Hearing impairment (HI) is one of the most common sensory disabilities with exceptionally high genetic heterogeneity. Of genetic HI cases, 30% are syndromic and 70% are nonsyndromic. For nonsyndromic (NS) HI, 77% of the cases are due to autosomal recessive (AR) inheritance. ARNSHI is usually congenital/prelingual, severe-to-profound, affects all frequencies and is not progressive. Thus far, 73 ARNSHI genes have been identified. Populations with high rates of consanguinity have been crucial in the identification of ARNSHI genes, and 92% (67/73) of these genes were identified in consanguineous families. Recent changes in genomic technologies and analyses have allowed a shift towards ARNSHI gene discovery in outbred populations. The latter is crucial towards understanding the genetic architecture of ARNSHI in diverse and understudied populations. We present an overview of the 73 ARNSHI genes, the methods used to identify them, including next-generation sequencing which revolutionized the field, and new technologies that show great promise in advancing ARNSHI discoveries.

听力障碍（HI）是最常见的感觉障碍之一，具有极高的遗传异质性。在遗传性 HI 病例中，30% 为综合征型，70% 为非综合征型。对于非综合征性 (NS) HI，77% 的病例是由于常染色体隐性 (AR) 遗传所致。ARNSHI 通常是先天性/语前性的，严重到严重，影响所有频率，并且不是进行性的。迄今为止，已鉴定出 73 个 ARNSHI 基因。近亲率高的人群对于 ARNSHI 基因的鉴定至关重要，92% (67/73) 的这些基因是在近亲家庭中鉴定出来的。基因组技术和分析的最新变化使得在远交种群中发现 ARNSHI 基因成为可能。后者对于了解不同和未充分研究的人群中 ARNSHI 的遗传结构至关重要。我们概述了 73 个 ARNSHI 基因、用于识别它们的方法，包括彻底改变该领域的下一代测序，以及在推进 ARNSHI 发现方面显示出巨大前景的新技术。