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Clinical and Audiological Features in Auditory Neuropathy
Colm Madden, FRCSI;
Michael Rutter, FRCS;
Lisa Hilbert, MA;
John H. Greinwald, Jr, MD;
Daniel I. Choo, MD
Arch Otolaryngol Head Neck Surg. 2002;128:1026-1030.
Objective To medically and audiologically characterize a population of children
diagnosed as having auditory neuropathy (AN).
Study Design Retrospective medical chart review.
Setting/Subjects We identified 22 patients from a pediatric otology clinic in a tertiary
care pediatric hospital setting.
Results A genetic factor in AN is suggested by our identification of 3 families
with 2 affected children and 2 other children with family histories that were
positive for hearing loss. Clinical features common among our population included
a history of hyperbilirubinemia (n = 11 [50%]), prematurity (n = 10 [45%]),
ototoxic drug exposure (n = 9 [41%]), family history of hearing loss (n =
8 [36%]), neonatal ventilator dependence (n = 8 [36%]), and cerebral palsy
(n = 2 [9%]). Full clinical and audiological data were available for 18 of
the 22 children, including otoacoustic emissions, auditory brainstem responses
with cochlear microphonics, and age-appropriate audiometric findings. Significantly,
9 of these 18 patients showed improvement in behavioral thresholds over time,
indicating that a subset of children with AN may recover useful hearing levels.
Also significant was the success of cochlear implantation in 4 children.
Conclusions Management of AN in children requires serial clinical and audiometric
evaluations, with a prominent role for behavioral testing. Prematurity, genetics,
and hyperbilirubinemia appear to be significant factors in the development
of AN; hyperbilirubinemia can be associated with spontaneous improvement of
hearing thresholds. For those children not benefiting from amplification or
FM systems, cochlear implantation remains a potentially successful method
of habilitation.
From the Center for Hearing and Deafness Research, Departments of Pediatric
Otolaryngology (Drs Madden, Rutter, Greinwald, and Choo) and Audiology (Ms
Hilbert), Children's Hospital Medical Center, Cincinnati, Ohio.
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