Auditory system hereditary disorder	Congenital anomaly of ear with impairment of hearing	Developmental hereditary disorder	Multiple malformation syndrome with facial defects as major feature	X-linked recessive hereditary disease
\|	\|	\|	\|	\|

X-linked external auditory canal atresia, dilated internal auditory canal, facial dysmorphism syndrome (disorder)
	X-linked external auditory canal atresia, dilated internal auditory canal, facial dysmorphism syndrome
	A rare syndromic genetic deafness with characteristics of congenital hearing loss, atresia or stenosis of the external auditory canal, dilated internal auditory canal, malformation of the inner ear (incomplete separation of the cochlea basal turn from the fundus of the internal auditory canal), in combination with abnormal auricular shape and facial dysmorphism (including thick eyebrows, ptosis, broad nasal root, and telecanthus). Intelligence is normal and developmental delay is absent.
	X-gebonden syndroom van atresie van uitwendige gehoorgang, wijde inwendige gehoorgang en faciale dysmorfie
	X-gebonden syndroom van atresie van meatus acusticus externus, wijde meatus acusticus internus en faciale dysmorfie

Id	1167372000
Status	Primitive

Has interpretation	Impaired
Interprets	Hearing

Associated morphology	Morphologically abnormal structure
Finding site	Ear structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Face structure
Occurrence	Congenital
Pathological process	Pathological developmental process

ICD-10 complex map reference set

Target	H91.8
Rule	TRUE
Advice	ALWAYS H91.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified