They coined the term auditory neuropathy. Starr et al 1 described 10 patients, 5 adults and 5 children, who demonstrated these findings and no other auditory diagnosis on results of clinical, audiological, or radiographic studies. Clinically, AN is defined as (1) hearing loss, usually bilateral, of any degree (2) normal outer hair cell function as evidenced by the presence of OAEs and/or CM (3) abnormal evoked potentials beginning with wave I of the ABR (4) poor speech perception and (5) absent acoustic reflexes to the ipsilateral and contralateral tones at a 110-dB hearing level. For those children not benefiting from amplification or FM systems, cochlear implantation remains a potentially successful method of habilitation.ĪUDITORY neuropathy (AN) is a hearing disorder characterized by an absent or severely abnormal auditory brainstem response (ABR), with preservation of the cochlear microphonics (CM) and otoacoustic emissions (OAEs). Prematurity, genetics, and hyperbilirubinemia appear to be significant factors in the development of AN hyperbilirubinemia can be associated with spontaneous improvement of hearing thresholds. Also significant was the success of cochlear implantation in 4 children.Ĭonclusions Management of AN in children requires serial clinical and audiometric evaluations, with a prominent role for behavioral testing. 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. 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. Clinical features common among our population included a history of hyperbilirubinemia (n = 11 ), prematurity (n = 10 ), ototoxic drug exposure (n = 9 ), family history of hearing loss (n = 8 ), neonatal ventilator dependence (n = 8 ), and cerebral palsy (n = 2 ). 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. Setting/Subjects We identified 22 patients from a pediatric otology clinic in a tertiary care pediatric hospital setting. Study Design Retrospective medical chart review. Objective To medically and audiologically characterize a population of children diagnosed as having auditory neuropathy (AN). Shared Decision Making and Communication. Scientific Discovery and the Future of Medicine.Health Care Economics, Insurance, Payment.Clinical Implications of Basic Neuroscience.Challenges in Clinical Electrocardiography.
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