A gene responsible for autosomal dominant auditory neuropathy (AUNA1) maps to 13q14-21

Abstract
AN may accompany peripheral neuropathy in a variety of dominant syndromes such as Charcot-Marie-Tooth disease2 and Freidreich’s ataxia.3 AN unassociated with peripheral neuropathy most commonly occurs as a sporadic or recessive trait,4– 6 but X linked recessive6 and autosomal dominant7 forms have also been described. We have mapped a gene responsible for autosomal dominant auditory neuropathy in a multigenerational family from the United States to a novel locus, AUNA1 (auditory neuropathy, dominant, 1) on 13q14–21.