Does Retinitis Pigmentosa (RP) Cause Hearing Loss?

If you have been diagnosed with standard (non-syndromic) retinitis pigmentosa (RP), the condition itself does not cause you to lose your hearing ^[1][2]. It is completely understandable to feel anxious after researching RP and stumbling across mentions of Usher Syndrome. However, standard RP and Usher Syndrome are different conditions. In non-syndromic RP, the genetic mutations only affect the retina in your eyes; this means that while you may experience typical age-related hearing changes later in life, your hearing is safe from the RP disease process ^[1].

Understanding the Difference: Syndromic vs. Non-Syndromic RP

When you read about RP, you will often see it divided into two main categories:

• Non-syndromic RP: This is the most common form of the condition. It means the retinitis pigmentosa occurs on its own and only affects your vision ^[1].
• Syndromic RP: This means the vision loss is part of a larger syndrome that affects other parts of the body. Usher Syndrome is the most common type of syndromic RP, and it specifically combines retinal degeneration with sensorineural hearing loss (hearing loss caused by issues in the inner ear or hearing nerve) ^[3][4].

Why You Unlikely Have Usher Syndrome

If you are an adult who was recently diagnosed with standard RP and you currently have normal hearing, it is highly unlikely that you will suddenly develop Usher Syndrome. Here is why:

• Hearing loss usually starts early: In the most common forms of Usher Syndrome (Types 1 and 2), the hearing loss is congenital—meaning it is present at birth—or it begins in early childhood ^[5][6].
• Different disease courses: If you had Usher Syndrome Types 1 or 2, you or your doctors would have likely noticed significant hearing or balance issues long before you were diagnosed with vision loss as an adult. Severe balance issues (often noticed as delayed walking in childhood) are a hallmark of Usher Type 1, whereas Type 2 usually does not cause balance problems.
• There is a rare exception: Usher Syndrome Type 3 does involve progressive hearing loss that can start later in life in late childhood or teenage years, and may sometimes be accompanied by later-onset balance issues or a decline in the sense of smell ^[7]. However, Type 3 is very rare overall, making up a very small percentage of Usher Syndrome cases.

The Role of Genetic Testing

Because there are more than 70 different genes associated with non-syndromic RP, doctors often recommend genetic testing ^[3][8]. Interestingly, changes in a specific gene called USH2A can cause either Usher Syndrome Type 2 (with hearing loss) or non-syndromic RP (without hearing loss) ^[9][10].

If genetic testing shows that your RP is caused by a mutation in the USH2A gene, but you have the non-syndromic form, you will not develop Usher-related hearing loss, though you may still experience normal age-related hearing changes just like anyone else ^[10]. Genetic testing is the most definitive way to understand exactly which form of RP you have and can provide immense peace of mind by ruling out syndromic conditions ^[1][11].

Next Steps for Your Peace of Mind

If you are still concerned about your hearing:

• Get a baseline hearing test: Ask your primary care doctor for a referral to an audiologist for a formal audiogram. This can establish a baseline for your hearing right now, which is reassuring and helpful for monitoring your overall health over time.
• Ask about genetic testing and counseling: If you haven’t already, discuss with your doctor whether genetic testing is appropriate to identify the exact cause of your RP. Working with a genetic counselor is highly recommended, as they are crucial for interpreting complex results.