[MIM 610 706]

Acronym for Labyrinthine Aplasia, Microtia and Microdontia.

Extremely rare. Autosomal recessive transmission of a mutation of the FGF3 gene (fibroblast growth factor 3) on chromosome 11.

Association of:

-        complete aplasia of the labyrinth, also known as Michel aplasia, which causes deafness;

-        type I microtia (generally bilateral)

-        microdontia

Anesthetic implications:

deafness; fragile teeth

References : 

-        Sensi A, Ceruti S, Trevisi P, Gualandi F, Busi M, Donati I, Neri M, Ferlini A, Martini A. 2011. 
LAMM syndrome with middle ear dysplasia associated with compound heterozygosity for FGF3 mutations. 
Am J Med Genet Part A 2011; 155:1096–1101.

-        Singh A, Tekin M, Falcone M, Kapoor S. 
Delayed presentation of rickets in a child with Labyritnhine Aplasia, Microtia and Microdontia (LAMM) syndrome. 
Indian Pediatr 2014; 51: 919-20.

Updated: February 2019