Auditory system hereditary disorder
Autosomal recessive hereditary disorder
Decreased hearing
Disorder of acoustic nerve
Hearing loss associated with syndrome
Hereditary degenerative disease of central nervous system
Inherited optic neuropathy
Mitochondrial cytopathy
Optic atrophy
|||||||||
Auditory neuropathy, optic atrophy syndrome (disorder)
Auditory neuropathy, optic atrophy syndrome
A rare mitochondrial disease with characteristics of bilateral auditory neuropathy and optic atrophy. Patients present hearing and visual impairment in the first or second decade of life, while psychomotor development is normal. Bilateral retinitis pigmentosa has been reported in association.
Id1222649004
StatusPrimitive
Finding siteStructure of auditory system
Has interpretationDecreased
InterpretsHearing
Associated morphologyAtrophy
Finding siteOptic nerve structure
Finding siteVestibulocochlear nerve structure
ICD-10 complex map reference set
TargetE88.8
RuleTRUE
AdviceALWAYS E88.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified