Tinnitus and Balance Issues in Alström Syndrome

Tinnitus (ringing or noise in the ears) and vestibular dysfunction (balance problems) are less commonly discussed features of Alström Syndrome but are real for some affected individuals. The exact frequency in Alström is not well-characterized in published literature, but reports from patient organizations and case series suggest a notable subset. This article covers what's known, what's anecdotal, and management options for both.

Tinnitus is the perception of sound — ringing, buzzing, hissing, humming — without an external source. It's common in any population with sensorineural hearing loss and is reported in some adults with Alström.¹

Tinnitus in Alström:

• Is most common in adults with established hearing loss
• May be constant or intermittent
• Can vary in pitch and loudness
• Is not specifically caused by Alström — it shares mechanisms with tinnitus in other sensorineural hearing losses
• Can be made more noticeable by quiet environments

Management

Approaches include:

• Hearing aids — by amplifying environmental sounds, hearing aids often reduce the perceptual prominence of tinnitus
• Sound therapy — masking sounds that distract from tinnitus, often built into modern hearing aid programs or available through dedicated devices
• Cognitive-behavioral therapy — research-supported for managing distress from tinnitus
• Tinnitus retraining therapy — combines counseling with sound therapy
• Avoiding tinnitus triggers — high salt, caffeine, certain medications, excessive noise exposure
• Sleep hygiene — sound machines or fans for nighttime, since tinnitus is often most noticeable in quiet bedrooms

There is no specific medication that reliably treats tinnitus. Various supplements and products are marketed for tinnitus; few have good evidence.

The inner ear includes both the cochlea (hearing) and the vestibular system (balance). Both share specialized hair cells, and both can be affected in conditions involving cochlear damage.²

In Alström, vestibular dysfunction is generally less severe than in conditions like Usher Syndrome Type 1 (where vestibular function is profoundly affected from birth). Most people with Alström don't have severe balance issues. Some report:

• Mild unsteadiness
• Difficulty with balance in dark environments
• Feeling off-balance after head movements
• Problems with balance on uneven surfaces

Balance is also affected by other Alström features — vision loss reduces visual input that helps balance, scoliosis can affect posture, and orthopedic findings (flat feet, brachydactyly) contribute. The full balance picture can be multi-factorial.

Vestibular evaluation

If balance is a significant concern, a vestibular evaluation can help identify whether the problem is from the inner ear, the vision system, or musculoskeletal issues. Tests include:

• Videonystagmography (VNG) — measures eye movements during balance tasks
• Rotational chair testing
• Posturography — measures how the body responds to balance challenges

Vestibular rehabilitation

If inner-ear involvement contributes to balance issues, vestibular rehabilitation therapy can help. A physical therapist trained in vestibular rehab provides exercises that train the brain to use available balance information more effectively.

Bring tinnitus or balance concerns to your audiology and otology team. Patterns to mention:

• When tinnitus started, and what it sounds like
• Whether it's interfering with sleep, concentration, or mood
• Specific situations where balance is most affected
• Any falls or near-falls
• Whether the symptoms are stable or getting worse

For balance concerns specifically, a referral to a vestibular specialist or physical therapist with vestibular expertise can help.

While Alström-related cochlear changes can't be prevented, you can reduce additional risk:

• Protect remaining hearing — avoid loud noise without ear protection
• Manage cardiovascular risk — diabetes, blood pressure, and lipid management protect the small blood vessels of the inner ear
• Adequate sleep — tinnitus is often more prominent with fatigue
• Stress management — stress can amplify tinnitus perception
• Stay active — physical activity supports overall vestibular and proprioceptive function

• Hearing Loss in Alström Syndrome
• Hearing Loss in Adults With Alström Syndrome
• Building Independence in a Child With Alström Syndrome
• Adult Life With Alström Syndrome

April 30, 2026.