Bilateral hearing loss and constricted visual fields

These are funduscopic images showing features of retinitis pigmentosa in a man in his early 20s with Usher syndrome (fig 1).bmj;378/aug03_2/e070672/F1F1f1Fig 1The patient was referred to the ophthalmology department after optometry assessment because he reported longstanding problems with his vision, which worsened at night and he bumped into objects. Visual field assessment showed substantial constriction. Slit lamp examination identified early posterior subcapsular cataracts, waxy disc pallor (arrowhead), arteriolar attenuation (white arrow), and bone spicules (dotted arrow). Given the patient’s history of bilateral sensorineural hearing loss from birth, combined with the fundal findings, the symptoms and signs were consistent with a diagnosis of Usher syndrome.Usher syndrome is an autosomal recessive condition characterised by profound congenital hearing impairment, early retinitis pigmentosa (usually evident within the first decade), and vestibular dysfunction. The condition can be categorised into types I-IV after visual, auditory, and vestibular examinations. Although Usher syndrome has an incidence of only…
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