Abstract：
:Temperature sensitive auditory neuropathy is a very rare and puzzling disorder. In the present study, we reported three unrelated 2 to 6 year-old children who were diagnosed as auditory neuropathy patients who complained of severe hearing loss when they had fever. Their hearing thresholds varied from the morning to the afternoon. Two of these patients' hearing improved with age, and one patient received positive results from cochlear implant. Genetic analysis revealed that these three patients had otoferlin (OTOF) homozygous or compound heterozygous mutations with the genotypes c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, or c.2382_2383delC/c.1621G>A, respectively. Our study suggests that these gene mutations may be the cause of temperature sensitive auditory neuropathy. The long term follow up results suggest that the hearing loss in this type of auditory neuropathy may recover with age.
journal_name
Hear Resjournal_title
Hearing researchauthors
Zhang Q,Lan L,Shi W,Yu L,Xie LY,Xiong F,Zhao C,Li N,Yin Z,Zong L,Guan J,Wang D,Sun W,Wang Qdoi
10.1016/j.heares.2016.01.008subject
Has Abstractpub_date
2016-05-01 00:00:00pages
53-63eissn
0378-5955issn
1878-5891pii
S0378-5955(16)00004-6journal_volume
335pub_type
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