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CASE REPORT
Year : 2022  |  Volume : 28  |  Issue : 1  |  Page : 62-64

Auditory neuropathy spectrum disorder in two siblings with congenital cytomegalovirus infections: Two rare cases


Department of Ear Nose Throat-Head and Neck Surgery, Faculty of Medicine, Universitas Indonesia/Dr. Cipto Mangunkusumo National Hospital, Jakarta, Indonesia

Correspondence Address:
Dr. Ronny Suwento
Department of Ear Nose Throat-Head and Neck Surgery, Faculty of Medicine, Universitas Indonesia/Dr. Cipto Mangunkusumo National Hospital, Jakarta
Indonesia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.indianjotol_25_21

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Auditory neuropathy spectrum disorder (ANSD) is a rare hearing disorder when signal transmission is disrupted on the auditory pathway. Infection is one of the multifactorial causes of ANSD. Only a few researchers have reported the cases of ANSD caused by congenital cytomegalovirus (CMV) infection, and fewer have presented cases involving siblings. In our study, the two patients were siblings, aged 3 years and 7 months and 8 months. They were born prematurely, had microcephaly; anti-CMV IgG levels were reactive. Distortion product otoacoustic emissions test was normal. A click auditory brainstem response showed that no wave was detected with an 80 dB stimulus, and a cochlear microphonic response was obtained. No abnormality in the middle ear was observed, and negative acoustic reflexes were noted. Hearing habilitation with a low-gain hearing aid, speech therapy, and motoric habilitation was planned, and the patients were referred to the pediatric neurology for CMV management.


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