Table 3.
Etiologies of Hearing Loss in Children with Mild-to-Severe Hearing Loss
| Etiology | Number | Percent Group | Percent CHL |
|---|---|---|---|
| Syndromic | (n = 27) | (n=307) | |
| Barakat | 1 | 4% | 0% |
| Brachio-oto-renal | 4 | 15% | 1% |
| Charcot-Marie-Tooth | 1 | 4% | 0% |
| Diamond-Blackfan | 1 | 4% | 0% |
| DiGeorge | 2 | 7% | 1% |
| Feingold | 1 | 4% | 0% |
| Goldenhar | 3 | 11% | 1% |
| Pendred | 6 | 22% | 2% |
| Pfeiffer | 1 | 4% | 0% |
| Pierre-Robin Sequence * | 1 | 4% | 0% |
| Stickler | 1 | 4% | 0% |
| Townes-Brocks | 1 | 4% | 0% |
| Treacher-Collins | 3 | 11% | 1% |
| 16p11.2 | 1 | 4% | 0% |
| Total syndromic | 27 | 9% | |
| Non-Syndromic Genetic | (n=30) | ||
| Connexin 26 | 18 | 60% | 6% |
| Non-syndromic EVA | 10 | 33% | 3% |
| OTOA | 1 | 3% | 0% |
| OTOF | 1 | 3% | 0% |
| Total non-syndromic genetic | 30 | 9% | |
| Environmental | (n=21) | ||
| Cisplatin Ototoxicity | 1 | 5% | 0% |
| CMV (Confirmed) | 4 | 19% | 1% |
| Gentamicin exposure | 14 | 67% | 5% |
| Meningitis | 2 | 10% | 1% |
| Total environmental | 21 | 7% | |
| Unspecified Congenital | (n=17) | ||
| Atresia | 2 | 12% | 1% |
| Auditory Neuropathy | 7 | 41% | 2% |
| Mondini Malformation | 7 | 41% | 2% |
| Superior Canal Dehiscence | 1 | 6% | 0% |
| Total Unspecified Congenital | 17 | 6% | |
| No other known etiology or risk factors except history of chronic otitis media with effusion (COME) | 15 | 5% | |
| Unknown etiology | 197 | 64.1% | |
| Total hearing loss | 307 |
*
Not a syndrome, but seen in several syndromes; CMV = Cytomegalovirus; EVA = Enlarged Vestibular Aqueduct