subcapsular lens opacity also was observed in that eye. A dense white congenital cataract and a retinal detachment were detected in the fellow eye. Conclusions: An inner retinal dysfunction, as evidenced by an abnormal ERG, may occur in NF-2. Further ERG evaluation of other patients with NF-2 is indicated to determine possible associations between NF-2 and selective b-wave reduction.