Standardization of Three Familiar Sound Recognition Tests in Hearing and Deaf Adult Populations.

OBJECTIVE: Recognition of familiar noises is crucial for understanding and reacting appropriately to our auditory environment. Its improvement is one of the benefits expected after cochlear implantation. The aim of this study was to standardize three environmental sounds noise recognition tests and to illustrate their application to a population of deaf adults with cochlear implants. METHOD: Norms were established on a sample of 126 normal-hearing adults divided into 6 age groups. Three familiar sound recognition tests were used: 1) the Blue Mouse "First Familiar Sounds" (BM), 2) the UCL-IRSA test (TI), and 3) the Bernadette Piérart Familiar Sounds Test (TBF). These tests were also administered to 61 implanted deaf ears. RESULTS: We observed a significant effect of age on the accuracy scores of the TI and TBF tests for the hearing group and on the time scores of the TI and BM tests. Overall, the performance of the deaf participants was poorer and more variable than that of the hearing participants. CONCLUSION: We have three tests that can be used in practice to measure the performance of deaf people (with cochlear implants) at different stages of their pre- and post-implant rehabilitation.

Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports.

Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids.

Mechanisms Linking Olfactory Impairment and Risk of Mortality.

Olfaction is a sense involved in a complex set of tasks, influencing eating behavior, increasing awareness of environmental hazards and affecting social communication. Surprisingly, smell disorders are very frequent, especially in the elderly population. Several recent studies conducted mostly in older subjects have demonstrated a strong association between olfactory impairment and overall mortality risk, with anosmia being even more predictive of 5 years mortality risk than cardiovascular disease. Presently, the underlying pathophysiology linking olfactory impairment to mortality remains unknown and only putative mechanisms are suggested. This review aims to examine the link between olfactory impairment and mortality and to discuss existing ideas on underlying existing mechanisms including, (1) the effect of olfactory loss on nutrition, life-threatening situations and social interactions, (2) associated neurodegenerative diseases, (3) accelerated brain aging, and (4) reflection of general health status being reflected in olfactory function.