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Updated: Jun 7, 2025
09:44
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
19.1K
Type 2 Usher Syndrome - A Cause for Sensorineural Hearing Loss.
B L Yatish Kumar1, Manjoo S Reddy2, N Vijayendra Simha1
1Ent-Hns, Eastpoint College of Medical Sciences & Research Center Virgonagar, Bidrahalli, Bengaluru, 560049 Karnataka India.
Summary
Usher syndrome, a genetic cause of deafness and blindness, requires early audiological and ophthalmological exams. Prompt diagnosis and management of Usher syndrome improve patient quality of life and vision.
Area of Science:
- Genetics
- Ophthalmology
- Audiology
Background:
- Usher syndrome is a leading genetic cause of deaf-blindness.
- It presents as sensorineural hearing loss and retinitis pigmentosa (RP).
- Inherited in an autosomal recessive pattern, it has three clinical types.
Purpose of the Study:
- To report on three cases of Usher syndrome.
- To highlight the importance of early diagnosis and management.
Main Methods:
- A retrospective study of three patients over five years.
- Evaluations included history, ENT, audiology, and ophthalmology.
Main Results:
- Three patients (2 male, 1 female, mean age 24) had moderate-to-severe bilateral hearing loss and RP.
- All patients received hearing aids and ophthalmic treatment.
Conclusions:
- Usher syndrome is a rare cause of early-onset hearing loss.
- Early audiological and ophthalmic screening improves quality of life and delays blindness.
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