Ataxias are a group of rare diseases. In the European Union, a disease is defined as rare if its prevalence is less than 5 per 10 000 people. Ataxias are rarer than this threshold, although reliable prevalence estimates are largely lacking....
✦ The floor
Discussion
Signed responses from readers of the wire.
No actionable change for audiology practice — audiologists should be aware that some ataxia syndromes involve auditory pathway involvement, but this article provides no new audiology-specific clinical guidance.
Rare neurological diseases like hereditary ataxias can include sensorineural hearing loss as a comorbidity, making basic familiarity with the diagnostic landscape marginally relevant for audiologists in multidisciplinary settings.
- 01Comprehensive review of ataxias covering diagnosis, genetics, and treatment options.
- 02Ataxias are rare neurological diseases affecting balance and coordination.
- 03Some hereditary ataxias (e.g., Friedreich's ataxia) can involve auditory pathway dysfunction.
- 04Published in Deutsches Ärzteblatt International (2026), a general medical journal.
- 05No audiology-specific data or hearing-focused outcomes are reported.
Ataxias represent a useful model for studying the challenges of diagnosing and managing rare neurological diseases.
opinionpartially supported- PMID
- 42839692
- DOI
- 10.3238/arztebl.m2026.0172.
- Journal
- Deutsches Ärzteblatt International
- Publication type
- review
- Evidence level
- 5
- Population
- Patients with hereditary and acquired ataxias
- Intervention
- Diagnostic and genetic evaluation approaches; treatment strategies for ataxias
Primary outcomes
Diagnostic accuracy; Genetic identification; Treatment response