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Definition

An X-linked recessive disorder characterized by postlingual sensorineural deafness with onset in early childhood, dystonia, spasticity, dysphagia, mental deterioration, paranoia and cortical blindness.

Acronym

MTS

Synonyms

DDS
DFN-1
Dystonia-deafness syndrome
Jensen syndrome
Opticoacoustic nerve atrophy with dementia
X-linked progressive deafness type 1

Keywords

Deafness
Dystonia

 
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