Usher Syndrome is distinct among inherited retinal conditions because it combines progressive retinal degeneration with sensorineural hearing loss, and in some forms, balance difficulties caused by inner-ear (vestibular) dysfunction. It is broadly divided into three clinical types, each with a different pattern and severity of hearing involvement and a different age at which retinal symptoms typically begin.
Because two separate sensory systems are involved, Somali families are guided through a coordinated evaluation that looks at hearing, balance, and vision together, rather than treating the retinal component in isolation.

Associate Professor, Vitreo-Retina Surgeon. 10+ Years Experience

Senior Consultant Ophthalmologist & Eye Surgeon· 26+ yrs experience

Consultant Ophthalmologist & Eye Surgeon, MD, DO .16+ Years Experience
Retinal management follows the same principles used for other rod-cone dystrophies, with stem cell therapy considered as a supportive option for eligible patients. Because the hearing component often requires separate management — including hearing aids or cochlear implant candidacy — the retinal care plan is designed to work alongside, not replace, audiology care that may already be underway or planned in Somalia.
“Our team focuses specifically on the retinal aspects of Usher Syndrome. Hearing management, including cochlear implant evaluation, is a separate specialty and we coordinate around whatever audiology care you already have in place or are pursuing locally.”
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It’s reasonable to ask for a retinal evaluation, particularly if Usher Syndrome Type I is suspected, since vision symptoms in that type can begin to appear from later childhood. Early baseline testing makes it easier to track any changes over time.”
“No — congenital hearing loss has many possible causes, and Usher Syndrome accounts for only a portion of them. Genetic testing alongside a retinal ERG is the most reliable way to confirm or rule out Usher Syndrome specifically.”
“Type is determined by the pattern and severity of hearing loss, presence or absence of balance issues, and age of retinal symptom onset, confirmed where possible by genetic testing. It does influence prognosis discussions, though the general framework for retinal evaluation and management is similar across types.”
