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Purpose <p>Usher syndrome (USH) is the most common condition in which hereditary hearing loss and progressive visual impairment occur together. USH is a clinically and genetically heterogeneous syndrome, divided categories according to the degree of hearing loss and the presence or absence of vestibular dysfunction, the age of onset of retinis pigmentosa: type 1 (USH1), type 2 (USH2), and type 3 (USH3) and atypical type. In this study, auditory influence according to the types of Usher syndrome will be examined.</p> Materials and methods <p>This systematic review was prepared in accordance with the PRISMA 2020 guidelines. Inclusion criteria include studies from 2010 to 2024, articles written in English, and only studies in individuals with Usher syndrome. Exclusion criteria are animal studies, articles not written in English, thesis studies, books, only gene studies and therapy methods. The remaining 31 articles were included in the study.</p> Results <p>In the included studies, hearing loss was diagnosed according to the ABR and audiometric evaluation results. Congenital moderate to profound hearing loss was observed in USH1 and USH2, while moderate-profound sensorineural hearing loss was observed in USH3 and USH4/Atypical USH. Delays in gross motor skills were reported in USH1 patients during infancy and childhood.</p> Conclusion <p>It was observed that people with USH have different degrees of sensorineural hearing loss depending on the type of syndrome. The type of amplification used is often a cochlear implant in USH1 patients and primarily a hearing aid in other types. It should be kept in mind that hearing aid users may also be candidates for CI due to the prognosis of the disease and regular audiologic follow-up should be performed in these patients. Based on the literature review, it is thought that genetic evaluation is necessary in newborns and children, regardless of the degree of hearing loss, especially if there is a family history of hearing and vision impairment.</p>

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Audiological findings in usher syndrome according to types

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年1月3日

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  • Meliha Başöz Behmen,
  • Rümeysanur Yılmaz,
  • Nida Tas Elibol

摘要

Purpose

Usher syndrome (USH) is the most common condition in which hereditary hearing loss and progressive visual impairment occur together. USH is a clinically and genetically heterogeneous syndrome, divided categories according to the degree of hearing loss and the presence or absence of vestibular dysfunction, the age of onset of retinis pigmentosa: type 1 (USH1), type 2 (USH2), and type 3 (USH3) and atypical type. In this study, auditory influence according to the types of Usher syndrome will be examined.

Materials and methods

This systematic review was prepared in accordance with the PRISMA 2020 guidelines. Inclusion criteria include studies from 2010 to 2024, articles written in English, and only studies in individuals with Usher syndrome. Exclusion criteria are animal studies, articles not written in English, thesis studies, books, only gene studies and therapy methods. The remaining 31 articles were included in the study.

Results

In the included studies, hearing loss was diagnosed according to the ABR and audiometric evaluation results. Congenital moderate to profound hearing loss was observed in USH1 and USH2, while moderate-profound sensorineural hearing loss was observed in USH3 and USH4/Atypical USH. Delays in gross motor skills were reported in USH1 patients during infancy and childhood.

Conclusion

It was observed that people with USH have different degrees of sensorineural hearing loss depending on the type of syndrome. The type of amplification used is often a cochlear implant in USH1 patients and primarily a hearing aid in other types. It should be kept in mind that hearing aid users may also be candidates for CI due to the prognosis of the disease and regular audiologic follow-up should be performed in these patients. Based on the literature review, it is thought that genetic evaluation is necessary in newborns and children, regardless of the degree of hearing loss, especially if there is a family history of hearing and vision impairment.