Table 3.

Phenotypic data for mouse and human Tecta alleles

			TectaC1509G/+ (ZA)	TectaC1619S/+ (ZA)	TectaL1820F,G1824D/+ (ZP)	TectaC1837G/+ (ZP)	TectaY1870C/+ (ZP)
Mouse	Structure of the organ of Corti	Tectorial membrane profile	Thicker and shorter	Distorted cross-sectional profile	‘Hump-backed’	‘Hump-backed’	‘Hump-backed’
		Limbal attachment	Reduced	Reduced	Much reduced	Much reduced	Much reduced
		Marginal band material	Dispersed and thinner	Hollow	Displaced	Displaced	Absent
		Hensen's stripe	ND	Less prominent	Malformed, detached	Malformed, detached	Absent
		Kimura's membrane	Fibrils loosely packed	Thinner, less distinct	Delaminated and hook-like	Delaminated and hook-like	Less firmly attached to TM
	Fluorescence microscopy	Tecta distribution	WT-like	Throughout the core of TM	Throughout the core of TM	Throughout the core of TM	Throughout the core of TM
		Tectb distribution	WT-like	WT-like	Reduced level in sulcal region	Reduced level in sulcal region	WT-like
	Transmission electron microscopy	Covernet fibrils	Covernet bundle is smaller	More numerous and smaller diameter	Partly separated from collagenous matrix	Partly separated from collagenous matrix	ND
	Hair cell	Hair-cell numbers	WT-like	WT-like	WT-like	WT-like	WT-like
		Hair-bundle attachment	1 Row	0 Rows of imprints in apical, 2 rows in basal	3 Rows of imprints in apical, 0 rows in basal	3 Rows of imprints in apical, 0 rows in basal	Imprints present, rows not evaluated
		Prestin level	Increased	WT-like	WT-like	WT-like	ND
	Auditory phenotype	ABR thresholds	Increased 25–40 dB	Increased ∼24 dB	Increased ∼30 dB	Increased ∼35 dB	ND
		Progression	ND	No	No	No	ND
		Seizure sensitivity	ND	Enhanced	Enhanced	Enhanced	ND
Human	Auditory phenotype	Auditory thresholds, severity	Increased, mild–moderate	Increased, mild–moderate	Increased, mild–severe	Increased, ND	Increased, moderate–severe
		Frequency range	High-frequency	High-frequency	Mid-frequency	Mid-frequency	Mid-frequency
		Progression	Yes	Yes	No	Yes	No

Table summarising the structural auditory phenotypes observed in mice carrying dominant, deafness-causing mutations in TECTA and the auditory phenotype of the corresponding human alleles. Data for the Tecta^C1619S, Tecta^{L1820F,G1824D} and Tecta^C1837G mice are from the current study; data for the Tecta^C1509G mouse are from Xia et al. (28), and data for the Tecta^Y1870C mouse are from Legan et al. (21). WT = wild type, ND = not determined, TM = tectorial membrane. Data for human alleles are from refs (11 –13) and (17).