Abstract

BackgroundThe aim of this article is to describe visual outcomes and posterior rehabilitation of the first Usher syndrome type II (USH2) patient receiving an Argus II (®) prosthesis.Case presentationWe present a case of a USH2 patient who underwent Argus II prosthesis surgery at the age of 53. He had hearing loss from birth and presented a very poor visual field with good light perception. He communicated through sign language translated by his interpreter, who explained all the information regarding the surgical procedure and who assisted in the posterior visual therapy.Sixteen months after surgery, the patient communicates more fluently with sign language and is able to identify letters with high contrast over 6 cm and words up to four letters.ConclusionsThis is the first case described in the literature of a USH2 patient receiving an Argus II prosthesis This is an alternative treatment for USH2 patients, whose interpreters are essential in the selection process and subsequent rehabilitation after surgery.

Highlights

  • The aim of this article is to describe visual outcomes and posterior rehabilitation of the first Usher syndrome type II (USH2) patient receiving an Argus II (®) prosthesis.Case presentation: We present a case of a USH2 patient who underwent Argus II prosthesis surgery at the age of 53

  • This is the first case described in the literature of a USH2 patient receiving an Argus II prosthesis This is an alternative treatment for USH2 patients, whose interpreters are essential in the selection process and subsequent rehabilitation after surgery

  • Usher syndrome type II (USH2) is a syndrome characterized by retinitis pigmentosa (RP) with significant visual reduction by the age of 50, sensor- neural hearing loss from birth and sometimes vestibular involvement [1]

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Summary

Introduction

Case presentation: We present a case of a USH2 patient who underwent Argus II prosthesis surgery at the age of 53 He had hearing loss from birth and presented a very poor visual field with good light perception. Usher syndrome type II (USH2) is a syndrome characterized by retinitis pigmentosa (RP) with significant visual reduction by the age of 50, sensor- neural hearing loss from birth and sometimes vestibular involvement [1]. It is clinically and genetically heterogeneous, and has an autosomal recessive inheritance [2]. Intelligible language development does not evolve in parallel with proper hearing, which necessitates the teaching from diagnosis of non-visual dependent rehabilitative strategies through sign language, involving hand contact with the help of an interpreter.

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