Presentation
The abnormalities of the inner ear cause a form of hearing loss called sensorineural deafness that is present from birth (congenital). [medlineplus.gov]
Routine ophthalmologic management of strabismus, if present, is indicated. [ncbi.nlm.nih.gov]
Entire Body System
- Asymptomatic
Heterozygotes (carriers) are asymptomatic and are not at risk of developing the disorder. [ncbi.nlm.nih.gov]
Jaw & Teeth
- Microdontia
Description Congenital deafness with labyrinthine aplasia, microtia, and microdontia (also called LAMM syndrome) is a condition that affects development of the ears and teeth. [medlineplus.gov]
[…] ear agenesis microtia and microdontia; Deafness congenital with inner ear agenesis microtia and microdontia; Deafness with LAMM; LAMM syndromeCongenital deafness with inner ear agenesis microtia and microdontia; Deafness congenital with inner ear agenesis [rarediseases.info.nih.gov]
Congenital deafness with labyrinthine aplasia, microtia, and microdontia A. [ncbi.nlm.nih.gov]
METATARSI RNG100 ALTRE ANOMALIE CONGENITE MULTIPLE CON RITARDO MENTALE SORDITA - NEFROPATIA DI ALPORT RN1360 SINDROME DI ALPORT SORDITA - PTOSI - ANOMALIE SCHELETRICHE RNG151 SINDROMI CON DISPLASIA ECTODERMICA SORDITA CON APLASIA LABIRINTICA, MICROTIA E MICRODONTIA [malattierare.regione.veneto.it]
Treatment
Evaluation of relatives at risk: It is recommended that sibs have hearing screening to allow early diagnosis and treatment of hearing impairment. Genetic counseling. LAMM syndrome is inherited in an autosomal recessive manner. [ncbi.nlm.nih.gov]
[…] microdontia; Deafness with LAMM; LAMM syndromeCongenital deafness with inner ear agenesis microtia and microdontia; Deafness congenital with inner ear agenesis microtia and microdontia; Deafness with LAMM; LAMM syndrome About the Disease Diagnosis & Treatment [rarediseases.info.nih.gov]
Etiology
Prevention of Secondary Complications Regardless of its etiology, uncorrected hearing loss has consistent sequelae: Auditory deprivation through age two years is associated with poor reading performance, poor communication skills, and poor speech production [ncbi.nlm.nih.gov]
Prevention
Injury prevention for children with disabilities. Phys Med Rehabil Clin N Am. 2002;13:891–906. [ncbi.nlm.nih.gov]