Overview
Annual audiology evaluation is a cornerstone of Alström care. Hearing changes gradually but predictably, and yearly testing tracks progression, optimizes hearing aids or cochlear implants, and informs school accommodations. This article explains what an annual audiology visit should include, what each test does, and how to make the most of these visits.
Why annual testing matters
Hearing loss in Alström progresses at roughly 1.23 dB per year on average in adults — a slow but cumulative change.¹ The 2020 international consensus guidelines recommend audiology evaluation at the time of diagnosis and annually thereafter.²
Annual testing:
- Documents the rate of change
- Updates hearing aid prescriptions or cochlear implant maps
- Identifies any new ear health issues (infections, fluid)
- Maintains records for school IEPs, work accommodations, and disability documentation
- Connects the family to current technology and services
What a comprehensive annual visit includes
The 2020 consensus guidelines specify the following components for annual audiology evaluation in people with Alström:³
Pure-tone audiometry
Measures the quietest sound the patient can hear at each frequency from 250 Hz to 8000 Hz. Results plotted on an audiogram. Done in a sound-treated room with headphones or earphones.
Speech audiometry
Measures the patient's ability to:
- Detect speech (Speech Reception Threshold)
- Understand speech (Word Recognition Score)
These functional measures often correlate better with daily-life impact than pure-tone thresholds alone.
Tympanometry
Measures middle-ear function — eardrum mobility and middle-ear pressure. Rules out conductive hearing loss from fluid or other middle-ear issues. Brief, painless, and tolerated by most ages.
Otoacoustic emissions (OAE)
Measures the response of the outer hair cells in the cochlea. Particularly relevant in Alström because outer hair cell dysfunction is a primary feature. OAEs are typically absent or reduced in Alström even when audiogram thresholds are still close to normal.⁴
Auditory brainstem response (ABR)
Measures how the brainstem responds to sound. Used in babies and young children who can't cooperate with behavioral audiometry, and to confirm auditory pathway integrity in patients being evaluated for cochlear implantation.
Speech-in-noise testing
Some clinics include testing of speech understanding in background noise — particularly relevant for school and work accommodation decisions.
Hearing aid or cochlear implant verification
For patients using devices, real-ear measurement and aided audiogram verify that current settings are optimal.
Special considerations by age
Babies (0–12 months)
ABR and OAE testing in a quiet sleeping baby. Behavioral testing emerges around 6 months as the baby becomes able to turn toward sound.
Toddlers (1–3 years)
Visual reinforcement audiometry (VRA) with the child looking toward a reinforcing toy when sound is presented. ABR and OAE if behavioral testing isn't adequate.
School age (3+ years)
Conditioned play audiometry (the child responds to sound with a play-based action). By age 4–5, conventional audiometry is often feasible.
Adolescents and adults
Standard audiometric battery as outlined above.
Preparing for the visit
Bring or have ready:
- Most recent audiology report
- Current hearing aids or cochlear implant processors and accessories
- List of current symptoms or concerns
- Any school or workplace accommodation documentation
- A list of questions
If your child has been having a particular difficulty (not hearing in noisy classrooms, asking to repeat more often, withdrawing socially), describe it specifically — these patterns often guide the audiologist's focus.
What questions to ask
Useful questions during the visit:
- "How does this audiogram compare with last year?"
- "What's the rate of change we're seeing?"
- "Should we adjust the hearing aid settings?"
- "Are there new technologies that might help?"
- "Do we need to update school or work accommodations?"
- "When should we start thinking about cochlear implants?"
Coordinating with the broader Alström team
The audiology team should be aware of:
- Recent vision changes (since combined vision and hearing loss has specific implications)
- Cardiac status and any planned procedures
- Current medications (some affect hearing)
- Educational and employment context
If your care is at a multidisciplinary Alström clinic, this coordination happens naturally. Otherwise, you can help bridge by sharing relevant information across providers.
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
How long does the annual audiology visit take?
Answer
A complete annual evaluation typically takes 60–90 minutes for an adult or older child. Pediatric evaluations can take longer, especially when behavioral testing is supplemented with objective measures.
Question
Is the testing painful or scary for a child?
Answer
The tests are not painful. Some young children find the testing booth or headphones uncomfortable initially. Pediatric audiologists are skilled at making the experience tolerable. ABR testing in babies is done while the baby is asleep or sedated.
Question
What if my child doesn't want to wear hearing aids?
Answer
Common in older children and teens. The audiology team can troubleshoot — checking comfort, sound quality, and social factors. Sometimes a cosmetic upgrade or a different style helps. Sometimes peer mentorship and counseling helps. The audiologist is your partner here.
Question
When should we add cochlear implant consultation?
Answer
When hearing aids no longer provide adequate benefit (typically when word recognition with optimally-fitted aids drops below 50% in the better ear), it's reasonable to seek a cochlear implant evaluation. Discuss with your audiologist.