Download Document

Comparison of Risk Factors Associated with Unilateral and
Bilateral Hearing Loss Identified by Newborn Hearing Screening
Jonathan N. Young MD 1, Joshua C. Yelverton MD 1, Laura M. Dominguez MD 1, Derek A. Chapman PhD2,4,
Shuhui Wang BS4, Arti Pandya, MD3, Kelley M. Dodson MD 1
Virginia Commonwealth University Medical Center Department s of 1Otolaryngology/Head and Neck
2
3
Surgery, Epidemiology and Community Health, Human and Molecular Genetics, Richmond, VA;
4Virginia Department of Health, Richmond, VA
ABSTRACT
Objective: To compare the incidence
of Joint Committee on Infant Hearing
(JCIH) risk factors and co-occurring
birth defects in children with
unilateral hearing loss (UHL) to
children with bilateral hearing loss
(BHL).
Methods: Retrospective review of
1375 children with confirmed hearing
loss identified through universal
newborn hearing screening (UNHS)
program in Virginia from 2002-2010.
Summary of Results: Of 493 children
with confirmed UHL, 96.1% were
identified through failed UNHS and
36% had one or more risk factor
reported. Of 882 children with
confirmed BHL, 95.7% were identified
through failed UNHS and 31% had
one or more risk factor reported. In
UHL, craniofacial anomalies (39.7%)
and neonatal indicators (22.4%) were
the most commonly reported risk
factors. In BHL, family history of
hearing loss was most commonly
reported (39.9%), followed by
neonatal indicators (25.7%).
Additional co-occurring birth defects
were identified in 35% of children
with UHL or BHL. For children with a
JCIH risk factor, co-occuring birth
defects were present in 69.9% with
UHL and in 53.6% with BHL. Children
with UHL most often had ear or
cardiovascular anomalies, while those
with BHL most often had
cardiovascular or musculoskeletal
anomalies.
Conclusion: The proportion of
children with confirmed UHL or BHL
and a JCIH risk factor or co-occurring
birth defect was similar at about 1/3.
However, the profile of risk factors
and co-occurring birth defects
differed. Further studies investigating
risk factors and co-morbid birth
defect associations with the etiology
of hearing loss are warranted.
Correspondence:
Jonathan N. Young, MD
VCU Medical Center
Department of Otolaryngology –
Head & Neck Surgery
Richmond, Virginia – USA
[email protected]
INTRODUCTION
Congenital hearing loss has a prevalence ranging
from 0.3 to 3.0 per 1,000 newborns.1-3,17 Universal
newborn hearing screening was initiated in order to
identify children with hearing loss. Before the advent of
universal newborn hearing screening (UNHS), the
diagnosis of HL was often delayed. Children with UHL
often went undiagnosed and unnoticed in children until
they reached elementary school. Early detection however
allows for early intervention, which is shown to play a key
role in cognitive and verbal development.
Research has shown that as many as 22% to 35% of
children with hearing loss fail at least one grade;
additionally, up to 20% are identified as having behavioral
problems.5-7 Multiple studies have shown that the speech
and language developmental delays can be prevented if
appropriate intervention for children with hearing loss is
initiated by 6 months of age.8-11,17
In 2007, the Joint Committee on Infant Hearing
(JCIH) released an updated position statement regarding
its principles and guidelines for early hearing detection
and intervention programs.4 The JCIH identified certain
risk factors that predispose infants to hearing loss. The
association between JCIH risk factors has been studied in
children with hearing loss; however, there has been no
delineation between the risk factor profiles of unilateral
and bilateral hearing loss.
Nearly half of all infants who do not pass the initial
hospital hearing screen do not receive timely and
appropriate follow-up care.12,13 The presence of a comorbid birth defect has been shown to lead to delay in
hearing screening, diagnosis, and intervention.1, 20-23 In
addition, the presence of a co-morbid birth defect
increases the chances that a child may need prolonged
mechanical ventilator support, and multiple studies have
demonstrated that mechanical ventilation greatly
increases the odds of SNHL.17-19
The aims of this study are to:
1. analyze and compare the JCIH risk factors
profiles of UHL and BHL
2. compare the prevalence of various co-morbid
birth defects with UHL and BHL.
RESULTS
Remainder of demographics is summarized in Figure 1.
Figure 1: Chart of infants born in Virginia
from 2002 to 2010.
 At least 1 JCIH risk factor was identified in 276 (31%) infants with confirmed BHL and 156 (32%) infants with confirmed UHL.
• Most common in BHL were family history of permanent hearing loss in 110 (39.9%) and neonatal indicators
(hyperbillirubinema, PPHN, and use of ECMO) in 25.7%.
• Most common in UHL were craniofacial anomalies in 62 (39.7%) and neonatal indicators (hyperbillirubinema, PPHN, and
use of ECMO) in 22.4%. (Summarized in Graph 1 and Table 1).
 Overall, 314 (35.6%) children with confirmed BHL and 161 (32.7%) children with confirmed UHL were found to have a cooccurring birth defect
• Most common were cardiovascular and musculoskeletal anomalies in BHL
• Most common cardiovascular anomalies followed by ear and respiratory anomalies in UHL. (Summarized in Graph 2 and
Table 2)
 148 (53.6%) children with confirmed BHL and a JCIH risk factor and 109 (69.9%) children with confirmed UHL and a JCIH risk
factor were found to have a co-occurring birth defect
• Most common in BHL were cardiovascular anomalies in 83 (56%) and musculoskeletal anomalies in 48 (32.0%)
• Most common in UHL were cardiovascular anomalies 49 (45%) followed by ear anomalies 43 (39%). (Summarized in
Graph 3 and Table 3)
Table 1: JCIH Risk Factors Present in Infants
Graph 1: JCIH Risk Factors Present in Infants Diagnosed
Diagnosed with BHL vs. UHL
with BHL vs. UHL
JCIH Risk Factors present in infants
diagnosed with BHL vs. UHL
METHODS AND MATERIALS
• Data was extracted regarding newborn hearing
screening and confirmatory diagnoses from the Virginia
Early Hearing Detection and Intervention (VEHDI) program
database for children born between January 1, 2002 and
December 31, 2010.1
• All newborns with a confirmed BHL, including those
newborns who passed their initial hearing screening and
required follow up due to a known risk factor were
analyzed
• Children were grouped into categories regarding status
of their newborn hearing screen, confirmatory test
outcome, and risk factor status.
• Hearing loss in this study was defined as having one of
the following International Classification of Diseases, Ninth
Revision, Clinical Modification (ICD-9-CM;14 codes reported
in both ears at a follow-up assessment by a licensed
audiologist: 389.0 (conductive hearing loss), 389.1
(sensorineural hearing loss), 389.2 (mixed hearing loss),
and 389.9 (undetermined hearing loss).
• JCIH risk factors were categorized as follows: (a)
craniofacial anomaly, (b) family history of permanent
childhood hearing loss, (c) head trauma requiring
hospitalization, (d)in utero infections such as CMV, herpes,
rubella, syphilis, and toxoplasmosis, (e) neonatal indicators
for hearing loss such as neonatal care of more than 5 days
or ECMO, assisted ventilation, exposure to ototoxic
medications (gentamycin or tobramycin) or loop diuretics
(furosemide), and hyperbilirubinemia that requires
exchange transfusion, and (f) stigmata of syndrome with
known hearing loss.
• Birth defect diagnoses were extracted from the
Virginia Congenital Anomalies Reporting System
(VaCARES) for children born within the same time period.
• ICD-9-CM codes were grouped into general categories
based on the organ system: (a) cardiovascular, (b) earspecific, (c) eye, (d) Endocrine/metabolic, (e)
gastrointestinal, (f) chromosomal, (g) genitourinary, (h)
integumentary, (i) musculoskeletal, (j) neurological, and (k)
respiratory tract.
DISCUSSION
Graph 2: Co-Occurring birth defects in Infants with
confirmed BHL vs. UHL
BHL (%)
UHL (%)
Family history of HL
39.9
18.6
Neonatal indicator
25.7
22.4
Stigmata of syndrome associated with
HL
19.6
18.6
Craniofacial anomaly
12.3
39.7
Parental/Caregiver Concern
11.6
7.1
In utero infections
6.2
5.8
Head Trauma
1.8
1.3
Syndromes associated with HL
1.1
0.6
Postnatal infection
0.7
1.9
Chemotherapy
0.0
0.6
Table 2: Co-occurring birth defects in
Infants with confirmed BHL vs. UHL
Co-Occurring birth defects in infants with BHL vs. UHL
BHL (%) UHL (%)
Cardiovascular
53
46
Musculoskeletal
28
16
Chromosomal
CNS
Metabolic & Endocrine
21
21
21
14
19
12
Respiratory
19
24
Genitourinary
Eye
Orofacial
17
16
16
12
11
4
Prenatal Exposures
13
14
Ear Anomalies
11
24
Gastrointestinal
10
7
6
4
2
1
Blood and Immune
Other
Graph 3: Co-Occurring birth defects in Infants with a JCIH Risk
Factor and BHL vs. UHL
Table 3: Co-occurring birth defects in Infants
with a JCIH risk factor and BHL vs. UHL
Co-Occurring Birth Defects in infants with
a JCIH Risk Factor and BHL vs. UHL
BHL (%)
UHL (%)
Cardiovascular
56
45
Musculoskeletal
32
11
Chromosomal
28
18
Orofacial
22
3
CNS
21
12
Genitourinary
20
9
Other
20
2
Respiratory
18
9
Ear Anomalies
17
39
Metabolic & Endocrine
Eye
Gastrointestinal
Prenatal Exposures
Blood and Immune
16
15
9
7
4
10
8
9
2
1
This study compares the risk factor profile associated
with UHL to that of BHL. The incidence of UHL in our study
was 0.3 per 1,000 newborns compared to an incidence of
0.5 per 1,000 newborns for BHL. This data was collected
from the Virginia Early Hearing Detection, an Intervention
program database for an 8-year period.
We found that almost one third of infants with
confirmed HL had a JCIH risk factor. A 2011 study by
Bielecki et al. assessed the incidence of JCIH risk factors in
children with sensorineural hearing loss (SNHL) without
defining laterality.16 That study reported that the most
common JCIH risk factors were syndromes associated with
hearing loss (15.52%) and mechanical ventilation for
longer than 5 days (11.45%). This differed from our
findings, where craniofacial anomalies (39.7%) and family
history of hearing loss (39.9%) were most prevalent for
UHL and BHL respectively. This difference could be
attributed to the laterality of hearing loss. Overall, our
data delineates the difference in profiles of JCIH risk
factors for UHL and BHL. We clearly show that both UHL
and BHL are associated with different risk factor profiles
for hearing loss in as many as a third of the children.
In a study from 2011, Chapman et al. also found an
incidence of co-occurring birth defects in about 1/3 of all
infants withUHL.1 Our study demonstrates that the most
common birth defect associated with UHL is
cardiovascular anomalies (46%), followed by ear and
respiratory anomalies (24% each). While BHL shares
cardiovascular anomalies (53%) as the most common birth
defect with UHL, the second most common birth defect
was musculoskeletal anomalies (28%).
A strength of this study lies in the use of populationbased data on hearing loss, risk factors, and birth defects,
which results in a large cohort for analysis. One weakness
of our study is the reliance on ICD-9-CM codes that could
not be verified through chart review. Inaccurate coding
could lead to under-reporting or false-positive results. In
order to circumvent some of these foreseeable
shortcomings, we grouped co-occurring birth defects into
systems. Both situations would make our results an
underestimate of the association that co-occurring birth
defects have on hearing loss.
CONCLUSIONS
1) About 31% of children with confirmed BHL and
confirmed UHL had a JCIH risk factor. Most commonly
reported risk factors were family history of hearing loss
and neonatal indicators in BHL, and craniofacial
anomalies and neonatal indicators in UHL.
2) About 1/3 of children with either BHL or UHL had a cooccurring birth defect. The most commonly associated
birth defect was a cardiovascular anomaly.
3) Over 50% of children with confirmed BHL or UHL and
a JCIH risk factor had a co-occurring birth defect. Most
common birth defect was cardiovascular.
4) It is important to recognize children at risk for hearing
loss and to perform confirmatory testing in a timely
manner despite distracting co-occurring birth defects.
5) The absence of JCIH risk and co-occurring birth defects
does not preclude the development of hearing loss.
6) Further studies are needed to define the etiology
underlying hearing loss and better define the risk
factor associations.
REFERENCES
1. Chapman D, Stampfel C, Bodurtha J, et al. Impact of co-occurring birth defects on the timing of newborn hearing screening and
diagnosis. Am J Audiol. 2011;20:132-139.
2. Borton S, Mauze E, Lieu JEC. Quality of life in children with unilateral hearing loss: a pilot study. Am J Audiol. 2010;19:61-72.
3. Dalzell L, Orlando M, MacDonald M, et al. The New York State universal newborn hearing screening demonstration project: ages of
hearing loss identification, hearing aid fitting, and enrollment in early intervention. Ear Hear. 2000;21:118-130.
4. Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics.
2007;120:898-921.
5. Lieu JEC. Speech-language and educational consequences of unilateral hearing loss in children. Archives of otolaryngology--head neck
surgery. 2004;130:524-530.
6. Bess FH, Tharpe AM. Unilateral hearing impairment in children. Pediatrics. 1984;74:206-216.
7. Bovo R, Martini A, Agnoletto M, et al. Auditory and academic performance of children with unilateral hearing loss. Scandinavian
audiology.Supplementum. 1988;30:71-74.
8. Kennedy C, McCann D, Campbell M, et al. Language ability after early detection of permanent childhood hearing impairment. N Engl J
Med. 2006;354:2131-2141.
9. Robinshaw HM. Early intervention for hearing impairment: differences in the timing of communicative and linguistic development. Br J
Audiol. 1995;29:315-334.
10. Sininger Y, Martinez A, Eisenberg L, Christensen E, Grimes A, Hu J. Newborn hearing screening speeds diagnosis and access to
intervention by 20-25 months. J Am Acad Audiol. 2009;20:49-57.
11. Yoshinaga Itano C, Sedey AL, Coulter DK, Mehl AL. Language of early- and later-identified children with hearing loss. Pediatrics.
1998;102:1161-1171.
12. White K. The current status of EHDI programs in the United States. Ment Retard Dev Disabil Res Rev. 2003;9:79-88.
13. White K. Early hearing detection and intervention programs: opportunities for genetic services. American journal of medical
genetics.Part A. 2004;130A:29-36.
14. World Health Organization. International Classification of Diseases, Ninth Revision, Clinical Modification. 1977;Geneva, Switzerland.
15. Kirby RS. Analytical resources for assessment of clinical genetics services in public health: current status and future prospects.
Teratology. 2000;61:9-16.
16. Bielecki I, Horbulewicz A, Wolan T. Risk factors associated with hearing loss in infants: an analysis of 5282 referred neonates. Int J
Pediatr Otorhinolaryngol. 2011;75:925-930.
17. Galambos R, Despland PA. The auditory brainstem response (ABR) evaluates risk factors for hearing loss in the newborn. Pediatr Res.
1980;14:159-163.
18. Martnez-Cruz C, Poblano A, Fernndez-Carrocera L. Risk factors associated with sensorineural hearing loss in infants at the neonatal
intensive care unit: 15-year experience at the National Institute of Perinatology (Mexico City). Arch Med Res. 2008;39:686-694.
19. van Dommelen P, Mohangoo AD, Verkerk PH, van der Ploeg,C P B., van Straaten HLM. Risk indicators for hearing loss in infants treated
in different neonatal intensive care units. Acta pædiatrica. 2010;99:344-349.
20. Bitsko R, Visser S, Schieve L, Ross D, Thurman D, Perou R. Unmet health care needs among CSHCN with neurologic conditions.
Pediatrics. 2009;124 Suppl 4:S343-S351.
21. Rosenberg D, Onufer C, Clark G, Wilkin T, Rankin K, Gupta K. The need for care coordination among children with special health care
needs in Illinois. Matern Child Health J. 2005;9:S41-S47.
22. Tippy K, Meyer K, Aronson R, Wall T. Characteristics of coordinated ongoing comprehensive care within a medical home in Maine.
Matern Child Health J. 2005;9:S13-S21.
23. Vohr B, Moore P, Tucker R. Impact of family health insurance and other environmental factors on universal hearing screen program
effectiveness. Journal of perinatology. 2002;22:380-385.
17. Martines F, Salvago P, Bentivegna D, Bartolone A, Dispenza F, Martiness E. Audiologic profile of infants at risk: Experience of a Western
Sicily tertiary care centre. Int J Pediatr Otorhinolaryngol. 2012;76;1285-1291.