TABLE 1.

Causative mutation versus CI outcome

Gene	Partition	Function	Successful CI Outcome
GJB2	Sensory	Connexin 26 gap junction	Yes (6–10,12,13,16)
SLC26A4	Sensory	Anion transporter in stria vascularis	Yes (6,13,14,16,26)
MYO7A	Sensory	Myosin in hair bundle organization	Yes (17)
CDH23	Sensory	Cell adhesion protein in tip linkage	Yes (6,17,22)
LOXHD1	Sensory	Stereocilia cell membrane protein	Yes (23)
ACTG1	Sensory	Primary actin isoform in hair cells	Yes (6,24)
OTOF	Synaptic	Otoferlin in synaptic vesicle release	Variable (2,6,16,33–36)
COCH	Synaptic	Extracellular matrix protein	Yes (42,44)
ROR1	Synaptic	Tyrosine kinase-like orphan receptor	Yes (2,39)
OPA1	Synaptic	GTPase in mitochondria	Yes (40,43)
TMPRSS3	Neural	Type II transmembrane serine protease 3	Variable (3,5,6,25,47–50)
TIMM8A	Neural	Mitochondrial translocase	No (2,51,52,55)
PCDH15	Neural	Cell adhesion Protein	No (55)
DFNB59	Neural	Pejvakin, protein of unknown function	No (55)