Hearing Loss in Children

Preface
The Child with Hearing Loss

Bradley W. Kesser, MD Margaret A. Kenna, MD, MPH

Editors

Perhaps no other field in otolaryngology has expanded as rapidly as the evaluation and
management of hearing loss in children. From safer and higher-resolution imaging
techniques to more focused genetic analyses, the old ratio of congenital sensorineural
hearing loss, 25-25-50, idiopathic-acquired-genetic, is rapidly changing as many idiopathic causes for hearing loss are being elucidated, and many specific causes of
genetic hearing loss have been discovered. With the widespread mandate of newborn
hearing screening in every state, children with hearing loss are being identified earlier
and, ideally, diagnosed by 3 months of age with intervention by 6 months of age. However, diagnosing the cause of hearing loss in the young child remains challenging, and
clinicians are left to ponder who should be tested, when, and what tests to order. Genotype-phenotype correlations and techniques from linkage algorithms to whole
genome screening have mapped deafness and other associated traits to specific chromosomes in the human genome, allowing researchers and clinicians to identify many
of the causes of both syndromic and nonsyndromic pediatric sensorineural hearing
loss. Interestingly, the genetic analysis of hearing loss has shed light on the structure,
physiology, function, and development of the cochlea and other inner ear structures.
With over 100 specific genes identified whose mutation causes hearing loss (with likely
more by the time this is published!), keeping up with the science is quite challenging for
clinicians (see the Hereditary Hearing Loss homepage at hereditaryhearingloss.org).
Whether to order computed tomography (CT) or magnetic resonance imaging (MRI)
is often a question asked at otolaryngology meetings in the evaluation of children with
hearing loss. The answer, of course, depends on the clinical question being asked and
the nature of the hearing loss. One article in this issue comprehensively discusses imaging for pediatric hearing loss, the ideal study, when to pursue it, and what to look for.
Cytomegalovirus (CMV) is now recognized as the most common viral cause of
congenital hearing loss and a likely cause of progressive hearing loss. Although the
optimal diagnostic test as well as management of CMV remains in flux, this common

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Preface

virus needs to be recognized as an important part of the newborn hearing loss

diagnostic effort.
Management options for children with hearing loss include any number of interventions from individualized education programs to surgery to hearing aids (conventional
and bone conducting), cochlear implantation, and even brain stem implants. Several
articles address these options for children with both conductive and sensorineural
hearing loss, including an entire article addressing the child with unilateral hearing
loss, an often difficult clinical scenario to assess and manage.
In summary, we asked the worlds leading pediatric otologic clinicians and scientists to present the most up-to-date, evidence-based recommendations to guide cliniciansotolaryngologists, pediatricians, audiologists, speech-language therapists,
and other hearing health care professionalsin the workup and habilitation of these
children. And what is coming down the road? Better cochlear implant technology
and coding strategies, auditory brainstem implant technology, and finally, gene therapy or stem cell therapy for sensorineural hearing loss are all exciting prospects for the
near and distant future.
We also cannot thank enough the authors who have generously donated their time
and expertise to make this issue of Otolaryngologic Clinics of North America a practical, informative, exciting, and relevant addition to the ever-expanding literature on
hearing loss in children, and who provide outstanding care for these children every
day.
Bradley W. Kesser, MD
Department of Otolaryngology-Head
and Neck Surgery
University of Virginia School of Medicine
Box 800713
Charlottesville, VA 22908-0713, USA
Margaret A. Kenna, MD, MPH
Department of Otolaryngology
and Communication Enhancement
Boston Childrens Hospital
300 Longwood Avenue, BCH 3129
Boston, MA 02115, USA
E-mail addresses:
Bwk2n@virginia.edu (B.W. Kesser)
margaret.kenna@childrens.harvard.edu (M.A. Kenna)