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Salt-and-Pepper Retinopathy in a Woman With Hearing Loss

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 58-year-old woman with interstitial cystitis presented for pentosan polysulfate (Elmiron) maculopathy screening. She reported no visual symptoms or ocular history, and routine eye examination results 6 months prior were reportedly normal. She was diagnosed with interstitial cystitis in 2004 and had been taking pentosan polysulfate, 100 mg twice daily, for 4½ years for a cumulative dose of 324 g. Her medical history was otherwise significant for moderate bilateral hearing loss detected in early childhood and type 2 diabetes, hyperlipidemia, and hypothyroidism treated with a therapeutic regimen of metformin, pravastatin, and levothyroxine supplementation, respectively.

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Congenital rubella syndrome

D. Obtain a more detailed medical history and provide an Amsler grid

Additional questioning revealed the patient’s mother was diagnosed with “German measles” early in pregnancy and expected her child might be blind and/or deaf. The patient was born in Canada and was small for gestational age, but had no apparent visual, auditory, or cardiac issues at birth. In early adulthood, when joining the military, the patient was tested and informed of her serologic rubella immunity. This patient’s presentation with early-onset hearing loss, granular retinal pigmentary mottling with punctate hyperpigmentation and hypopigmentation (“salt and pepper”), and irregularly distributed speckled fundus autofluorescence changes is consistent with congenital rubella syndrome (CRS).

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Article Information

Corresponding Author: Nieraj Jain, MD, Department of Ophthalmology, Emory University School of Medicine, 1365B Clifton Rd NE, Ste 2400, Atlanta, GA 30322 (nieraj.jain@emory.edu).

Published Online: April 1, 2021. doi:10.1001/jamaophthalmol.2020.4624

Conflict of Interest Disclosures: None reported.

Funding/Support: Dr Jain receives salary support through Foundation Fighting Blindness Career Development Award CD-C-0917-0748-EEC.

Role of the Funder/Sponsor: The Foundation Fighting Blindness had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Hanif  AM , Armenti  ST , Taylor  SC ,  et al.  Phenotypic spectrum of pentosan polysulfate sodium-associated maculopathy.   JAMA Ophthalmol. 2019;137(11):1275-1282. doi:10.1001/jamaophthalmol.2019.3392 PubMedGoogle ScholarCrossref
2.
Pearce  WA , Chen  R , Jain  N .  Pigmentary maculopathy associated with chronic exposure to pentosan polysulfate sodium.   Ophthalmology. 2018;125(11):1793-1802. doi:10.1016/j.ophtha.2018.04.026 PubMedGoogle ScholarCrossref
3.
Gregg  NM .  Congenital cataract following German measles in the mother.   Aust N Z J Ophthalmol. 1991;19(4):267-276.PubMedGoogle Scholar
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Bouthry  E , Picone  O , Hamdi  G , Grangeot-Keros  L , Ayoubi  JM , Vauloup-Fellous  C .  Rubella and pregnancy.   Prenat Diagn. 2014;34(13):1246-1253. doi:10.1002/pd.4467 PubMedGoogle ScholarCrossref
5.
Givens  KT , Lee  DA , Jones  T , Ilstrup  DM .  Congenital rubella syndrome.   Br J Ophthalmol. 1993;77(6):358-363. doi:10.1136/bjo.77.6.358 PubMedGoogle ScholarCrossref
6.
Arnold  JJ , McIntosh  ED , Martin  FJ , Menser  MA .  A fifty-year follow-up of ocular defects in congenital rubella.   Aust N Z J Ophthalmol. 1994;22(1):1-6. doi:10.1111/j.1442-9071.1994.tb01687.x PubMedGoogle ScholarCrossref
7.
Reef  SE , Frey  TK , Theall  K ,  et al.  The changing epidemiology of rubella in the 1990s.   JAMA. 2002;287(4):464-472. doi:10.1001/jama.287.4.464 PubMedGoogle ScholarCrossref
8.
Phadke  VK , Bednarczyk  RA , Salmon  DA , Omer  SB .  Association between vaccine refusal and vaccine-preventable diseases in the united states.   JAMA. 2016;315(11):1149-1158. doi:10.1001/jama.2016.1353 PubMedGoogle ScholarCrossref
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 Controlling rubella and preventing congenital rubella syndrome.   Wkly Epidemiol Rec. 2010;85(42):413-418.PubMedGoogle Scholar
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Centers for Disease Control and Prevention (CDC).  Three cases of congenital rubella syndrome in the postelimination era.   MMWR Morb Mortal Wkly Rep. 2013;62(12):226-229.PubMedGoogle Scholar
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