Crisly.nl

World Hearing Day 2026

2026-03-03T00:00:00+01:00

Today is World Hearing Day, observed every 3 March. The WHO’s theme for 2026 is “From communities to classrooms: hearing care for all children” — a call to act before hearing loss shapes a child’s educational trajectory in ways that become increasingly hard to reverse.

The numbers that frame this are striking. Approximately 90 million children aged 5–19 live with hearing loss globally. More than 60% of childhood hearing loss is preventable through cost-effective public health measures — screening, treatment of chronic ear infections, noise protection. And yet in most health systems, hearing loss in children is identified late, addressed inconsistently, and rarely discussed at all in the spaces where children actually spend their time: classrooms, playgrounds, living rooms.

That last one is where something interesting happened in the weeks leading up to today.

George Pig gets a hearing aid

In February 2026, the National Deaf Children’s Society announced a partnership with Peppa Pig to introduce a deaf storyline — and the character carrying it is not a new face but a familiar one: George Pig, Peppa’s younger brother, revealed to have moderate single-sided deafness.

The storyline follows George through a hearing test, his first hearing aid fitting, and the experience of beginning to understand the world differently. The NDCS worked with the production team to ensure the portrayal is clinically accurate in ways that matter: it shows explicitly that a hearing aid provides greater access to sound rather than restoring normal hearing, and that listening in noise — a crowded room, multiple voices at once — remains genuinely difficult even with amplification.

George Pig with his hearing aid, from the NDCS-partnered Peppa Pig storyline airing March 2026.

Why does this matter clinically? Because over 54,000 children in the UK live with deafness or hearing loss, and nearly 25% have single-sided deafness — exactly George’s profile. Research consistently shows that children who see their own experience reflected in characters they love feel less isolated and more willing to wear their devices in front of peers. Stigma around hearing aids in children is real and measurable; it delays consistent use and therefore delays the language and cognitive development that depends on consistent auditory input. A single episode of Peppa Pig, watched by millions of children across dozens of countries, can shift the social framing of “wearing a hearing aid” faster than any clinical leaflet. The NDCS found that 98% of parents believe deaf representation in children’s media positively impacts deaf children’s wellbeing — and probably that of hearing children’s understanding too.

A photograph worth more than most awareness campaigns

There is an older image that has done similar work for adults.

Harold Whittles, hearing sound for the first time as a hearing aid is placed in his ear. Photograph: Jack Bradley, 1974. One of the most reproduced images in the history of audiology.

Jack Bradley’s 1974 photograph of Harold Whittles — a small boy with a look of pure astonishment as a hearing aid is placed in his ear for the first time — has circulated for fifty years and still stops people. It captures something that is very difficult to convey in words: that sound, for someone who has not reliably had it, is not just information. It is texture, presence, connection.

That photograph was taken in an era when hearing screening for newborns was far from routine, when the median age of diagnosis for moderate hearing loss in many countries was three to four years — an entire window of language acquisition already narrowed. The situation has improved substantially: universal newborn hearing screening programmes now exist across most high-income countries. But access is uneven, follow-through is imperfect, and in middle- and low-income settings the gap between a child’s first suspicion of hearing difficulty and a confirmed diagnosis and fitting remains measured in years rather than weeks.

What “early identification” actually means

The WHO message is “act now so no child is left behind due to ear or hearing problems.” This is easy to say and genuinely complex to implement. Early identification of childhood hearing loss requires several things to work in sequence: a screening moment (newborn, school entry, or opportunistic), a referral pathway that is followed, diagnostic capacity that is accessible, and then — crucially — a fitted, appropriate device that a child will actually wear.

Each of those steps has its own failure mode. A child can pass a newborn screen and develop hearing loss later, as happens in conditions like TMPRSS3-related hearing loss where onset is post-lingual and progression can be rapid. A child can be diagnosed correctly but then fall through gaps in the follow-up system. Or a child can be fitted with a hearing aid that is technically adequate but socially intolerable — because their classmates have never seen one on a character they know and like.

That last failure mode is the one Peppa Pig just addressed.

The bigger picture for hereditary hearing loss

For families where hearing loss has a genetic basis — which accounts for roughly half of all childhood hearing loss cases — the conversation is more complicated still. Genetic hearing loss is not one condition; it is hundreds of conditions, each with its own natural history, its own rate of progression, its own likelihood of affecting vestibular function or other systems. The audiological phenotype of a child with USH2A-related Usher syndrome looks nothing like that of a child with a RIPOR2 deletion; their trajectories, their implant candidacy timelines, and their communication needs differ in ways that a one-size-fits-all “hearing loss” framing obscures.

This is where the genotype–phenotype work described elsewhere on this site connects to the World Hearing Day theme. The natural history studies — the CRUSH cohort in Usher syndrome type 2A, the multi-centre TMPRSS3 cohort, the DFNA9 audiometric meta-analysis — exist partly as clinical science and partly as the evidence base that should inform when a child is counselled about cochlear implantation, what progression they and their parents should be prepared for, and what therapeutic options might become relevant in the coming decade.

That evidence base is still being built. But days like today — when a cartoon pig goes to a hearing clinic, and millions of children watch with the particular attention they give to something that is new and safe at the same time — are part of how the ground gets prepared for it to matter.

World Hearing Day 2026 resources: WHO campaign page · NDCS Peppa Pig partnership

Sources:

A New Digital Ecology

2026-03-03T00:00:00+01:00

There is a particular kind of cognitive dissonance that comes with having a website that only shows one face of you. For a few years, crisly.nl was essentially a publications list with a blog attached — useful, but flat. My clinical and research identity was there in outline, but the connections between projects were invisible, the design felt inherited rather than chosen, and the private side of the site (photography, animation, the occasional detour through astrophotography or cycling) sat in a separate aesthetic universe from the work pages, as if the two halves were embarrassed to be seen together.

What I wanted was something closer to an ecosystem: a site where a visitor could arrive at a research write-up about Usher syndrome genetics and, without feeling they had wandered off-map, end up looking at storm photographs from Gelderland. Not because those things are the same, but because they come from the same person, made with the same disposition towards attention and detail. A digital ecology, to use a term I’ve been turning over — a space where different kinds of output coexist in a shared environment, shaped by consistent design rather than rigid category walls.

This post documents what changed in the latest round of work.

Research as narrative, not inventory

The biggest structural addition is a Projects section — five pages, one per research theme, each building a coherent story out of what would otherwise be a scatter of publications:

Hereditary Hearing Loss & Rare Genetic Disorders
Genotype–Phenotype Correlations & Natural History
Cochlear Implantation & Auditory Outcomes
Remote Care & Digital Audiology
Clinical Trials & Inner-Ear Therapeutics

Each project page has a findings table (gene, phenotype, key result, reference), a narrative summary, a linked list of related blog write-ups, and a per-page bibliography rendered by jekyll-scholar from the same .bib file that feeds the publications page. The result is something that reads more like a research dossier than a filtered publication list.

What I find interesting about this structure is that it makes the connections visible. The natural history work on DFNA9 and Usher syndrome type 2A isn’t just a set of papers — it’s the explicit scientific prerequisite for the AON therapies that colleagues Erwin van Wijk and Erik de Vrieze are now moving into clinical application. A publications list can’t show that. A project page can.

The projects are also wired into the navigation as a dropdown, and linked bidirectionally from the resume’s focus areas — so the same information is reachable from multiple entry points depending on what the visitor is looking for.

The design layer: runtime flexibility

The visual redesign was driven by one core idea: separate what the compiler knows from what the browser decides.

Jekyll compiles Sass to CSS at build time. The traditional Feeling Responsive approach bakes a palette (a set of hex colours) into the compiled stylesheet, and that’s what users see. Changing the palette means rebuilding the site. That’s fine for production, but it makes experimentation slow and it means every visitor gets the same colour experience regardless of context or preference.

The new approach adds a second layer on top of the compiled styles: CSS custom properties (var(--primary), var(--secondary), etc.) that the browser evaluates at paint time. Dark mode works by switching html[data-theme="dark"] — the browser re-evaluates all the custom properties instantly, no recompilation. The same mechanism drives a palette switcher in the navigation bar: four small coloured dots (Moonlight, Verdant, Claret, Meridian) that swap the full colour scheme at runtime, with the choice persisted to localStorage so it survives page reloads.

The practical consequence is that the site can have four complete colour identities without four builds. The compiled stylesheet contains all of them as html[data-palette="X"] override blocks; only the active one wins on any given page load.

Colour palettes

Four palettes are available via the dot switcher in the navigation bar. The dark-mode values are derived at runtime using the same lightness-scaling formula as the Sass compiler.

Palette	Light primary	Light secondary	Light accent	Dark primary	Dark secondary	Dark accent
D · Moonlight
E · Verdant
F · Claret
G · Meridian

A small but meaningful downstream effect: any component that uses color-mix(in srgb, var(--secondary) 12%, white 88%) rather than a hardcoded hex automatically responds to both the palette switcher and dark mode. The project page TOC pills, the resume focus area pills, the navigation gradient stripe — all theme-aware, all zero-maintenance as new palettes are added.

The smaller things

A few changes that don’t warrant their own section but add up:

Navigation order was adjusted to reflect how I actually think about the site: Start → Blog → Photos → Projects → Publications → Resume. The research-heavy end is to the right; the more personal and recent content is to the left.

The projects index now displays entries in the same visual format as the blog listing — subheadline, title, thumbnail image, description blurb, read-more link — rather than a flat card list. The TOC jump-to pills above the listing use the same token-driven styling as the resume focus pills, so they track whatever palette and light/dark mode is active.

The hero name on the front page was cleaned up — weight reduced, gradient removed, and the font size aligned to match the widget headers below it. The negative letter-spacing that was clipping the right edge of long descenders is gone.

Why Jekyll

The question I get most often about the site is some version of: “why not just use WordPress?” Or Substack, or Ghost, or Webflow, or one of the many platforms designed to make publishing easy. The honest answer is that easy and controllable are not the same thing, and for a site that needs to hold a bibliography engine, a custom runtime colour system, a structured research collection, and a photography archive all without feeling like it’s straining at its seams — control matters more than ease.

Jekyll is fast in two senses. The generated site is entirely static: no PHP, no database queries, no server-side rendering on request. Every page is pre-built HTML served directly from a CDN. On a standard connection, pages load in under a second; on mobile on a flaky network, the difference between a static site and a CMS-backed one is the difference between “readable” and “I’ll come back later”. The build step itself — Sass compilation, Liquid templating, bibliography rendering — takes about five seconds for this site. That’s an acceptable price for having a fully automated system that produces optimised output every time.

Jekyll is flexible in the sense that matters for an academic personal site: it gets out of the way when you know what you want. The bibliography integration runs through jekyll-scholar, which processes .bib files and renders per-page reference lists with <a class="citation" href="#"></a> tags that work exactly like LaTeX — familiar territory. Jekyll Collections handle the structured project pages with typed front matter and clean URLs. Liquid templating is limited by design (no arbitrary code execution), which sounds like a constraint but mostly means that the templates stay readable and the site stays auditable. YAML front matter means every page carries its own metadata: title, projects membership, image, category, tags — and Liquid can filter and sort on any of it without a database.

The aesthetics are entirely mine. This is probably the real reason. A platform like Webflow or Squarespace imposes visual opinions through its component system; fighting those opinions is where most of the effort goes. With Jekyll and SCSS, the design is built from the bottom up: every spacing value, every colour token, every responsive breakpoint is something I chose. The runtime palette switcher described above — CSS custom properties overridden by html[data-palette] attribute selectors — would be awkward or impossible to implement inside a managed CMS. Here it’s just SCSS and fifteen lines of JavaScript. The constraint is having to write it; the freedom is that it then does exactly what I wanted.

The tradeoff is real: there is no admin panel, no drag-and-drop, no autosave. New content goes through a text editor and a jekyll build. For someone who writes everything in Markdown anyway and uses a bibliography manager (Zotero) that exports .bib, this is not a hardship — it’s just the existing workflow, with one more step at the end.

What this is, eventually

None of this is finished. The remote care and clinical trials project pages are still placeholder content. Several blog posts about ongoing work (RIPOR2 follow-up, TMPRSS3 CI outcomes, the AC102 programme) exist only as drafts. The photography section deserves more curatorial attention than a reverse-chronological dump.

But the structure is right, and the design feels genuinely mine. That’s enough to work from.

Hearing Loss and Vestibular Function in Usher Syndrome Type 2A

2026-03-01T00:00:00+01:00

Background

Usher syndrome type 2A (USH2a) is caused by pathogenic variants in the USH2A gene and is the most common form of Usher syndrome. Clinically, it presents with a moderate-to-severe congenital sensorineural hearing loss combined with progressive retinitis pigmentosa that typically becomes symptomatic in adolescence. Unlike Usher type 1, the vestibular system is generally considered intact in USH2a — but how intact, and over how long?

Figure 1. Diagram summarizing the Usher syndrome subtypes — differing by hearing severity at birth, vestibular involvement, and onset of retinitis pigmentosa. Taken from Wijn et al. (2025) Ear and Hearing (figure 1).

These are not idle questions. As gene therapy approaches for retinal disease advance and cochlear implantation becomes more routine in this population, we increasingly need robust natural history data: how fast does the hearing loss actually progress, what happens to speech understanding, and should we be looking at the vestibular system more systematically?

The CRUSH study (a prospective, longitudinal natural history study at Radboudumc, see e.g., Radboudumc - Usher Syndrome Research; and CRUSH Database - Usher Syndrome Knowledge Portal) was designed to answer exactly these questions. Our paper (Wijn et al., 2025), led by Dirk Wijn and with contributions from Mirthe Fehrmann, Sybren Robijn, Hedwig Velde, Jeroen Smits, and others, reports on 4 years of structured follow-up in 33 USH2a patients and 2 patients with USH2A-associated non-syndromic retinitis pigmentosa (nsRP).

Hearing Loss Progression

The headline finding for pure-tone audiometry is a statistically significant decline of 2.3 dB in PTA 0.5–4 kHz over 4 years (P = 0.017). This is a modest but real signal and importantly, one that exceeds what we would expect from presbycusis alone. The progression was most pronounced at the mid-to-high frequencies (see fig 2), consistent with the cochlear regions most vulnerable in USH2A-related pathology.

Figure 2. Age-related typical audiograms (ARTA) for all USH2a patients in the CRUSH cohort, illustrating the characteristic sloping configuration and the degree of inter-individual variability across the frequency range. Taken from Wijn et al. (2025) Ear and Hearing (figure 3).

What makes this clinically useful is that these are prospectively collected measurements from a single center, using standardized protocols across all time points. Cross-sectional comparisons in genetics clinics often underestimate progression because of population heterogeneity; a within-subject longitudinal design is far more sensitive and meaningful for counseling individual patients.

One finding worth highlighting in conversations with patients: speech reception thresholds (SRT) remained relatively stable despite the decline in pure-tone thresholds. This means patients will notice slightly deteriorating audiograms at routine visits, but their functional communication ability, as captured by speech testing, holds up better than the pure-tone picture might suggest. This distinction matters enormously for patient-facing counseling, and for timing decisions around cochlear implantation.

The two nsRP patients (same USH2A pathogenic variants, no clinical RP) had normal hearing, which is a useful datapoint for genetic counseling in family members with isolated retinal disease.

Vestibular Phenotype: Mostly Intact Semicircular Canals, but Subtle Otolith Dysfunction

The vestibular results are, in some ways, the most interesting part of the study, and the part most likely to change clinical practice.

As expected for USH2a, semicircular canal function was largely preserved: video head impulse testing (vHIT), velocity step tests, and caloric reflex tests were all within normal limits in more than 90% of patients. Patient-reported balance problems on the Dizziness Handicap Inventory (DHI) were also absent or minor.

This is reassuring — but the otolith picture is different.

Cervical VEMPs (cVEMP) — assessing saccular function — were abnormal in 34% of patients
Ocular VEMPs (oVEMP) — assessing utricular function — were abnormal in 75% of patients

These findings indicate subclinical otolith organ dysfunction in a substantial proportion of USH2a patients who report no balance complaints and have intact canal function. This dissociation between patient-reported symptoms and objective test findings is clinically important: if we rely only on symptoms or vHIT results, we will miss a significant proportion of patients with vestibular pathology.

What This Means in Practice

1. Annual audiological follow-up is warranted. A 2.3 dB decline over 4 years in the speech frequency range is small but real, and the mid-to-high frequency progression is consistent with cochlear base involvement. Stable SRT is reassuring but should not replace pure-tone tracking, both are essential when CI timing is under consideration.

2. Cochlear implant timing deserves proactive discussion. In USH2a, progressive hearing loss and progressive vision loss are on parallel trajectories. Cochlear implantation is optimally performed while audiovisual learning and integration are still available. The longitudinal hearing data from CRUSH now provide a firmer empirical basis for those timing conversations.

3. VEMPs belong in the USH2a assessment protocol. The high prevalence of abnormal oVEMPs (75%) in the absence of subjective balance problems argues for routine VEMP testing in USH2a — not because patients complain, but because the underlying organ is silently compromised. This may have relevance for fall risk in the context of concurrent visual field loss, and is relevant if CI fitting approaches that rely on vestibular input are under consideration.

4. Distinguish the nsRP phenotype. Patients with USH2A-associated nsRP carry the same variants without clinical RP, and had normal hearing in this sample. For genetic counseling in such families, this reassurance about hearing, with the caveat that systematic follow-up is still indicated, is clinically useful.

Wijn, D. H., Fehrmann, M. L. A., Robijn, S. M. M., Velde, H. M., Smits, J. J., van Wijk, E., Beynon, A. J., Cals, F. L. J., Hoyng, C. B., Yzer, S., Lanting, C. P., & Pennings, R. J. E. (2025). From Sound to Stability: Lessons Learned From the CRUSH Study on Hearing Loss Progression and Vestibular Phenotype in Usher Syndrome Type 2A. Otology & Neurotology, 10.1097/MAO.0000000000004851. https://doi.org/10.1097/MAO.0000000000004851

May Holiday Adventures Berlin (improved)

2026-01-11T00:00:00+01:00

Spring sunshine, lush evenings, and a city in motion—Berlin in May was pure magic. Over the holidays, we set out to discover the city on two wheels and see it from fresh angles.

Cycling Highlights

We kicked off by biking across the wide-open expanse of the Tempelhofer Feld, Berlin’s repurposed airport-turned-urban playground. There’s nothing quite like riding down a former runway with the wind in your face and city skyline ahead.

From there, one of the most scenic rides started in Ostkreuz, weaving along the Spree River. We crossed bridge after bridge—each offering a different slice of Berlin life—before arriving at the Reichstag, where the glass dome shimmered under a blue sky. Of course, no riverside ride is complete without a stop at the East Side Gallery. The massive murals never get old, with their bold colors and historical echoes.

Urban Culture

We also visited the RAW-Gelände, a gritty, creative sprawl of clubs, galleries, and a fantastic skatepark. The energy here is unmistakable—raw and authentic, with a laid-back skater vibe and murals layered across every wall.

For food and chill, alternative vibes, Friedrichshain was a favorite. It’s packed with cafés, street food, indie bars, and green spaces (Boxhagener Platz) offering a more local vibe than central Berlin. If you’re visiting, it’s worth spending a day just wandering here—and steering clear of the tourist traps around places like Checkpoint Charlie and the overly commercial parts of Alexanderplatz.

Future and Fiction

For a change of pace, we stepped into the Spy Museum, diving into Cold War tech, disguises, and tales of espionage. Then it was off to the Futurium, where you can interact with possible futures and rethink everything from mobility to AI.

One final visual treat was the Lighthouse of Digital Art – The Grand Tour: an immersive show of our solar system, our own Milky Way galaxy, but also way, way beyond. A truly mesmerizing experience.

Berlin and Nature—Best of Both Worlds

The best part? We combined city exploration with a quiet stay in nature. It’s perfectly doable to use public transport—train RE7 takes you straight into the city—while camping in Naturpark Dahme-Heideseen, near the peaceful lake Schmöldesee. Wake up to birdsong, spend the day in Berlin, and return to a peaceful campsite. Highly recommended.

Phase 1 Audiological Outcomes of Intratympanic AC102

2025-11-19T00:00:00+01:00

Overview

In this first-in-human phase 1 trial, we evaluated the audiological effects of intratympanic AC102, a novel inner-ear therapy delivered in a thermosensitive gel, in 42 normal-hearing volunteers. The main safety message is clear: AC102 is safe and well-tolerated. Furthermore, cochlear function remained intact, with no permanent sensorineural threshold shifts or deterioration in BERA or otoacoustic emissions.

From an audiological standpoint, the most notable finding was a short-lived, volume-dependent conductive hearing loss predominantly at high frequencies (4–8 kHz), reflecting a mechanical middle-ear effect of the injected gel rather than drug-related toxicity.

Key Audiological Findings

No permanent sensorineural changes: Bone conduction thresholds, BERA, and TEOAE/DPOAE remained stable throughout follow-up.
Transient high-frequency conductive loss: Air conduction thresholds increased temporarily, most clearly between 4 and 8 kHz, and returned to baseline within days.
Volume-dependent effect, independent of AC102: The magnitude of the conductive shift scaled with injected volume (50–800 μl) and occurred for both placebo gel and AC102, indicating a mechanical middle-ear effect rather than drug toxicity.
Mild, procedure-related symptoms only: Transient ear discomfort, brief vertigo, tinnitus, and minor bleeding at the injection site were observed, resolving within about a week.

Volume–Effect Relationship (FIG. 4)

Figure 4. The average air conduction threshold versus the injected volume of either placebo or AC102 on the day of injection. Each panel showcases the frequency tested, ranging from 0.25 to 8.0 kHz, and displays the threshold measured after injection for the injected ear (threshold at TP day 1 in dB HL). The color code in each panel represents the volume cohorts, with red representing 50 μl, blue/white triangle representing 100 μl, orange circle representing 200 μl, green triangle representing 400 μl, and purple diamond representing 800 μl. The x-axis displays the exact volume injected for each participant, and the y-axis shows the effect of the injected volume on the threshold, with the most significant impact seen between 4 and 8 kHz. For each frequency, a linear fit with the confidence interval around the mean is presented (fit in black, confidence interval in gray).

Take-Home for Audiology

Intratympanic AC102 in a thermosensitive gel can be delivered without permanent cochlear damage, as evidenced by stable BC, BERA, and OAEs.
Clinically, you can expect a short-term, high-frequency air–bone gap whose size depends on the injected volume and resolves within days.
Patients should be counseled about transient “muffled” high-frequency hearing and mild procedure-related discomfort, but reassured that long-term thresholds are not adversely affected.
A phase 2 trial is currently ongoing, focusing on the efficacy of AC102 in sudden sensorineural hearing loss (SSNHL). Because of the tight time window after onset, our local recruitment is modest, but we are closely involved in the clinical and methodological design.
After many negative trials and a few promising successes (e.g. gene-specific approaches such as OTOF), the field urgently needs an effective treatment for SSNHL to mitigate the profound impact that patients report in daily life.

Genetic Insights into Cochlear Implant Outcomes

2025-06-09T00:00:00+02:00

Overview

What determines how well someone hears after receiving a cochlear implant (CI)? In our newly published study, Cochlear Implantation Outcomes in Genotyped Subjects with Sensorineural Hearing Loss, we analyzed data from 220 individuals with hereditary sensorineural hearing loss (SNHL), each with a known genetic diagnosis involving one or more of 31 nuclear or mitochondrial genes.

Our goal: to understand how cochlear implant outcomes relate to both genetic causes and individual characteristics—and to assess whether the cochlear site-of-lesion (as inferred from gene function) helps explain variability in performance.

Figure 1. Pre- and post-synaptic components of the cochlea. The transverse section through the middle turn of the cochlea shows the pre-synaptic components of the cochlea in blue, including the stria vascularis and organ of Corti, and the post-synaptic parts in red, including the spiral ganglion neurons that eventually form the cochlear nerve. Figure created in BioRender. Fehrmann, M. (2025) https://BioRender.com/e54j431.

Key Findings at a Glance

Most genotyped CI recipients performed very well. The median phoneme score at 65 dB SPL in quiet was 90%, with many individuals reaching scores above 95%.
Early implantation is beneficial. Individuals who received their implant before age 6 performed better and showed less variability.
Cochlear site-of-lesion did not explain variability. Models with site-of-lesion (pre-synaptic, post-synaptic, mitochondrial) as a factor explained virtually none of the variance in CI outcomes (R² = 0.000, p = 0.938).
Subject-specific factors matter most. A multivariate regression model including age at implantation, sex, CI experience (years), hearing aid use before implantation, and self-reported SNHL duration explained 19% of the variability in CI outcomes.
Findings align with existing literature. Results are consistent with earlier work (e.g., Lazard et al.) emphasizing the strong influence of auditory history and environmental factors.

CI Outcomes per Gene

We visualized CI outcomes per gene in Figure 2 below, which shows median phoneme scores and individual data points for each gene, ranked from best to worst outcomes. While most genes were associated with good performance, a few (e.g., MYO3A, OTOF, USH1C, OPA1) showed wider variability and lower median scores. Yet these poorer outcomes were largely explained by subject-level factors such as prelingual deafness with late implantation, lack of auditory stimulation, or psychosocial factors.

We excluded genes with dual site-of-lesion associations from this figure, including COCH (N = 37 ears), CLRN1 (N = 6), and WFS1 (N = 7), to avoid bias due to uncertain categorization.

Figure 2. CI outcomes per gene, sorted by median phoneme score (high-to-low) and color by site-of-lesion. Dots represent individual ears.

Implications for Clinical Practice

These findings offer reassurance to clinicians and patients alike: most individuals with hereditary hearing loss can expect excellent CI outcomes, regardless of the precise genetic etiology.

However, timing and support are critical. Early implantation, consistent use of hearing aids prior to surgery, and a strong support system were all associated with better outcomes. In contrast, delayed implantation, prolonged auditory deprivation, and limited psychosocial support were common among lower performers.

The site-of-lesion hypothesis remains a promising framework for future research, but current data suggest it has limited predictive value when applied clinically. Improved models—possibly integrating neural integrity tests or human-derived organoid models—are needed to fully understand genotype-phenotype relationships in auditory rehabilitation.

Our study reinforces a central message in auditory care: while genes provide important diagnostic information, they are only part of the story. A personalized, timing-sensitive, and supportive approach remains key to maximizing cochlear implant outcomes.

About the Dissertation — PhDone

This work was part of the doctoral research by Mirthe Fehrmann, who successfully defended her PhD dissertation titled From Genes to Hearing Outcomes: Cochlear Implantation in Hereditary Hearing Loss and the Search for Predictive Factors on June 3, 2025, at Radboud University. Needless to say, I’m proud of her achievements (7 papers (!), see below), her ability to manage supervisors, and for a beautiful thesis (see cover below) providing an in-depth analysis of genetic and individual factors shaping CI success.

Supervised by Prof. Mylanus, Prof. Pennings, Dr. Huinck, and myself, the dissertation is now available via the Radboud repository. You can read the full dissertation at https://hdl.handle.net/2066/319710.

Publications

Journal Articles

Fehrmann, M., Beynon, A., Huinck, W., Pennings, R., Mylanus, E., & Lanting, C. (2025). The Potential of Electrocochleography in Explaining the Variability in Cochlear Implant Outcomes: A Scoping Review. International Journal of Audiology, 1–15. https://doi.org/10.1080/14992027.2025.2459223
Fehrmann, M. L. A., Haer-Wigman, L., Kremer, H., Yntema, H. G., Thijssen, M. E. G., Mylanus, E. A. M., Huinck, W. J., Lanting, C. P., & Pennings, R. J. E. (2025). Cochlear Implantation Outcomes in Genotyped Subjects with Sensorineural Hearing Loss. Journal of the Association for Research in Otolaryngology. https://doi.org/10.1007/s10162-025-00987-0
Wijn, D. H., Fehrmann, M. L. A., Robijn, S. M. M., Velde, H. M., Smits, J. J., van Wijk, E., Beynon, A. J., Cals, F. L. J., Hoyng, C. B., Yzer, S., Lanting, C. P., & Pennings, R. J. E. (2025). From Sound to Stability: Lessons Learned From the CRUSH Study on Hearing Loss Progression and Vestibular Phenotype in Usher Syndrome Type 2A. Otology & Neurotology, 10.1097/MAO.0000000000004851. https://doi.org/10.1097/MAO.0000000000004851
Colbert, B. M., Lanting, C., Smeal, M., Blanton, S., Dykxhoorn, D. M., Tang, P.-C., Getchell, R. L., Velde, H., Fehrmann, M., Thorpe, R., Chapagain, P., Elkhaligy, H., Kremer, H., Yntema, H., Haer-Wigman, L., Redfield, S., Sun, T., Bruijn, S., Plomp, A., … Liu, X. Z. (2024). The Natural History and Genotype–Phenotype Correlations of TMPRSS3 Hearing Loss: An International, Multi-Center, Cohort Analysis. Human Genetics, 143(5), 721–734. https://doi.org/10.1007/s00439-024-02648-3
Fehrmann, M. L. A., Meijer, F. J. A., Mylanus, E. A. M., Pennings, R. J. E., Lanting, C. P., & Huinck, W. J. (2024). Evaluating Cochlear Implant Outcomes in DFNA9 Subjects: A Comprehensive Study on Cerebral White Matter Lesions and Vestibular Abnormalities. European Archives of Oto-Rhino-Laryngology. https://doi.org/10.1007/s00405-024-08933-1
Fehrmann, M. L. A., Lanting, C. P., Haer-Wigman, L., Mylanus, E. A. M., Huinck, W. J., & Pennings, R. J. E. (2024). Good Cochlear Implantation Outcomes in Subjects with Mono-Allelic WFS1- Associated Sensorineural Hearing Loss – a Case Series. International Journal of Audiology, 1–9. https://doi.org/10.1080/14992027.2024.2411579
Fehrmann, M. L. A., Lanting, C. P., Haer-Wigman, L., Yntema, H. G., Mylanus, E. A. M., Huinck, W. J., & Pennings, R. J. E. (2024). Long-Term Outcomes of Cochlear Implantation in Usher Syndrome. Ear and Hearing, 10.1097/AUD.0000000000001544. https://doi.org/10.1097/AUD.0000000000001544
Fehrmann, M. L. A., Huinck, W. J., Thijssen, M. E. G., Haer-Wigman, L., Yntema, H. G., Rotteveel, L. J. C., Widdershoven, J. C. C., Goderie, T., Van Dooren, M. F., Hoefsloot, E. H., Van Der Schroeff, M. P., Mylanus, E. A. M., DOOFNL consortium, Van Dooren, M. F., Kant, S. G., De Gier, H. H. W., Hoefsloot, E. H., Van Der Schroeff, M. P., Rotteveel, L. J. C., … Pennings, R. J. E. (2023). Stable Long-Term Outcomes after Cochlear Implantation in Subjects with TMPRSS3 Associated Hearing Loss: A Retrospective Multicentre Study. Journal of Otolaryngology - Head & Neck Surgery, 52(1), 82. https://doi.org/10.1186/s40463-023-00680-3

Advancing Teleaudiology with AI: Jan-Willem Wasmann's Thesis Defence

2025-05-12T00:00:00+02:00

Toward AI-Aided Teleaudiology: A Milestone in Digital Hearing Care

On 18th of March 2025, Jan-Willem Wasmann successfully defended his PhD thesis, Toward AI-Aided Teleaudiology, a comprehensive exploration of how artificial intelligence and teleaudiology can revolutionize hearing healthcare. It was a proud moment to witness a dedicated colleague achieve this milestone after years of impactful research.

Expanding Access to Hearing Care

Worldwide, approximately 1.5 billion people experience some level of hearing loss, yet many lack access to timely and effective hearing care. Jan-Willem’s thesis addresses this gap by proposing innovative ways to deliver hearing services remotely using AI-driven solutions. From smartphone-based self-administered tests for cochlear implant users to speech-to-text applications for those with moderate to severe hearing loss, his research underscores the potential of digital tools to make hearing care more accessible and convenient.

Self-Administered Tests for Cochlear Implant Users

One of the core findings of the thesis is the potential for cochlear implant users to evaluate their hearing performance at home. By using self-administered auditory tests, these individuals can monitor their progress and fine-tune their implant settings without frequent clinic visits. This advancement not only empowers users but also alleviates the burden on clinical resources.

Speech-to-Text Apps: Bridging Communication Gaps

Jan-Willem also evaluated the effectiveness of free speech-to-text applications that convert spoken words into text in real time. For people with moderate to severe hearing loss, these apps can significantly improve communication in daily life. According to the study, the accuracy of these apps is sufficient to assist users in following conversations more effectively.

Next Steps in AI-Driven Audiology

The insights gained from Jan-Willem’s research lay the groundwork for further advancements in AI-aided teleaudiology. The thesis not only demonstrates how AI can support hearing care but also highlights areas for future exploration, including the integration of more advanced algorithms to refine self-assessment tools and enhance speech-to-text accuracy.

Congratulations, Jan-Willem, on this remarkable achievement! It has been a privilege to supervise such a talented and committed researcher. Explore his full thesis at Radboud University Press.

DOI: https://doi.org/10.54195/9789493296657

Disclaimer: This blog post was generated with the assistance of AI, based on the content and key messages from Jan-Willem Wasmann’s thesis.

Meta Information

Model Information:

Model Version: GPT-4.0, May 2025
Release Date: 2025-05-01
Model Type: Transformer-based language model
Parameters: 1.76 trillion parameters
Training Data: Multimodal datasets, emphasizing health sciences, auditory research, and AI/teleaudiology domains.
Fine-Tuning: Targeted fine-tuning on academic communication, technical writing, and structured content generation.
Content Structuring Algorithms: Integration of thematic analysis, key point extraction, and logical flow structuring for consistency.

Token Analysis and Processing:

Total Tokens Processed: Approximately 890
Context Analysis: 15% (Identifying key themes, user role, and content structure)
Content Generation: 70% (Drafting blog content, structuring narrative)
Metadata and Transparency: 10% (Adding meta information and disclaimers)
Revisions and Iterations: 5% (Integrating user feedback and adjustments)

Transparency and Reproducibility:

Input Data: Thesis title, DOI, key findings, and user instructions.
User Role: Co-supervisor perspective, incorporating personal reflections.
Jekyll Template: Applied structure for layout consistency, tags, and categories.
AI Output: Clearly marked as AI-generated, with disclaimers and metadata for transparency.
Reproducibility Parameters: Input data, context, and structure can be replicated using the same content and instructions.

May Holiday Adventures Berlin

2025-05-05T00:00:00+02:00

Spring sunshine, lush evenings, and a city in motion—Berlin in May was pure magic. Over the holidays, we set out to discover the city on two wheels and see it from fresh angles.

Cycling Highlights

Urban Culture

For food and chill, alternative vibes, Friedrichshain was a favorite. It’s packed with cafés, street food, indie bars, and green spaces (Boxhagener Platz) offering a more local vibe than central Berlin. If you’re visiting, it’s worth spending a day just wandering here—and steering clear of the tourist traps around places like Checkpoint Charlie and the overly commercial parts of Alexanderplatz.

Future and Fiction

Berlin and Nature—Best of Both Worlds

<ul class="clearing-thumbs small-block-grid-4" data-clearing>
	
	<li><a href="https://crisly.nl/images/IMG20250428101314.jpg"><img data-caption="Berlin HbF" src="https://crisly.nl/images/IMG20250428101314-thumb.jpg" alt="Berlin HbF"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430114510.jpg"><img data-caption="Graffiti details at East Side Gallery" src="https://crisly.nl/images/IMG20250430114510-thumb.jpg" alt="Graffiti details at East Side Gallery"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250428110244.jpg"><img data-caption="Brandenburger Tor" src="https://crisly.nl/images/IMG20250428110244-thumb.jpg" alt="Brandenburger Tor"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430114816.jpg"><img data-caption="Graffiti details at East Side Gallery (Japanese sector)" src="https://crisly.nl/images/IMG20250430114816-thumb.jpg" alt="Graffiti details at East Side Gallery (Japanese sector)"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250428111855.jpg"><img data-caption="Holocaust Memorial" src="https://crisly.nl/images/IMG20250428111855-thumb.jpg" alt="Holocaust Memorial"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430114936.jpg"><img data-caption="Graffiti details at East Side Gallery (Feet)" src="https://crisly.nl/images/IMG20250430114936-thumb.jpg" alt="Graffiti details at East Side Gallery (Feet)"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250428142650.jpg"><img data-caption="Tempelhof park cycling" src="https://crisly.nl/images/IMG20250428142650-thumb.jpg" alt="Tempelhof park cycling"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430144318.jpg"><img data-caption="Berliner Dom" src="https://crisly.nl/images/IMG20250430144318-thumb.jpg" alt="Berliner Dom"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250428160657.jpg"><img data-caption="Gedenkstätte Berliner Mauer Bernauer Str" src="https://crisly.nl/images/IMG20250428160657-thumb.jpg" alt="Gedenkstätte Berliner Mauer Bernauer Str"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430144727.jpg"><img data-caption="Rotes Rathaus Berlin" src="https://crisly.nl/images/IMG20250430144727-thumb.jpg" alt="Rotes Rathaus Berlin"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250428160707.jpg"><img data-caption="Gedenkstätte Berliner Mauer Bernauer Str" src="https://crisly.nl/images/IMG20250428160707-thumb.jpg" alt="Gedenkstätte Berliner Mauer Bernauer Str"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430150005.jpg"><img data-caption="Karl Marx str" src="https://crisly.nl/images/IMG20250430150005-thumb.jpg" alt="Karl Marx str"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250428161219.jpg"><img data-caption="Gedächtniskirche" src="https://crisly.nl/images/IMG20250428161219-thumb.jpg" alt="Gedächtniskirche"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430162447.jpg"><img data-caption="Skatehall berlin RAW gelände" src="https://crisly.nl/images/IMG20250430162447-thumb.jpg" alt="Skatehall berlin RAW gelände"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250429112255.jpg"><img data-caption="Potsdamer Platz" src="https://crisly.nl/images/IMG20250429112255-thumb.jpg" alt="Potsdamer Platz"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250430175942.jpg"><img data-caption="The Lighthouse Digital Art show" src="https://crisly.nl/images/IMG20250430175942-thumb.jpg" alt="The Lighthouse Digital Art show"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250429154545.jpg"><img data-caption="New Media Art Center RAW gelände" src="https://crisly.nl/images/IMG20250429154545-thumb.jpg" alt="New Media Art Center RAW gelände"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250501125802.jpg"><img data-caption="Lift detail in Futurium" src="https://crisly.nl/images/IMG20250501125802-thumb.jpg" alt="Lift detail in Futurium"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250429154604.jpg"><img data-caption="RAW gelände graffiti" src="https://crisly.nl/images/IMG20250429154604-thumb.jpg" alt="RAW gelände graffiti"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250501132010.jpg"><img data-caption="Transportation ideas at Futurium" src="https://crisly.nl/images/IMG20250501132010-thumb.jpg" alt="Transportation ideas at Futurium"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250429154701.jpg"><img data-caption="RAW gelände graffiti" src="https://crisly.nl/images/IMG20250429154701-thumb.jpg" alt="RAW gelände graffiti"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250501145608.jpg"><img data-caption="Museum Island" src="https://crisly.nl/images/IMG20250501145608-thumb.jpg" alt="Museum Island"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250429154922 (1).jpg"><img data-caption="RAW gelände graffiti" src="https://crisly.nl/images/IMG20250429154922 (1)-thumb.jpg" alt="RAW gelände graffiti"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250501192428 (1).jpg"><img data-caption="Friedrichshain views" src="https://crisly.nl/images/IMG20250501192428 (1)-thumb.jpg" alt="Friedrichshain views"></a></li>
	
	<li><a href="https://crisly.nl/images/IMG20250429191530.jpg"><img data-caption="Friedrichshain views" src="https://crisly.nl/images/IMG20250429191530-thumb.jpg" alt="Friedrichshain views"></a></li>
	
</ul>

First Light with the Carbonstar 150

2025-04-16T00:00:00+02:00

Exciting first light! These are the first images taken with my new Apertura Carbonstar 150 Newtonian telescope. This compact and powerful f/5 imaging Newtonian performed beautifully under early spring skies.

Gear & Setup

Telescope: Apertura Carbonstar 150 Newtonian
Mount: Sky-Watcher EQ6-R PRO
Camera: ZWO ASI 1600MM-PRO (cooled @ -10C)
Filters: ZWO H-alpha, OIII, SII
Guiding: ZWO ASI120MM Mini + Apertura 32mm Guide Scope
Acquisition: ZWO ASIAIR Plus
Post-processing: PixInsight

The Orion Region

The Orion constellation offers some of the most iconic nebulae for astrophotographers — and M42 remains a personal favorite. Capturing the details in its bright core while revealing faint outer regions is always a balancing act. This image is a blend of multiple narrowband exposures (H-alpha, OIII, SII).

Flame & Horsehead

Positioned near the bright star Alnitak (the easternmost star in Orion’s belt), the Flame Nebula and the Horsehead Nebula offer a dramatic contrast between glowing gas and dark dust. The Carbonstar handled this complex scene very well, especially in revealing the flame’s textured gas clouds.

Reflections from First Light

The Carbonstar 150 Newtonian is light, sharp, and super fun to handle. Collimation was straightforward, and combined with the ASIAIR system, imaging was smooth from start to finish. I’m already looking forward to more deep-sky targets (more to come)!

North America Nebula

2023-07-20T00:00:00+02:00

The North America Nebula (NGC7000) is a vast and captivating celestial wonder, spanning an area more than ten times that of the full moon. Though too faint for our eyes to perceive, we can marvel at its ethereal beauty through the lens of photography. Capturing this breathtaking sight, however, demands specialized equipment and precise techniques (see the list below).

Equipment Used for Astrophotography

Mount: Sky-Watcher EQ6-R PRO
Camera: ZWO ASI 1600MM-PRO (cooled @ -10C)
Refractor: William Optics RedCat II 51mm F4.9 telescope
Filters: ZWO 1.25″ 7nm Narrowband H-alpha, OIII, SII filters
Focuser: ZWO New Electronic Automatic Focuser
Guidecam: ZWO ASI 120MM mini
Image acquisition and control: ZWO ASIAIR Plus WiFi
Post-processing: PixInsight and tutorial (see this link)

For my astrophotography, I rely on an equatorial mount with two main axes, RA and DEC (Sky-Watcher EQ6-R PRO, for instance). These axes, accompanied by a guiding camera, play a crucial role in tracking the stars, ensuring they don’t blur during long exposures. Additionally, an electronic focuser aids in maintaining focus, especially during the start of the sequence and in response to temperature fluctuations (which can necessitate (re)focusing).

Over time, I’ve improved on my process, employing a cooled camera and specialized filters. This combination allows me to capture crisp and intricate monochrome images, something almost essential in highly light-polluted city skies (although it is possible, as I’ve shown before). These monochrome frames become the foundation for mesmerizing (false) color images, using techniques like the Hubble palette, which projects SHO images onto RGB colors.

The Captivating Moments in Astrophotography

Astrophotography is filled with captivating moments that make this endeavor so rewarding. Firstly, the initial setup and calibration of all the equipment, including proper polar alignment of the mount and telescope, may seem daunting at first, but with experience, it becomes a relaxing routine. Secondly, framing the perfect target and planning the image sequence requires thoughtful consideration. Often, I decide in advance which target to image based on its location (north or south) and the current season.

One of the most profound moments is when the first raw image (e.g., 300s exposure) appears on the screen. Especially when I started this hobby, this was a crucial moment - the realization of producing an astrophoto of objects otherwise invisible, revealing their size and level of detail in the night sky.

And last but not least is the post-processing stage, where I meticulously refine the images, bringing out all the intricate details and creating awe-inspiring visuals. Here, the Forax false-color processing technique comes into play, adding an artistic touch and enhancing the vibrant colors of the nebula in a unique and captivating manner.

Witness the Beauty: The Cygnus Wall

If you’re curious about achieving such breathtaking results, like the captivating reds and blues in the Cygnus Wall, you can watch a tutorial here.

The North America Nebula is a truly awe-inspiring sight in the night sky, and I’m grateful to be able to share its beauty through the art of astrophotography.

Images: 300s exposures of H-alpha, OIII, SII. Darks, flats, and bias frames were also acquired in the same time-window as acquisition.