Rare diseases · Sign or symptom
Posteriorly rotated ears
Ears rotated toward back of head
HP:0000358
What it means
A type of abnormal location of the ears in which the position of the ears is characterized by posterior rotation (the superior part of the ears is rotated towards the back of the head, and the inferior part of the ears towards the front).
Angle formed by the line perpendicular to the Frankfurt plane and the medial longitudinal axis of the ear (the two most remote points of the ear) greater than two standard deviations above the mean for age.
Rare diseases that can present with this253
Very common80–99%
80- 14q11.2microdeletion syndrome
- 14q22q23microdeletion syndrome
- 15q overgrowth syndrome
- 2p21microdeletion syndrome
- 49,XXXYY syndrome
- 49,XYYYY syndrome
- 6q terminal deletion syndrome
- Acrocraniofacial dysostosis
- Acrofacial dysostosis, Palagonia type
- Agnathia-holoprosencephaly-situs inversus syndrome
- Antley-Bixler syndrome
- Aortic arch anomaly-facial dysmorphism-intellectual disability syndrome
- AREDYLD syndrome
- Arthrogryposis multiplex congenita-whistling face syndrome
- Autosomal recessive faciodigitogenital syndrome
- Autosomal recessive omodysplasia
- B3GALT6-related spondylodysplastic Ehlers-Danlos syndrome
- Beemer-Ertbruggen syndrome
- Blepharophimosis-intellectual disability syndrome, SBBYS type
- Braddock syndrome
- Branchio-oculo-facial syndrome
- Cerebrofaciothoracic dysplasia
- CK syndrome
- Congenital osteogenesis imperfecta-microcephaly-cataracts syndrome
- Cooper-Jabs syndrome
- Crane-Heise syndrome
- C syndrome
- Deafness-genital anomalies-metacarpal and metatarsal synostosis syndrome
- Developmental delay-language impairment-dopa responsive dystonia-parkinsonism syndrome due to 2q24 microdeletion
- Diabetic embryopathy
- Difference of sex development-intellectual disability syndrome
- Distal deletion 17q syndrome
- Distal deletion 19p syndrome
- Distal deletion 9p syndrome
- Distal duplication 18q syndrome
- Distal limb deficiencies-micrognathia syndrome
- Donnai-Barrow syndrome
- Endocardial fibroelastosis
- Facial dysmorphism-macrocephaly-myopia-Dandy-Walker malformation syndrome
- Fetal hydantoin syndrome
- Fetal minoxidil syndrome
- Fryns syndrome
- Hartsfield syndrome
- Hernández-Aguirre Negrete syndrome
- Hypertelorism-hypospadias-polysyndactyly syndrome
- Imperforate oropharynx-costovertebral anomalies syndrome
- Isolated rhombencephalosynapsis
- Kapur-Toriello syndrome
- Lateral meningocele syndrome
- Marden-Walker syndrome
- Microcephalic osteodysplastic primordial dwarfism types I and III
- Microcephalic primordial dwarfism, Montreal type
- Microtriplication 11q24.1 syndrome
- Monosomy 5p syndrome
- Multiple pterygium-malignant hyperthermia syndrome
- Musculocontractural Ehlers-Danlos syndrome
- Neonatal adrenoleukodystrophy
- Neurofibromatosis-Noonan syndrome
- Non-distal duplication 10q syndrome
- Noonan syndrome
- Noonan syndrome-like disorder with loose anagen hair
- Orofaciodigital syndrome type 4
- Osteosclerotic bone dysplasia
- Paternal uniparental disomy of chromosome X syndrome
- Penile agenesis
- Pentasomy X syndrome
- PHAVER syndrome
- Postaxial acrofacial dysostosis
- Progeroid syndrome, Petty type
- Prominent glabella-microcephaly-hypogenitalism syndrome
- Sanjad-Sakati syndrome
- Schwartz-Jampel syndrome
- Short stature-craniofacial anomalies-genital hypoplasia syndrome
- Shprintzen-Goldberg syndrome
- Spondyloepimetaphyseal dysplasia, aggrecan type
- Tetrasomy 5p syndrome
- Thakker-Donnai syndrome
- Thin ribs-tubular bones-dysmorphism syndrome
- Trichorhinophalangeal syndrome type 2
- Triploidy syndrome
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Ear, posterior angulation, increased · Posteriorly angulated ears · Posteriorly rotated · Posteriorly rotated auricles · Posteriorly-angulated ears · Posteriorly-rotated ears
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.