Complete Restoration of Hearing Loss and Cochlear Synaptopathy via Minimally Invasive, Single-Dose, and Controllable Middle Ear Delivery of Brain-Derived Neurotrophic Factor-Poly(dl-lactic acid-co-glycolic acid)-Loaded Hydrogel.

Noise-induced hearing loss (NIHL) often accompanies cochlear synaptopathy, which can be potentially reversed to restore hearing. However, there has been little success in achieving complete recovery of sensorineural deafness using nearly noninvasive middle ear drug delivery before. Here, we present a study demonstrating the efficacy of a middle ear delivery system employing brain-derived neurotrophic factor (BDNF)-poly-(dl-lactic acid-co-glycolic acid) (PLGA)-loaded hydrogel in reversing synaptopathy and restoring hearing function in a mouse model with NIHL. The mouse model achieved using the single noise exposure (NE, 115 dBL, 4 h) exhibited an average 20 dBL elevation of hearing thresholds with intact cochlear hair cells but a loss of ribbon synapses as the primary cause of hearing impairment. We developed a BDNF-PLGA-loaded thermosensitive hydrogel, which was administered via a single controllable injection into the tympanic cavity of noise-exposed mice, allowing its presence in the middle ear for a duration of 2 weeks. This intervention resulted in complete restoration of NIHL at frequencies of click, 4, 8, 16, and 32 kHz. Moreover, the cochlear ribbon synapses exhibited significant recovery, whereas other cochlear components (hair cells and auditory nerves) remained unchanged. Additionally, the cochlea of NE treated mice revealed activation of tropomyosin receptor kinase B (TRKB) signaling upon exposure to BDNF. These findings demonstrate a controllable and minimally invasive therapeutic approach that utilizes a BDNF-PLGA-loaded hydrogel to restore NIHL by specifically repairing cochlear synaptopathy. This tailored middle ear delivery system holds great promise for achieving ideal clinical outcomes in the treatment of NIHL and cochlear synaptopathy.

An Italian case series' description of thiamine responsive megaloblastic anemia syndrome: importance of early diagnosis and treatment.

BACKGROUND: Individuals with thiamine-responsive megaloblastic anemia (TRMA) mainly manifest macrocytic anemia, sensorineural deafness, ocular complications, and nonautoimmune diabetes. Macrocytic anemia and diabetes may be responsive to high-dosage thiamine treatment, in contrast to sensorineural deafness. Little is known about the efficacy of thiamine treatment on ocular manifestations. CASES PRESENTATION: Our objective is to report data from four Italian TRMA patients: in Cases 1, 2 and 3, the diagnosis of TRMA was made at 9, 14 and 27 months. In 3 out of 4 subjects, thiamine therapy allowed both normalization of hyperglycemia, with consequent insulin suspension, and macrocytic anemia. In all Cases, thiamine therapy did not resolve the clinical manifestation of deafness. In Cases 2 and 3, follow-up showed no blindness, unlike Case 4, in which treatment was started for megaloblastic anemia at age 7 but was increased to high doses only at age 25, when the genetic diagnosis of TRMA was performed. CONCLUSIONS: Early institution of high-dose thiamine supplementation seems to prevent the development of retinal changes and optic atrophy in TRMA patients. The spectrum of clinical manifestations is broad, and it is important to describe known Cases to gain a better understanding of this rare disease.

A Case Report of Riboflavin Treatment and Cochlear Implants in a 4-Year-Old Girl with Progressive Hearing Loss and Delayed Speech Development: Brown-Vialetto-Van Laere Syndrome.

BACKGROUND Brown-Vialetto-Van Laere (BVVL) syndrome is a rare autosomal recessive disorder caused by mutations in intestinal riboflavin transporter genes, resulting in a motor neuron disorder of childhood, which can be associated with sensorineural deafness. This report describes a 4-year-old Polish girl with progressive hearing loss and delayed speech development diagnosed with Brown-Vialetto-Van Laere syndrome who was treated with riboflavin (vitamin B2) and cochlear implants. CASE REPORT The case report concerns a girl from Poland who, at the age of 2 years 10 months, developed progressive atypical neurological symptoms of unknown etiology: ataxia of the upper and lower limbs, gait abnormalities, generalized muscle weakness, visual and hearing problems, and regression of speech development. A karyotype study (whole-exome sequencing) revealed alterations within SLC52A2, leading to the diagnosis of Brown-Vialetto-Van Laere syndrome and initiation of high-dose riboflavin treatment. As a 4-year-old child, she presented to the Institute of Physiology and Pathology of Hearing - World Hearing Center in Poland with progressive hearing loss and speech regression. Hearing tests revealed bilateral profound sensorineural hearing loss with auditory neuropathy. Surgical treatment was applied in the form of bilateral cochlear implantation. CONCLUSIONS This report shows the importance of genetic testing in infants who present with atypical symptoms or signs. In this case, the diagnosis of Brown-Vialetto-Van Laere syndrome resulted in timely correction of the genetic riboflavin (vitamin B2) deficiency and improved hearing following the use of cochlear implants.

The Occupational Health and Safety of Sign Language Interpreters Working Remotely During the COVID-19 Pandemic.

INTRODUCTION: The COVID-19 pandemic has caused a dramatic shift in work conditions, bringing increased attention to the occupational health of remote workers. We aimed to investigate the physical and mental health of sign language interpreters working remotely from home because of the pandemic. METHODS: We measured the physical and mental health of certified interpreters who worked remotely 10 or more hours per week. We evaluated associations within the overall sample and compared separate generalized linear models across primary interpreting settings and platforms. We hypothesized that physical health would be correlated with mental health and that differences across settings would exist. RESULTS: We recruited 120 interpreters to participate. We calculated scores for disability (mean score, 13.93 [standard error of the mean (SEM), 1.43] of 100), work disability (mean score, 10.86 [SEM, 1.59] of 100), and pain (mean score, 3.53 [SEM, 0.29] of 10). Shoulder pain was most prevalent (27.5%). Respondents had scores that were not within normal limits for depression (22.5%), anxiety (16.7%), and stress (24.2%). Although disability was not associated with depression, all other outcomes for physical health were correlated with mental health (r ≥ 0.223, P ≤ .02). Educational and community/freelance interpreters trended toward greater adverse physical health, whereas educational and video remote interpreters trended toward more mental health concerns. CONCLUSION: Maintaining the occupational health of sign language interpreters is critical for addressing the language barriers that have resulted in health inequities for deaf communities. Associations of disability, work disability, and pain with mental health warrant a holistic approach in the clinical treatment and research of these essential workers.

First report of quality of life in adults with neurofibromatosis 2 who are deafened or have significant hearing loss: results of a live-video randomized control trial.

PURPOSE: To test the feasibility, acceptability, and preliminary efficacy of a mind-body program for patients with neurofibromatosis 2 (NF2) who are deaf or have significant hearing loss (d3RP-NF2) against an attention placebo control (dHEP-NF2) in a single-blind randomized control trial. Both were delivered using Communication Access Real-Time Translation and live group videoconferencing. METHODS: Forty-five adults with NF2 were randomized. Co-primary outcomes were physical quality of life (QoL) and psychological QoL and secondary outcomes were social QoL and environmental QoL, all measured with the World Health Organization Quality of Life Abbreviated Instrument (WHOQOL-BREF). Assessments were conducted at baseline, post-treatment, and six-month follow-up. RESULTS: Forty-one participants (91%) completed the intervention, and 29 (64%) completed the six-month follow up. Participants in the d3RP-NF2 showed significantly greater improvements from baseline to post-treatment on physical QoL (14.79, 95% CI 5.41-24.18; p ≤ 0.001), psychological QoL (18.77, 95% CI 7.09-30.44, p ≤ 0.001), and environmental QoL (13.25, 95% CI 1.10-25.39, p = 0.03) compared to the dHEP-NF2. Social QoL also significantly increased in the d3RP-NF2 (16.32, 95% CI 6.66-25.97, p = 0.001), but improvement was not beyond the dHEP-NF2. Gains in QoL were clinically meaningful and maintained at the 6-month follow-up for d3RP-NF2 participants across all QoL domains. There were more treatment responders in the d3RP-NF2 compared to the dHEP-NF2. CONCLUSIONS: The d3RP-NF2 was well accepted, highly feasible, and resulted in sustained improvements in QoL in patients with NF2 who are deaf or have significant hearing loss.

Disruption in neural phase synchrony is related to identification of inattentional deafness in real-world setting.

Individuals often have reduced ability to hear alarms in real world situations (e.g., anesthesia monitoring, flying airplanes) when attention is focused on another task, sometimes with devastating consequences. This phenomenon is called inattentional deafness and usually occurs under critical high workload conditions. It is difficult to simulate the critical nature of these tasks in the laboratory. In this study, dry electroencephalography is used to investigate inattentional deafness in real flight while piloting an airplane. The pilots participating in the experiment responded to audio alarms while experiencing critical high workload situations. It was found that missed relative to detected alarms were marked by reduced stimulus evoked phase synchrony in theta and alpha frequencies (6-14 Hz) from 120 to 230 ms poststimulus onset. Correlation of alarm detection performance with intertrial coherence measures of neural phase synchrony showed different frequency and time ranges for detected and missed alarms. These results are consistent with selective attentional processes actively disrupting oscillatory coherence in sensory networks not involved with the primary task (piloting in this case) under critical high load conditions. This hypothesis is corroborated by analyses of flight parameters showing greater maneuvering associated with difficult phases of flight occurring during missed alarms. Our results suggest modulation of neural oscillation is a general mechanism of attention utilizing enhancement of phase synchrony to sharpen alarm perception during successful divided attention, and disruption of phase synchrony in brain networks when attentional demands of the primary task are great, such as in the case of inattentional deafness.

Cortical Representation of Interaural Time Difference Is Impaired by Deafness in Development: Evidence from Children with Early Long-term Access to Sound through Bilateral Cochlear Implants Provided Simultaneously.

Accurate use of interaural time differences (ITDs) for spatial hearing may require access to bilateral auditory input during sensitive periods in human development. Providing bilateral cochlear implants (CIs) simultaneously promotes symmetrical development of bilateral auditory pathways but does not support normal ITD sensitivity. Thus, although binaural interactions are established by bilateral CIs in the auditory brainstem, potential deficits in cortical processing of ITDs remain. Cortical ITD processing in children with simultaneous bilateral CIs and normal hearing with similar time-in-sound was explored in the present study. Cortical activity evoked by bilateral stimuli with varying ITDs (0, ±0.4, ±1 ms) was recorded using multichannel electroencephalography. Source analyses indicated dominant activity in the right auditory cortex in both groups but limited ITD processing in children with bilateral CIs. In normal-hearing children, adult-like processing patterns were found underlying the immature P1 (∼100 ms) response peak with reduced activity in the auditory cortex ipsilateral to the leading ITD. Further, the left cortex showed a stronger preference than the right cortex for stimuli leading from the contralateral hemifield. By contrast, children with CIs demonstrated reduced ITD-related changes in both auditory cortices. Decreased parieto-occipital activity, possibly involved in spatial processing, was also revealed in children with CIs. Thus, simultaneous bilateral implantation in young children maintains right cortical dominance during binaural processing but does not fully overcome effects of deafness using present CI devices. Protection of bilateral pathways through simultaneous implantation might be capitalized for ITD processing with signal processing advances, which more consistently represent binaural timing cues.SIGNIFICANCE STATEMENT Multichannel electroencephalography demonstrated impairment of binaural processing in children who are deaf despite early access to bilateral auditory input by first finding that foundations for binaural hearing are normally established during early stages of cortical development. Although 4- to 7-year-old children with normal hearing had immature cortical responses, adult patterns in cortical coding of binaural timing cues were measured. Second, children receiving two cochlear implants in the same surgery maintained normal-like input from both ears, but this did not support significant effects of binaural timing cues in either auditory cortex. Deficits in parieto-occiptal areas further suggested impairment in spatial processing. Results indicate that cochlear implants working independently in each ear do not fully overcome deafness-related binaural processing deficits, even after long-term experience.

AAC intervention using a VOCA for deaf children with multiple disabilities who received cochlear implantation.

OBJECTIVES: The aim of this study was to examine the efficacy of a new habilitation approach, augmentative and alternative communication (AAC) intervention using a voice output communication aid (VOCA), in improving speech perception, speech production, receptive vocabulary skills, and communicative behaviors in children with cochlear implants (CIs) who had multiple disabilities. METHODS: Five children with mental retardation and/or cerebral palsy who had used CIs over two years were included in this study. Five children in the control group were matched to children who had AAC intervention on the basis of the type/severity of their additional disabilities and chronological age. They had limited oral communication skills after cochlear implantation because of their limited cognition and oromotor function. The children attended the AAC intervention with parents once a week for 6 months. We evaluated their performance using formal tests, including the monosyllabic word tests, the articulation test, and the receptive vocabulary test. We also assessed parent-child interactions. We analyzed the data using a one-group pretest and posttest design. RESULTS: The mean scores of the formal tests performed in these children improved from 26% to 48% in the phoneme scores of the monosyllabic word tests, from 17% to 35% in the articulation test, and from 11 to 18.4 in the receptive vocabulary test after AAC intervention (all p < .05). Some children in the control group showed improvement in the speech perception, speech production, and receptive vocabulary tests for 6 months, but the differences did not achieve statistical significance (all p > .05). The frequency of spontaneous communicative behaviors (i.e., vocalization, gestures, and words) and imitative words significantly increased after AAC intervention (p < .05). CONCLUSIONS: AAC intervention using a VOCA was very useful and effective on improving communicative skills in children with multiple disabilities who had very limited oral communication skills after cochlear implantation.

Brown-Vialetto-van Laere and Fazio-Londe overlap syndromes: a clinical, biochemical and genetic study.

Brown-Vialetto-van Laere (BVVL) and Fazio-Londe (FL) are rare and clinically overlapping motor neurons syndromes. Recently BVVL has been associated with mutations in C20orf54/hRFT2 and defective riboflavin transport. We compared clinical and laboratory features of 6 patients (age range 11-17 years), with features of BVVL and FL overlap syndromes. Patients were assessed as following: blood levels of riboflavin and redox status, electrophysiological, neuroradiological and pulmonary studies, ALS functional rating scale and molecular genetic analysis. Two patients manifested deafness at ages of 3 and 10 years, and developed later subacute progressive ponto-bulbar palsy. A third patient markedly improved after intravenous immunoglobulins (IVIG), but then relapsed remaining unresponsive to treatment; he was not deaf although had abnormal auditory evoked responses (BAERs). The remaining 3 patients had no deafness, although likewise manifested subacute progressive ponto-bulbar palsy. We found hRFT2 mutations in 3/6 patients manifesting deafness or abnormal BAERs. No patient had reduced riboflavin blood levels. However, on riboflavin supplementation (10mg/kg/day) the most severely affected BVVL patient stopped progression of symptoms following 8 months of treatment. BVVL and FL are severe progressive diseases with overlapping symptoms although only hRFT2 mutated patients manifest deafness. Riboflavin supplementation seems to stabilize and improve progression of the disease.

Speech perception, rapid temporal processing, and the left hemisphere: a case study of unilateral pure word deafness.

The mechanisms and functional anatomy underlying the early stages of speech perception are still not well understood. One way to investigate the cognitive and neural underpinnings of speech perception is by investigating patients with speech perception deficits but with preserved ability in other domains of language. One such case is reported here: patient NL shows highly impaired speech perception despite normal hearing ability and preserved semantic knowledge, speaking, and reading ability, and is thus classified as a case of pure word deafness (PWD). NL has a left temporoparietal lesion without right hemisphere damage and DTI imaging suggests that he has preserved cross-hemispheric connectivity, arguing against an account of PWD as a disconnection of left lateralized language areas from auditory input. Two experiments investigated whether NL's speech perception deficit could instead result from an underlying problem with rapid temporal processing. Experiment 1 showed that NL has particular difficulty discriminating sounds that differ in terms of rapid temporal changes, be they speech or non-speech sounds. Experiment 2 employed an intensive training program designed to improve rapid temporal processing in language impaired children (Fast ForWord; Scientific Learning Corporation, Oakland, CA) and found that NL was able to improve his ability to discriminate rapid temporal differences in non-speech sounds, but not in speech sounds. Overall, these data suggest that patients with unilateral PWD may, in fact, have a deficit in (left lateralized) temporal processing ability, however they also show that a rapid temporal processing deficit is, by itself, unable to account for this patient's speech perception deficit.

Transtympanic electrical stimulation for immediate and long-term tinnitus suppression.

Tinnitus is a common symptom which often becomes disabling, affecting the emotional and psychosocial dimensions of life. There are many reports describing tinnitus suppression or attenuation through electrical stimulation of the ear, provided either by cochlear implants or by transtympanic stimulation. Our study project aims to assess the effects of electrical promontory stimulation (EPS) on persistent disabling tinnitus. We enrolled 11 patients affected by postlingual monoaural or binaural profound hearing loss and disabling tinnitus in the worse ear. EPS was performed with direct continuous positive current delivered by an active platinum-iridium needle electrode connected to a promontory stimulator device. The short-term effect on tinnitus was assessed during and immediately after the stimulation. Long-term effects were estimated after one month by comparing pre- and post-EPS Tinnitus Handicap Inventory (THI) scores. Immediately after EPS, five patients (45.4%) reported complete suppression and four (36.4%) reported attenuation of tinnitus. Two patients (18.2%) said it was unchanged. After one month, the THI score was reduced in five patients (45.4%) and remained unchanged in the other six patients (54.6%). The beneficial effects of EPS on tinnitus might be explained by interference with tinnitus generating circuits such as the dorsal cochlear nucleus and the inferior colliculus and by modification of cortical activity. EPS is to be considered a worthwhile attempt at tinnitus suppression, and could help select candidates for the positioning of an implantable electrical stimulator that might provide longer-term beneficial effect on tinnitus.

Ataxia with vitamin E deficiency associated with deafness.

Ataxia with vitamin E deficiency (AVED) is a rare autosomal recessive disorder, usually with a phenotype resembling Friedreich ataxia, caused by selective impairment of gastrointestinal vitamin E absorption. Vitamin E supplementation improves symptoms and prevents disease progress. In North Africa and Southern Europe, AVED is as common as Friedreich ataxia. There are no reported cases from Turkey. We herein report a 16-year-old Turkish girl with AVED, who was found to have total deletion of the TTPA gene as well as sensorineural deafness, and we present her follow-up data after vitamin E therapy.

Neural tonotopy in cochlear implants: an evaluation in unilateral cochlear implant patients with unilateral deafness and tinnitus.

In cochlear implants, the signal is filtered into different frequency bands and transmitted to electrodes along the cochlea. In this study the frequency-place function for electric hearing was investigated as a means to possibly improve speech coding by delivering information to the appropriate cochlear place. Fourteen subjects with functional hearing in the contralateral ear have been provided with a MED-EL cochlear implant in the deaf ear in order to reduce intractable tinnitus. Pitch scaling experiments were performed using single-electrode, constant-amplitude, constant-rate stimuli in the implanted ear, and acoustic sinusoids in the contralateral ear. The frequency-place function was calculated using the electrode position in the cochlea as obtained from postoperative skull radiographs. Individual frequency-place functions were compared to Greenwood's function in normal hearing. Electric stimulation elicited a low pitch in the apical region of the cochlea, and shifting the stimulating electrode towards the basal region elicited increasingly higher pitch. The frequency-place function did not show a significant shift relative to Greenwood's function. In cochlear implant patients with functional hearing in the non-implanted ear, electrical stimulation produced a frequency-place function that on average resembles Greenwood's function. These results differ from previously derived data.

[Conception and realisation of a prosody test battery]. / Konzeption und Realisierung einer Prosodie-Testbatterie.

BACKGROUND: Prosody reflects rhythmic and melodic aspects in speech and is one of the quality measures that shows--apart from pure speech understanding--a rising interest in the assessment of technical hearing aids, especially of cochlear implants. At present, there is no adequate test battery for the German speaking population. The test battery presented in this study aims to fill this gap. METHODS: The test battery consists of four different modules addressing different prosodic cues, namely duration, question vs statement, sentence stress, and speaker gender. One part of the test battery aims at reflecting everyday situations and was realized with six different speakers representing various pitch frequencies and speak rates. Another goal of the tests is to detect very small differences in prosody perception. Therefore, natural utterances were artificially modified by changing the underlying acoustic parameters (e.g. duration, fundamental frequency). Measurements were performed with 12 normal listeners in order to investigate the properties of the tests. RESULTS: As expected, the normal listeners revealed discrimination rates of close to 100% in that part of the test battery using natural utterances. For the part with the modified stimuli, all four modules revealed very steep discrimination functions indicating that the normal listeners were able to use very small changes in the acoustic parameters as cues for prosody perception. Moreover, the slope of the discrimination function revealed very low variability. CONCLUSIONS: Together with preliminary data from cochlear implant recipients who clearly showed different psychometric functions, the test battery seems suitable for a comprehensive evaluation of prosody perception. This facilitates future examinations of speech processing beyond the pure understanding of speech.

Effectiveness of sulphur spa therapy with politzer in the treatment of rhinogenic deafness.

Several studies have focused on the usefulness of sulphur, radioactive and bromo-iodine mineral waters in the treatment of chronic inflammatory lower and upper respiratory processes. The purpose of this study was to evaluate the tolerability, effectiveness and impact on quality of life of sulphur spa therapy with Politzer in subjects with chronic inflammatory processes responsible for the onset or persistence of rhinogenic deafness. The study was performed on 27 subjects (mean age 62 +/- 2.2 years, range: 28-88) with chronic catarrhalis otitis, chronic rhino-sinusitis and pharyngeal inflammation. These patients underwent 12 sessions of humid-hot inhalation, with vapour jet 20 cm from the face, at 38 degrees C for 10 min, followed by Politzer with sulphur sodium chloride bicarbonate alkaline mineral water from "Rosapepe" Spa, in Contursi (Salerno, Italy). Middle ear function and possible social recovery (based on Giaccai and Gardenghi guidelines) of the patients were assessed, at the beginning and end of the spa therapy. Results, at the end of this treatment, showed a significant (p < 0.05) increase in audiometric curves corresponding to the normal ventilation of the tympanic box (incidence of 24% before therapy and 33% thereafter) and a decrease in pathological curves. Moreover, a significant (p < 0.05) reduction in the percentage of auditory loss was recorded (N = 41; 19.7% +/- 2.5 --> 13.9% +/- 1.9) and improved hearing, at the frequencies required for daily activities: 500-1000 and 2000 Hz (31.1 dB +/- 1.7 --> 26.8 dB +/- 1.5). No adverse effects to the spa therapy were observed during the study. In conclusion, the results of this study are in agreement with data in the literature, demonstrating that associated spa therapy with Politzer and inhalation have a positive impact on the therapeutic strategy of chronic inflammatory processes, responsible for the onset or persistence of rhinogenic deafness, in order to enhance and combine with the already consolidated pharmacological approaches.

Neurolinguistic development in deaf children: the effect of early language experience.

Recent investigations have indicated a relationship between the development of cerebral lateralization for processing language and the level of development of linguistic skills in hearing children. The research on cerebral lateralization for language processing in deaf persons is compatible with this view. We have argued that the absence of appropriate input during a critical time window creates a risk for deaf children that the initial bias for left-hemisphere specialization will be distorted or disappear. Two experiments were conducted to test this hypothesis The results of these investigations showed that children educated early and intensively with cued speech or with sign language display more evidence of left-hemisphere specialization for the processing of their native language than do those who have been exposed later and less intensively to those languages.

[Chronic complex tinnitus: therapeutic results of inpatient treatment in a tinnitus clinic]. / Ergebnisse stationärer Therapie bei Patienten mit chronisch komplexem Tinnitus.

BACKGROUND: In-patient treatment of patients with chronic tinnitus is necessary only when these patients have a severe psychosomatic co-morbidity and suffer severely. However this therapeutic approach has to be supervised and evaluated properly. METHODS: We present data and results of 1841 patients suffering from chronic tinnitus. Due to the severity of the symptom and psycho-neurotic side effects in-patient treatment was necessary. Therapy lasted 5 - 6 weeks, the main aspect was an intensive psychotherapeutic evaluation and stabilisation next to retraining and habituation programmes. Relaxation techniques were taught. Patients suffered from their tinnitus more than six month; 95 % further suffered from hearing-loss, mainly in high frequencies. The study evaluates results of patients from October 1994 until June 2000. Basis of the study was the evaluation of a specific tinnitus-questionnaire (TQ), published by Hallam in the UK and translated by Goebel and Hiller in Germany. Data was recorded at registration in our clinic, 4 - 6 months later during admission and at the end of the therapy. Final data was gained during a special meeting or questioning 6 months after dismissal from the clinic. RESULTS: Patients that suffered most showed the greatest improvement; directly after therapy there was a highly significant improvement in the TQ for an average of 13.01 points. Highly significant improvements were found in all the TQ-subscales respectively. Only 10 % of the patients did not show any improvement at all. CONCLUSION: Therapy of most severe cases of chronic tinnitus is possible, using an integrated concept of otologic and psychosomatic treatments. With large numbers of patients and sufficient data a thorough and necessary evaluation of this therapy can be achieved.

Auditory deprivation modifies biological rhythms in the golden hamster.

To assess to what extent auditory sensory deprivation affects biological rhythmicity, sleep/wakefulness cycle and 24 h rhythm in locomotor activity were examined in golden hamsters after bilateral cochlear lesion. An increase in total sleep time as well as a decrease in wakefulness (W) were associated to an augmented number of W episodes, as well as of slow wave sleep (SWS) and paradoxical sleep (PS) episodes in deaf hamsters. The number of episodes of the three behavioural states and the percent duration of W and SWS increased significantly during the light phase of daily photoperiod only. Lower amplitudes of locomotor activity rhythm and a different phase angle as far as light off were found in deaf hamsters kept either under light-dark photoperiod or in constant darkness. Period of locomotor activity remained unchanged after cochlear lesions. The results indicate that auditory deprivation disturbs photic synchronization of rhythms with little effect on the clock timing mechanism itself.

Functional magnetic resonance imaging may avoid misdiagnosis of cochleovestibular nerve aplasia in congenital deafness.

OBJECTIVE: To investigate a narrow internal auditory canal (IAC) syndrome using functional magnetic resonance imaging (fMRI) of the auditory cortex. STUDY DESIGN: The study design was a case report. The follow-up period lasted 18 months. SETTING: The study was carried out in the audiology clinic of an ear, nose, and throat department and in the department of pediatric neuroradiology at a university hospital. MAIN OUTCOME MEASURES: Age-appropriate observational audiometry, objective audiovestibular tests, computed tomography (CT), magnetic resonance imaging (MRI), and (fMRI) of the auditory cortex were performed to analyze in detail the profound deafness of a young child. RESULTS: Audiovestibular examination demonstrated both measurable hearing and normal vestibulo-ocular reflex, and CT showed narrow IACs combined with normal labyrinths. Axial MR images completed by sagittal sections perpendicular to the IAC delineated a single nerve that was initially supposed to be the facial nerve. No cochleovestibular nerve was identified. However, fMRI performed with the patient under general anesthesia demonstrated activation of the primary auditory cortex during 1-kHz monaural stimulation on the left side. CONCLUSIONS: The absence of cochleovestibular nerve on MR studies cannot exclude connections between the inner ear and the central auditory pathways. This might be caused by a lack of spatial resolution of anatomical MR studies. The single nerve delineated within the IAC might also carry both facial and cochleovestibular fibers. Functional MRI can assess the cortical response to acoustic stimuli when aplasia of the cochleovestibular nerve is suspected. This case study illustrates a novel and atypical presentation of cochlear nerve dysplasia.