Fukushima et al. (1999) reported a family in which 13 members over 4 generations had a form of autosomal dominant nonsyndromic hearing loss, designated DFNA16, in which the phenotype included rapidly progressing and fluctuating hearing loss that appeared ... Fukushima et al. (1999) reported a family in which 13 members over 4 generations had a form of autosomal dominant nonsyndromic hearing loss, designated DFNA16, in which the phenotype included rapidly progressing and fluctuating hearing loss that appeared to respond to steroid therapy. Hearing-impaired persons stated that they had normal or nearly normal hearing until age 9 to 10 years.