Giant retinal tear and retinal detachment with underlying retinitis pigmentosa and hearing loss

Few retinal detachments have been described in patients with retinitis pigmentosa and allied retinal disorders, with only two cases (both with hearing loss) reported in association with giant retinal tears. To further characterise clinical characteristics of giant retinal tear associated with retinitis pigmentosa, we reviewed the course of four eyes of three patients. Unexpectedly, all three individuals also suffered from congenital sensorineural hearing loss. One suffered from associated myopathy. Despite aggressive surgical management, three of the four eyes became blind. The diagnosis of retinal detachment preceded the diagnosis of photoreceptor dystrophy in two of the three patients. To date, giant retinal tears occurring with underlying retinitis pigmentosa have been described in five young individuals, all of whom had associated congenital sensorineural hearing loss.