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Spondyloepiphyseal dysplasia is a disorder characterized by abnormalities of growth. Previous studies of this disorder have identified a significant incidence of associated hearing loss. Hearing loss has been reported to occur in 25 to 30% of affected patients. To date, all reports of associated hearing loss have indicated the presence of a sensorineural component. In this article, we report the case of a child who was diagnosed with spondyloepiphyseal dysplasia congenita and who was found to have a significant conductive hearing loss with a Carhart's notch, indicating the likely presence of stapes footplate fixation. We also review the diagnosis of this condition and the literature associated with hearing loss as it occurs with this disorder. [ABSTRACT FROM AUTHOR]

Recent work suggests that hair cells are not the most vulnerable elements in the inner ear; rather, it is the synapses between hair cells and cochlear nerve terminals that degenerate first in the aging or noise-exposed ear. This primary neural degeneration does not affect hearing thresholds, but likely contributes to problems understanding speech in difficult listening environments, and may be important in the generation of tinnitus and/or hyperacusis. To look for signs of cochlear synaptopathy in humans, we recruited college students and divided them into low-risk and high-risk groups based on self-report of noise exposure and use of hearing protection. Cochlear function was assessed by otoacoustic emissions and click-evoked electrocochleography; hearing was assessed by behavioral audiometry and word recognition with or without noise or time compression and reverberation. Both groups had normal thresholds at standard audiometric frequencies, however, the high-risk group showed significant threshold elevation at high frequencies (10–16 kHz), consistent with early stages of noise damage. Electrocochleography showed a significant difference in the ratio between the waveform peaks generated by hair cells (Summating Potential; SP) vs. cochlear neurons (Action Potential; AP), i.e. the SP/AP ratio, consistent with selective neural loss. The high-risk group also showed significantly poorer performance on word recognition in noise or with time compression and reverberation, and reported heightened reactions to sound consistent with hyperacusis. These results suggest that the SP/AP ratio may be useful in the diagnosis of “hidden hearing loss” and that, as suggested by animal models, the noise-induced loss of cochlear nerve synapses leads to deficits in hearing abilities in difficult listening situations, despite the presence of normal thresholds at standard audiometric frequencies. [ABSTRACT FROM AUTHOR]

Purpose: To estimate real-environment iPod listening levels for listeners in 4 environments to gain insight into whether average listeners receive dosages exceeding occupational noise exposure guidelines as a result of their listening habits. Method: The earbud outputs of iPods were connected directly into the inputs of a digital recorder to make recordings of listening levels. These recordings were used to estimate listening levels using reference recordings made in a real ear. Recordings were made in 4 environments with a wide range of background noises: (a) a library, (b) a student center, (c) busy streets, and (d) the subway. Results: None of the 64 listeners were estimated to exceed allowable occupational dosages, with a maximum dose of 7.57% based on Occupational Safety and Health Administration (OSHA; 1998) methods and 10.83% based on National Institute for Occupational Safety and Health (NIOSH; 1998) methods. Conclusions: All of the listeners surveyed were exposed to dosages well below OSHA and NIOSH occupational regulations. Although this does not guarantee individual safety, the results do not support the widespread concern regarding the safety of common iPod usage. However, measurements made in this study agree with the finding that iPod output can exceed safe levels and further support recommendations to monitor and limit listening volume and listening duration. [ABSTRACT FROM AUTHOR]