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.

several anonymous reviewers, and the many audiences who have heard parts of this work at conferences and colloquia.

A probabilistic phonotactic grammar based on the probabilities of the constituents contained in a dictionary of English was used to generate multisyllabic nonwords. English-speaking listeners evaluated the wordlikeness of these patterns. Wordlikeness ratings were higher for nonwords containing high-probability constituents and were also higher for nonwords with fewer syllables. Differences in the processing of these same nonwords that partially reflected their perceived wordlikeness were also found in a recognition memory task. Nonwords with higher probability constituents yielded better recognition memory performance, suggesting that participants were able to use their knowledge of frequently occurring lexical patterns to improve recognition. These results suggest that lexical patterns provide the foundation of an emergent phonological competence used to process nonwords in both linguistic and metalinguistic tasks.

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