Multi-site diagnosis and management of 260 patients with Auditory Neuropathy/Dys-synchrony (Auditory Neuropathy Spectrum Disorder^*)

Abstract

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.

Sumario

Se resumen los resultados de las pruebas y los datos del tratamiento de 260 pacientes con diagnóstico de Espectro de desórdenes de la Neuropatía Auditiva (ANSD). En 85 de estos pacientes se probó el uso de auxiliares auditivos y 49 pacientes recibieron un implante coclear. Aproximadamente 15% reportaron algún beneficio con los auxiliares auditivos para la adquisición del lenguaje mientras que el 85% de los que recibieron un implante reportaron una mejoría en la comprensión y la adquisición del lenguaje. Aproximadamente 5% (13/260) de la población total desarrolló lenguaje normal sin intervención. Los paciente fueron diagnosticados en nuestro laboratorio (n=66) o referidos de algún otro lado (n=194) y todos mostraron ausencia o anormalidad importante de los potenciales evocados (ABR), frecuentemente con una microfónica coclear “timbrante” y con presencia o historia de emisiones otoacústicas. La etiología o las condiciones co-existentes incluidas fueron: genéticas (n=41), neuropatías periféricas (n=20), ictericia perinatal y/o anoxia y/o prematurez (n=74). Estos pacientes representan 10% o más de los pacientes con hipoacusia; su trayectoria en el proceso de adquisición del lenguaje es generalmente impredecible a partir de sus audiogramas.

Key Words:

• Auditory neuropathy
• Auditory dys-synchrony
• Auditory neuropathy spectrum disorder
• Diagnosis, management
• Auditory brainstem response
• Cochlear microphonics
• Otoacoustic emissions
• Hearing aids
• Cochlear implants
• Cued speech
• Middle-ear muscle reflexes

Acknowledgements

We appreciate the help of grants and support from: NIH NIDCD, BMDR 1549; the Oberkotter, Marriott, Lions, and Deafness Research Foundations; Kam's Fund for Hearing Research as part of the LSUHSC Foundation; The Kenneth and Frances Barnes Bullington Professorship to Dr. Berlin; and help from the donors who established the Charles I. Berlin PhD Chair in Molecular and Genetic Hearing Science. This Chair was most recently occupied at LSUHSC by Dr. Bronya JB Keats. We appreciate the patients and their families who came for testing and stayed for coffee. Data were collected and shared by many audiologists too numerous to mention, but among the most diligent and steadfast were Harriet L. Berlin, Shanda Brashears Morlet, and Melanie Thibodeaux. Thanks are due to Annette Hurley, Ph.D., and Robert Turner, Ph.D., for valuable editorial suggestions. Administrative support through the many years of data collection came from Sue Mason, Nancie Roark, and Frances Billes. At Vanderbilt University, Andrea Hillock, Christopher Spankovich, Rita Anelli, and Christine Williams assisted with recent data review and compilation.

Declaration of interest: We have no financial or related ownership of Parents List Serve on yahoo.com