Tumors of the pineal region typically present with symptoms and signs of mass effect and increased intracranial pressure. However, although rare and can be overlooked, hearing impairment is a potential clinical finding in these cases. The authors describe a 24-year-old male who presented to the emergency room complaining of bilateral hearing impairment. Brain computed tomography showed a pineal region tumor. Histopathological examination demonstrated features consistent with germinoma. This case reports a rare presentation rarely seen in the literature and in practice as evident by the conducted literature review. Therefore, we highlight the importance of considering hearing impairment as a presenting symptom of pineal region tumors since prompt recognition and intervention, as demonstrated in this case, can lead to successful outcomes.

Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant neurodegenerative disorder characterized by progressive cerebellar ataxia associated with retinal degeneration. The disease is rare in Japan, and this is the first full description of clinicopathological findings in a Japanese autopsy case of genetically confirmed SCA7 having 49 cytosine-adenine-guanine (CAG) trinucleotide repeats in the ataxin 7 gene. A 34-year-old Japanese man with no family history of clinically apparent neurodegenerative diseases presented with gait disturbance, gradually followed by truncal instability with progressive visual loss by the age of 42 years. He became wheelchair-dependent by 51 years old, neurologically exhibiting cerebellar ataxia, slow eye movement, slurred and scanning speech, lower limb spasticity, hyperreflexia, action-related slowly torsional dystonic movements in the trunk and limbs, diminished vibratory sensation in the lower limbs, auditory impairment, and macular degeneration. Brain magnetic resonance imaging revealed atrophy of the brainstem and cerebellum. He died of pneumonia at age 60 with a 26-year clinical duration of disease. Postmortem neuropathological examination revealed pronounced atrophy of the spinal cord, brainstem, cerebellum, external globus pallidus (GP), and subthalamic nucleus, microscopically showing neuronal cell loss and fibrillary astrogliosis with polyglutamine-immunoreactive neuronal nuclei and/or neuronal nuclear inclusions (NNIs). Degeneration was also accentuated in the oculomotor system, auditory and visual pathways, upper and lower motor neurons, and somatosensory system, including the spinal dorsal root ganglia. There was a weak negative correlation between the frequency of nuclear polyglutamine-positive neurons and the extent of neuronal cell loss. Clinicopathological features in the present case suggest that neurological symptoms, such as oculomotor, auditory, visual, and sensory impairments, are attributable to degeneration in their respective projection systems affected by SCA7 pathomechanisms and that dystonic movement is related to more significant degeneration in the external than internal GP.

We report a case in which we quantified the maturation of the central auditory pathway in children with hearing loss and associated disabilities; the audiological intervention was performed using the BAHA softband. The hearing aid was applied according to the international clinical protocols. The presented case reveals the importance of using the P1 CAEP biomarker in clinical practice along with a neuropsychological evaluation to assess the maturation of the central auditory pathways and to objectively quantify the results of auditory rehabilitation in children with hearing loss and associated disabilities.

Substrate gleaning is a foraging strategy in which bats use a mixture of echolocation, prey-generated sounds, and vision to localize and hunt surface-dwelling prey. Many substrate-gleaning species depend primarily on prey-generated noise to hunt. Use of echolocation is limited to general orientation and obstacle avoidance. This foraging strategy involves a different set of selective pressures on morphology, behavior, and auditory system organization of bats compared to the use of echolocation for both hunting and navigation. Gleaning likely evolved to hunt in cluttered environments and/or as a counterstrategy to reduce detection by eared prey. Gleaning bats simultaneously receive streams of echoes from obstacles and prey-generated noise, and have to segregate these acoustic streams to attend to one or both. Not only do these bats have to be exquisitely sensitive to the soft, low frequency sounds produced by walking/rustling prey, they also have to precisely localize these sounds. Gleaners typically use low intensity echolocation calls. Such stealth echolocation requires a nervous system that is attuned to low intensity sound processing. In addition, landing on the ground to hunt may bring gleaners in close proximity to venomous prey. In fact, at least 2 gleaning bat species are known to hunt highly venomous scorpions. While a number of studies have addressed adaptations for echolocation in bats that hunt in the air, very little is known about the morphological, behavioral, and neural specializations for gleaning in bats. This review highlights the novel insights gleaning bats provide into bat evolution, particularly auditory pathway organization and ion channel structure/function relationships. Gleaning bats are found in multiple families, suggesting convergent evolution of specializations for gleaning as a foraging strategy. However, most of this review is based on recent work on a single species - the pallid bat (Antrozous palli dus) - symptomatic of the fact that more comparative work is needed to identify the mechanisms that facilitate gleaning behavior.

The relevance of left dorsal and ventral fiber pathways for syntactic and semantic comprehension is well established, while pathways for prosody are little explored. The present study examined linguistic prosodic structure building in a patient whose right arcuate/superior longitudinal fascicles and posterior corpus callosum were transiently compromised by a vasogenic peritumoral edema. Compared to ten matched healthy controls, the patient\'s ability to detect irregular prosodic structure significantly improved between pre- and post-surgical assessment. This recovery was accompanied by an increase in average fractional anisotropy (FA) in right dorsal and posterior transcallosal fiber tracts. Neither general cognitive abilities nor (non-prosodic) syntactic comprehension nor FA in right ventral and left dorsal fiber tracts showed a similar pre-post increase. Together, these findings suggest a contribution of right dorsal and inter-hemispheric pathways to prosody perception, including the right-dorsal tracking and structuring of prosodic pitch contours that is transcallosally informed by concurrent syntactic information.

OBJECTIVE: To determine the types and to assess the role of auditory evoked potentials and otoacoustic emissions in early detection of hearing abnormalities in Behçet\'s disease (BD) patients. Their correlations with disease activity were also considered.
METHODS: Thirty patients with BD and thirty apparently sex- and age-matched healthy volunteers were included in this study. Auditory evaluation included pure tone audiometry (PTA), otoacoustic emissions (TEOAEs, DPOAE), auditory brainstem response test (ABR) and cortical auditory evoked potentials (tone and speech CAEPs) for all patients and control.
RESULTS: The highest abnormality of CAEP latencies elicited by (500Hz and 1000 Hz) as well as speech stimuli (da and ga) among our BD patients was delayed P1 and N1 waves at 80 dB with greater bilateral affection, as well as significant differences between patients and controls. All our BD patients had a smaller amplitude of distortion product OAE (DPOAE) and S/N ratio at 1, 2, 4, 6 kHZ compared with controls and the differences were highly statistically significant (p=0.0001).
CONCLUSIONS: Being one of the autoimmune inner ear diseases (AIED), BD has a definite hearing impairment, even in the presence of normal hearing sensitivity, as evidenced by PTA. BD patients had a sub-clinical cochlear pathology which was not affected by disease activity or different organ affection. DPOAE (S/N ratio) proved to be a sensitive test in detecting minimal changes in cochlear pathology and the latencies of CAEPs (tone and speech) measures were considered as sensitive indicators (100%) of early detection of hearing impairment in BD patients.

BACKGROUND: Hypothyroidism, a common endocrinological disorder is quite prevalent in its subclinical state in the adult population. Nervous system involvement is frequent in hypothyroidism with documentation of peripheral and central conduction delays as abnormal latency prolongations in Brainstem Auditory Evoked Potential (BAEP) records. Subclinical hypothyroidism however, has been less extensively studied for investigating the involvement of the auditory functions.
OBJECTIVE: To assess the auditory functions and Central Nervous System (CNS) involvement in the patients with subclinical hypothyroidism by recording BAEP.
METHODS: The study comprised of 50 females (25 females with subclinical hypothyroidism and 25 age-matched healthy females) in the age-group of 30-50 years. BAEP absolute and Interpeak Latencies (IPLs) (I, III and V, I-III, III-V and I-V) were compared between the two groups by unpaired t-test. The p<0.05 was considered as statistically significant.
RESULTS: Mean BAEP absolute latencies (III and V) increased in the subjects with subclinical hypothyroidism as compared to controls (p<0.001) (both the ears) and wave I absolute latency also increased with p<0.001 (both the ears) by unpaired t-test. Among IPLs (interpeak latencies), III-V and I-V IPLs exhibited increase (p<0.01), while I-III IPL did not vary with statistical significance (both the ears) in the two groups.
CONCLUSIONS: Patients with subclinical hypothyroidism were found to demonstrate central as well as peripheral auditory pathway affections. BAEPs can prove valuable and sensitive tests to detect involvement of the CNS and auditory dysfunctions earlier in hypothyroidism.

In single sided deafness, treatment with transcranial CROS makes pseudo stereophonic listening possible. This leads to improved speech understanding in noise. However, several reports show large variations between the individual results of the benefit by speech audiometry in noise. One possible factor is the duration and changes in the auditory pathway between the onset of deafness to CROS supply. 18 patients with single sided deafness have tested a transcranial CROS supply. The speech intelligibility thresholds were evaluated in 2 spatial hearing situations each with CROS and unaided. When speech was arriving at 45° from the deaf ear and noise at 45° from the hearing ear, a significant improvement of speech reception threshold for 50% intelligibility was detected by CROS supply compared the medians. Patients with longer duration of deafness showed higher benefit by an average of -4.0 dB, in contrast to patients with short duration of deafness. In the reversed situation, that speech on the hearing ear and noise on the deaf ear was a significant deterioration observed, an average of 3.1 dB at longer deafened patients. In addition, a highly significant correlation was found with the individual changes in the speech recognition threshold between the two situations. The duration of deafness is an essential factor in the individual benefit estimate for treatment with transcranial CROS supply. The time frame for audimetrically proven changes in the auditory pathway as a result of unilateral auditory deprivation can be estimated to about 1-3 years. Patients with short duration of deafness have low or no benefit from transcranial CROS supply.

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Advances in technology and expanding candidacy guidelines have motivated many clinics to consider children with precipitously sloping high-frequency hearing loss as candidates for cochlear implants (CIs). A case study is presented of a pediatric CI patient whose hearing thresholds were preserved within 10 dB of preimplant levels (125-750 Hz) after receiving a fully inserted 31.5-mm electrode array at one ear. The primary goal of this study was to explore the possible benefit of using both a hearing aid (HA) and a CI at one ear while using a HA at the opposite ear. The authors find that although the use of bilateral hearing aids with a CI may only provide a slight benefit, careful attention must be paid to the coordinated fitting of devices, especially at the ear with two devices.