Type 2 Usher Syndrome - A Cause for Sensorineural Hearing Loss

Usher syndrome is a genetic condition involving Sensorineural hearing loss and Retinitis pigmentosa (RP). Although considered a rare disease, it is the most frequent cause of deaf- blindness in humans. It is inherited in an autosomal recessive pattern. It is divided clinically into three significant groups: Type I, Type 2 and Type 3. A retrospective study was carried out for three patients over a period of 5 years in a medical college hospital presenting with non-progressive hearing loss and progressive diminished vision. History, complete ENT, audiological and ophthalmological evaluation was performed. 3 patients (2 males, 1 Female) with a mean age 24 years had bilateral moderate to severe sensorineural hearing loss and bilateral retinitis pigmentosa. All patients were rehabilitated with hearing aids, and medical treatment was given for ophthalmic issues. Usher syndrome is the rare possibility of bilateral hearing loss since early childhood. Early detection of this condition with audiological and ophthalmic examination will improve the quality of life, improve hearing and delay the progression of complete blindness.