Table 2.

Hearing loss profiles.

ID	Fhx	NBHS	Onset	R Severity	R Audiogram Shape	R PTA 1	L Severity 2	L Audiogram Shape	L PTA	HI	Age at HI
1	None	F	Birth	Severe-to-profound	Flat	92	Severe-to-profound	Flat	92	CI	8 mo
2	Brother	F	Birth	Moderately severe-to-profound	Sloping	77	Severe	Flat	83	CI	10 mo
3	None	P	3 yr	Mild-to-moderate	Sloping	27	Mild-to-moderate	Sloping	32	HA	3 yr
4	None	F	2 yr	Moderate-to-moderately severe	Cookie-bite	52	Moderate-to-moderately severe	Sloping	50	HA	2 yr
5	None	P	3 yr	Moderate-to-severe	Sloping	52	Mild-to-severe	Sloping	55	HA	3 yr
6	Cousin	F	Birth	Profound	Cookie-bite	65	Profound	Cookie-bite	65	CI	23 mo

¹ Average threshold from unaided PTA was calculated from audiometric measures conducted with unaided hearing across 500, 1000, and 2000 Hz. ² Normal, −10 to 15 dB; Slight, 16 to 25 dB; Mild, 26 to 40 dB; Moderate, 41 to 55 dB; Moderately severe, 56 to 70 dB; Severe, 71 to 90 dB; Profound, 91+ dB [30]. Abbreviations: Fhx, family history of early-onset hearing loss; PTA, pure tone average; HI, hearing intervention; CI, cochlear implantation; HA, hearing aid.