|
1
|
(HPO:0000407)
|
Sensorineural hearing impairment |
|
|
|
|
524 / 7739
|
|
2
|
(HPO:0008527)
|
Congenital sensorineural hearing impairment |
|
|
|
|
165 / 7739
|
|
3
|
(HPO:0008625)
|
Severe sensorineural hearing impairment |
|
|
|
|
150 / 7739
|
|
4
|
(HPO:0004463)
|
Absent brainstem auditory responses |
|
|
|
|
2 / 7739
|
|
5
|
(HPO:0008529)
|
Absence of acoustic reflex |
|
|
|
|
2 / 7739
|
|
6
|
(OMIM)
|
U- or bowl-shaped audiogram |
|
|
|
|
1 / 7739
|
|
7
|
(OMIM)
|
Normal otoacoustic emissions (OAE), indicating intact outer ear hair cell function |
|
|
|
|
1 / 7739
|
|
8
|
(OMIM)
|
OAE responses may decrease with age or use of hearing aids |
|
|
|
|
1 / 7739
|
|
9
|
(HPO:0000007)
|
Autosomal recessive inheritance |
|
|
|
|
2538 / 7739
|