@entity70271 or degeneration ( @entity528 ) is considered the most common cause of profound @entity393 , and accounts for approximately 70% of cases 2 with @entity74 . A five-generation family with @entity6363 associated with @entity70271 has recently been identified. The diagnosis of classical @entity528 was based on histopathological findings in the temporal bones of the proband, a 61-year-old profoundly deaf male. Auditory structures in the brainstem of the proband were also studied. Twenty-two members of the family were contacted for surveys and blood samples. Of these, 6 males and 2 females have @entity393 . Complete audiological evaluation was done on 12 family members, and prior audiologic records of the proband and affected family members were available for study. Affected family members suffer a mild bilateral high-frequency @entity393 during childhood and adolescence, and progress to moderate-to-profound @entity2319 in the second and third decades of life. The family is suitable for linkage analysis and does not map to previously reported loci harboring autosomal dominant, @entity6363 genes. The genetic study of this family will be helpful in identifying the genes which, when mutated, result in @entity528 .

A five-generation family with late-onset progressive @entity6363 due to XXXX .

@entity6363 :: ('MESH:C537845', 'Disease') :: ['hereditary hearing impairment', 'nonsyndromic hereditary hearing impairment'] @entity2319 :: ('MESH:D003638', 'Disease') :: ['deafness'] @entity74 :: ('MESH:D030342', 'Disease') :: ['hereditary deafness'] @entity70271 :: ('MESH:C536432', 'Disease') :: ['cochleosaccular degeneration', 'Cochleosaccular dysplasia'] @entity393 :: ('MESH:D034381', 'Disease') :: ['congenital hearing impairment', 'hearing loss', 'hearing impairment'] @entity528 :: ('MESH:D009410', 'Disease') :: ['Scheibe degeneration']
@entity70271 :: (MESH:C536432,Disease) :: ['cochleosaccular degeneration']
