Gordon 2015
Gordon 2015
Gordon 2015
Deafness affects ∼2 in 1000 children and is one of the most common abstract
congenital impairments. Permanent hearing loss can be treated by fitting
hearing aids. More severe to profound deafness is an indication for cochlear
implantation. Although newborn hearing screening programs have increased a
Archie’s Cochlear Implant Laboratory, The Hospital for Sick
the identification of asymmetric hearing loss, parents and caregivers of Children, Department of Otolaryngology–Head and Neck
Surgery, bUniversity of Toronto, Toronto, Canada; cHearing,
children with single-sided deafness are often hesitant to pursue therapy for Speech, and Language Center, Sheba Medical Center, Tel
the deaf ear. Delayed intervention has consequences for recovery of hearing. It Hashomer, dDepartment of Communication Disorders,
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv,
has long been reported that asymmetric hearing loss/single-sided deafness Israel; and eCluster of Excellence Hearing4all, Institute of
compromises speech and language development and educational outcomes AudioNeuroTechnology, Hannover, Germany; fDepartment of
Experimental Otology, ENT Clinics, School of Medicine,
in children. Recent studies in animal models of deafness and in children Hannover Medical University, Hannover, Germany; and
consistently show evidence of an “aural preference syndrome” in which single- g
School of Behavioral and Brain Sciences, The University of
sided deafness in early childhood reorganizes the developing auditory Texas at Dallas, Dallas, Texas
pathways toward the hearing ear, with weaker central representation of the Drs Gordon, Henkin, and Kral co-proposed the article
deaf ear. Delayed therapy consequently compromises benefit for the deaf ear, to the editorial office, cowrote the manuscript, and
revised the manuscript; and all authors approved the
with slow rates of improvement measured over time. Therefore, asymmetric final manuscript as submitted.
hearing needs early identification and intervention. Providing early effective
www.pediatrics.org/cgi/doi/10.1542/peds.2014-3520
stimulation in both ears through appropriate fitting of auditory prostheses,
DOI: 10.1542/peds.2014-3520
including hearing aids and cochlear implants, within a sensitive period in
development has a cardinal role for securing the function of the impaired Accepted for publication Feb 9, 2015
ear and for restoring binaural/spatial hearing. The impacts of asymmetric Address correspondence to Karen Gordon,
Department of Otolaryngology–Head and Neck
hearing loss on the developing auditory system and on spoken language Surgery, Archie’s Cochlear Implant Laboratory, The
development have often been underestimated. Thus, the traditional minimalist Hospital for Sick Children, Room 6D08, 555
approach to clinical management aimed at 1 functional ear should be modified University Ave, Toronto, ON, Canada M5G 1X8. E-mail:
[email protected]
on the basis of current evidence.
PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online,
1098-4275).
Copyright © 2015 by the American Academy of
Deafness is one of the most common clinical implications. Screening Pediatrics
congenital impairments.1,2 Newborn programs will also miss children who
FINANCIAL DISCLOSURE: The authors have indicated
hearing screening programs, acquire deafness in 1 ear from
they have no financial relationships relevant to this
implemented in many countries, have infection, trauma, or worsening of article to disclose.
decreased the age at diagnosis of preexisting hearing loss.3–6 Acquired
FUNDING: Dr Kral has been supported by the
hearing loss. When hearing loss occurs unilateral deafness can go unidentified Deutsche Forschungsgemeinschaft (Cluster of
in only 1 ear, the screening result until educational, social, or other Excellence Hearing4all). Dr Gordon’s work has been
may be overlooked or dismissed as impairments push families and supported by the Canadian Institutes of Health
unimportant, particularly when caregivers to seek medical consult. Research and the SickKids Foundation, including the
Bastable-Potts Clinician-Scientist Award in Hearing
hearing in the opposite ear is normal Because the prevalence of permanent
Impairment. Dr Henkin’s work has been supported
(unilateral or single-sided deafness). unilateral hearing loss in neonates is by the Shauder grant, Sackler Faculty of Medicine,
The consequence will be a failure to reported to vary from 0.45 to 2.7 in Tel Aviv University, Israel.
intervene until long after major 10007,8 and estimates in school-aged POTENTIAL CONFLICT OF INTEREST: The authors have
developmental effects have set in, children range from 30 to 56 in indicated they have no potential conflicts of interest
which causes significant negative 1000,9,10 awareness of medical to disclose.
cortex,95 was softened to “aural compared with the left ear.103,104,108 auditory brainstem, the first point of
preference.”91 More extensive effects, including binaural integration in the ascending
Supporting evidence for recruitment of additional cortical pathways, is already affected.
a developmental change toward areas/networks, were observed in Brainstem responses rapidly change
abnormal cortical aural preference is children with unilateral hearing over the first year of unilateral CI use
available in humans with asymmetric loss,109–113 in line with the increasing in children with early-onset
hearing who did not receive effects found in younger cats90,91 deafness.118,119 When the opposite
treatment. Various imaging and the language, cognitive, and (second) ear was implanted after this
techniques have been used, including educational challenges these children period (.1.5 years), the brainstem
electroencephalography and are reported to have (discussed in responses from this ear remained
functional MRI. A number of studies “Current State of the Problem” abnormally prolonged despite up to 3
documented a stronger than normal section above). years of bilateral implant use
representation of the hearing ear at Further support for abnormal aural (Fig 3120,121). By contrast, bilateral
the cortex ipsilateral to the hearing preference comes from children who implantation with minimal or no
ear in adult onset of single-sided receive bilateral CIs sequentially. delay in early development promoted
deafness.96–101 This change resulted Although expected cortical symmetric maturation of the
in a more symmetric activation of electrophysiologic responses were responses for both ears.120 Cortical
both auditory cortices from the measured from the first implanted responses of sequentially implanted
hearing ear.101–107 The differences ear, responses from the second, later- children revealed a reduction in
from normal were larger when the implanted ear remained normal contralateral aural preference,
loss occurred in the right ear abnormal.114–117 The developing consistent with the animal studies
FIGURE 3
A, EABR wave eV evoked by the CI-1 (right implant) and CI-2 (left implant) are at similar latencies to the BD in a child receiving both implants
simultaneously. B, A period of unilateral CI use before bilateral cochlear implantation reduces wave eV latency evoked by CI-1 and the response from
CI-2 remains delayed despite 2 years of bilateral CI use. The BD is delayed relative to the CI-1–evoked wave eV. C, CI-2–evoked wave eVs are
significantly prolonged relative to CI-1 when the period of unilateral CI use exceeds 2 years. Plots were reprinted from ref 120, Fig 3. BD, binaural
difference component; EABR, electrically evoked auditory brainstem response; eII, the second wave of the EABR; eIII, the third wave of the EABR; eV, the
fifth wave of the EABR.
in the auditory cortices of children as adolescents suggest that there are 2. asymmetric speech understanding
implanted sequentially with a long continued difficulties in processing in each ear that is resistant to
delay.122 Although these children input from the second-treated ear. treatment (ie, persisting after
learned to detect large changes in Overall, the data indicate that an early compensation of the initial asym-
binaural timing cues after long period of monaural hearing as brief as metry); and
periods of bilateral implant 1.5 years has long-lasting 3. deficits in binaural hearing, in-
experience, they continued to judge consequences. cluding sound localization, re-
input as coming from the side of their sistant to therapy of the weaker
first implant more often than children ear.
receiving bilateral implants RECOMMENDATIONS FOR
simultaneously.134 Furthermore, IDENTIFICATION AND TREATMENT OF Awareness of the problem is
ASYMMETRIC HEARING LOSS important. On the basis of recent
although speech perception was
gained in the weaker ear, the progress Combining the available evidence, evidence, a more aggressive approach
was slow and did not match the we propose the existence of an to treating asymmetric hearing loss in
stronger ear even after 5 to 9 years of “aural preference syndrome,” children appears to be justified, with
implant use.58 Poor speech characterized by a combination of the following objectives:
perception55 together with absent following factors: 1. early identification of hearing loss
cortical binaural interaction135 in 1. asymmetric hearing during that is more pronounced in 1 ear
children receiving the second implant development; than the other;
CAR SIGNALS: My wife and I have been looking at new cars during the past week. We
do not own a car made in this millennium, and were looking for a reliable all-wheel
drive car that was safe in the snow. We were fairly open-minded about what we
wanted, so we looked at many different cars from diverse manufacturers. We also
got lots of interesting advice from friends and family. My daughter scoffed when I
told her I wanted a specific car in red, retorting that the particular model was clearly
not sporty, was designed for old men like me, and that I should stick with a basic color.
For a brief time, my wife decided that she wanted a white car because it would send
a message (or at least until I told her that white is the most common color of new cars
sold in the US and the world). It seems that what type of car one purchases and even
the color can say a lot about the buyer.
As reported in The New York Times (Real Estate: April 9, 2015), wealthy car drivers
have very particular buying preferences which vary by zip code. For example, in
Beverly Hills, luxury car buyers prefer Mercedes, while in the San Francisco Bay area
they prefer the Tesla Model S, and in New York City they prefer the BMW X5. Surveys
completed by more than 300,000 car buyers, between September 2013 and August
2014, showed that car choice correlates with a myriad of personal characteristics
and interests. It turns out that bridge and poker are popular pursuits among
Mercedes-Benz S Class buyers, while New York BMW X5 buyers enjoy bowling more
than those who buy other luxury brand cars.
I certainly do not plan to fill out a survey after we finally purchase a car, but if I do,
I am pretty sure that the most important reason that we purchased the car is because
my wife liked it the most.
Noted by WVR, MD
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References This article cites 144 articles, 5 of which you can access for free at:
http://pediatrics.aappublications.org/content/136/1/141#BIBL
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