Submit Manuscript | http://medcraveonline.com Introduction The aim of this study is to present a case of progresive sensorineural hearing loss with deterioration of visual acuity. Usher syndrome (US) is a rare but the most common cause of inherited combined vision and hearing loss. In order to establish a recent diagnosis of US genetic examination must be performed. Early detection for US in children with sensorineural hearing loss is very important. Psychological, educational and sociological assistance is also important. The patient may develop symptoms of retinitis pigmentosa in second or third decade of his life. The necessity of thorough investigation for detecting other systemic abnormalities should be emphasized. There is no effcient treatment of this syndrome. Case presentation A 45-year-old Caucasian man with high frequency sensorineural hearing loss (Figure 1) and deterioration of visual acuity. He gave a history of hearing loss for 10 years. His medical history was not signifcant and denied a history of ocular or head trauma, taking any medications. There is no family history. He underwent standard ENT and ophthalmologic examination. Visual feld examination disclosed peripheral ring scotoma. Electrophysiological studies was performed Ophthalmoscopy (Figure 2) demonstrated pigment in the retina. The presence of progresive sensorineural hearing loss indicated the necessity of performing the genetic study for US. Apart from a mild loss in visual acuity that had been relatively stable for several years, our patient did not have any other signifcant ocular symptoms. J Otolaryngol ENT Res. 2018;10(3):162‒164. 162 ©2018 Cetinkaya et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and build upon your work non-commercially. Usher syndrome: reason for combined hearing and vision loss Volume 10 Issue 3 - 2018 Erdem Atalay Cetinkaya,1 Mehmet Fatih Kucuk2 1 ENT Department, Antalya Ataturk State Hospital, Turkey 2 Ophthalmology Department, Antalya Ataturk State Hospital, Turkey Correspondence: Erdem Atalay Cetinkaya, ENT Department, Antalya Ataturk Devlet Hastenesi, KBB Klinigi, Antalya, 07000 Turkey, Tel 00905334110305, Email drerdemcetinkaya@gmail.com Received: February 04, 2018 | Published: June 19, 2018 Abstract Usher syndrome (US), also known as, retinitis pigmentosa-dysacusis syndrome, is an rare genetic condition but represents the most common cause of inherited combined vision and hearing loss. It is inherited in an autosomal recessive pattern. There are many gene proteins have been identifed, that all of them are very important for the function of the inner ear stereocilia . Early and accurate detection of US is crucial and it is generally performed through hearing, balance, vision tests and genetic study. This study is a brief clinical and genetic analysis of US patients. Keywords: usher, sensorineural hearing loss ,retinitis pigmentosa, clinical genetics Journal of Otolaryngology-ENT Research Case Report Open Access Figure 1 Pure tone audiometry presents bilateral moderetaly severe high frequency sensorineural hearing loss.