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GTR Home > Conditions/Phenotypes > Deafness with labyrinthine aplasia, microtia, and microdontia

Deafness with labyrinthine aplasia, microtia, and microdontia

Synonyms
Congenital Deafness with Inner Ear Agenesis, Microtia, and Microdontia; DEAFNESS, CONGENITAL, WITH LABYRINTHINE APLASIA, MICROTIA, AND MICRODONTIA; Deafness with labyrinthine aplasia microtia and microdontia (LAMM)
Modes of inheritance
Autosomal recessive inheritance (Orphanet)

Summary

Excerpted from the GeneReview: Congenital Deafness with Labyrinthine Aplasia, Microtia, and Microdontia
Congenital deafness with labyrinthine aplasia, microtia, and microdontia (LAMM syndrome) is characterized by: profound bilateral congenital sensorineural deafness associated with inner ear anomalies (most often bilateral complete labyrinthine aplasia); microtia (type I) that is typically bilateral (although unilateral microtia and normal external ears are observed on occasion); and microdontia (small teeth). Individuals with LAMM syndrome commonly have motor delays during infancy presumably due to impaired balance from inner ear (vestibular) abnormalities. Growth, physical development, and cognition are normal.
Full text of GeneReview (by section):
Summary
Diagnosis
Clinical Characteristics
Genetically Related (Allelic) Disorders
Differential Diagnosis
Management
Genetic Counseling
Resources
Molecular Genetics
Chapter Notes
References
Authors:
Jessica Ordonez
Mustafa Tekin
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Available tests

18 tests are in the database for this condition.

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Clinical tests (18 available)

Molecular Genetics Tests

Genes See tests for all associated and related genes

  • Also known as: HBGF-3, INT2, FGF3
    Summary: fibroblast growth factor 3

Clinical features

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Imported from Human Phenotype Ontology (HPO)

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