Morphology studies of the human fetal cochlea in turner syndrome.

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.