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Light eye colour linked to deafness after meningitis

Research pointers: Light eye colour linked to deafness after meningitis
Bacterial meningitis is the most common cause of profound deafness acquired in childhood. Previously there have been no strong indicators of why some survivors of meningitis experience hearing loss whereas others recover fully.
The link between pigmentation and damage to hearing after exposure to ototoxic substances and noise is well documented. People with brown eyes are more likely to experience hearing loss after exposure to cisplatin. It is assumed that people with dark eyes also have more melanin in the inner ear than those with light eyes, and melanin causes the retention of ototoxic derivatives within the cochlea. 1 A higher melanin content in the cochlea also protects against the effects of noise; those with dark eyes are less likely to develop hearing loss associated with noise. 2
Participants, methods, and results
Eye colour was examined in 133 deaf patients with cochlear implants, either by the author's direct observation or by requesting the information by mail. Results were obtained for 130 patients aged from 2 to 80 years (mean 28 years); three patients failed to reply after two letters. The classification of eye colour is subjective; shades of blue, green, grey, and hazel are difficult to distinguish. The classification I used was therefore simply "dark" or "light." "Dark" included pure brown eyes, usually of non.white people, and all other shades of brown. "Light" included blue, green, grey, and hazel eyes.
Overall, 32 patients were deafened by meningitis (table). Of the 98 patients whose deafness was not due to meningitis, 26 (27%) had dark eyes and 72 (73%) had light eyes. This is almost identical to figures obtained from the National Study of Hearing (A Davis, personal communication). From that sample of 1598 adults in the United Kingdom, 447 (28%) had dark eyes and 1151 (72%) had light eyes.
Only two (6%) of the patients in the meningitis group, however, had dark eyes, with 30 (94%) having light eyes. The difference in proportions of eye colour between the survivors of meningitis and the UK adult population was significant. The odds ratio showed that people with light eyes were 5.8 times as likely to be deafened by meningitis than those with dark eyes (95% confidence interval 1.4 to 24.4).
Comment
People with light eyes are more likely to be deafened by meningitis than those with dark eyes. I propose that a higher melanin content protects the inner ear from damage caused by meningitis. However it is possible that the data are misleading. Perhaps people with light eyes are more vulnerable to meningitis or those with dark eyes are more likely to die from meningitis, thus skewing the data for eye colour in the survivors. There is much published evidence that black people have a higher incidence of meningitis than white people, 3 although the reasons for this may be unrelated to pigmentation. Further research may suggest a genetic basis; perhaps the genes encoding eye colour are in linkage disequilibrium with the genes determining the inflammatory response to infection. I thank ME Lutman for advice, RL Booth for statistics assistance, G Jones for microbiology input, and GP Clarke for initial motivation of my interest in this topic.

Hearing Screening in Children

Hearing Screening in Children
Older Infants and Toddlers
Infants and toddlers (7 months through 2 years) should be screened for hearing loss as needed, requested, mandated, or when conditions place them at risk for hearing disability.

Infants not tested as newborns should be screened before three months of age. Other infants should be screened who received neonatal intensive care or special care, or who display other indicators that place them at risk for hearing loss.

Older infants and toddlers who have a greater chance of hearing loss because of certain risk factors should also be screened. This screening should be done even if an initial hearing screening is passed because some causes of hearing loss do not take effect until later in the child's development. These children' s hearing should be monitored at least every 6 months until 3 years of age, and at regular intervals thereafter dependent upon the risk factor.

Risk Factors for Hearing Loss in Children

Parental, caregiver and/or health care provider concerns regarding hearing, speech, language, and/or developmental delay based on observation and/or standardized developmental screening.

1.Family history of permanent childhood hearing loss.

2.Characteristics or other findings associated with a syndrome known to include a sensorineural and/or conductive hearing loss.

3.Infections associated with sensorineural hearing loss including bacterial meningitis, mumps.

4.In utero infections such as cytomegalovirus, herpes, rubella, syphilis, and toxoplasmosis.

5.Neonatal indicators - specifically hyperbilirubinemia at a serum level requiring exchange transfusion, persistent pulmonary hypertension of the newborn associated with mechanical ventilation, and conditions requiring the use of extracorporeal membrane oxygenation (ECMO)

6.Syndromes associated with progressive hearing loss such as neurofibromatosis, osteopetrosis, and Usher' s syndrome

7.Neurodegenerative disorders, such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich' s ataxia and Charcot-Marie-Tooth syndrome.

8.Head trauma

9.Recurrent or persistent otitis media with effusion for at least 3 months.

10.Anatomic disorders that affect eustachian tube function

11.Neurofibromatosis type II or neurodegenerative disorders

Screening Techniques for Infants, Toddlers and Children

Screening procedures to detect hearing impairment that exceeds 20-30 dB HL are applicable to this age group. Two screening methods are suggested as the most appropriate tools for children who are functioning at a development age of 7 months to 3 years, visual reinforcement audiometry (VRA) and conditioned play audiometry (CPA). Both of these methods are behavioral techniques that require involvement and cooperation of the child.

Visual reinforcement audiometry (VRA) is the method of choice for children between 6 months and 2 years of age. The child is trained to look toward (localize) a sound source. When the child gives a correct response, e.g., looking to a source of sound when it is presented, the child is "rewarded" through a visual reinforcement such as a toy that moves or a flashing light.

Conditioned play audiometry (CPA) can be used as the child matures. It is widely used between 2 and 3 years of age. The child is trained to perform an activity each time a sound is heard. The activity may be putting a block in a box, placing pegs in a hole, putting a ring on a cone, etc. The child is taught to wait, listen, and respond.

With both of these methods, sounds of different frequencies are presented at a sound level that children with normal hearing can hear.

It is ideal if the child will allow earphones to be placed on his or her head so that independent information can be obtained for each ear. If the child refuses earphone placement or earphone placement is otherwise not possible, sounds are presented through speakers inside a sound booth. Since sound field screening does not give ear specific information, a unilateral hearing loss (hearing loss in only one ear) may be missed.

Alternative procedures, such as otoacoustic emissions (OAEs) or auditory brainstem response (ABR) may be used if the child is unable to be conditioned. See hearloss.htm for additional information on tests used to evaluate hearing problems.



What happens if a toddler does not pass the screening?

A toddler who does not pass the screening should be rescreened or referred for audiologic evaluation. Confirmation of hearing status should be obtained within 1 month, but no later than 3 months, after the initial screening.



Hearing Screening in Preschoolers

The goal of screening for hearing loss in preschoolers (ages 3-5 years) is to identify children most likely to have hearing loss that may interfere with communication, development, health, or future school performance. In addition, because hearing loss in this age range is so often associated with middle ear disease, it is also recommended that children in this age group be screened for outer and middle ear disorders (acoustic emmittance screening).

Some children may pass an initial hearing screening, but still be at risk for hearing loss that fluctuates, is progressive (gets worse over time), or is acquired later in development.

Risk Factors for Hearing Loss in Preschoolers

1.Parental, caregiver and/or health care provider concerns regarding hearing, speech, language, and/or developmental delay based on observation and/or standardized developmental screening.

2.Family history of permanent childhood hearing loss.

3.Characteristics or other findings associated with a syndrome known to include a sensorineural and/or conductive hearing loss.

4.Infections associated with sensorineural hearing loss including bacterial meningitis, mumps.

5.In utero infections such as cytomegalovirus, herpes, rubella, syphilis, and toxoplasmosis.

6.Neonatal indicators -- specifically hyperbilirubinemia at a serum level requiring exchange transfusion, persistent pulmonary hypertension of the newborn associated with mechanical ventilation, and conditions requiring the use of extracorporeal membrane oxygenation (ECMO)

7.Syndromes associated with progressive hearing loss such as neurofibromatosis, osteopetrosis, and Usher' s syndrome

8.Neurodegenerative disorders, such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich' s ataxia and Charcot-Marie-Tooth syndrome.

9.Head trauma

10.Recurrent or persistent otitis media with effusion for at least 3 months.

11.Ototoxic medications, including but not limited to chemotherapeutic agents or aminoglycosides used in multiple courses or in combination with loop diuretics

12.Apgar scores of 0-4 at 1 minute or 0-6 at 5 minutes

13.Neurofibromatosis type II or neurodegenerative disorders

14.Anatomic disorders that affect eustachian tube function

Screening Techniques for Preschoolers

Screening procedures to detect unilateral or bilateral sensorineural and/or conductive hearing loss greater than 20 dB HL in the frequency region from 1000 through 4000 Hz are applicable to this age group.

Conditioned play audiometry (CPA) is the most commonly employed procedure.

Acoustic Immittance screening may include tympanometry, acoustic reflex, and static acoustic impedance.

Tympanometry introduces air pressure into the ear canal making the eardrum move back and forth. A special machine then measures the mobility of the eardrum. Tympanograms, or graphs, are produced which show stiffness, floppiness, or normal eardrum movement.

Acoustic reflex testing measures the response of a tiny ear muscle that contracts when a loud sound occurs. The loudness level at which the acoustic reflex occurs and/or the absence of the acoustic reflex give important diagnostic information.

Static acoustic impedance testing measures estimate the physical volume of air in the ear canal. This test is useful in identifying a perforated eardrum or whether ear ventilation tubes are still open.

What happens if a preschooler does not pass the screening?

If the child cannot be conditioned to the play audiometry, the child will be screened using infant-toddler procedures or will be recommended for a more in-depth audiologic assessment.

If the child did condition and did not pass the screening, then referral for audiological assessment by an ASHA-certified audiologist will be made.

Hearing status of children referred after screening should be confirmed within 1 month, but no later than 3 months, after the initial screening.



Hearing Screening for School Age Children and Adolescents (5-18 years)

School-age children should be screened for hearing loss as needed, requested, mandated, or when conditions place them at risk for hearing disability. Screening for hearing loss identifies the school-age children most likely to have hearing impairment that may interfere with development, communication, health, and education. School age children with even minimal hearing loss are at risk for academic and communication difficulties.

Periodic screenings are recommended because of the increased potential for hearing loss due to overexposure to high levels of noise and the importance of identifying children at risk for hearing impairment that may affect their future educational, vocational, or social opportunities.

School age children should be screened at the following times:

1.on first entry into school

2.every year from kindergarten through 3rd grade

3.in 7th grade

4.in 11th grade

5.upon entrance into special education

6.upon grade repetition

7.upon entering a new school system without evidence of having passed a previous hearing screening

School age children who already receive regular audiologic management need not participate in a screening program.



Hearing screening should be done in other years when:

1.Parent/care provider, health care provider, teacher, or other school personnel have concerns regarding hearing, speech,

language, or learning abilities.

2.There is family history of late or delayed onset hereditary hearing loss.

3.Otitis media with effusion (fluid in the middle ear) recurs or persists for at least 3 months.

4.There are skull or facial abnormalities, especially those that can cause changes to the structure of the pinna and ear canal.

5.Characteristics or other findings occur that are associated with a syndrome known to include hearing loss.

6.Head trauma occurs with loss of consciousness.

7.There is reported exposure to potentially damaging noise levels or to drugs that frequently cause hearing loss.



Screening techniques used for school-age students

Screening procedures to detect unilateral or bilateral sensorineural and/or conductive hearing loss greater than 20 dB HL in the frequency region from 1000 through 4000 Hz are applicable to this age group.

Conventional audiometry, in which students are instructed to raise their hand (or point to the appropriate ear) when they hear a tone, is the commonly used procedure. Conditioned play audiometry (CPA) is also used.

Who should perform the screening?

Screening practitioners should be limited to:

1.Audiologists holding a Certificate of Clinical Competence (CCC-A) from the American Speech-Language Hearing

Association and state licensure where applicable.

2.Speech-Language Pathologists holding a Certificate of Clinical Competence (CCC-SLP) from the American Speech-Language Hearing Association and state licensure where applicable.

3.Support personnel under supervision of a certified audiologist.

What happens if a school-age student does not pass the screening?
1.The student should be reinstructed, earphones repositioned, and rescreened in the same sessio
2.If the student does not pass the rescreening, he or she should be referred for audiologic assessment.
3.Hearing status of referred students should be confirmed within one month, and no later than 3 months, after initial screening.

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Meningitis Support |Strtococcal Meningitis. |Meningococcal Menigitis and Septicaemia. |Bacterial Meningitis. |MENINGITITS IN YOUNG PEOPLE |TB MENINGTITS |MENINGITIS IN BABY'S |Desease Trends |Vaccines |Men C |Hib vaccination |MONKEYS BOOST MENINGITIS HOPES |'Sneaky' bacteria cause meningitis |Light eye colour linked to deafness after meningitis |COMPLEMENT DEFICIENCIES |Following meningitis |Antibiotics and other Drugs |Disclaimer |Copyright |Mobile Number |Epilepsy |After effects |Memory problems |fungal meningitis |Viral meningitis |Meningitis and the West Nile Virus |Antibiotics and other Drugs |For Professional Medical Advice |PNEUMOCOCCAL MENINGITIS |Am I at Risk? |Stories |My story |In Memory of a lost loved one... |Types |Contact Us By Email. |Privacy |MRSA |Contact Information for Meningitis support |Links for Meningitis support |Message Board |Guestbook