Autosomal recessive

Locus (OMIM)

Location

Gene (OMIM)

Important reference

DFNB1

13q12

GJB2

Guilford et al., 1994 Kelsell et al., 1997

DFNB2

11q13.5

MYO7A

Guilford et al., 1994 ; Liu et al., 1997 ; Weil et al., 1997

DFNB3

17p11.2

MYO15A

Friedman et al., 1995 ; Wang et al., 1998

DFNB4

7q31

SLC26A4

Baldwin et al., 1995 ; Li et al., 1998

DFNB5 (see note 1)

14q12

unknown

Fukushima et al., 1995

DFNB6

3p14-p21

TMIE

Fukushima et al., 1995 ; Naz et al, 2002

DFNB7/11

9q13-q21

TMC1

Jain et al., 1995 ; Scott et al., 1996 ; Kurima et al., 2002

DFNB8/10

21q22

TMPRSS3

Veske et al., 1996  ;  Bonné-Tamir et al., 1996 Scott et al., 2001  

DFNB9 (see note 2)

2p22-p23

OTOF

Chaib et al., 1996 ;  Yasunaga et al., 1999

DFNB10

see DFNB8   

 

 

DFNB11

see DFNB7

 

 

DFNB12

10q21-q22

CDH23

Chaib et al., 1996 ;  Bork et al., 2001 

DFNB13

7q34-36

unknown

Mustapha et al., 1998

DFNB14

7q31

unknown

Mustapha et al., 1998

DFNB15

3q21-q25
19p13

 

GIPC3  

Chen et al., 1997

Charizopoulou et al., 2011

DFNB16

15q21-q22

STRC

Campbell et al., 1997 ; Verpy et al., 2001

DFNB17

7q31

unknown

Greinwald et al., 1998

DFNB18

11p14-15.1

USH1C

Jain et al., 1998 Ouyang et al., 2002 ; Ahmed et al., 2002

DFNB18B 11p15.1 OTOG Scharders et al., 2012

DFNB19

18p11

unknown

The Molecular Biology of Hearing and Deafness meeting Bethesda, October 8-11, 1998 (Green et al., abstract 108) 

DFNB20

11q25-qter

unknown

Moynihan et al., 1999

DFNB21

11q

TECTA

Mustapha et al., 1999

DFNB22

16p12.2

OTOA

Zwaenepoel et al ., 2002

DFNB23

10p11.2-q21

PCDH15

Ahmed et al, 2003

DFNB24

11q23

RDX

Khan et al., 2007

DFNB25

4p13

GRXCR1  

Schraders et al., 2010  

DFNB26  (see note 3)

4q31

unknown

Riazuddin et al., 2000

DFNB27

2q23-q31

unknown

Pulleyn et al., 2000

DFNB28

22q13

TRIOBP

Walsh et al., 2000 ; Shahin et al, 2006 ; Riazuddin et al, 2006

DFNB29

21q22

CLDN14

Wilcox et al., 2001

DFNB30

10p11.1

MYO3A

Walsh et al., 2002

DFNB31

9q32-q34

WHRN

Mustapha et al., 2002 ; Mburu et al., 2003

DFNB32

1p13.3-22.1

GPSM2

Masmoudi et al., 2003 ; Walsh et al., 2010

DFNB33

9q34.3

unknown

Medlej-Hashim et al., 2002

DFNB35

14q24.1-24.3

ESRRB

Ansar et al., 2003 ; Collin et al., 2008

DFNB36

1p36.3

ESPN

Naz et al., 2004

DFNB37

6q13

MYO6

Ahmed et al., 2003

DFNB38

6q26-q27

unknown

Ansar et al., 2003

DFNB39

7q21.1

HGF

Schultz et al., 2009

DFNB40

22q

unknown

Delmaghani et al., 2003

DFNB42

3q13.31-q22.3

ILDR1

Aslam et al., 2005 ;  Borck et al., 2011

DFNB44

7p14.1-q11.22

ADCY1

Ansar et al., 2004 ; Santos-Cortez et al., 2014

DFNB45

1q43-q44

unknown

Bhatti et al., 2008

DFNB46

18p11.32-p11.31

unknown

Mir et al., 2005

DFNB47

2p25.1-p24.3

unknown

Hassan et al., 2005

DFNB48

15q23-q25.1

CIB2

Ahmad et al., 2005

DFNB49

5q12.3-q14.1.

MARVELD2 / BDP1

Ramzan et al., 2004 ; Riazuddin et al., 2006 ; Girotto et al., 2013

DFNB51

11p13-p12

unknown

Shaikh et al., 2005

DFNB53

6p21.3

COL11A2

Chen et al., 2005

DFNB55

4q12-q13.2

unknown

Irshad et al., 2005

DFNB59

2q31.1-q31.3

PJVK

Delmaghani et al., 2006

DFNB60 5q23.2-q31.1 SLC22A4 Ben Said et al., 2016

DFNB61

7q22.1

SLC26A5

Liu et al., 2003

DFNB62

12p13.2-p11.23

unknown

Ali et al., 2006

DFNB63

11q13.2-q13.4

LRTOMT/COMT2

Du et al., 2008 ; Ahmed et al., 2008 

DFNB65

20q13.2-q13.32

unknown

Tariq et al., 2006

DFNB66 6p21.2-22.3 DCDC2 Grati et al., 2015

DFNB66/67

6p21.31

LHFPL5

Tlili et al., 2005 ; Shabbir et al., 2006 ; Kalay et al., 2006

DFNB68

19p13.2

S1PR2

Santos et al., 2006 Santos-Cortez et al., 2016

DFNB71

8p22-21.3

unknown

Chishti et al., 2009

DFNB72

19p13.3

GIPC3  

Ain et al., 2007 ; Rehman et al., 2011

DFNB73

1p32.3

BSND 

Riazuddin et al., 2009 

DFNB74

12q14.2-q15

MSRB3  

Waryah et al., 2009 ; Ahmed et al., 2011

DFNB76 19q13.12 SYNE4 Horn et al., 2013

DFNB77

18q12-q21

LOXHD1 

Grillet et al., 2009

DFNB79 9q34.3 TPRN Rehman et al., 2010
DFNB80 2p16.1-p21 unknown Mosrati et al., 2013
DFNB81 19p unknown Rehman et al., 2011
DFNB82 see DFNB32  
DFNB83 see DFNA47    
DFNB84 12q21.2 PTPRQ / OTOGL Schraders et al., 2010 ; Yariz et al., 2012
DFNB85 17p12-q11.2 unknown Shahin et al., 2010
DFNB86 16p13.3 TBC1D24 Ali et al., 2012Rehman et al., 2014
DFNB88 2p12-p11.2 ELMOD3 Jaworek et al., 2013
DFNB89 16q21-q23.2 KARS Basit et al., 2011
DFNB90 7p22.1-p15.3 unknown Ghazanfar et al., 2011
DFNB91 6p25 SERPINB6 Sirmaci et al., 2010
DFNB93

11q12.3-11q13.2

CABP2

Tabatabaiefar et al., 2011

DFNB94
NARS2 Simon et al., 2015
DFNB95 19p13 GIPC3 Charizopoulou et al., 2011
DFNB96 1p36.31-p36.13 unknown Ansar et al., 2011
DFNB97 7q31.2-q31.31 MET Mujtaba et al., 2015
DFNB98 21q22.3-qter TSPEAR Delmaghani et al., 2012
DFNB99 17q12 TMEM132E Li et al., 2015
DFNB101 5q32 GRXCR2 Imtiaz et al., 2014
DFNB102 12p12.3 EPS8 Behlouli et al., 2014
DFNB103 6p21.1 CLIC5 Seco et al., 2015
DFNB105
1p21.2-1p21.1 CDC14A Delmaghani et al., 2016




 


Note 1: DFNB5 was reported originally as DFNB4.

Note 2:  DFNB9 was reported originally as DFNB6 .

Note 3: DFNB26 is suppressed by dominant modifier DFNM1.