Hearing Loss in Alström Syndrome: Timeline and Care

Hearing loss is one of the major features of Alström Syndrome. Around 70% of children with Alström develop sensorineural hearing loss in the first decade of life, and almost everyone with Alström has some degree of hearing loss by adulthood.¹ Hearing loss in Alström is gradual, predictable, and well-managed with the right care. This article covers the typical timeline, why hearing changes, and how families and adults navigate the changes.

Hearing loss in Alström is sensorineural — it comes from problems in the inner ear (cochlea) or the auditory nerve, rather than from blockages in the outer or middle ear. Specifically, the dysfunction in Alström affects:

• Outer hair cells in the cochlea — these amplify quiet sounds before they're processed
• Possibly the stria vascularis — the structure that maintains the special chemistry of cochlear fluids²

The hearing loss is bilateral (both ears) and predominantly symmetric. It progresses gradually over years.

Birth to age 3

Most babies with Alström pass newborn hearing screenings — hearing is generally normal at birth. This distinguishes Alström from Usher Syndrome Type 1 (where hearing loss is congenital).

Ages 3 to 10

Hearing loss appears in around 70% of children during the first decade. It usually begins as mild high-frequency loss and gradually involves more frequencies. Parents often notice:

• The child not responding when called from another room
• Asking "what?" frequently
• Turning up the TV volume
• Difficulty hearing in noisy environments
• Speech delay or articulation issues

Ages 10 to 20

Hearing loss progresses through adolescence. By the late teens, many young people with Alström have moderate to severe sensorineural hearing loss. Hearing aids are often fitted during this period.³

Adulthood

A 2022 study from the UK National Alström Service tracked adults and found hearing deteriorated at about 1.23 dB per year on average. Earlier studies found progression of approximately 10–15 dB per decade.⁴ Most adults with Alström have moderate to severe hearing loss and benefit from amplification or, in some cases, cochlear implantation.

The functional impact varies by stage:

Mild loss

Difficulty hearing soft speech, especially with background noise; may miss parts of conversations. School-age children may struggle in busy classrooms.

Moderate loss

Conversational speech becomes difficult without amplification; phone calls are challenging; hearing aids are usually beneficial.

Severe loss

Even amplified speech can be hard to understand; reliance on hearing aids is significant; lip-reading and visual cues become more important.

Profound loss

Hearing aids may not provide enough benefit; cochlear implants may be considered; communication moves toward more visual or tactile methods.

For people with Alström, the additional context is that vision is also changing. The combination — declining hearing alongside declining vision — is what makes Alström deafblindness; communication strategies adapt accordingly.

Annual audiology evaluation is recommended. A typical visit includes:

• Pure-tone audiometry — measures hearing across frequencies; produces an audiogram
• Speech audiometry — measures speech understanding at different volumes
• Tympanometry — measures middle-ear function (rules out conductive hearing loss)
• Otoacoustic emissions (OAEs) — measures outer hair cell function; abnormal in Alström
• Auditory brainstem response (ABR) — measures the brainstem response to sound; useful in young children⁵

In babies and very young children, ABR and OAE testing are most useful. Pure-tone audiometry becomes feasible around age 3–4 with cooperative testing.

Most children with Alström benefit from digital behind-the-ear or receiver-in-canal hearing aids. Modern hearing aids:

• Provide multi-channel amplification tuned to the specific audiogram
• Include directional microphones for noisy environments
• Stream audio from phones, TVs, and FM/DM systems
• Connect to school accessibility systems (FM, soundfield amplification)

Pediatric audiologists fit hearing aids for children with Alström much as they would for any child with sensorineural hearing loss. Adjustments are made over time as hearing changes.⁶

For people with severe to profound hearing loss where hearing aids aren't providing enough benefit, cochlear implants are an established option. Important points for Alström specifically:

• Cochlear implants work in Alström. A 2020 case series from Italy described excellent outcomes in patients with Alström who received cochlear implants.⁷
• Candidacy is individual. A cochlear implant team evaluates the specific person's situation — current hearing, language skills, motivation, family support.
• Younger is generally better. Cochlear implant outcomes are typically best when implantation happens before language delays accumulate.
• The Alström-specific advantage: because the dysfunction is largely in the outer hair cells (cochlea), cochlear implants — which bypass the cochlea and stimulate the auditory nerve directly — can work very well.

We cover this in detail in Cochlear Implants in Alström Syndrome.

As hearing changes, families adapt:

• Get the listener's attention before speaking
• Reduce background noise in conversations
• Face the person and ensure good lighting for any residual visual support
• Speak clearly at a normal pace — neither too fast nor exaggerated
• Repeat or rephrase when needed
• Use captioning on TV and video; CART services for events
• Learn basic sign language if relevant to the family

For people with combined hearing and vision loss, tactile communication methods (tactile signing, print-on-palm, Pro-Tactile communication) become important — see Communication Methods for a Deafblind Child With Alström.

• Alström Syndrome Symptoms by Stage
• Hearing Aids for Kids With Alström
• Cochlear Implants in Alström Syndrome
• Annual Audiology Tests for Alström Syndrome
• Communication Methods for a Deafblind Child With Alström

April 30, 2026.