[Small abnormality of the middle ear--a genetically-induced defect?].
Conductive and sensorineural hearing losses are of genetic origin in 20% to 60% of cases. In general, genetic abnormalities are more often expressed as a sensorineural hearing loss than as a conductive hearing loss. At present several genes for sensorineural hearing loss have been isolated. The most common genetically transmitted forms for isolated conductive hearing losses are otosclerosis and small malformations of the ossicles. To date no genes responsible for these deformations have been isolated. We present a family with four siblings having conductive hearing losses caused by ossification of the stapedial tendon. This finding is suggestive of an autosomal recessive inheritance. The early diagnosis of an hereditary conductive hearing loss contains the possibility for permitting normal development of speech.
Klinik für HNO-Krankheiten, Plastische Kopf- und Halschirurgie, Klinikum Nord-Heidberg, Hamburg.
C
C
Thies
K
K
Sperling
A
A
Reis
M
M
Handrock
D000328:Adult; D001301:Audiometry, Pure-Tone; D002869:Chromosome Aberrations / Q000235:genetics*; D025063:Chromosome Disorders; D005260:Female; D005808:Genes, Recessive / Q000235:genetics*; D006314:Hearing Loss, Conductive / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D009999:Ossification, Heterotopic / Q000175:diagnosis / Q000235:genetics*; D010375:Pedigree; D013199:Stapes / Q000002:abnormalities*; D013710:Tendons
Die kleine Fehlbildung des Mittelohres--ein genetisch bedingter Defekt?