TABLE 1:

Clinical and imaging data of patients with congenital hearing loss

Patient	Age (y)/ Sex	Significant MH	History (R/L)	Audiology (R/L)	MR IAC Size	MR Findings, Inner Ear and Nerves
C1	4/M	Hirschprung syndrome	NAD/HL of unknown duration	NAD/no response	L small/R NAD	R NAD/L modiolar deficiency, no L VIII n, clumped vestibular n
C2	8/M	Premature birth, cleft lip and palate	NAD/HL of unknown duration	NAD/severe profound mixed HL	L small/R NAD	R NAD/L modiolar deficiency, no L VIII n
C3	44/M	Negative	Long-standing HL/progressive HL	Audiology not available	R small/L NAD	R EELDS, modiolar deficiency, no R VIII n/L NAD
C4	3/M	CHARGE syndrome	Bilateral HL since birth	Profound bilateral SNHL	Bilateral small	Bilateral severe cochleovestibular dysplasia, no R or L VIII n
C5	5/F	Meconium intussusception	Bilateral HL since birth	Profound bilateral SNHL	Bilateral small	Bilateral severe cochleovestibular dysplasia, no R or L VIII n
C6	4/M	Marked developmental delay, lactic acidosis	Bilateral HL at age 9 months	Could not perform	Bilateral small	Bilateral dysplastic cochlea, bilateral small clumped VIII n
C7	47/F	Scarlet fever as a child	Bilateral HL at age 3 y	No response/profound SNHL	Bilateral NAD	Bilateral EELDS with modiolar deficiency, bilateral small VIII n
C8	5/F	Negative	Long-standing HL/NAD	Profound SNHL/NAD	R small/L NAD	R “isolated” cochlea, no R VIII n/L NAD
C9	3/F	In utero CVA, motor and developmental delay	Bilateral progressive HL	No response bilaterally	Bilateral small	Bilateral modiolar deficiency, no R or L VIII n
C10	6/F	Negative	Bilateral HL since birth	No response bilaterally	Bilateral small	Bilateral severe dysplasia with no cochlea, no R or L VIII n
C11	12/F	In utero toxoplasmosis exposure	NAD/HL (at age 5 y)	NAD/moderate profound SNHL	L small/R NAD	R NAD/L modiolar deficiency, no L VIII n
C12	8/M	Negative	HL since birth/NAD	Profound SNHL/NAD	R small/L NAD	R isolated, modiolar deficiency, no R VIII n or Vinf/L NAD

Note.—MH indicates medical history; CVA, cerebrovascular accident; R, right; L, left; NAD, no abnormality detected; HL, hearing loss; SNHL, sensorineural hearing loss; IAC, internal auditory canal; VIII n, cochlear nerve; n, nerve; EELDS, enlarged endolymphatic duct and sac; Vinf, inferior vestibular nerve.