Overview
Hearing loss continues through adulthood for most people with Alström Syndrome. The 2022 UK National Alström Service study tracked adults and found progression of about 1.23 dB per year on average — a slow but steady decline.¹ This article covers what hearing loss looks like across adulthood, when to upgrade hearing technology, and how adults living with Alström adapt over time.
What hearing changes look like in adulthood
Most adults entering their 20s with Alström have moderate hearing loss already, often using hearing aids. Through their 20s, 30s, and beyond, hearing typically:
- Continues to decline gradually across all frequencies
- May reach severe-to-profound levels in middle age for some
- Combines with vision loss to create the deafblindness picture
- Eventually exceeds what hearing aids alone can compensate for in many cases
Individual variation is real. Some adults remain stable for years; some progress faster. Annual audiometry tracks the trajectory.
When to upgrade hearing technology
Updating hearing aid prescriptions
The audiogram changes annually for most adults with Alström, and hearing aids should be reprogrammed accordingly. Many audiologists recommend a verification visit every 6–12 months to ensure aids are optimally adjusted.
Replacing hearing aids
Hearing aid hardware typically lasts 5–7 years before manufacturer support and parts become limited. Replacement during this window is often appropriate. With each generation, technology improves — better noise reduction, better Bluetooth integration, smaller form factors.
Considering cochlear implants
When hearing aids no longer provide adequate benefit — often defined as word recognition with optimally-fitted aids dropping below 50% in the better ear — cochlear implant evaluation is appropriate. Cochlear implants in adults with Alström have shown excellent outcomes in published reports.² We cover this in Cochlear Implants in Alström Syndrome.
Adding accessory technology
- Bluetooth streaming to phone, TV, and computer
- Remote microphones for one-on-one conversations in noise (Roger system, similar)
- Captioning services for video content
- Vibrating alerting devices for doorbells, alarms
Adapting work and home environments
At work
Common workplace accommodations under disability law (ADA in the US, similar elsewhere) include:
- Captioned phone services
- Remote microphone accessories provided by employers
- Quiet workspace away from open-plan noise
- Captioning for video meetings (now standard on most platforms)
- Email and chat-based communication preferences when speech is difficult
Many adults with Alström work successfully across diverse fields with these adaptations.
At home
Communication patterns shift toward:
- Quiet environments for important conversations
- Direct face-to-face when possible (during the years when residual vision still helps)
- Repetition and rephrasing as natural parts of conversation
- Captioned TV and video — easy to enable on most modern devices
- Smart-home alerting (visual or vibrating alerts for doorbells, smoke alarms, baby monitors)
In the community
- Identification (such as a deafblind ID card or alerting bracelet) helps in unfamiliar situations
- Reservation of accessible seating in restaurants and venues
- Communication cards explaining preferred methods to strangers
Annual audiology in adulthood
Annual audiology testing in adulthood includes the same components as pediatric testing — pure-tone audiometry, speech audiometry, OAEs, tympanometry — plus:
- Hearing aid verification and adjustment
- Discussion of evolving needs and any cochlear implant considerations
- Updates to accommodations documentation
Coordination with the broader Alström care team continues to matter, particularly cardiac status before any planned cochlear implant surgery.
Mental health and hearing changes
The combination of changing hearing alongside changing vision can be psychologically demanding. Common experiences:
- Increased social fatigue from communication effort
- Withdrawal from group settings that were previously enjoyable
- Frustration with peers and family who haven't adjusted to communication needs
- Periods of grief revisiting as functions decline
Mental-health support — whether through formal therapy, peer support, or other means — helps. We cover this in Mental Health and Wellbeing in Alström Syndrome.
Common questions
Frequently asked questions
Short answers grounded in the article and the underlying references, so families can quickly understand the main point without losing the medical meaning.
Question
Is the rate of hearing loss the same for everyone with Alström?
Answer
No — there's individual variation. Some adults are relatively stable for years; others progress faster. Annual testing tracks your specific trajectory.
Question
Will my speech be affected by hearing loss?
Answer
Adults with Alström who developed hearing in childhood and used hearing aids typically maintain spoken language well. Speech may become slightly less clear over years if hearing loss is severe and unmanaged, which is one reason ongoing audiology care matters.
Question
Can I still use the phone?
Answer
Most adults with Alström use phones with hearing aids using Bluetooth streaming. Captioned phone services (CapTel, similar) provide real-time text alongside the call. As hearing changes, video relay services and text-based communication become more central.
Question
Is there a point where hearing aids stop helping?
Answer
Yes — when word recognition with optimally-fitted aids drops significantly. At that point cochlear implant evaluation is reasonable. Many adults with Alström use a combination of hearing aids in one ear and a cochlear implant in the other, or bilateral cochlear implants.