Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
Abstract
Objective. To determine the type of hearing loss, incidence of
the lost to follow-up rate, and the time to diagnose sensorineural hearing loss (SNHL) in children with Down syndrome (DS) identified from a statewide database.
Study Design. Case series with chart review.
Setting. Pediatric referral center.
Subjects and Methods. Three hundred forty-four patients
with DS born in Utah between January 2002 and December
2006 were identified using the Utah Department of Healths
Newborn Hearing Screening database and birth defects
registry.
Results. Three hundred thirty-two patients were included in
the study. Eighty-seven infants (26.2%) did not pass their
newborn hearing screening (NBS). Thirty-three of these
children (37.9%) had a conductive hearing loss attributed to
serous otitis media. Five infants had SNHL; 3 children were
diagnosed with a mixed hearing loss (MHL). The average
time to diagnose a sensorineural hearing loss was 485 6
601 days. One child who passed his NBS was subsequently
found to have an SNHL. More than 43% of the newborns
with DS who passed their NBS developed a conductive
hearing loss requiring insertion of ventilation tubes. Eightyfour percent of newborns with DS who did not undergo
NBS did not have any apparent subsequent audiologic
testing.
Conclusion. Patients with DS present with a relatively high
incidence of conductive hearing loss, MHL, and SNHL and a
higher lost to follow-up rate compared to patients without
DS. The authors were not able to diagnose SNHL within
the 90-day period recommended by the Joint Committee
on Infant Hearing.
Keywords
newborn hearing screening, Down syndrome, sensorineural
hearing loss
Received September 20, 2010; revised August 25, 2011; accepted
September 9, 2011.
Otolaryngology
Head and Neck Surgery
146(1) 135140
American Academy of
OtolaryngologyHead and Neck
Surgery Foundation 2012
Reprints and permission:
sagepub.com/journalsPermissions.nav
DOI: 10.1177/0194599811425156
http://otojournal.org
1
Division of OtolaryngologyHead and Neck Surgery, University of Utah,
Salt Lake City, Utah, USA
2
University of Utah School of Medicine, Salt Lake City, Utah, USA
3
Utah Department of Health, Salt Lake City, Utah, USA
4
Audiology, Primary Childrens Medical Center, Salt Lake City, Utah, USA
This article was presented at the 2010 AAO-HNSF Annual Meeting & OTO
EXPO; September 26-29, 2010; Boston, Massachusetts.
Corresponding Author:
Albert H. Park, MD, Division of OtolaryngologyHead and Neck Surgery,
University of Utah, 50 North Medical Drive, 3C 120, Salt Lake City, UT
84132, USA
Email: pcapark@ihc.com
136
Newborns with DS
Alive
n = 332
DS Failed NBS
n = 87
Normal Hearing
n = 16
(18.4%)
Conductive HL
n = 33
(37.9%)
Sensorineural HL
n=5
(5.7%)
DS Passed NBS
n = 232
Mixed HL
n=3
(3.4%)
DS No NBS
n = 13
Indeterminant
n=8
(9.2%)
No Follow-up
n = 22 (25.3%)
Figure 1. Distribution of normal and hearing loss in the Down syndrome (DS) population. HL, hearing loss; NBS, newborn hearing
screening.
Methods
Subjects
Three hundred forty-four newborns with DS born in Utah
between 2002 and 2006 were identified using the Utah Birth
Defects Registry. Several outcome measures were determined
using the Utah Department of Health Hi*Track information
management system and the Intermountain Healthcare (IHC)
electronic medical record system. The Utah Newborn
Hearing Screening Program has been considered a very successful initiative, with more than 97% of the 53,080 newborns delivered being screened for 2010 with an overall
initial state pass rate of 95.8%. The Utah Department of
Health is responsible for data collection and management,
follow-up, and technical assistance for the state screening
program. It helped establish several audiological diagnostic
sites throughout the state that can provide complete diagnostic services to newborns and infants. The IHC is the largest
health care provider in the Intermountain West. More than 20
hospitals, including the only childrens hospital in Utah,
Primary Childrens Medical Center, are owned by the IHC. It
provides insurance to 19% of all Utah residents.
Outcome measures included newborn hearing screening
results, otoscopic examinations, behavioral audiometry, tympanometry/immitance, otoacoustic emissions, auditory brainstem response (ABR) testing thresholds, magnetic resonance
and computed tomography temporal bone imaging, time to
diagnosis of hearing loss, time to hearing loss treatment, and
surgical treatment. Institutional review board (IRB) approval
was obtained from the Utah Department of Health,
University of Utah, and Primary Childrens Medical Center.
The measures of hearing were categorized as normal hearing, conductive hearing loss, sensorineural hearing loss,
mixed hearing loss, indeterminant hearing result, and lost to
follow-up. Normal hearing results included behavioral thresholds at 500, 1000, 2000, and 4000 Hz better than 20 dB or
Results
Newborn Hearing Screening Data for DS and Non-DS
Newborns in Utah
Of 258,289 children born in the state of Utah between 2002
and 2006, 344 (0.13%) had Down syndrome, including 199
(57.8%) males and 146 females (42.2%). Twelve (3.5%)
died during the newborn period, and no additional data were
collected on these subjects. Three hundred thirty-two children had otoscopic and audiologic data available for analysis. Two hundred thirty-two newborns (69.9%) with DS
passed their newborn hearing screening (NBS; Figure 1).
This percentage is lower than the 92.8% of all infants
(234,545) who passed during this period. Eighty-seven
(26%) failed their screening, and 13 (3.9%) did not undergo
screening. The percentage of all infants not screened was
less than the DS group at 1.8% (4741 infants).
Park et al
137
Newborns with DS
Alive
n = 332
DS Failed NBS
n = 87
Normal Hearing
n = 124
(53.4%)
Conductive HL
n = 101
(43.5%)
DS Passed NBS
n = 232
DS No NBS
n = 13
Sensorineural HL
n=1
(0.4%)
Indeterminant
n=1
(0.4%)
No Follow-up
n=5
(2.2%)
Figure 2. Distribution of hearing loss in patients with Down syndrome (DS) who passed their newborn hearing screening (NBS). HL, hearing loss.
congenital heart problems may have delayed a timely diagnosis for this infant. Eight children (9.2%) had an indeterminant diagnosis. Twenty-two children (25.3%) were lost to
follow-up. Community otolaryngologists treated some of
these infants, and their audiograms could not be found in
the IHC electronic medical record system.
138
Newborns with DS
Alive
n = 332
DS Normal Hearing
n = 141
(42.5%)
DS Failed NBS
n = 87
DS Passed NBS
n = 232
DS No NBS
n = 13
Conductive HL
n = 135
(88.2%)
DS Hearing Loss
n = 153
(46.1%)
Sensorineural HL
n=6
(3.9%)
DS no follow-up
n = 38
(11.4%)
Mixed HL
n=3
(2.0%)
Indeterminant
n=9
(5.9%)
Conductive HL
n=4
(30.8%)
No Follow-up
n=8
(61.5%)
Discussion
Of the 332 children with DS evaluated, 42.5% had normal
hearing, 46.1% had a hearing loss, and 11.4% were lost to
follow-up. SNHL was detected in 3.9% of hearing loss
patients; a mixed hearing loss was less common at 2.0%.
Our reported rate of conductive hearing loss is similar to a
number of prior studies.6,11-14 Our reported rate of SNHL is
slightly lower than Davies 4% rate.14
More than 96% of all infants with DS born in Utah
between 2002 and 2006 underwent NBS. It is not clear why
13 DS infants did not undergo hearing screening. For other
Park et al
139
Disclosures
Competing interests: None.
Sponsorships: None.
Funding source: None.
References
1. Blaser S, Propst EJ, Martin D, et al. Inner ear dysplasia is
common in children with Down syndrome (trisomy 21).
Laryngoscope. 2006;116:2113-2119.
2. Shott SR. Down syndrome: common otolaryngologic manifestations. Am J Med Genet C Semin Med Genet. 2006;142:131-140.
3. Strome M. Downs syndrome: a modern otorhinolaryngological perspective. Laryngoscope. 1981;91:1581-1594.
4. Shibahara Y, Sando I. Congenital anomalies of the eustachian
tube in Down syndrome: histopathologic case report. Ann Otol
Rhinol Laryngol. 1989;98:543-547.
140