Recent clinical trials targeting congenital hearing loss have demonstrated the promise of gene therapy as a curative approach. However, whether the therapeutic system can deliver agents to the inner ear efficiently and precisely remains one of the key determinants of therapeutic efficacy. Based on the unique anatomical and physiological features of the inner ear, this review systematically summarizes the development process of targeted inner ear drug delivery strategies from basic research to clinical translation, with a focus on analyzing the impacts of different inner ear delivery pathways on treatment safety and effectiveness. Meanwhile, drawing on multiple gene therapy clinical trials for OTOF-related deafness, it highlights the practical value of targeted inner ear delivery strategies in clinical translation and the emergence of Inner Ear Surgery in the current landscape. This review provides a systematic perspective on targeted inner ear delivery strategies, offering a clear reference framework for relevant practitioners and facilitating the development and translation of gene therapy for congenital hearing loss. Furthermore, the introduction of the Inner Ear Surgery concept is poised to herald a new paradigm in otology.