Deafness-intellectual disability syndrome, Martin-Probst type

Orpha code: 85321OMIM code: 300519

Definition

A rare X-linked syndromic intellectual disability characterized by congenital sensorineural hearing loss, varying degrees of intellectual disability, short stature, and dysmorphic facial features (such as telecanthus, epicanthic folds, broad nasal root, malar hypoplasia, low-set ears, dental anomalies, and micrognathia). Additional reported manifestations include microcephaly, renal and genitourinary abnormalities, widely spaced, hypoplastic nipples, and adult onset of progressive pancytopenia.

Disease data
Classification

Malformation syndrome

Synonyms
Hearing loss-intellectual disability syndrome, Martin-Probst type
Zespół głuchota sprzężona z chromosomem X - niepełnosprawność intelektualna
Zespół Martina i Probsta
Martin-Probst syndrome
X-linked deafness-intellectual disability syndrome syndrome
X-linked hearing loss-intellectual disability syndrome syndrome
ORPHA code
85321
OMIM code
300519
ICD10 code
Q87.8
ICD11 code
-

No additional description.

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