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Hearing Loss with Vision Impairment: Usher Syndrome. A Case of the East Democratic Republic of Congo.


ABSTRACT: Usher syndrome (USH) is a clinically heterogeneous condition characterized by sensorineural hearing loss, progressive retinal degeneration, and vestibular dysfunction. There are two phenotypically recognizable types of Usher syndrome described in the literature. Usher type 1 individual have no vestibular function and profound sensorineural hearing loss. Usher type 2 individuals have a normal vestibular function and mild-to-severe hearing loss with visual impairment that is presented later in life. We are reporting a case of 35 years old gentleman with hearing loss and visual impairment presented to the ENT clinic at the tertiary care center. Clinical evaluations as well as comprehensive testing of hearing, vestibular function, and visual function have confirmed USH. It's a rare but serious

SUBMITTER: Edouard AM 

PROVIDER: S-EPMC10646019 | biostudies-literature | 2023 Dec

REPOSITORIES: biostudies-literature