A novel COCH mutation, V104del, impairs folding of the LCCL domain of cochlin and causes progressive hearing loss

According to this view, accumulation of deposits in vestibular and cochlear nerve channels leads to strangulation and progressive degeneration of the dendrites, and loss of cochlear and vestibular neurones. [...]we amplified exon 4 and 5 of the mutant COCH gene separately by using 5[variant prime]-GCGGGATCCGTCGACCGCTCCCATTGCTATCACATG-3[variant prime] (sense) and 5[variant prime]-GAGTTGCTGATTACTCCCCTGTGGACAGCAGCCCCA-3[variant prime] (antisense) primers for exon 4, and 5[variant prime]-TGGGGCTGCTGTCCACAGGGGAGTAATCAGCAACTC-3[variant prime](sense) and 5[variant prime]-GCGAAGCTTACTCGAGAGTTACTGTGAAAGAAGCAG-3[variant prime] (antisense) primers for exon 5.