ArticleProceedings of the National Academy of Sciences of the United States of America2016
ROR1 is essential for proper innervation of auditory hair cells and hearing in humans and mice.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers, 2 of them syntheses that pooled it.
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Who cites it
26 citing papers in PubMed, 2 syntheses or guidelines pooled it, 65 citations in OpenAlex.
- Is auditory neuropathy an appropriate term? A systematic literature review on its aetiology and pathogenesis.Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-facciale · 2021Pooled it
- Cochlear Implantation Outcomes in Post Synaptic Auditory Neuropathies: A Systematic Review and Narrative Synthesis.The journal of international advanced otology · 2020Pooled it
- Novel candidate genes for vestibular function identified through GWAS in the hybrid mouse diversity panel.BMC genomics · 2026Article
- A Structural Variant in the 5' Regulatory Region ofInternational archives of otorhinolaryngology · 2026Article
- Retrocochlear Auditory Dysfunctions (RADs) and Their Treatment: A Narrative Review.Audiology research · 2025Review
- Unlocking the potential: advancements and future horizons in ROR1-targeted cancer therapies.Science China. Life sciences · 2024Review
- Jag1 represses Notch activation in lateral supporting cells and inhibits an outer hair cell fate in the medial cochlea.Development (Cambridge, England) · 2024Article
- Article
- Impaired AIF-CHCHD4 interaction and mitochondrial calcium overload contribute to auditory neuropathy spectrum disorder in patient-iPSC-derived neurons with AIFM1 variant.Cell death & disease · 2023Article
- Clinical and genome-wide association analysis of chemoradiation-induced hearing loss in nasopharyngeal carcinoma.Human genetics · 2023Article
- ROR1: an orphan becomes apparent.Blood · 2022Review
- Genetics of Inner Ear Malformations: A Review.Audiology research · 2021Review
- NCOA3 identified as a new candidate to explain autosomal dominant progressive hearing loss.Human molecular genetics · 2021Article
- Small fish, big prospects: using zebrafish to unravel the mechanisms of hereditary hearing loss.Hearing research · 2020Article
- Practical aspects of inner ear gene delivery for research and clinical applications.Hearing research · 2020Review
- The emerging role of Wnt5a in the promotion of a pro-inflammatory and immunosuppressive tumor microenvironment.Cancer metastasis reviews · 2020Review
- Long-range cis-regulatory elements controlling GDF6 expression are essential for ear development.The Journal of clinical investigation · 2020Article
- Ectopic expression of ROR1 prevents cochlear hair cell loss in guinea pigs with noise-induced hearing loss.Journal of cellular and molecular medicine · 2020Article
- Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports.Journal of clinical medicine · 2020Review
- An Autocrine Wnt5a Loop Promotes NF-κB Pathway Activation and Cytokine/Chemokine Secretion in Melanoma.Cells · 2019Article
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Authors and funding
21 authors at 6 institutions in 5 countries.
Funding
Abstract
Hair cells of the inner ear, the mechanosensory receptors, convert sound waves into neural signals that are passed to the brain via the auditory nerve. Little is known about the molecular mechanisms that govern the development of hair cell-neuronal connections. We ascertained a family with autosomal recessive deafness associated with a common cavity inner ear malformation and auditory neuropathy. Via whole-exome sequencing, we identified a variant (c.2207G>C, p.R736T) in ROR1 (receptor tyrosine kinase-like orphan receptor 1), cosegregating with deafness in the family and absent in ethnicity-matched controls. ROR1 is a tyrosine kinase-like receptor localized at the plasma membrane. At the cellular level, the mutation prevents the protein from reaching the cellular membrane. In the presence of WNT5A, a known ROR1 ligand, the mutated ROR1 fails to activate NF-κB. Ror1 is expressed in the inner ear during development at embryonic and postnatal stages. We demonstrate that Ror1 mutant mice are severely deaf, with preserved otoacoustic emissions. Anatomically, mutant mice display malformed cochleae. Axons of spiral ganglion neurons show fasciculation defects. Type I neurons show impaired synapses with inner hair cells, and type II neurons display aberrant projections through the cochlear sensory epithelium. We conclude that Ror1 is crucial for spiral ganglion neurons to innervate auditory hair cells. Impairment of ROR1 function largely affects development of the inner ear and hearing in humans and mice.
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