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. 2017 Nov 28;7:16504. doi: 10.1038/s41598-017-16676-9

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© The Author(s) 2017

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Pedigrees and audiological phenotype of the three probands. Pure tone audiograms of three ANSD subjects display moderate to severe sensorineural hearing loss, with poor speech discrimination score. DPOAE responses are observed, whereas ABR waveforms are absent or abnormal (a) SH191-430, (b) SH222-518, and (c) SB284-563. The patient SB284-563 shows detectable wave on ABR at 90 dB stimulus on the right ear and no response in both OAE and ABR after two-years. (PTA pure tone audiometry; SDS speech discrimination score; DPOAE distortion product optoacoustic emission; ABR auditory brainstem response; NA not available).