Understanding Usher Syndrome: Usher syndrome is a disorder characterized by the partial or total loss of hearing combined with progressive vision loss. The word syndrome refers to a disease or disorder that presents more than one symptom. Because the vestibular system is greatly affected, diagnosis of Usher Syndrome is heavily reliant on the observation and evaluation of a patient’s senses, specifically hearing, vision, and balance.
While Usher Syndrome accounts for roughly 50 percent of deaf-blindness in adults, the disorder itself is relatively rare. Approximately 400,000 people are affected by the disorder worldwide; 16,000 of which reside in the United States.
Based on the severity of symptoms and the age at which they manifest, there are three types of Usher Syndrome: Usher Type I, Usher Type II, and Usher Type III. Existence of a fourth type is a hotly debated issue among medical specialists.
To learn more about the different types of Usher Syndrome, check out the infographic below:
Understanding Usher Syndrome: Type I
Patients affected by Type I Usher syndrome experience profound deafness in both ears and severe balance problems at birth. Deterioration of vision oftentimes begins in early childhood with patients noting difficulty seeing at night before the age of 10.
Understanding Usher Syndrome: Type II
Hearing loss in patients with Type II Usher syndrome varies from moderate to severe at the time of birth. Unlike Type I, these patients benefit from the use of hearing aids and have normal balance. Additionally, patients often times do not experience vision problems until their teenage years.
Understanding Usher Syndrome: Type III
Patients with Type III Usher syndrome are born with normal hearing and balance and typically do not encounter problems until puberty. These patients usually become reliant on hearing aids near mid to late adulthood. Problems with vision are progressive, with patients usually being declared legally blind by mid-adulthood.
Understanding Usher Syndrome: Type IV
Little is known about Type IV Usher syndrome. Like other types of the disorder, Type IV is characterized by hearing loss and progressive vision problems. Type IV affects mostly males.