Table 1:

Selected hereditary syndromes commonly associated with SNHL

Inner Ear Malformations on ImagingInner Ear Malformations Not Common on Imaging
Alagille syndromeAlport syndrome
Branchio-oto-renal syndromeBiotinidase deficiency
CHARGE syndromeJervell and Lange-Nielsen syndrome
Klippel-Feil syndromeNorrie syndrome
Pendred syndromeStickler syndrome
Waardenburg syndromeUsher syndrome
X-linked hearing loss with stapes gusher