--- license: permission_granted topic_id: companion_breed_health_rottweiler_nonsyndromic_hearing_loss_dog category: companion-breed-health title: "Rottweiler — Nonsyndromic hearing loss (hereditary; OMIA-verified breed predisposition)" lang: en source: "OMIA (Online Mendelian Inheritance in Animals, University of Sydney) breed-specific hereditary-disorder record, saved verbatim to source_file; C1 substring-verified. Pulled 2026-08-02 from local OMIA database dump." source_file: pdf-raw/breed-health/rottweiler_nonsyndromic_hearing_loss_4508.txt date_parsed: 2026-08-02 tokens_estimated: 526 verification: method: substring_match claims: 6 passed: 6 date: 2026-08-02 recovered: false path: companion-breed-health/companion_breed_health_rottweiler_nonsyndromic_hearing_loss_dog/01_companion_breed_health_rottweiler_nonsyndromic_hearing_loss_dog.md source_document: "OMIA — Online Mendelian Inheritance in Animals (University of Sydney)" citation: authority: "OMIA — Online Mendelian Inheritance in Animals (University of Sydney)" title: "Rottweiler — Nonsyndromic hearing loss (hereditary; OMIA-verified breed predisposition)" url: "https://omia.org/OMIA002336/9615/" retrieved: "2026-08-02" ref: "OMIA breed-specific hereditary-disorder records (omia.org), derived from local OMIA database dump; dataset: https://doi.org/10.25910/2AMR-PV70" doc_type: "academic animal-genetics database (breed-specific disorder entries)" needs_review: false
Rottweiler — Nonsyndromic hearing loss (hereditary; OMIA-verified breed predisposition)
Source: first-hand OMIA (University of Sydney) breed-specific hereditary-disorder record, saved verbatim to source_file; every claim below is a C1 byte-substring of it.
Claims
Breed: Rottweiler (Dog)Disorder: Nonsyndromic hearing lossMode of inheritance: Autosomal recessiveClin feat: Hytönen et al. (2021): Sensorineural bilateral deafness was diagnosed in four Rottweiler siblings (one female and three males) in a litter of ten puppies using brainstem auditory evoked response (BAER) testing. BAER testing was performed either at 4 (n = 2), 5, or 19 months of age, and no auditory response was detected in any of them. However, owners’ observations suggested that the puppies had already been affected by hearing impairment at a few weeks of age. No other clinical signs were observed.Defect: yesPrevalence: Hytönen et al. (2021): The allele frequency in the population [of Rottweilers], excluding the affected family, was 2.6% and carrier frequency 5.3%. An additional sample of dogs submitted for commercial genetic testing was screened for the LOXHD1 variant to explore its distribution across breeds. All 28,116 tested dogs representing 374 breeds, breed varieties or designer dog mixes were found homozygous for the wild-type allele (Online Resource 7). Finally, the variant was also screened in a larger study sample of 771,864 dogs submitted to genetic testing, including breed detection assessment. A variant carrier frequency of 0.08% and allele frequency of 0.04% were observed in this dataset. Interestingly, six dogs were found homozygous for the LOXHD1 variant. We were able to contact the owners of 4/6 of the homozygous dogs and the owners reported profound hearing loss or deafness in all of them. One of the deaf dogs did not show any immediate Rottweiler ancestry, while one was a purebred Rottweiler and two were mixed-breed with Rottweiler ancestry. Altogether, of the dogs carrying at least one copy of the deafness candidate variant, 63.4% showed evidence of Rottweiler ancestry in their immediate three-generation pedigree going back to great-grandparents, providing further support for a link between this specific breed background and the presence of the variant.
Associated gene(s)
Derived from OMIA database dump (omia.xml, local); structured field, not verbatim prose. First-hand values below are verbatim substrings of the topic's pdf-raw/omia/<phene_id>.txt source file.
- Gene: Entrez Gene ID 388254896 (no symbol in OMIA GeneSynonym) — OMIA Phene_Gene
- OMIA molecular-genetics note: Using "a combined approach of homozygosity mapping and genome sequencing to dissect the genetic background of the disorder . . . . [Hytönen et al. (2021)] identified a fully segregating missense variant [chr7:44,806,821G>C; p.(G1914A)] in LOXHD1, a gene that is known to be essential for cochlear hair cell function and associated with nonsyndromic hearing loss in humans and mice."
Evidence (references)
Derived from OMIA database dump (omia.xml, local); structured field, not verbatim prose. First-hand values below are verbatim substrings of the topic's pdf-raw/omia/<phene_id>.txt source file.
- 1996. Aetiology, prevalence and diagnosis of deafness in dogs and cats [Review]. Br Vet J — PubMed:PMID8634862 | DOI:10.1016/s0007-1935(96)80083-2 — OMIA Phene_Article / Article
- 2021. Missense variant in LOXHD1 is associated with canine nonsyndromic hearing loss. Hum Genet — PubMed:PMID33983508 | DOI:10.1007/s00439-021-02286-z — OMIA Phene_Article / Article
- 2001. An original inner ear neuroepithelial degeneration in a deaf Rottweiler puppy. Hear Res — PubMed:PMID11744282 | DOI:10.1016/s0378-5955(01)00354-9 — OMIA Phene_Article / Article
- 2023. Genome sequencing of 2000 canids by the Dog10K consortium advances the understanding of demography, genome function and architecture. Genome Biol — PubMed:PMID37582787 | DOI:10.1186/s13059-023-03023-7 — OMIA Phene_Article / Article
- 2023. An overview of canine inherited neurological disorders with known causal variants. Animals (Basel) — PubMed:PMID38003185 | DOI:10.3390/ani13223568 — OMIA Phene_Article / Article
Comparative medicine (human OMIM)
Derived from OMIA database dump (omia.xml, local); structured field, not verbatim prose. First-hand values below are verbatim substrings of the topic's pdf-raw/omia/<phene_id>.txt source file.
- OMIM:613079 (type: trait) — OMIA Group_OMIM (via OMIA_ID)
- OMIM:613072 (type: gene) — OMIA Group_OMIM (via OMIA_ID)
verification_derived: method: derived_from_dataset source: "OMIA database dump (omia.xml, local); fields Gene/Variant/Article/OMIM" note: "Structured fields (gene, variant rsID/protein change, PubMed/DOI references, OMIM cross-link) extracted from OMIA dump and presented with first-hand values; not verbatim prose."