Re: Incidence of hearing loss in families

[Guest guest]

Hi Kerryn

The following is from studies rather than personal experience and it applies

only to deafness due to Connexin 26. Our son is deaf from connexin 26 and this

is from the articles etc i read when i found out the reason for his deafness. We

would like to have another child, deaf or hearing. As we are both carriers of

the connexin 26 gene, there's a 25% chance that another child would be deaf and

75% that s/he would be hearing. If another child is deaf, the degree of hearing

loss of a future child cannot be predicted based on that of Dominic's

(profound). But at any rate it will be severe enough to need hearing aids or a

cochlear implant. Let me know if you want details of the references below.

Best wishes,

Cristina

DEAFNESS DUE TO CONNEXIN 26

How severe is the hearing loss?

Approx 60% of children with connexin 26-related deafness have severe-profound or

profound hearing loss (Cohn et al, 1999). However, the hearing deficit can vary

from mild to profound (Cohn et al, 1999; Denoyelle et al, 1999), and there is no

consistent audiologic phenotype (Cohn et al, 1999; Cohn and Kelley, 1999;

Mueller et al, 1999; Wilcox et al, 1999; Wilcox et al, 2000). At any rate, all

children have some degree of hearing loss (Cohn et al, 1999).

The severity of the hearing loss is extremely variable and cannot be predicted,

not even among siblings. Variation in the severity of deafness between siblings

is common (Denoyelle et al, 1999; Murgia et al, 1999).

Which frequencies are affected?

Usually all frequencies. Connexin 26-related deafness has flat or sloping

audiometric curves (Denoyelle et al, 1999). Loss of hearing in the high

frequency range (4000-8000 Hz) is a characteristic feature (Wilcox et al, 2000).

Is the hearing loss progressive?

Sometimes. In most cases, the hearing loss is not progressive (Mueller et al,

1999; Murgia et al, 1999), at least up to young adulthood (Denoyelle et al,

1999). However, in up to a third of cases, progressive and asymmetrical hearing

loss has been noted (Cohn et al, 1999; Cohn and , 1999).

Are there other associated problems?

Generally, no. Connexin 26-related deafness is associated with a radiologically

normal inner ear (Denoyelle et al, 1999, Cohn et al, 1999; Cohn and Kelley,

1999) and normal vestibular responses (Cohn and Kelley, 1999). The children do

not experience balance problems and learn to sit and walk at age-appropriate

times ( and Van Camp, 2001). There are no associations with thyroid

dysfunction, heart conduction defects, urinalyses, dysmorphic features, or

retinal abnormality (Cohn et al, 1999).

Value of genetic testing

The limited research available shows that parents with normal hearing who have

deaf children view genetic testing for deafness as positive and beneficial (Weir

Brunger et al, 2000). While individual genetic information and counselling are

essential, a few general points can be made.

Genetic testing can help identify a cause for the child's hearing impairment.

Testing for mutations in the GJB2 gene can detect >95% of deafness-causing

mutations of this gene. However, if the GJB2 mutation is not found, this does

not rule out the diagnosis of connexin-26 related deafness or of other possible

genetic causes. Nowadays, at least 40 loci (places) for non-syndromic hearing

impairment have been identified ( and Van Camp, 1999). The genes

responsible have been characterized for two of them, one being GJB2/DFNB1. Then

there are other, syndromic hearing impairments, where deafness accompanies other

signs or symptoms, such as in Waardenburg, Usher or Pendred syndrome. This is an

area of rapid research in genetics and other tests might be available in the

near future.

Genetic testing for mutations in the GJB2 gene has prognostic value. Both

parents of a child with connexin 26-related deafness carry a copy of the GJB2

mutation. In each pregnancy, a couple who have a child with connexin 26-related

deafness have a 25% chance of having a child with hearing loss due to Cx26, a

50% chance of having a child with normal hearing who is a carrier, and a 25%

chance of having a child with normal hearing who is not a carrier. The parents'

siblings have a 50% chance of also being carriers.

A child who has connexin 26-related deafness will have two copies of the mutated

GJB2 gene and will definitely pass on one of them to their offspring. Whether

their offspring are deaf or not will then depend on chance and the laws of

genetics (mode of transmission). If their partner is deaf due to connexin 26 all

children will be deaf, whereas if their partner has a different form of

recessive deafness all their children will have normal hearing (Nance et al,

2000). If their partner is hearing or only a carrier, their offspring will be

hearing.

Theoretically, a mutation in the GJB2 gene can be detected early on in

pregnancy, via amniocentesis or chorion villus sampling. Prenatal testing for

at-risk pregnancies is not carried out at present. At this moment in time, when

gene therapy is still years of research away, the only option for avoiding the

birth of a child with connexin 26-related deafness would be termination of

pregnancy. A UK study of self-selected participants who were either deaf, hard

of hearing or deafened, or were hearing individuals with a deaf parent or child

found that some of those who would take up prenatal diagnosis (21%, 39% and 49%

of these groups, respectively) would consider a termination of pregnancy if the

fetus was found to be deaf (6%, 11% and 16%, respectively) (Middleton et al,

2001). Of those who responded in a US study of hearing parents who had a deaf

child, no parents stated that they would terminate an affected pregnancy (Weir

Brunger et al, 2000).

Prenatal testing could help prepare parents for a deaf child (e.g. by getting

information on all available options for communication). Even if it can be known

that a next child has connexin 26-related deafness, it is not possible to tell

beforehand how severely the hearing will be affected. At this point in time,

there simply is no way of knowing the severity of the hearing loss in a future

child based on the hearing levels of another family member with connexin

26-related deafness. In all cases, prenatal testing could lead to an early

diagnosis of children at birth.

[Guest guest]

Hi Kerryn

The following is from studies rather than personal experience and it applies

only to deafness due to Connexin 26. Our son is deaf from connexin 26 and this

is from the articles etc i read when i found out the reason for his deafness. We

would like to have another child, deaf or hearing. As we are both carriers of

the connexin 26 gene, there's a 25% chance that another child would be deaf and

75% that s/he would be hearing. If another child is deaf, the degree of hearing

loss of a future child cannot be predicted based on that of Dominic's

(profound). But at any rate it will be severe enough to need hearing aids or a

cochlear implant. Let me know if you want details of the references below.

Best wishes,

Cristina

DEAFNESS DUE TO CONNEXIN 26

How severe is the hearing loss?

Approx 60% of children with connexin 26-related deafness have severe-profound or

profound hearing loss (Cohn et al, 1999). However, the hearing deficit can vary

from mild to profound (Cohn et al, 1999; Denoyelle et al, 1999), and there is no

consistent audiologic phenotype (Cohn et al, 1999; Cohn and Kelley, 1999;

Mueller et al, 1999; Wilcox et al, 1999; Wilcox et al, 2000). At any rate, all

children have some degree of hearing loss (Cohn et al, 1999).

The severity of the hearing loss is extremely variable and cannot be predicted,

not even among siblings. Variation in the severity of deafness between siblings

is common (Denoyelle et al, 1999; Murgia et al, 1999).

Which frequencies are affected?

Usually all frequencies. Connexin 26-related deafness has flat or sloping

audiometric curves (Denoyelle et al, 1999). Loss of hearing in the high

frequency range (4000-8000 Hz) is a characteristic feature (Wilcox et al, 2000).

Is the hearing loss progressive?

Sometimes. In most cases, the hearing loss is not progressive (Mueller et al,

1999; Murgia et al, 1999), at least up to young adulthood (Denoyelle et al,

1999). However, in up to a third of cases, progressive and asymmetrical hearing

loss has been noted (Cohn et al, 1999; Cohn and , 1999).

Are there other associated problems?

Generally, no. Connexin 26-related deafness is associated with a radiologically

normal inner ear (Denoyelle et al, 1999, Cohn et al, 1999; Cohn and Kelley,

1999) and normal vestibular responses (Cohn and Kelley, 1999). The children do

not experience balance problems and learn to sit and walk at age-appropriate

times ( and Van Camp, 2001). There are no associations with thyroid

dysfunction, heart conduction defects, urinalyses, dysmorphic features, or

retinal abnormality (Cohn et al, 1999).

Value of genetic testing

The limited research available shows that parents with normal hearing who have

deaf children view genetic testing for deafness as positive and beneficial (Weir

Brunger et al, 2000). While individual genetic information and counselling are

essential, a few general points can be made.

Genetic testing can help identify a cause for the child's hearing impairment.

Testing for mutations in the GJB2 gene can detect >95% of deafness-causing

mutations of this gene. However, if the GJB2 mutation is not found, this does

not rule out the diagnosis of connexin-26 related deafness or of other possible

genetic causes. Nowadays, at least 40 loci (places) for non-syndromic hearing

impairment have been identified ( and Van Camp, 1999). The genes

responsible have been characterized for two of them, one being GJB2/DFNB1. Then

there are other, syndromic hearing impairments, where deafness accompanies other

signs or symptoms, such as in Waardenburg, Usher or Pendred syndrome. This is an

area of rapid research in genetics and other tests might be available in the

near future.

Genetic testing for mutations in the GJB2 gene has prognostic value. Both

parents of a child with connexin 26-related deafness carry a copy of the GJB2

mutation. In each pregnancy, a couple who have a child with connexin 26-related

deafness have a 25% chance of having a child with hearing loss due to Cx26, a

50% chance of having a child with normal hearing who is a carrier, and a 25%

chance of having a child with normal hearing who is not a carrier. The parents'

siblings have a 50% chance of also being carriers.

A child who has connexin 26-related deafness will have two copies of the mutated

GJB2 gene and will definitely pass on one of them to their offspring. Whether

their offspring are deaf or not will then depend on chance and the laws of

genetics (mode of transmission). If their partner is deaf due to connexin 26 all

children will be deaf, whereas if their partner has a different form of

recessive deafness all their children will have normal hearing (Nance et al,

2000). If their partner is hearing or only a carrier, their offspring will be

hearing.

Theoretically, a mutation in the GJB2 gene can be detected early on in

pregnancy, via amniocentesis or chorion villus sampling. Prenatal testing for

at-risk pregnancies is not carried out at present. At this moment in time, when

gene therapy is still years of research away, the only option for avoiding the

birth of a child with connexin 26-related deafness would be termination of

pregnancy. A UK study of self-selected participants who were either deaf, hard

of hearing or deafened, or were hearing individuals with a deaf parent or child

found that some of those who would take up prenatal diagnosis (21%, 39% and 49%

of these groups, respectively) would consider a termination of pregnancy if the

fetus was found to be deaf (6%, 11% and 16%, respectively) (Middleton et al,

2001). Of those who responded in a US study of hearing parents who had a deaf

child, no parents stated that they would terminate an affected pregnancy (Weir

Brunger et al, 2000).

Prenatal testing could help prepare parents for a deaf child (e.g. by getting

information on all available options for communication). Even if it can be known

that a next child has connexin 26-related deafness, it is not possible to tell

beforehand how severely the hearing will be affected. At this point in time,

there simply is no way of knowing the severity of the hearing loss in a future

child based on the hearing levels of another family member with connexin

26-related deafness. In all cases, prenatal testing could lead to an early

diagnosis of children at birth.

[Guest guest]

Hi Kerryn

The following is from studies rather than personal experience and it applies

only to deafness due to Connexin 26. Our son is deaf from connexin 26 and this

is from the articles etc i read when i found out the reason for his deafness. We

would like to have another child, deaf or hearing. As we are both carriers of

the connexin 26 gene, there's a 25% chance that another child would be deaf and

75% that s/he would be hearing. If another child is deaf, the degree of hearing

loss of a future child cannot be predicted based on that of Dominic's

(profound). But at any rate it will be severe enough to need hearing aids or a

cochlear implant. Let me know if you want details of the references below.

Best wishes,

Cristina

DEAFNESS DUE TO CONNEXIN 26

How severe is the hearing loss?

Approx 60% of children with connexin 26-related deafness have severe-profound or

profound hearing loss (Cohn et al, 1999). However, the hearing deficit can vary

from mild to profound (Cohn et al, 1999; Denoyelle et al, 1999), and there is no

consistent audiologic phenotype (Cohn et al, 1999; Cohn and Kelley, 1999;

Mueller et al, 1999; Wilcox et al, 1999; Wilcox et al, 2000). At any rate, all

children have some degree of hearing loss (Cohn et al, 1999).

The severity of the hearing loss is extremely variable and cannot be predicted,

not even among siblings. Variation in the severity of deafness between siblings

is common (Denoyelle et al, 1999; Murgia et al, 1999).

Which frequencies are affected?

Usually all frequencies. Connexin 26-related deafness has flat or sloping

audiometric curves (Denoyelle et al, 1999). Loss of hearing in the high

frequency range (4000-8000 Hz) is a characteristic feature (Wilcox et al, 2000).

Is the hearing loss progressive?

Sometimes. In most cases, the hearing loss is not progressive (Mueller et al,

1999; Murgia et al, 1999), at least up to young adulthood (Denoyelle et al,

1999). However, in up to a third of cases, progressive and asymmetrical hearing

loss has been noted (Cohn et al, 1999; Cohn and , 1999).

Are there other associated problems?

Generally, no. Connexin 26-related deafness is associated with a radiologically

normal inner ear (Denoyelle et al, 1999, Cohn et al, 1999; Cohn and Kelley,

1999) and normal vestibular responses (Cohn and Kelley, 1999). The children do

not experience balance problems and learn to sit and walk at age-appropriate

times ( and Van Camp, 2001). There are no associations with thyroid

dysfunction, heart conduction defects, urinalyses, dysmorphic features, or

retinal abnormality (Cohn et al, 1999).

Value of genetic testing

The limited research available shows that parents with normal hearing who have

deaf children view genetic testing for deafness as positive and beneficial (Weir

Brunger et al, 2000). While individual genetic information and counselling are

essential, a few general points can be made.

Genetic testing can help identify a cause for the child's hearing impairment.

Testing for mutations in the GJB2 gene can detect >95% of deafness-causing

mutations of this gene. However, if the GJB2 mutation is not found, this does

not rule out the diagnosis of connexin-26 related deafness or of other possible

genetic causes. Nowadays, at least 40 loci (places) for non-syndromic hearing

impairment have been identified ( and Van Camp, 1999). The genes

responsible have been characterized for two of them, one being GJB2/DFNB1. Then

there are other, syndromic hearing impairments, where deafness accompanies other

signs or symptoms, such as in Waardenburg, Usher or Pendred syndrome. This is an

area of rapid research in genetics and other tests might be available in the

near future.

Genetic testing for mutations in the GJB2 gene has prognostic value. Both

parents of a child with connexin 26-related deafness carry a copy of the GJB2

mutation. In each pregnancy, a couple who have a child with connexin 26-related

deafness have a 25% chance of having a child with hearing loss due to Cx26, a

50% chance of having a child with normal hearing who is a carrier, and a 25%

chance of having a child with normal hearing who is not a carrier. The parents'

siblings have a 50% chance of also being carriers.

A child who has connexin 26-related deafness will have two copies of the mutated

GJB2 gene and will definitely pass on one of them to their offspring. Whether

their offspring are deaf or not will then depend on chance and the laws of

genetics (mode of transmission). If their partner is deaf due to connexin 26 all

children will be deaf, whereas if their partner has a different form of

recessive deafness all their children will have normal hearing (Nance et al,

2000). If their partner is hearing or only a carrier, their offspring will be

hearing.

Theoretically, a mutation in the GJB2 gene can be detected early on in

pregnancy, via amniocentesis or chorion villus sampling. Prenatal testing for

at-risk pregnancies is not carried out at present. At this moment in time, when

gene therapy is still years of research away, the only option for avoiding the

birth of a child with connexin 26-related deafness would be termination of

pregnancy. A UK study of self-selected participants who were either deaf, hard

of hearing or deafened, or were hearing individuals with a deaf parent or child

found that some of those who would take up prenatal diagnosis (21%, 39% and 49%

of these groups, respectively) would consider a termination of pregnancy if the

fetus was found to be deaf (6%, 11% and 16%, respectively) (Middleton et al,

2001). Of those who responded in a US study of hearing parents who had a deaf

child, no parents stated that they would terminate an affected pregnancy (Weir

Brunger et al, 2000).

Prenatal testing could help prepare parents for a deaf child (e.g. by getting

information on all available options for communication). Even if it can be known

that a next child has connexin 26-related deafness, it is not possible to tell

beforehand how severely the hearing will be affected. At this point in time,

there simply is no way of knowing the severity of the hearing loss in a future

child based on the hearing levels of another family member with connexin

26-related deafness. In all cases, prenatal testing could lead to an early

diagnosis of children at birth.

[Guest guest]

Hi Kerryn. I have thought about this issue often and my views on it have

shifted with time. I too, have a 20 month old. HIs name is and he was

born with a severe/profound hearing loss. He wore hearing aids since 3

months old. He just got an implant 2 weeks ago and will be activated at the

end of the month.

He was diagnosed with the newborn hearing screen and it came as a surprise.

We have no hearing loss in our family. There was no illness or unusual

circumstances during pregancy. was full term... actually 10 days past

due date. I had a c-section and he was perfectly healthy upon delivery.

Once he was diagnosed it was recommended we go through the genetic

counseling. We tested negative for Connexin and they found no reason to test

for the other known genes because displayed no other symptoms/signs that

go along with these. So the cause is a mystery to us.

Sometimes we think things would be so much easier if was not HI. We

wrestled with the thought of having another HI child. IN the beginning we

were overwhelmed with all the new things we were learning and the special

attention we thought we would have to give . We didn't know if we would

have the time, attention, money to devote to another child hearing or not.

But in time we learned so much and through experience we found that is

so much like any other hearing child. There are many challenges, but we

decided to go ahead and have another baby. We thought it would be great for

to have a sibling. I am now expecting a baby in mid September. The

thought that this baby could be hearing impaired does not strike me as

devastating because we've been through it and know what to expect, but at the

same time I really hope he is not HI. The professionals seem to think there

is no reason to think the baby would be HI, but I think if it happened once

it could happen again. It could be genetic afterall and perhaps it's a gene

they just haven't identified yet.

I know I will be very anxious to know the results of the newborn hearing

screen this time around whereas with my first baby I had no idea what was

going on with the hearing screen. I will let you all know the results.

Kearns

[Guest guest]

Hi Kerryn. I have thought about this issue often and my views on it have

shifted with time. I too, have a 20 month old. HIs name is and he was

born with a severe/profound hearing loss. He wore hearing aids since 3

months old. He just got an implant 2 weeks ago and will be activated at the

end of the month.

He was diagnosed with the newborn hearing screen and it came as a surprise.

We have no hearing loss in our family. There was no illness or unusual

circumstances during pregancy. was full term... actually 10 days past

due date. I had a c-section and he was perfectly healthy upon delivery.

Once he was diagnosed it was recommended we go through the genetic

counseling. We tested negative for Connexin and they found no reason to test

for the other known genes because displayed no other symptoms/signs that

go along with these. So the cause is a mystery to us.

Sometimes we think things would be so much easier if was not HI. We

wrestled with the thought of having another HI child. IN the beginning we

were overwhelmed with all the new things we were learning and the special

attention we thought we would have to give . We didn't know if we would

have the time, attention, money to devote to another child hearing or not.

But in time we learned so much and through experience we found that is

so much like any other hearing child. There are many challenges, but we

decided to go ahead and have another baby. We thought it would be great for

to have a sibling. I am now expecting a baby in mid September. The

thought that this baby could be hearing impaired does not strike me as

devastating because we've been through it and know what to expect, but at the

same time I really hope he is not HI. The professionals seem to think there

is no reason to think the baby would be HI, but I think if it happened once

it could happen again. It could be genetic afterall and perhaps it's a gene

they just haven't identified yet.

I know I will be very anxious to know the results of the newborn hearing

screen this time around whereas with my first baby I had no idea what was

going on with the hearing screen. I will let you all know the results.

Kearns

[Guest guest]

Hi Kerryn. I have thought about this issue often and my views on it have

shifted with time. I too, have a 20 month old. HIs name is and he was

born with a severe/profound hearing loss. He wore hearing aids since 3

months old. He just got an implant 2 weeks ago and will be activated at the

end of the month.

He was diagnosed with the newborn hearing screen and it came as a surprise.

We have no hearing loss in our family. There was no illness or unusual

circumstances during pregancy. was full term... actually 10 days past

due date. I had a c-section and he was perfectly healthy upon delivery.

Once he was diagnosed it was recommended we go through the genetic

counseling. We tested negative for Connexin and they found no reason to test

for the other known genes because displayed no other symptoms/signs that

go along with these. So the cause is a mystery to us.

Sometimes we think things would be so much easier if was not HI. We

wrestled with the thought of having another HI child. IN the beginning we

were overwhelmed with all the new things we were learning and the special

attention we thought we would have to give . We didn't know if we would

have the time, attention, money to devote to another child hearing or not.

But in time we learned so much and through experience we found that is

so much like any other hearing child. There are many challenges, but we

decided to go ahead and have another baby. We thought it would be great for

to have a sibling. I am now expecting a baby in mid September. The

thought that this baby could be hearing impaired does not strike me as

devastating because we've been through it and know what to expect, but at the

same time I really hope he is not HI. The professionals seem to think there

is no reason to think the baby would be HI, but I think if it happened once

it could happen again. It could be genetic afterall and perhaps it's a gene

they just haven't identified yet.

I know I will be very anxious to know the results of the newborn hearing

screen this time around whereas with my first baby I had no idea what was

going on with the hearing screen. I will let you all know the results.

Kearns

[Guest guest]

Hi Kerryn,

Here are mine...

Hubby and I -- normal hearing

1st child (9 yo daughter) -- progressive, moderate sloping to

severe/profound loss, bi-laterally -- not confirmed but loss " likely "

genetic

2nd child (7 yo daughter) -- normal hearing

3rd child (3 yo son) -- normal hearing

4th child (9 mth old son) -- one ear normal hearing -- other ear was normal

at newborn screen but in followup OAR results were inconclusive so we'll

retest. I suspect his hearing is normal

Side note: I wish I knew more about the study of genetics but a doctor once

mentioned to me that it is also possible for a fetus's gene to

" spontaneously mutate " and cause a hearing loss in a child -- even though

there is no genetic risk for it and neither parent carries any gene (even

recessively) for loss. In this case, it would be highly unlikely to have

another child with a hearing loss. We have tested negative for everything

genetic they can test for...

When we were expecting our last two children, the thought did cross our

minds that they could be born with hearing loss - and possibly worse than

their sister's. But my OB/GYN made a comment that will stick with me

forever -- When I mentioned my concern, she smiled and said " The way I see

it, you already have a hearing impaired child so God already knows you have

the qualifications to do this -- you are uniquely qualified as far as

mothers go. " There are days I don't feel qualified (ha!) but her point was

made...if it happened, we would deal with it. I just love these kids so

darn much -- can't imagine life without them.

Pam

> I was wondering if I could do my own little survey: Could everyone on the

list (who can be bothered), who has a child with a genetic (or likely

genetic) hearing loss, with 1 or more siblings, hearing or hearing impaired,

let me know each child's hearing status.

[Guest guest]

Hi Kerryn,

Here are mine...

Hubby and I -- normal hearing

1st child (9 yo daughter) -- progressive, moderate sloping to

severe/profound loss, bi-laterally -- not confirmed but loss " likely "

genetic

2nd child (7 yo daughter) -- normal hearing

3rd child (3 yo son) -- normal hearing

4th child (9 mth old son) -- one ear normal hearing -- other ear was normal

at newborn screen but in followup OAR results were inconclusive so we'll

retest. I suspect his hearing is normal

Side note: I wish I knew more about the study of genetics but a doctor once

mentioned to me that it is also possible for a fetus's gene to

" spontaneously mutate " and cause a hearing loss in a child -- even though

there is no genetic risk for it and neither parent carries any gene (even

recessively) for loss. In this case, it would be highly unlikely to have

another child with a hearing loss. We have tested negative for everything

genetic they can test for...

When we were expecting our last two children, the thought did cross our

minds that they could be born with hearing loss - and possibly worse than

their sister's. But my OB/GYN made a comment that will stick with me

forever -- When I mentioned my concern, she smiled and said " The way I see

it, you already have a hearing impaired child so God already knows you have

the qualifications to do this -- you are uniquely qualified as far as

mothers go. " There are days I don't feel qualified (ha!) but her point was

made...if it happened, we would deal with it. I just love these kids so

darn much -- can't imagine life without them.

Pam

> I was wondering if I could do my own little survey: Could everyone on the

list (who can be bothered), who has a child with a genetic (or likely

genetic) hearing loss, with 1 or more siblings, hearing or hearing impaired,

let me know each child's hearing status.

[Guest guest]

Hi Kerryn,

Here are mine...

Hubby and I -- normal hearing

1st child (9 yo daughter) -- progressive, moderate sloping to

severe/profound loss, bi-laterally -- not confirmed but loss " likely "

genetic

2nd child (7 yo daughter) -- normal hearing

3rd child (3 yo son) -- normal hearing

4th child (9 mth old son) -- one ear normal hearing -- other ear was normal

at newborn screen but in followup OAR results were inconclusive so we'll

retest. I suspect his hearing is normal

Side note: I wish I knew more about the study of genetics but a doctor once

mentioned to me that it is also possible for a fetus's gene to

" spontaneously mutate " and cause a hearing loss in a child -- even though

there is no genetic risk for it and neither parent carries any gene (even

recessively) for loss. In this case, it would be highly unlikely to have

another child with a hearing loss. We have tested negative for everything

genetic they can test for...

When we were expecting our last two children, the thought did cross our

minds that they could be born with hearing loss - and possibly worse than

their sister's. But my OB/GYN made a comment that will stick with me

forever -- When I mentioned my concern, she smiled and said " The way I see

it, you already have a hearing impaired child so God already knows you have

the qualifications to do this -- you are uniquely qualified as far as

mothers go. " There are days I don't feel qualified (ha!) but her point was

made...if it happened, we would deal with it. I just love these kids so

darn much -- can't imagine life without them.

Pam

> I was wondering if I could do my own little survey: Could everyone on the

list (who can be bothered), who has a child with a genetic (or likely

genetic) hearing loss, with 1 or more siblings, hearing or hearing impaired,

let me know each child's hearing status.

[Guest guest]

Hi Kerryn,

I have no known hearing loss in my family and had my daughter 6yrs old

now whom has a severe bilateral SNL. We went back and forth with the

concept of having another child. Boy am I glad I did. my son does not

have a hearing loss and all the work with my daughter (reading, reading,

reading) is rubbing off on him (he's now 3) They have normal sibling

fights and they have the best of times together. My question was,

could I handle physically, mentally and fairly, another child. (Did I

mention mentally?) Maybe this next child would be challanged more. I

sure am glad I did! Best wishes to you.

/Mass

[Guest guest]

Hi Kerryn,

I have no known hearing loss in my family and had my daughter 6yrs old

now whom has a severe bilateral SNL. We went back and forth with the

concept of having another child. Boy am I glad I did. my son does not

have a hearing loss and all the work with my daughter (reading, reading,

reading) is rubbing off on him (he's now 3) They have normal sibling

fights and they have the best of times together. My question was,

could I handle physically, mentally and fairly, another child. (Did I

mention mentally?) Maybe this next child would be challanged more. I

sure am glad I did! Best wishes to you.

/Mass

[Guest guest]

Hi Kerryn,

I have no known hearing loss in my family and had my daughter 6yrs old

now whom has a severe bilateral SNL. We went back and forth with the

concept of having another child. Boy am I glad I did. my son does not

have a hearing loss and all the work with my daughter (reading, reading,

reading) is rubbing off on him (he's now 3) They have normal sibling

fights and they have the best of times together. My question was,

could I handle physically, mentally and fairly, another child. (Did I

mention mentally?) Maybe this next child would be challanged more. I

sure am glad I did! Best wishes to you.

/Mass

[Guest guest]

Kerryn,

I am a hearing mother to two children with hearing loss. My husband is also

hearing and we have no evidence of hearing loss in either family. When

ny (6yrs old) was diagnosed at 2 yrs old, we were just fine with it. He

was diagnosed with a mild to mod. loss. We thought he would have the same

loss his whole life. When he started losing his hearing and couldn't hear

at all is when we had a hard time. He would ask us if he was ever going to

hear again. That's a hard question to answer. He was 3 1/2 at the time.

He now has a severe/profound loss. My daughter 3 1/2 now was diagnosed

just recently. I think she had a mild loss to begin with and had a drop

just 3 month ago and then another 2 weeks ago which brought her down to 60

db in one ear and 80 db loss in the other. She is now at 60db loss in both

ears and goes down to a 90 db loss. Yes, it's strange but her hearing did

improve. At any time she or my son can lose hearing. Would we have

another child??? We sure would if my Arthritis wasn't so bad. It would be

just fine and dandy with us if we had another D/HOH child. We use TC at

home now. We've been through it. It's much easier with the second child.

Don't get me wrong, we would love for our children to be hearing but if this

is the only thing that happens to them, that's great!! It could be worse.

We know what to expect and what road to take. Hope this helps you.

Donna

Mother to ny 6.5, Severe/profound HA

3.5, moderate HA

>

To everyone,

>

> Since was diagnosed, some people have asked whether we will have more

children or not. We have been told we have a 1 in 8 chance of future

children being hearing impaired (HI), but know this is not a reason not to

have more children. We want a sibling for and HI or not, a second baby

will be exactly that. We certainly wouldn't have not had had we been

told before she was born that she would be HI - she is too precious!

>

> However, the concept of having another HI child does cross our minds. I

understand the incidence of HI is more likely once you have one HI child,

but was wondering whether it makes any difference if you have, say, one

child who is severely HI, does this mean if you have another HI child, is

that child more likely to be severely HI, or could it be any degree of loss?

>

> I was wondering if I could do my own little survey: Could everyone on the

list (who can be bothered), who has a child with a genetic (or likely

genetic) hearing loss, with 1 or more siblings, hearing or hearing impaired,

let me know each child's hearing status.

> Eg. Child 1 - severe hearing impairment

> Child 2 - hearing

>

> or: Child 1 - moderate hearing impairment

> Child 2 - severe hearing impairment

> Child 3 - hearing

>

> If one or both of the parents are hearing impaired, could you also please

mention this.

>

> I am hoping to get a better idea of the likelihood of having more than 1

hearing impaired child and if so, whether the degree of loss is likely to be

similar or the same as previously HI children. Naturally, this information

will in no way change my mind to have another child and I know there are no

guarantees what will happen, but I was just interested to see the results. I

would really appreciate your time and trouble.

> Kind regards,

> Kerryn (Mum to , 20 months, moderate HI)

>

>

>

[Guest guest]

Kerryn,

I am a hearing mother to two children with hearing loss. My husband is also

hearing and we have no evidence of hearing loss in either family. When

ny (6yrs old) was diagnosed at 2 yrs old, we were just fine with it. He

was diagnosed with a mild to mod. loss. We thought he would have the same

loss his whole life. When he started losing his hearing and couldn't hear

at all is when we had a hard time. He would ask us if he was ever going to

hear again. That's a hard question to answer. He was 3 1/2 at the time.

He now has a severe/profound loss. My daughter 3 1/2 now was diagnosed

just recently. I think she had a mild loss to begin with and had a drop

just 3 month ago and then another 2 weeks ago which brought her down to 60

db in one ear and 80 db loss in the other. She is now at 60db loss in both

ears and goes down to a 90 db loss. Yes, it's strange but her hearing did

improve. At any time she or my son can lose hearing. Would we have

another child??? We sure would if my Arthritis wasn't so bad. It would be

just fine and dandy with us if we had another D/HOH child. We use TC at

home now. We've been through it. It's much easier with the second child.

Don't get me wrong, we would love for our children to be hearing but if this

is the only thing that happens to them, that's great!! It could be worse.

We know what to expect and what road to take. Hope this helps you.

Donna

Mother to ny 6.5, Severe/profound HA

3.5, moderate HA

>

To everyone,

>

> Since was diagnosed, some people have asked whether we will have more

children or not. We have been told we have a 1 in 8 chance of future

children being hearing impaired (HI), but know this is not a reason not to

have more children. We want a sibling for and HI or not, a second baby

will be exactly that. We certainly wouldn't have not had had we been

told before she was born that she would be HI - she is too precious!

>

> However, the concept of having another HI child does cross our minds. I

understand the incidence of HI is more likely once you have one HI child,

but was wondering whether it makes any difference if you have, say, one

child who is severely HI, does this mean if you have another HI child, is

that child more likely to be severely HI, or could it be any degree of loss?

>

> I was wondering if I could do my own little survey: Could everyone on the

list (who can be bothered), who has a child with a genetic (or likely

genetic) hearing loss, with 1 or more siblings, hearing or hearing impaired,

let me know each child's hearing status.

> Eg. Child 1 - severe hearing impairment

> Child 2 - hearing

>

> or: Child 1 - moderate hearing impairment

> Child 2 - severe hearing impairment

> Child 3 - hearing

>

> If one or both of the parents are hearing impaired, could you also please

mention this.

>

> I am hoping to get a better idea of the likelihood of having more than 1

hearing impaired child and if so, whether the degree of loss is likely to be

similar or the same as previously HI children. Naturally, this information

will in no way change my mind to have another child and I know there are no

guarantees what will happen, but I was just interested to see the results. I

would really appreciate your time and trouble.

> Kind regards,

> Kerryn (Mum to , 20 months, moderate HI)

>

>

>

[Guest guest]

Kerryn,

I am a hearing mother to two children with hearing loss. My husband is also

hearing and we have no evidence of hearing loss in either family. When

ny (6yrs old) was diagnosed at 2 yrs old, we were just fine with it. He

was diagnosed with a mild to mod. loss. We thought he would have the same

loss his whole life. When he started losing his hearing and couldn't hear

at all is when we had a hard time. He would ask us if he was ever going to

hear again. That's a hard question to answer. He was 3 1/2 at the time.

He now has a severe/profound loss. My daughter 3 1/2 now was diagnosed

just recently. I think she had a mild loss to begin with and had a drop

just 3 month ago and then another 2 weeks ago which brought her down to 60

db in one ear and 80 db loss in the other. She is now at 60db loss in both

ears and goes down to a 90 db loss. Yes, it's strange but her hearing did

improve. At any time she or my son can lose hearing. Would we have

another child??? We sure would if my Arthritis wasn't so bad. It would be

just fine and dandy with us if we had another D/HOH child. We use TC at

home now. We've been through it. It's much easier with the second child.

Don't get me wrong, we would love for our children to be hearing but if this

is the only thing that happens to them, that's great!! It could be worse.

We know what to expect and what road to take. Hope this helps you.

Donna

Mother to ny 6.5, Severe/profound HA

3.5, moderate HA

>

To everyone,

>

> Since was diagnosed, some people have asked whether we will have more

children or not. We have been told we have a 1 in 8 chance of future

children being hearing impaired (HI), but know this is not a reason not to

have more children. We want a sibling for and HI or not, a second baby

will be exactly that. We certainly wouldn't have not had had we been

told before she was born that she would be HI - she is too precious!

>

> However, the concept of having another HI child does cross our minds. I

understand the incidence of HI is more likely once you have one HI child,

but was wondering whether it makes any difference if you have, say, one

child who is severely HI, does this mean if you have another HI child, is

that child more likely to be severely HI, or could it be any degree of loss?

>

> I was wondering if I could do my own little survey: Could everyone on the

list (who can be bothered), who has a child with a genetic (or likely

genetic) hearing loss, with 1 or more siblings, hearing or hearing impaired,

let me know each child's hearing status.

> Eg. Child 1 - severe hearing impairment

> Child 2 - hearing

>

> or: Child 1 - moderate hearing impairment

> Child 2 - severe hearing impairment

> Child 3 - hearing

>

> If one or both of the parents are hearing impaired, could you also please

mention this.

>

> I am hoping to get a better idea of the likelihood of having more than 1

hearing impaired child and if so, whether the degree of loss is likely to be

similar or the same as previously HI children. Naturally, this information

will in no way change my mind to have another child and I know there are no

guarantees what will happen, but I was just interested to see the results. I

would really appreciate your time and trouble.

> Kind regards,

> Kerryn (Mum to , 20 months, moderate HI)

>

>

>

[Guest guest]

it is also possible for a fetus's gene to

> " spontaneously mutate " and cause a hearing loss in a child -- even though

> there is no genetic risk for it and neither parent carries any gene (even

> recessively) for loss. In this case, it would be highly unlikely to have

> another child with a hearing loss.

Yes, i've come across that as well. When we found out Dominic's deafness is due

to connexin 26 we asked the geneticist about the possibility that it was a

compeltely new occurrence in our family. I'd read that in some cases the

deafness is sporadic, a totally isolated occurrence within the family. Mutations

in the GJB2 gene (connexin 26) have a prevalence of 10-37% in sporadic cases

(Murgia et al, 1999; Estivill et al, 1998). The geneticist was cagey about

providing any figures or discussing it further. We have only tested Dominic so

far, we haven't done any tests on ourselves to see whether we are indeed

carriers. Until we do so, however, we'll assume we are and that a next child

will have a 1:4 possibility of having some degree of hearing loss.

Cristina

[Guest guest]

it is also possible for a fetus's gene to

> " spontaneously mutate " and cause a hearing loss in a child -- even though

> there is no genetic risk for it and neither parent carries any gene (even

> recessively) for loss. In this case, it would be highly unlikely to have

> another child with a hearing loss.

Yes, i've come across that as well. When we found out Dominic's deafness is due

to connexin 26 we asked the geneticist about the possibility that it was a

compeltely new occurrence in our family. I'd read that in some cases the

deafness is sporadic, a totally isolated occurrence within the family. Mutations

in the GJB2 gene (connexin 26) have a prevalence of 10-37% in sporadic cases

(Murgia et al, 1999; Estivill et al, 1998). The geneticist was cagey about

providing any figures or discussing it further. We have only tested Dominic so

far, we haven't done any tests on ourselves to see whether we are indeed

carriers. Until we do so, however, we'll assume we are and that a next child

will have a 1:4 possibility of having some degree of hearing loss.

Cristina

[Guest guest]

it is also possible for a fetus's gene to

> " spontaneously mutate " and cause a hearing loss in a child -- even though

> there is no genetic risk for it and neither parent carries any gene (even

> recessively) for loss. In this case, it would be highly unlikely to have

> another child with a hearing loss.

Yes, i've come across that as well. When we found out Dominic's deafness is due

to connexin 26 we asked the geneticist about the possibility that it was a

compeltely new occurrence in our family. I'd read that in some cases the

deafness is sporadic, a totally isolated occurrence within the family. Mutations

in the GJB2 gene (connexin 26) have a prevalence of 10-37% in sporadic cases

(Murgia et al, 1999; Estivill et al, 1998). The geneticist was cagey about

providing any figures or discussing it further. We have only tested Dominic so

far, we haven't done any tests on ourselves to see whether we are indeed

carriers. Until we do so, however, we'll assume we are and that a next child

will have a 1:4 possibility of having some degree of hearing loss.

Cristina

[Guest guest]

We have three sons - the two older are hearing and the youngest is

HI - so if you think about the Connexin 26 having a 1 in 4 chance of

causing a HI, then would a fourth child - statistically - have no

hearing loss? Hmmm. Well, we decided three was enough so we will

never knowm. Also, we have no known cause for his loss yet since he

is newly diagnosed. Medically we find no reason so we want to do the

genetic testing. I feel we owe that to all three of our children so

if we are carriers they and their spouses can be tested if and when

they decide to have children - if they want to. Would I have had

more children if the first was HI? Absolutely, my kids are great and

are all unique in their own ways.

[Guest guest]

We have three sons - the two older are hearing and the youngest is

HI - so if you think about the Connexin 26 having a 1 in 4 chance of

causing a HI, then would a fourth child - statistically - have no

hearing loss? Hmmm. Well, we decided three was enough so we will

never knowm. Also, we have no known cause for his loss yet since he

is newly diagnosed. Medically we find no reason so we want to do the

genetic testing. I feel we owe that to all three of our children so

if we are carriers they and their spouses can be tested if and when

they decide to have children - if they want to. Would I have had

more children if the first was HI? Absolutely, my kids are great and

are all unique in their own ways.

[Guest guest]

We have three sons - the two older are hearing and the youngest is

HI - so if you think about the Connexin 26 having a 1 in 4 chance of

causing a HI, then would a fourth child - statistically - have no

hearing loss? Hmmm. Well, we decided three was enough so we will

never knowm. Also, we have no known cause for his loss yet since he

is newly diagnosed. Medically we find no reason so we want to do the

genetic testing. I feel we owe that to all three of our children so

if we are carriers they and their spouses can be tested if and when

they decide to have children - if they want to. Would I have had

more children if the first was HI? Absolutely, my kids are great and

are all unique in their own ways.

[Guest guest]

> so if you think about the Connexin 26 having a 1 in 4 chance of

> causing a HI, then would a fourth child - statistically - have no

> hearing loss? Hmmm.

That's not the way things work statistically, unfortunately. EACH child

would have a 1 in 4 chance, including the 4th child.

Hugs,

Kay

[Guest guest]

> so if you think about the Connexin 26 having a 1 in 4 chance of

> causing a HI, then would a fourth child - statistically - have no

> hearing loss? Hmmm.

That's not the way things work statistically, unfortunately. EACH child

would have a 1 in 4 chance, including the 4th child.

Hugs,

Kay

[Guest guest]

> so if you think about the Connexin 26 having a 1 in 4 chance of

> causing a HI, then would a fourth child - statistically - have no

> hearing loss? Hmmm.

That's not the way things work statistically, unfortunately. EACH child

would have a 1 in 4 chance, including the 4th child.

Hugs,

Kay

[Guest guest]

Myself and husband both carriers of Connexin 26 gene,

neither side had history of hearing loss.

First child has moderate to severe hearing loss

Second child has profound hearing loss