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It comprises branchiootorenal syndrome and branchiootic syndrome, distinguished by the presence or absence of renal abnormalities. Pathogenic variants have been discovered in the following genes:\n                    <jats:italic>EYA1<\/jats:italic>\n                    ,\n                    <jats:italic>SIX5<\/jats:italic>\n                    ,\n                    <jats:italic>SIX1<\/jats:italic>\n                    and\n                    <jats:italic>SALL1<\/jats:italic>\n                    . As the otological phenotype in BORSD is inconsistently reported, we performed a systematic review to provide an up\u2010to\u2010date overview, correlated with the genotype. Forty publications were included, describing 295 individual patients. HL was diagnosed in 95%, usually bilateral and mixed\u2010type, and differed among the different genes involved. Mixed moderate\u2010to\u2010severe HL was the predominant finding in patients with\n                    <jats:italic>EYA1<\/jats:italic>\n                    involvement, regardless of the presence of renal abnormalities. The sensorineural HL of profound severity was more prevalent in patients with\n                    <jats:italic>SIX1<\/jats:italic>\n                    mutations. No significant differences among different mutation types or location within the genes could be observed. Structural otological manifestations, ranging from periauricular to inner ear anomalies, were common in both genes. Especially periauricular anomalies were more common and more severe in\n                    <jats:italic>EYA1<\/jats:italic>\n                    . In summary, otological differences among the different genes involved in BORSD are observed, so the molecular analysis is strongly advised.\n                  <\/jats:p>","DOI":"10.1111\/cge.13949","type":"journal-article","created":{"date-parts":[[2021,2,24]],"date-time":"2021-02-24T08:01:55Z","timestamp":1614153715000},"page":"3-13","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Otological manifestations in branchiootorenal spectrum disorder: A systematic review and meta\u2010analysis"],"prefix":"10.1111","volume":"100","author":[{"given":"Anhai","family":"Chen","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology Xiangya Hospital Central South University  Changsha Hunan China"},{"name":"Key Laboratory of Otolaryngology Major Disease Research of Hunan Province  Changsha Hunan China"},{"name":"National Clinical Research Centre for Geriatric Disorders, Department of Geriatrics Xiangya Hospital, Central South University  Changsha Hunan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8447-8647","authenticated-orcid":false,"given":"Jian","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology Xiangya Hospital Central South University  Changsha Hunan China"},{"name":"Key Laboratory of Otolaryngology Major Disease Research of Hunan Province  Changsha Hunan China"},{"name":"National Clinical Research Centre for Geriatric Disorders, Department of Geriatrics Xiangya Hospital, Central South University  Changsha Hunan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederic R. 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