ABSTRACT
The purposes of this review are to facilitate (i) the differential diagnosis of genetic hearing impairment phenotypes, (ii) the consideration of treatment opportunities, (iii) the counselling, and (iv) the guidance for genotyping efforts of newly defined nonsyndromal hearing impairment traits. Illustrative examples are given as for as possible in figures based on previously reported data, our own original data, or original data communicated to us. In previous review papers, we dealt only with nonsyndromal autosomal-dominant SNHI (4-9). The present review not only updates and extends these reviews regarding autosomal-dominant SNHI disorders (linked to DFNA loci) but also covers autosomal-recessive SNHI (DFNB loci) and Xlinked SNHI disorders (DFN loci) as well as a newly identified Y-linked disorder.
