This study describes 5 infants who were diagnosed with auditory neuropathy (AN) associated with severe to profound neural hearing loss shortly after birth. However, on repetition of the tests 7–12 months later, all infants showed full or partial recovery. The follow-up electrophysiological patterns were characterized by the appearance of wave I, followed by wave III and V, reflecting synchronization of auditory pathways and improvement in auditory nerve function. Suspected causative or contributory factors were neonatal hyperbilirubinemia, hypoxia, ischemia, and central nervous system immaturity, alone or in combination. These findings indicate that lack of an auditory brain stem response does not necessarily mean no hearing and that the situation where AN exists can improve. Thus, clinicians should be made aware that although cochlear implants may yield better auditory performance when applied early, they should be considered a therapeutic option only after repeated measures have proved persistent AN, and no child should be considered for an implant until a behavioral measure of hearing has been obtained.

The objective of the study was to investigate the prevalence of congenital and early-onset hearing loss, and the influence of the known risk factors for hearing loss on infants in Jordan and Israel. Subjects were a total of nearly 17000 infants from both countries, including infants with and without risk factors for hearing loss. The hearing screening protocol included distortion product otoacoustic emission, followed in case of repeated OAE referral or high risk (HR) infant by diagnostic auditory brainstem responses. The results indicate that the prevalence and severity of hearing loss amongst Jordanian infants (1.37%) is remarkably higher as compared to the Israeli infants (0.48%). The overall prevalence of bilateral SNHL was seven times more in the Jordanian infants, 18 times in non-risk, and three times in the HR infants relative to the Israeli infants. Risk factors including family history, hyperbilirubinemia, bacterial meningitis, and associated syndromes were more prevalent amongst Jordanian infants. This unique study underscores the importance of sharing and exchanging information to create empirical data to guide health-care providers in adapting protocols to the local constraints in developing countries. Sumario Objetivo: Investigar la prevalencia de hipoacusias congénitas o de inicio temprano y la influencia de los factores de riesgo conocidos para sordera en niños de Jordania e Israel. Sujetos y materiales: Cerca de 17.000 infantes de ambos países participaron en el estudio, incluyendo niños con factores de riesgo y sin ellos. El protocolo de evaluación auditiva incluyó Emisiones Otoacústicas por Productos de Distorsión (DOPAE), seguido, en el caso de referencias repetidas de OAE, por Respuestas Auditivas del Tallo Cerebral (ABR). Resultados: La prevalencia y la severidad de los trastornos auditivos en niños de Jordania (1.37%) es marcadamente más alto en comparación con los niños israelitas (0.48%), especialmente en el caso de la SNHL bilateral, donde la prevalencia global en los niños jordanos era 7 veces mayor, 18 veces en los niños sin riesgo y 3 veces mayor en los infantes con HR, comparado con los israelitas. Los factores de riesgo, que incluían historia familiar, hiperbilirrubinemia, meningitis bacteriana y síndromes asociados, fueron más prevalentes entre los niños jordanos. Este estudio singular subraya la importancia de compartir e intercambiar información, para crear datos empíricos que guíen a los proveedores de salud en la adaptación de los protocolos a las limitaciones locales de cada país.

Article Dysfunction of the Auditory Efferent System in Patients with Traumatic Brain Injuries with Tinnitus and Hyperacusis was published on September 1, 2005 in the journal Journal of Basic and Clinical Physiology and Pharmacology (volume 16, issue 2-3).

Eleven infants who were fed a thiamine-deficient formula for a mean of 3 months were evaluated for immediate and long-term auditory abnormalities. At presentation, 8 infants had auditory neuropathy spectrum disorder (ANSD), which resolved with supplementary thiamine in 5 children, was permanent in 2 children, and deteriorated in 1 patient who died at the age of 7 years. An additional patient had an auditory pattern corresponding to that of auditory neuropathy of brain stem origin. The 2 remaining patients had unilateral cochlear hearing loss. Six to 8 years later, all patients with transient ANSD had normal audiograms, 2 patients had unilateral cochlear hearing loss, and the rest had neural hearing loss. All survivors had a language developmental delay and impaired speech intelligibility of varying degrees, especially in the presence of background noise. Thiamine is crucial for normal auditory development and function, and its deficiency may be considered an acquired metabolic cause of ANSD in infants.