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JAMA Ophthalmology Clinical Challenge
September 9, 2021

Middle-aged Woman With Bilateral Macular Atrophy, Hearing Loss, and Headache

Author Affiliations
  • 1Vita-Salute San Raffaele University, Milan, Italy
  • 2Department of Ophthalmology, Vita-Salute University, IRCCS Ospedale San Raffaele Scientific Institute, Milan, Italy
  • 3Department of Neurology, IRCCS Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, Bologna, Italy
JAMA Ophthalmol. 2021;139(11):1238-1239. doi:10.1001/jamaophthalmol.2021.0944

A 45-year-old woman was referred to the medical retina service for evaluation of bilateral macular atrophy noted by her general ophthalmologist. Medical history was positive for bilateral sensorineural hearing loss with cochlear origin as determined by an otolaryngologist and headache since age 35 years. She had no history of longer-term use of medications. There was no family history of hearing and vision loss in all immediate family members. Her best-corrected visual acuity was 20/20 OD and 20/63 OS. Her pupils were round and reactive. The anterior segment was unremarkable. Fundus examination confirmed the presence of bilateral macular atrophy. Fundus autofluorescence revealed a fairly symmetric loss of autofluorescence in the macula and in the regions surrounding the optic disc (Figure). In proximity of these areas of reduced autofluorescence, a speckled fundus autofluorescence pattern of alternating spots of increased and decreased autofluorescence was also noted. Optical coherence tomography displayed the presence of a bilateral atrophy of the outer retina and retinal pigment epithelium (Figure). The patient’s family underwent a complete ophthalmologic evaluation, which did not display any alteration.

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