A rare, genetically highly heterogeneous otorhinolaryngologic disease, resulting from inner and/or middle ear or hearing nerve anomalies, typically characterized by bilateral, severe to profound hearing loss (mean sensorineural hearing impairment of 60 dB or more for 500-, 1,000-, and 2,000-Hz frequency tones in the better ear) which occurs before the onset of speech development and is not associated with visible external ear abnormalities or any other medical problems. It is usually nonprogressive and impedes oral language acquisition.
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X-linked mixed hearing loss with perilymphatic gusher
MONDO:0010576
postlingual non-syndromic genetic hearing loss
MONDO:0016298
X-linked nonsyndromic hearing loss
MONDO:0019586
autosomal dominant nonsyndromic hearing loss
MONDO:0019587
hearing loss, autosomal recessive
MONDO:0019588
nonsyndromic deafness, Y-linked
MONDO:0033304