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.