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Hyperostosis cranialis interna(HCIN)

MedGen UID:
327093
Concept ID:
C1840404
Disease or Syndrome
Synonym: Hyperostosis cranalis interna
SNOMED CT: Hyperostosis cranialis interna (1217210001)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): SLC39A14 (8p21.3)
 
HPO: HP:0005890
Monarch Initiative: MONDO:0007765
OMIM®: 144755
Orphanet: ORPHA443098

Definition

Hyperostosis cranialis interna (HCIN) is a bone disorder characterized by endosteal hyperostosis and osteosclerosis of the calvaria and the skull base. The progressive bone overgrowth causes entrapment and dysfunction of cranial nerves I, II, V, VII, and VIII (Waterval et al., 2010). [from OMIM]

Clinical features

From HPO
Ocular pain
MedGen UID:
57490
Concept ID:
C0151827
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to the eye.
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Tinnitus
MedGen UID:
52760
Concept ID:
C0040264
Disease or Syndrome
Tinnitus is an auditory perception that can be described as the experience of sound, in the ear or in the head, in the absence of external acoustic stimulation.
Abnormal vestibular function
MedGen UID:
334848
Concept ID:
C1843865
Finding
An abnormality of the functioning of the vestibular apparatus.
Anosmia
MedGen UID:
1950
Concept ID:
C0003126
Finding
An inability to perceive odors. This is a general term describing inability to smell arising in any part of the process of smelling from absorption of odorants into the nasal mucous overlying the olfactory epithelium, diffusion to the cilia, binding to olfactory receptor sites, generation of action potentials in olfactory neurons, and perception of a smell.
Headache
MedGen UID:
9149
Concept ID:
C0018681
Sign or Symptom
Cephalgia, or pain sensed in various parts of the head, not confined to the area of distribution of any nerve.
Chiari type I malformation
MedGen UID:
196689
Concept ID:
C0750929
Congenital Abnormality
Arnold-Chiari type I malformation refers to a relatively mild degree of herniation of the posteroinferior region of the cerebellum (the cerebellar tonsils) into the cervical canal with little or no displacement of the fourth ventricle. It is characterized by one or both pointed (not rounded) cerebellar tonsils that project 5 mm below the foramen magnum, measured by a line drawn from the basion to the opisthion (McRae Line)
Hyposmia
MedGen UID:
473584
Concept ID:
C2364082
Finding
A decreased sensitivity to odorants (that is, a decreased ability to perceive odors).
Mastoiditis
MedGen UID:
7480
Concept ID:
C0024904
Disease or Syndrome
Inflammation of the mucosal lining of the mastoid antrum and mastoid air cell system of the mastoid process.
Facial palsy
MedGen UID:
87660
Concept ID:
C0376175
Disease or Syndrome
Facial nerve palsy is a dysfunction of cranial nerve VII (the facial nerve) that results in inability to control facial muscles on the affected side with weakness of the muscles of facial expression and eye closure. This can either be present in unilateral or bilateral form.
Hyperostosis cranialis interna
MedGen UID:
327093
Concept ID:
C1840404
Disease or Syndrome
Hyperostosis cranialis interna (HCIN) is a bone disorder characterized by endosteal hyperostosis and osteosclerosis of the calvaria and the skull base. The progressive bone overgrowth causes entrapment and dysfunction of cranial nerves I, II, V, VII, and VIII (Waterval et al., 2010).
Calvarial hyperostosis
MedGen UID:
350147
Concept ID:
C1863351
Finding
Excessive growth of the calvaria.
Osteosclerosis of the base of the skull
MedGen UID:
867264
Concept ID:
C4021624
Finding
An increase in bone density affecting the basicranium (base of the skull).
Proptosis
MedGen UID:
41917
Concept ID:
C0015300
Disease or Syndrome
An eye that is protruding anterior to the plane of the face to a greater extent than is typical.
Optic atrophy
MedGen UID:
18180
Concept ID:
C0029124
Disease or Syndrome
Atrophy of the optic nerve. Optic atrophy results from the death of the retinal ganglion cell axons that comprise the optic nerve and manifesting as a pale optic nerve on fundoscopy.
Epiphora
MedGen UID:
57518
Concept ID:
C0152227
Disease or Syndrome
Abnormally increased lacrimation, that is, excessive tearing (watering eye).
Reduced visual acuity
MedGen UID:
65889
Concept ID:
C0234632
Finding
Diminished clarity of vision.
Raised intraocular pressure
MedGen UID:
68606
Concept ID:
C0234708
Finding
Intraocular pressure that is 2 standard deviations above the population mean.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHyperostosis cranialis interna

Conditions with this feature

Hyperostosis cranialis interna
MedGen UID:
327093
Concept ID:
C1840404
Disease or Syndrome
Hyperostosis cranialis interna (HCIN) is a bone disorder characterized by endosteal hyperostosis and osteosclerosis of the calvaria and the skull base. The progressive bone overgrowth causes entrapment and dysfunction of cranial nerves I, II, V, VII, and VIII (Waterval et al., 2010).
Ghosal hematodiaphyseal dysplasia
MedGen UID:
344739
Concept ID:
C1856465
Congenital Abnormality
Ghosal hematodiaphyseal dysplasia (GHDD) is an autosomal recessive disorder characterized by increased bone density with predominant diaphyseal involvement and aregenerative corticosteroid-sensitive anemia (summary by Genevieve et al., 2008).

Recent clinical studies

Etiology

Waterval JJ, van Dongen TM, Stokroos RJ, De Bondt BJ, Chenault MN, Manni JJ
AJNR Am J Neuroradiol 2012 Mar;33(3):453-61. Epub 2011 Dec 22 doi: 10.3174/ajnr.A2830. PMID: 22194361Free PMC Article
Waterval JJ, Stokroos RJ, Bauer NJ, De Bondt RB, Manni JJ
Am J Med Genet A 2010 Mar;152A(3):547-55. doi: 10.1002/ajmg.a.33205. PMID: 20140965

Diagnosis

Waterval JJ, Bischoff MP, Stokroos RJ, Anteunis LJ, Hilkman DM, Kingma H, Manni JJ
Clin Neurol Neurosurg 2013 Sep;115(9):1701-8. Epub 2013 Apr 23 doi: 10.1016/j.clineuro.2013.03.008. PMID: 23622937
Waterval JJ, Van Dongen TM, Stokroos RJ, Teule JG, Kemerink GJ, Brans B, Nieman FH, Manni JJ
Eur J Nucl Med Mol Imaging 2011 May;38(5):884-93. Epub 2010 Nov 16 doi: 10.1007/s00259-010-1655-2. PMID: 21079950Free PMC Article
Waterval JJ, Stokroos RJ, Bauer NJ, De Bondt RB, Manni JJ
Am J Med Genet A 2010 Mar;152A(3):547-55. doi: 10.1002/ajmg.a.33205. PMID: 20140965
Manni JJ, Huygen PL, Noten JF, Kuijpers W
Acta Otolaryngol 1992;112(1):75-82. doi: 10.3109/00016489209100786. PMID: 1575042

Therapy

Waterval JJ, Stokroos RJ, Dings J, Van Overbeeke JJ, Manni JJ
Clin Neurol Neurosurg 2011 Dec;113(10):904-8. Epub 2011 Jun 12 doi: 10.1016/j.clineuro.2011.05.005. PMID: 21665359

Prognosis

Waterval JJ, Bischoff MP, Stokroos RJ, Anteunis LJ, Hilkman DM, Kingma H, Manni JJ
Clin Neurol Neurosurg 2013 Sep;115(9):1701-8. Epub 2013 Apr 23 doi: 10.1016/j.clineuro.2013.03.008. PMID: 23622937
Waterval JJ, van Dongen TM, Stokroos RJ, De Bondt BJ, Chenault MN, Manni JJ
AJNR Am J Neuroradiol 2012 Mar;33(3):453-61. Epub 2011 Dec 22 doi: 10.3174/ajnr.A2830. PMID: 22194361Free PMC Article
Waterval JJ, Van Dongen TM, Stokroos RJ, Teule JG, Kemerink GJ, Brans B, Nieman FH, Manni JJ
Eur J Nucl Med Mol Imaging 2011 May;38(5):884-93. Epub 2010 Nov 16 doi: 10.1007/s00259-010-1655-2. PMID: 21079950Free PMC Article
Waterval JJ, Stokroos RJ, Bauer NJ, De Bondt RB, Manni JJ
Am J Med Genet A 2010 Mar;152A(3):547-55. doi: 10.1002/ajmg.a.33205. PMID: 20140965

Clinical prediction guides

Borra VM, Waterval JJ, Stokroos RJ, Manni JJ, Van Hul W
Calcif Tissue Int 2013 Jul;93(1):93-100. Epub 2013 May 3 doi: 10.1007/s00223-013-9732-8. PMID: 23640157
Waterval JJ, van Dongen TM, Stokroos RJ, De Bondt BJ, Chenault MN, Manni JJ
AJNR Am J Neuroradiol 2012 Mar;33(3):453-61. Epub 2011 Dec 22 doi: 10.3174/ajnr.A2830. PMID: 22194361Free PMC Article
Manni JJ, Huygen PL, Noten JF, Kuijpers W
Acta Otolaryngol 1992;112(1):75-82. doi: 10.3109/00016489209100786. PMID: 1575042

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