Usher Syndrome in Grand Rapids, Michigan

Usher syndrome is the most common genetic cause of combined vision and hearing impairment and deafblindness. People with Usher syndrome usually have:

  • Partial or total hearing loss
  • Gradual vision loss caused by retinitis pigmentosa (RP)
  • In some cases, problems with balance and coordination

In Grand Rapids and across West Michigan, early diagnosis and coordinated care through local specialists (such as those at Corewell Health – Spectrum Health, Trinity Health Grand Rapids, Metro Health – University of Michigan Health, and Mercy Health clinics) can make a major difference in quality of life.

What Is Usher Syndrome?

Usher syndrome is a genetic condition present from birth, even though symptoms may not appear until later in childhood or adolescence. It affects:

  • Hearing (inner ear)
  • Vision (retina in the back of the eye)
  • Balance (vestibular system in the inner ear)

There is currently no cure for Usher syndrome, but there are many treatments and supports in Grand Rapids to help people manage hearing, vision, and balance changes over time.

Types of Usher Syndrome

Usher syndrome is grouped into three main types based on when symptoms start and how severe they are:

  • Type 1 (US1)
  • Type 2 (US2)
  • Type 3 (US3)

Types 1 and 2 together account for about 5% of all children born with a hearing impairment.

Usher Syndrome Type 1 (US1)

US1 is usually the most severe form and symptoms start early in life.

Typical features of US1:

  • Hearing
    • Profound deafness from birth
    • Speech difficulties are common
    • Children usually need sign language (such as American Sign Language – ASL) or other visual communication methods
  • Balance
    • Balance is often significantly affected
    • Children may be late to sit, stand, and walk
    • May appear clumsy or unsteady, especially on uneven surfaces or in the dark (a common issue in West Michigan’s long winter evenings)
  • Vision
    • Retinitis pigmentosa usually begins in early childhood, often around age 10
    • Night blindness is often the first sign (difficulty seeing in dim light, such as during Michigan’s dark winter mornings and evenings)
    • Over time, peripheral (side) vision narrows, leading to “tunnel vision”

US1 is further divided into several subtypes based on the specific gene involved, but the day-to-day symptoms are usually similar.

Usher Syndrome Type 2 (US2)

US2 is generally milder than Type 1 in terms of hearing and balance, but vision loss from RP still occurs.

Typical features of US2:

  • Hearing
    • Moderate hearing loss in the lower sound frequencies
    • Severe hearing loss in the higher sound frequencies
    • Many people benefit from hearing aids and speech therapy
  • Balance
    • Usually normal in childhood
    • Some people may develop balance issues later in life
  • Vision
    • Signs of retinitis pigmentosa (such as blind spots or night blindness) often begin in the teenage years
    • Vision loss may slowly progress throughout adulthood

There are three known subtypes of US2, each linked to different genes.

Usher Syndrome Type 3 (US3)

US3 is less common globally and in Michigan. It is more frequent in people of Finnish descent, but can occur in any ethnic group.

Typical features of US3:

  • Hearing
    • Hearing is usually normal at birth
    • Progressive hearing loss develops over time and becomes measurable by puberty
  • Balance
    • Balance is close to normal in early life
    • Balance function often deteriorates as the person gets older
  • Vision
    • Retinitis pigmentosa typically begins in the teenage years
    • Vision loss progresses gradually over adulthood

Only one subtype of US3 has been identified so far.

How Usher Syndrome Affects Vision, Hearing, and Balance

Retinitis Pigmentosa (RP) and Vision Loss

RP is an eye disease that damages the retina, the light-sensitive tissue at the back of the eye.

Common stages of RP:

  1. Night blindness
    • Trouble seeing in low light (for example, walking outside in winter evenings in Grand Rapids, or in dim restaurants)
  2. Loss of peripheral vision
    • Side vision slowly shrinks, leading to “tunnel vision”
  3. Advanced vision loss
    • Central vision may eventually be affected, making reading and recognizing faces difficult

Hearing Loss

Hearing loss in Usher syndrome can be:

  • Congenital (present at birth) – more common in Types 1 and 2
  • Progressive (worsening over time) – typical in Type 3

Hearing loss may affect speech development in children and communication in school, work, and social settings. Early access to audiology services in Grand Rapids is important.

Balance Problems

Balance problems in Usher syndrome happen because of damage to the vestibular system in the inner ear.

The brain relies on three main systems for balance:

  • Inner ear (vestibular system) – fluid-filled canals that detect head movement
  • Eyes �� provide information about the environment
  • Proprioception – sensors in muscles, joints, and skin that sense body position

When the vestibular system is affected, a person may:

  • Feel unsteady or dizzy
  • Have trouble walking in the dark or on icy or uneven surfaces (a frequent challenge during snowy and slippery West Michigan winters)
  • Be slower to reach motor milestones in childhood (sitting, standing, walking)

Genetic Causes of Usher Syndrome

Usher syndrome is a genetic condition, meaning it is caused by changes (mutations) in certain genes.

  • Humans have 46 chromosomes, including:
    • 2 sex chromosomes (X and Y)
    • 44 other chromosomes (autosomes)
  • Around 30,000–40,000 genes are arranged on these chromosomes
  • Each gene comes in pairs – one from each parent

Researchers believe there are about 15 different genes that can cause Usher syndrome. Different gene changes lead to different types and subtypes of Usher syndrome.

Inheritance: Autosomal Recessive Pattern

Usher syndrome follows an autosomal recessive inheritance pattern.

This means:

  • A child must inherit two copies of a changed Usher gene (one from each parent) to develop the condition
  • If a person has only one changed gene, they are a carrier but usually do not have symptoms
  • If both parents are carriers of the same Usher gene change, in each pregnancy there is:
    • 25% chance (1 in 4) the child will have Usher syndrome
    • 50% chance (1 in 2) the child will be a carrier
    • 25% chance the child will not carry the changed gene

If parents are carriers of two different Usher gene changes, their child will not develop Usher syndrome but may be a carrier of both.

Diagnosis of Usher Syndrome in Grand Rapids

Early diagnosis can help families in West Michigan plan for school, communication, mobility, and safety—especially during darker, icy seasons when vision and balance issues can be more challenging.

Common tests include:

  • Eye tests
    • To check for retinitis pigmentosa
    • May include visual field testing, retinal imaging, and electroretinography (ERG)
  • Hearing tests (audiology)
    • To measure the type and degree of hearing loss
  • Balance tests
    • Such as electronystagmography (ENG) or videonystagmography (VNG) to assess the vestibular system
  • Genetic testing
    • To confirm the type of Usher syndrome and identify the specific gene involved

In Grand Rapids, these tests may be arranged through:

  • Pediatric and adult audiology and ophthalmology services at Corewell Health – Spectrum Health, Trinity Health Grand Rapids, or Metro Health – U of M Health
  • Genetic counseling and testing services available through local hospital systems and Kent County Health Department referrals

Treatment and Management in Grand Rapids, MI

There is currently no cure for Usher syndrome. Treatment focuses on managing hearing, vision, and balance problems, and supporting communication, learning, and independence.

Managing Hearing Loss

Options may include:

  • Hearing aids
    • For mild to severe hearing loss, especially in US2 and US3
  • Cochlear implants
    • For people with profound hearing loss, more common in US1
  • Assistive listening devices and adaptive technology
    • FM systems in classrooms
    • Bluetooth devices and phone apps
  • Communication training
    • American Sign Language (ASL) instruction
    • Speech therapy
    • Lip-reading and other communication strategies

Local resources in the Grand Rapids area include:

  • Audiology and cochlear implant programs at:
    • Corewell Health – Spectrum Health
    • Trinity Health Grand Rapids
    • Metro Health – U of M Health
  • School-based services through Grand Rapids Public Schools and other Kent County districts (Individualized Education Programs – IEPs and 504 plans)

Managing Vision Loss

Vision care for Usher syndrome focuses on maximizing remaining sight and supporting safe mobility.

Options may include:

  • Low-vision services
    • Magnifiers, high-contrast materials, special lighting
    • Large-print or electronic reading devices
  • Orientation and mobility training
    • Learning to move safely with reduced vision
    • White cane training and travel skills (especially important in snow, ice, and low-light conditions common in Michigan)
  • Braille instruction
    • For those with significant vision loss
  • Adaptive technology
    • Screen readers, text-to-speech software, accessible smartphones and tablets

Local and regional vision resources for West Michigan residents include:

  • Vision rehabilitation services at major Grand Rapids health systems
  • Guiding Eyes/Guide Dog services (regional and national programs that serve Michigan residents)
  • Support and referrals through Kent County Health Department and Grand Rapids Public Health programs

Managing Balance Problems

For people with vestibular (balance) issues, treatment may include:

  • Vestibular rehabilitation therapy
    • Exercise-based program run by a vestibular physiotherapist
    • Aims to improve balance, coordination, and reduce dizziness
    • Especially helpful for children learning to walk and for adults navigating icy sidewalks and uneven outdoor terrain in West Michigan
  • Physical therapy
    • Strength and coordination exercises
    • Fall-prevention strategies

These services are available through many outpatient rehabilitation clinics in Grand Rapids affiliated with:

  • Corewell Health – Spectrum Health
  • Trinity Health Grand Rapids
  • Metro Health – U of M Health
  • Independent physical therapy and vestibular rehab centers in Kent County

Support Services for People Living with Usher Syndrome in West Michigan

Living with Usher syndrome can be emotionally and practically challenging for individuals and families. Support services can help with education, employment, daily living, and mental health.

Possible supports include:

  • Community support groups
    • Local and online groups for people with hearing and vision loss
    • National organizations such as:
      • Usher Syndrome Coalition
      • AADB (American Association of the DeafBlind)
      • AFB (American Foundation for the Blind)
  • Disability and adaptive services
    • Michigan-based organizations that support people with hearing and vision impairments
    • Vocational rehabilitation and job support through Michigan Rehabilitation Services
  • School and college accommodations
    • IEPs and 504 plans
    • Interpreters, captioning, note-taking support, assistive technology

In the Grand Rapids area, you can ask your:

  • Primary care provider or pediatrician
  • Hospital social worker
  • Kent County Health Department

for up-to-date referrals to local and statewide support programs.

Genetic Counseling and Testing in Grand Rapids

If you or your child has been diagnosed with Usher syndrome—or if it runs in your family—genetic counseling can be very helpful.

Genetic counselors are trained in both genetics and counseling. They can:

  • Explain what Usher syndrome is and how it is inherited
  • Review your family history and discuss your personal risk
  • Discuss genetic testing options for you and other family members
  • Help you understand what test results mean for:
    • Your health
    • Your children’s health
    • Future pregnancy planning
  • Provide emotional support and connect you with local and national resources

You may consider genetic counseling if:

  • Usher syndrome or early hearing/vision loss occurs in your family
  • You are planning a pregnancy and want to understand your risks
  • You are considering prenatal testing or preimplantation genetic testing (PGT) with IVF

In Grand Rapids and West Michigan, genetic counseling services are typically available through:

  • Corewell Health – Spectrum Health genetics clinics
  • Trinity Health Grand Rapids genetics services
  • Referrals from your family doctor, pediatrician, or ophthalmologist

Living With Usher Syndrome in Grand Rapids, MI

With the right supports, many people with Usher syndrome in West Michigan:

  • Attend mainstream schools and colleges
  • Work in a wide range of careers
  • Use technology and mobility skills to live independently
  • Stay active year-round, including adapting to Michigan’s:
    • Long, dark winters
    • Snow and ice (which can worsen balance and mobility challenges)
    • Glare from snow and water around the Great Lakes, which can be difficult for people with light sensitivity

Planning ahead for seasonal changes—such as using good lighting, non-slip footwear, and safe travel routes—can improve safety and confidence.

When to Seek Help in Grand Rapids

Talk to your doctor, pediatrician, or eye/ear specialist if you notice:

  • Your child is not responding to sound or has delayed speech
  • Your child is late to sit, stand, or walk
  • You or your child have trouble seeing at night or bump into objects in dim light
  • Progressive hearing loss or balance problems develop over time
  • There is a family history of Usher syndrome, early deafness, or retinitis pigmentosa

They can refer you to:

  • Audiology and ophthalmology specialists
  • Genetic counseling
  • Vestibular rehabilitation
  • Local support services in Grand Rapids and Kent County

Key Points About Usher Syndrome

  • Usher syndrome is the most common genetic cause of combined hearing and vision loss and deafblindness.
  • It is divided into three main types – Type 1, Type 2, and Type 3 – based on symptom severity and age of onset.
  • Symptoms usually include:
    • Hearing impairment or deafness
    • Progressive vision loss from retinitis pigmentosa (RP)
    • In some cases, balance problems
  • Usher syndrome is caused by changes in several different genes and follows an autosomal recessive inheritance pattern.
  • There is no cure, but in Grand Rapids, MI, people can access:
    • Hearing aids, cochlear implants, and communication training
    • Low-vision services, orientation and mobility training, and adaptive technology
    • Vestibular rehabilitation for balance problems
    • Genetic counseling and community support services

If you live in the Grand Rapids area and are concerned about Usher syndrome, start by contacting your primary care physician or pediatrician. They can help coordinate testing, referrals, and ongoing care with local specialists and support programs.