OBJECTIVES: Turner syndrome (TS) is the most frequent sex chromosome abnormality, and sensorineural hearing loss is common. We aimed to determine whether there are consistent morphologic cochlear abnormalities during gestational development that could be associated with TS. DESIGN: The histology of nine fetal temporal bones of TS autopsied after spontaneous abortion was studied. RESULTS: Gross morphologic examination of the TS cochleae failed to reveal a pattern of structural abnormalities that would explain the development of sensorineural hearing loss. Mondini-like cochlear dysplasia was observed in one 13-wk-old TS fetus. CONCLUSION: We could not demonstrate a consistent pattern of cochlear malformations.
OBJECTIVES:Turner syndrome (TS) is the most frequent sex chromosome abnormality, and sensorineural hearing loss is common. We aimed to determine whether there are consistent morphologic cochlear abnormalities during gestational development that could be associated with TS. DESIGN: The histology of nine fetal temporal bones of TS autopsied after spontaneous abortion was studied. RESULTS: Gross morphologic examination of the TS cochleae failed to reveal a pattern of structural abnormalities that would explain the development of sensorineural hearing loss. Mondini-like cochlear dysplasia was observed in one 13-wk-old TS fetus. CONCLUSION: We could not demonstrate a consistent pattern of cochlear malformations.
Authors: Siddharth K Prakash; Adrianna K San Roman; Melissa Crenshaw; Barbara Flink; Kimberly Earle; Evan Los; Åsa Bonnard; Angela E Lin Journal: Am J Med Genet C Semin Med Genet Date: 2019-01-11 Impact factor: 3.908
Authors: Marsha L Davenport; Jackson Roush; Chunhua Liu; Anthony J Zagar; Erica Eugster; Sharon Travers; Patricia Y Fechner; Charmian A Quigley Journal: Horm Res Paediatr Date: 2010-04-27 Impact factor: 2.852