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. Author manuscript; available in PMC: 2015 May 1.
Published in final edited form as: Ear Hear. 2014 May-Jun;35(3):e84–e91. doi: 10.1097/AUD.0000000000000008

Table 1.

Onset Affected frequencies Severity Progression
Autosomal dominant inheritance
DFNA4 (CEACAM16) (Zheng et al. 2011) Postlingual All Moderate Yes, +/- 50 dB HL
DFNA8/12 (TECTA) (Hildebrand et al. 2011; Plantinga et al. 2006) Variable (depending on affected domain) Mid or high, depending on affected protein domain. Mild to severe Variable (depending on cysteine-replacing substitutions)
DFNA13 (COLL11A2) (De Leenheer et al. 2002; McGuirt et al. 1999) Congenital Mid, occasionally high Mild to moderate None
Autosomal recessive inheritance
DFNB21 (TECTA) (Meyer et al. 2007) Prelingual All, mid frequency dip Moderate to profound None
DFNB53 (COLL11A2) (Chen et al. 2005) Prelingual All Profound None
DFNB18B (OTOG) Prelingual All Mild to moderate Mild
DFNB84B (OTOGL) Prelingual All Mild to moderate None