Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder).

Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these patients, and 49 patients tried cochlear implants. Approximately 15% reported some benefit from hearing aids for language learning, while improvement in speech comprehension and language acquisition was reported in 85% of patients who were implanted. Approximately 5% (13/260) of the total population developed normal speech and language without intervention. Patients were diagnosed at our laboratory (n=66) or referred from other sites (n=194), and all showed absent/grossly abnormal auditory brainstem responses (ABR), often 'ringing' cochlear microphonics, and the presence or history of otoacoustic emissions. Etiologies and co-existing conditions included genetic (n=41), peripheral neuropathies (n=20), perinatal jaundice and/or anoxia and/or prematurity (n=74). These patients comprise 10% or more of hearing impaired patients; their language acquisition trajectories are generally unpredictable from their audiograms.

Absent or elevated middle ear muscle reflexes in the presence of normal otoacoustic emissions: a universal finding in 136 cases of auditory neuropathy/dys-synchrony.

We extracted a subpopulation of 136 patients (from our database of 257 AN/AD subjects) in whom middle ear muscle reflexes had been measured. None showed normal reflexes at all frequencies tested. Only three subjects showed any reflexes at 95 dB HL or below, but never at both 1 and 2 kHz in both ears whether ipsilaterally or contralaterally elicited. All the other reflex measures in these remaining 133 patients were either absent or observed above 100 dB HL, which is incongruous with their normal otoacoustic emissions throughout the frequency bands. Therefore, we urge colleagues to test ipsilateral middle ear muscle reflex at least at 1 kHz and 2 kHz in any perinatal hearing screening that depends solely on otoacoustic emissions. If the emissions are present and the reflexes are absent or elevated, an ABR may be required to properly intervene, because the management of AN/AD patients often differs drastically from what the behavioral audiogram or the ABR suggest.