Table 1.

Clinical Descriptions and Hearing Loss (HL) Genes with Variants in Cochlear Implant Patients^11-15

Patient No.	Prenatal History	Other Childhood Disease	Temporal Bone Findings	HL gene with variant
1	Maternal joint pain during pregnancy	Unremarkable	Superior semicircular canal dehiscence and otitis media, left	KCNQ4
2	Unremarkable	Unremarkable	EVA, bilatera	SLC26A4
3	Maternal fever at 6 mos AOG	Microscopic hematuria; primary complex	Normal	CDH23
4	Unremarkable	Delayed motor development; white matter disease by MRI	Normal	WFS1?
5	Unremarkable	Primary Koch infection	Normal	MYO15A
6	Maternal diabetes at 6 mos AOG	Unremarkable	Malformed cochlea, bilateral; absent cochlear and inferior estibular nerves, right	SLC9A3R1
7	Unremarkable	Unremarkable	EVA, bilateral	COL4A3
8	Unremarkable	Unremarkable	EVA, bilateral	SLC26A4
9	Unremarkable	Global developmental delay; left foot inversion	Normal	MYH14
10	Unremarkable	Maternal urinary tract infection and eclampsia during pregnancy	EVA, lef	IST1 a
11	Unremarkable	Global developmental delay	Normal	SLC12A2b
12	Unremarkable	Sepsis at 19 days of age and was prescribed various antibiotics, one of which was Amikacin	Normal	MYO7A b
13	Unremarkable	Turbinate hypertrophy secondary to papillary allergic rhinitis with nodule in nasopharynx	Normal	CLDN9 b
14	Unremarkable	Unremarkable	Normal	GREB1Lb;CBLN3a
15	Unremarkable	Unremarkable	EVA; Otitis media, left	GDPD5 a

^avariant is found in candidate gene¹⁵;

^bvariant is novel and found in a known HL gene¹⁵

Abbreviations: AOG, age of gestation; EVA, enlarged vestibular aqueduct