Clinical Manifestation and Progressive Sensorineural Hearing Loss in the Patients with Retinitis Pigmentosa

Abstract

Retinitis Pigmentosa is hereditary disorder characterized by progresive loss of photo receptors and retinal pigment epithelial function. Aim: Prospective examination of the patients with RP and retrospective examination of medical records of patients with retinitis pigmentosa. Material an Method: Typical familiar presentation of the desease (9 patients) and sporadic cases (15 patients) were examined from January 2006 do December 2016. Most od the cases are still following in the Clinic for eye disease in Skopje. The visual acuity, slip lamp and fundus examination, visual field, FFA, red cam investigation and OCT of the posterior segment were done in some of the patients. In 24 patients, condition of the hearing was studied with vestibular analyzation. Results: Clinical symptoms, FFA angiogram, OCT slides and visual fields results are discussed. The arterial attenuation, retinal bone-spicule pigmentation and waxy disc pallor were present in all patients. Other ocular features include the following: drusen of the PNO 33%, posterior sub capsular cataract 81%, keratoconus 54%, myopia 83%, open-angle glaucoma37,5% and vitreous opacification 66%. Neural sensorial hearing loss was noticed in 60% of the patients. RP patients with hearing loss have alteration of vestibular function of peripheral type in 80% and mixed type in 20%. Discussion: Visual prognosis depends of involvement of the macula-maculopathy and opacifications of the lens. Almost half of the patients with RP have social inadequate hearing (45% in our study). Conclusion: Dissolving the ocular complication and continuous following of the patients with retinitis pigmentosa in important for saving the vision acuity. Importance of examination of visual functions in cases of neurosensory hearing loss of unknown genesis is underlined.