The development of a central hearing impairment is described in a young girl with risk factors for hearing impairment that included mosaic Down syndrome, leukemia, and chemotherapy. This case is unusual in the prospective regularity with which hearing was assessed from birth. The diagnosis is electrical status epilepticus in slow wave sleep, a rare childhood disorder, which was associated with ...

Mastocytosis refers to a heterogeneous group of rare disorders characterized by an abnormal accumulation of mast cells in one or more organ systems. Cutaneous mastocytosis (CM) is the most frequent form in children and is characterized by hyperpigmented macules or papules symmetrically distributed over the trunk, and less so over the limbs, neck, and scalp. Two published articles have reported ...

This critical review examines the efficacy of hearing amplification on speech perception results when used as a habilitation method for children with Auditory Neuropathy Spectrum Disorder in eight studies. Study designs include 3 case series studies, 2 nonrandomized clinical trials, 2 case-control studies, and a retrospective single group study. Overall, the evidence provided by these studies i...

Background and Objective: Hearing loss as a sensory disorder is among the most common developmental disorders. Based on the universal neonatal screening results, the incidence of hearing deficit is 1–3 per 1000 live births in the well-baby nursery population and 2–4 in 100 newborns in the intensive care community. The aim of this study was to highlight the main risk factors for hearing loss bas...

Sjögren's syndrome (SS) is a cell-mediated immune disorder primarily affecting the exocrine glands and hearing loss may be the first otological manifestation of this autoimmune disease. In order to assess the degree of sensorineural hearing loss in SS, 22 female patients were examined by means of standard audiometric tests (pure-tone audiometry, acoustic reflexes and impedance testing) and usin...

Hearing loss is the most frequent sensory disorder in humans. Auditory hair cells (HCs) are postmitotic at late-embryonic differentiation and postnatal stages, and their damage is the major cause of hearing loss. There is no measurable HC regeneration in the mammalian cochlea, and the maintenance of cell function is crucial for preservation of hearing. Here we generated mice deficient in autoph...

background: previous studies have shown that interaural-time-difference (itd) training can improve localization ability. surprisingly little is, however, known about localization training vis-à-vis speech perception in noise based on interaural time difference in the envelope (itd env). we sought to investigate the reliability of an itd env-based training program in speech-in-noise perception a...

To assess patterns of hearing loss and asymmetry in major depressive disorder (MDD), pure-tone and brief-click audiometric thresholds were measured in 59 inpatients with MDD and 40 normal control subjects. For both tasks, patients had higher bilateral thresholds, with marked hearing loss for the highest pure-tone frequency. At lower frequencies, patients displayed significant asymmetry, with po...

Deficits in central auditory processing may occur in a variety of clinical conditions including traumatic brain injury, neurodegenerative disease, auditory neuropathy/dyssynchrony syndrome, neurological disorders associated with aging, and aphasia. Deficits in central auditory processing of a more subtle nature have also been studied extensively in neurodevelopmental disorders in children with ...

In 1991, Arnold Starr and colleagues described an 11-year-old child with the paradoxic findings of absent auditory brainstem responses in the presence of normal cochlear microphonics (CMs) and otoacoustic emissions (OAEs). The child demonstrated severely impaired speech understanding despite a mild loss of hearing sensitivity. Further evaluation showed impaired temporal processing as exhibited ...