CLINICAL MANIFESTATION AND PROGRESSIVE SENSORINEURAL HEARING LOSS IN THE PATIENTS WITH RETINITIS PIGMENTOSA

Abstract

Retinitis pigmentosa (RP) is an inherited retinal disorder characterized by progressive loss of photoreceptors and retinal pigment epithelium. RP is characterized by impaired night vision, slowly progressive peripheral to central visual field loss, and often a decline in visual acuity. Aim: Clinical examination of patients with retinitis pigmentosa. Material and method: From January 2006 to December 2017, 24 patients with retinitis pigmentosa (RP) were examined. The visual acuity, slip lamp and fundus examination, visual field, red cam investigation, FFA and OCT of the posterior segment were done. In the patients, the hearing condition was examined with vestibular testing. Most of the cases are still followed in the University Eye Clinic in Skopje. Results: Clinical symptoms, FFA angiograms, OCT slides and visual fields results are discussed. All 24 patients showed either arteriolar attenuation, retinal bone-spicule pigmentation and/or waxy disc pallor, which result in progressive vision loss. 18 patients showed familiar characteristic presentation of the disease and 6 patients showed sporadic cases. Myopia was found in 62,5% (15 patients) and keratoconus in 25% (6 patients). On the posterior segment, fundus, drusen of the PNO in 20,8% (5 patients) and macular abnormalities (macular edema, macular atrophy, cellophane maculopathy) in 16,7% (4 patients) was observed. Vitreous opacification 66,7% (16 patients) and cataract 62,5% (15 patients) were present and lead to additional deterioration of vision acuity. The open-angle glaucoma was found in 12,5% (3 patients). The results of the sensorineural hearing tests suggest that most of the patients with RP have difficulty communicating and socially inadequate hearing. Sensorineural hearing loss was observed in 62,5% (15 patients). In these 15 patients with hearing loss, alteration of vestibular function of peripheral type was found in 80% (12 patients) and of mixed type in 20% (3 patients). Conclusion: Treating the retinitis pigmentosa visual and hearing manifestations, as well as continuous following of the RP-affected patients is vital for preserving the vision acuity, auditory acuity and quality of life. Patients with neurosensory hearing loss of unknown genesis should necessarily undergo a preliminary eye examination to confirm or exclude the RP diagnosis.