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AbstractObjectiveDesignStudy sampleResultsConclusionThis study aimed to evaluate long-term cochlear implant (CI) outcomes in individuals with mono-allelic pathogenic variants in WFS1, which is associated with both Wolfram-like syndrome and DFNA6/14/38.Retrospective case series.Seven CI recipients, ranging from eight months to 58 years of age, were included in the study, including four with Wolfram-like syndrome and three with DFNA6/14/38. A total of ten cochlear implantations were performed among these subjects.At one-year post-implantation, a mean phoneme score of 90 ± 9% at 65 dB SPL in quiet was found, which remained stable up to ten years post-implantation with a mean phoneme score of 94 ± 6%. Despite these excellent outcomes, one subject achieved no speech recognition with CI and eventually became a non-user. This individual had a prolonged absence of auditory stimulation prior to implantation and encountered multiple challenges during rehabilitation.Individuals with Wolfram-like syndrome or DFNA6/14/38 demonstrate consistently good outcomes following implantation, which remain stable over time. These findings affirm cochlear implantation as an effective rehabilitation option for these individuals. Furthermore, the stable and good CI outcomes contradict the suggested link between WFS1-associated sensorineural hearing loss and auditory neuropathy. [ABSTRACT FROM AUTHOR]

AbstractObjectiveDesignStudy sampleResultsConclusionThis study aimed to explore the functional integrity of vestibulo-masseteric and sacculo-collic reflex pathways in individuals with Auditory Neuropathy (AN). The study also aimed at finding the correlation between mVEMP and cVEMP response parameters for AN and healthy individuals.Standard group comparison research designIndividuals with AN (n = 20); age-gender matched healthy individuals (n = 20) were recruited. Each participant underwent routine audiological evaluation; mVEMP and cVEMP testing. mVEMP and cVEMP were recorded using 500 Hz tone burst stimulus for all the participants.Most of the AN individuals had no mVEMP (ipsilateral & contralateral − 60%; bilateral − 50%) and cVEMP (60%) responses. There was no significant association (p > 0.05) between the duration of AN with mVEMP and cVEMP findings. However, a significant correlation (p < 0.05) was found for EMG rectified amplitude of mVEMP and cVEMP in AN.The results of this study suggested an impaired function of the vestibulomassteric and sacculocollic reflex pathways in individuals with AN. Vestibular evaluation should be included as a part of the regular test battery for individuals with AN. [ABSTRACT FROM AUTHOR]

AbstractObjectivesDesignStudy SampleResultsConclusionsA case of sudden sensorineural hearing loss following use of sildenafil was examined in detail over a period of three days from first report to recovery.Case study. The subject presented with sudden sensorineural hearing loss and diplacusis a day after onset. Testing involved detailed interview, standard audiometry, detailed inter-octave audiometry, and measurement of detailed psychophysical frequency tuning curves during a two day recovery period.One male aged in his thirties with otherwise normal hearing.Although standard audiometry was within normal limits, detailed inter-octave audiometry and psychophysical frequency tuning curves were consistent with a punctate unilateral intra-cochlear lesion that resolved over a period of three days.This is the first report of such a frequency-specific audiometric shift and diplacusis after sildenafil, and is not consistent with previous reports of direct ototoxic pharmacological effects. We propose that the lesion was most likely caused by a cochlear bleed, and may have been due to physical exertion rather than a direct pharmaceutical effect. The study highlights the important role of additional diagnostic testing that can be easily achieved in a clinical setting with minimal equipment [ABSTRACT FROM AUTHOR]

Objective: It has recently been discussed whether hearing screening and hearing threshold assessment can accurately be completed using automated ASSR methods for children with auditory neuropathy spectrum disorder (ANSD). Possible causes for the claimed potential failures were investigated here. Design: The study is based on the analysis of stored ASSR raw data. Study sample: This study reviewed raw ASSR data from 274 patients with a total of 5809 individual recordings. Results: Cochlear microphonics (CM) were found in 18 of the 274 patient records. Four of these 18 were obtained from patients with ANSD. One patient with ANSD without click auditory brainstem responses up to 100 dBnHL demonstrated clear ASSR responses from 65 dBnHL upwards. Where click stimulation suggests an auditory nerve defect, narrow-band chirps were shown to evoke ASSR in certain patients. CMs are elicited by narrow-band chirps in the same way as by broadband stimuli. CM residuals as well as a presumed enlarged wave I with absent neural responses, always accompanied by CM, were found as possible causes of misinterpretation at high stimulus levels. A CM detector was created. Conclusions: The CM detector, indicating the presence of CM, will prevent misinterpretation of clinical ASSR results. [ABSTRACT FROM AUTHOR]

The objective of this study was to determine the prevalence, risk factors, and audiological characteristics of auditory neuropathy spectrum disorder (ANSD) in the pediatric population. A retrospective review of medical charts was conducted for children visiting two hospitals in Saudi Arabia. Medical records of 1025 patients with sensorineural hearing loss (SNHL) were reviewed. We analyzed the databases for results of audiological examinations, risk factors, and outcomes of intervention including hearing aid (HA) and cochlear implantation (CI). Out of 1025 children with SNHL, 101 patients (9.85%) were identified to have ANSD. Audiological characteristics of the ANSD group revealed a severe-to-profound degree of hearing loss, all showed type A tympanogram and absent reflexes, absent auditory brainstem response (ABR) findings with present cochlear microphonic while otoacoustic emissions were absent in 54.5% of patients. The most prevalent risk factors for ANSD in this group were family history of hearing loss, consanguinity, hyperbilirubinemia, and low birth weight. Pure tone and speech detection thresholds improved significantly with CI compared to HA use in this sample of patients with ANSD. This study shows that ANSD is not extremely rare among Saudi children with severe to profound hearing loss, with a prevalence of 9.85%. [ABSTRACT FROM AUTHOR]

The purpose of this paper is to describe a child with auditory neuropathy spectrum disorder (ANSD) associated with Brown-Vialetto-Van Laere (BVVL) syndrome, which is a rare, inherited, neurodegenerative disorder that is caused by defects in riboflavin transporter genes. We report the audiological and clinical profile of a child who presented with a complaint of sudden loss of speech understanding associated with an atypical form of ANSD. He was later diagnosed with BVVL. An 11-year-old boy with ANSD associated with BVVL. The patient's severe neurological symptoms improved within a year of supplementation with high doses of riboflavin. His fluctuating hearing loss and 0% WDS remained unchanged. The patient was able to use hearing aids without any discomfort after treatment initiation, but he stopped using them again due to a lack of benefit in speech understanding. Although cochlear implantation was recommended, the patient and his family decided not to consider it for another year since they still had hope for complete recovery. Sudden-onset ANSD can be the earliest sign of undetected BVVL syndrome. Early detection of BVVL is crucial since all symptoms can be reversible with an early intervention of high doses of riboflavin supplementation. [ABSTRACT FROM AUTHOR]

ObjectiveThe high energy demand of the auditory and visual pathways render these sensory systems prone to diseases that impair mitochondrial function. Primary open-angle glaucoma, a neurodegenerative disease of the optic nerve, has recently been associated with a spectrum of mitochondrial abnormalities. This study sought to investigate auditory processing in individuals with open-angle glaucoma. DESIGN/STUDY SAMPLE: Twenty-seven subjects with open-angle glaucoma underwent electrophysiologic (auditory brainstem response), auditory temporal processing (amplitude modulation detection), and speech perception (monosyllabic words in quiet and background noise) assessment in each ear. A cohort of age, gender and hearing level matched control subjects was also tested.ResultsWhile the majority of glaucoma subjects in this study demonstrated normal auditory function, there were a significant number (6/27 subjects, 22%) who showed abnormal auditory brainstem responses and impaired auditory perception in one or both ears.ConclusionsThe finding that a significant proportion of subjects with open-angle glaucoma presented with auditory dysfunction provides evidence of systemic neuronal susceptibility. Affected individuals may suffer significant communication difficulties in everyday listening situations.

Objective: The primary goal of this study was to characterise the cochlear and neural components of hearing loss in a large cohort of people with Charcot-Marie Tooth neuropathy who reported hearing difficulties. Design: A full complement of audiologic measures including behavioral, physiologic and subjective assessments were administered. Study sample: Seventy-nine participants completed the study. Forty-four people had CMT1, 27 had CMT2 and four had CMT-INT. Results: Pure tone average was related to age and the absence of high-frequency DPOAE energy, suggesting a strong cochlear component. Acoustic reflexes were often elevated or absent and many participants exhibited abnormal ABR waveforms, suggesting additional neural hearing loss components. Participants with an abnormal or absent ABR wave V exhibited poorer speech perception abilities. There was an association between a prolonged ABR wave I latency and an abnormal or absent ABR wave V with a higher Charcot-Marie Tooth Neuropathy Score (indicating greater disability). Conclusions: The hearing abilities of people with CMT are highly variable. While there were strong neural hearing loss components, speech perception abilities were not disproportionately affected in most participants. Therefore, a hearing aid trial is recommended. ABR responses may be a useful tool for monitoring the progression of CMT over time. [ABSTRACT FROM AUTHOR]

Objective: The present study attempted to compare the aided benefit using low-cut modified amplification and channel-free hearing aids in individuals with auditory neuropathy spectrum disorder (ANSD). It was also attempted to determine these effects in good and poor performers with ANSD. Design: Cross-sectional within group pretest, post-test design. Study sample: Twenty-five individuals with acquired ANSD were selected for the study. The study sample included 11 males and 14 females between the age ranges of 17-40 years (mean age of 24.6 years). Results: The results of the repeated measures analysis of variance (ANOVA) showed that aided benefit was significantly higher with the channel-free hearing aid. Mixed ANOVA results showed that the improvement was more in good performers than poor performers with ANSD. Multiple regression analyses showed that speech identification scores are a strong predictor of aided benefit. Conclusions: The results of the present study suggest that channel-free hearing aids and low-cut modified amplification can be used as an efficient alternative technique during hearing aid fitting for individuals with ANSD. However, further evidence-based studies on a larger group are essential to validate the results. [ABSTRACT FROM AUTHOR]

Objective: The individual outcome after cochlear implantation in children with auditory synaptopathy/neuropathy (AS/AN) is difficult to predict. A tool for preoperative assessment would be helpful for counseling parents. This study evaluates the outcome after CI in children with AS/AN and with sensorineural hearing loss (SNHL), and correlates it with the preoperative ECochG results in order to find specific parameters of prognostic value.Design: The improvement of auditory behavior after CI was retrospectively assessed using the LittlEARS questionnaire and quantified in a score (LS). This score was correlated with the CAP/SP ratio in the preoperative ECochG. The score was further correlated with the patient’s age six months following CI.Study sample:Nine children with AS/AN were compared to nine children with SNHL.Results: Both groups showed a significant improvement in LS following CI. There was a significant positive correlation between the CAP/SP ratio and the improvement in LS in all children. The correlation between age and LS was significantly negative in the SNHL group and positive in the AS/AN group.Conclusion: All children with AS/AN and SNHL benefit to a similar extent from CI. The preoperatively assessed CAP/SP ratio has a prognostic value for the development of auditory behavior following CI. [ABSTRACT FROM PUBLISHER]

Objective: To evaluate the prevalence of auditory neuropathy (AN) in late preterms treated with phototherapy for hyperbilirubinemia. Design: Prospective observational study comprising late preterms treated with phototherapy for hyperbilirubinemia. Newborns were screened with combined transient-evoked otoacoustic emissions (TEOAEs) / automated auditory brainstem responses (AABR). Infants who failed screening underwent diagnostic (ABR). Infants were all re-evaluated with AABR at one year. Study sample: Eighty-five infants with a mean serum total bilirubin concentration of 22.3 ± 1.76 mg/dl; severe-hyperbilirubinemia (SH), and 102 infants with a mean serum total bilirubin concentration of 18.6 ± 1.26 mg/dl; non-severe hyperbilirubinemia (NSH) were included. Results: From 85 late preterms with SH, six (7.1%) failed screening and underwent diagnostic ABR for six weeks. AN was diagnosed in two (2%) infants with SH. Four (3.9%) of the 102 controls with NSH demonstrated failure at TEOAE/AABR. No AN was diagnosed in the control group at the diagnostic ABR. No statistically significant difference was found between infants treated with phototherapy for SH and NSH with regard to AN/AD either in the postnatal period or at one year. No correlation was found between serum bilirubin levels and ABR latencies or thresholds. Conclusions: AN (2%) in late preterms treated with phototherapy for severe-hyperbilirubinemia was not higher than in those with non-severe hyperbilirubinemia. [ABSTRACT FROM AUTHOR]

Objective: This paper reviews the current literature on involvement of the vestibular division of the eighth cranial nerve in peripheral neuropathies. The literature abounds with references to auditory neuropathy, which is frequently related to more generalized neuropathies, but there is a marked paucity of work regarding vestibular neuropathy. A brief overview of neuropathies and the anatomy of the vestibulocochlear nerve provide the background for a review of the literature of vestibular nerve involvement in a range of neuropathies. Design: A literature search including textbooks, and peer-reviewed published journal articles in online bibliographic databases was conducted. Study sample: Two databases for medical research were included in this review. Results: The review of the literature indicates that vestibular involvement is a common and consistent finding in many peripheral neuropathies of different aetiologies. Specifically, if patients present unsteadiness/ataxia out of proportion to objective signs of somatosensory loss or muscle weakness. Conclusion: This review concludes that vestibular neuropathy, is common in peripheral neuropathy and should be identified to optimize patient management and rehabilitation. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this paper was to describe a child with brainstem juvenile pilocytic astrocytoma (JPA) and associated auditory symptoms. Design: Case study of a 5-year-old female who presented with a complaint of headaches, accompanied with a history of symptoms that could be associated with a left unilateral congenital auditory neuropathy. She was later diagnosed with a large, left-sided posterior fossa mass determined to be a JPA. Study sample: Case study of a child identified at birth with a constellation of symptoms consistent with unilateral auditory neuropathy spectrum disorder (ANSD). Results: The early onset symptoms, that can be the same for ANSD or for a brainstem tumor that involves the auditory nerve, suggests that the JPA was congenital. This child, if initially followed by neurology may not have been identified earlier with this tumor, however a baseline MRI or monitoring neuroimaging may have been available for reference or led to earlier detection of the lesion. It may not have changed her management, however, this case stresses the importance of the recommendations in the 'Guidelines for Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder' that include a referral to a neurologist for a baseline assessment and neuroimaging. Conclusion: An undetected JPA can have the same audiological clinical presentation as a unilateral ANSD. This case description highlights the need to heighten awareness for neurological and radiological monitoring in cases of ANSD, especially when the presentation is unilateral. [ABSTRACT FROM AUTHOR]

Abstract Objective: Audiological data from a population based epidemiological investigation were studied on elderly persons. Specific diagnoses of otological and audiological disorders, which can result in hearing loss, were searched for. Design: A retrospective register study. Study sample: Three age cohorts, 474 70- and 75-year olds ('younger'), and 252 85-year olds ('older'), were studied. Clinical pure tone and speech audiometry was used. Data from medical files were included. Results: Conductive hearing loss was diagnosed in 6.1% of the 'younger' elderly persons, and in 10.3% of the 'older' ones. Specific diagnoses (chronic otitis media and otosclerosis) were established in about half of the cases. Sensorineural hearing loss, other than age-related hearing loss and noise induced hearing loss, was diagnosed in 3.4 % and 5.2% respectively. Severely impaired speech recognition, possibly reflecting age-related auditory neuropathy, was found in 0.4% in the 'younger' group, and in 10% in the 'older' group. Bilateral functional deafness was present in 3.2% of the 85-year-old persons, but was not present in the 70-75-year group. Conclusion: The incidence of probable age-related auditory neuropathy increases considerably from 70-75 to 85 years. There are marked differences between 'younger' and 'older' elderly persons regarding hearing loss that severely affects oral communication. Sumario Objetivo: Se estudiaron los datos audiológicos obtenidos en una investigación epidemiológica basada en grupos de ancianos. Se buscaron diagnósticos específicos de problemas otológicos o audiológicos que pueden determinar pérdidas auditivas. Diseño: Estudio retrospectivo de registros. Muestra: Se estudiaron tres cohortes de grupos de edad: 474 'jóvenes' de 70 y 75 años y 252 'viejos' de 85 años de edad. Se realizó audiometría clínica por tonos puros y logoaudiometría. Se incluyeron los datos de los expedientes clínicos. Resultados: Se diagnosticó pérdida auditiva conductiva en el 6.1% de los ancianos 'jóvenes' y en el 10.3% de los 'viejos'. Se estableció el diagnóstico especifico (otitis media crónica y otoesclerosis) en cerca de la mitad de los casos. Se diagnosticó pérdida auditiva neurosensorial, aparte de pérdidas relacionadas con la edad o inducidas por ruido, en el 3.4% y en el 5.2% de los casos, respectivamente. Se encontró reconocimiento del lenguaje severamente afectado, posiblemente reflejando neuropatía auditiva relacionada con la edad, en el 0.4% de los 'jóvenes' y en el 10% de los 'viejos'. La sordera funcional bilateral estuvo presente en el 3.2% de las personas de 85 años, pero no se observó en el grupo de personas de 70-75 años. Conclusión: La incidencia de neuropatía auditiva probablemente relacionada con la edad, aumenta considerablemente de los 70-75 a los 85 años. Hay marcadas diferencias relacionadas con las pérdidas auditivas entre ancianos más 'jóvenes' y más 'viejos', que afectan severamente la comunicación oral. [ABSTRACT FROM AUTHOR]

Abstract Objective: Disrupted auditory nerve activity in auditory neuropathy (AN) significantly impairs the sequential processing of auditory information, resulting in poor speech perception. This study investigated the ability of AN subjects to perceive temporally modified consonant-vowel (CV) pairs and shed light on their phonological awareness skills. Design: Four Arabic CV pairs were selected: /ki/-/gi/, /to/-/do/, /si/-/sti/ and /so/-/zo/. The formant transitions in consonants and the pauses between CV pairs were prolonged. Rhyming, segmentation and blending skills were tested using words at a natural rate of speech and with prolongation of the speech stream. Study sample: Fourteen adult AN subjects were compared to a matched group of cochlear-impaired patients in their perception of acoustically processed speech. Results: The AN group distinguished the CV pairs at a low speech rate, in particular with modification of the consonant duration. Phonological awareness skills deteriorated in adult AN subjects but improved with prolongation of the speech inter-syllabic time interval. Conclusions: A rehabilitation program for AN should consider temporal modification of speech, training for auditory temporal processing and the use of devices with innovative signal processing schemes. Verbal modifications as well as visual imaging appear to be promising compensatory strategies for remediating the affected phonological processing skills. Sumario Objetivo: En la neuropatía auditiva (AN) la disrupción de la actividad del nervio auditivo impide significativamente el procesamiento secuencial de la información auditiva con la consecuente pobre percepción del habla. Este estudio investiga la habilidad de los sujetos con neuropatía auditiva para percibir pares temporalmente modificados de consonante vocal (CV) y nos da luz sobre sus habilidades en el reconocimiento fonológico. Diseño: Se seleccionaron cuatro pares de CV en árabe: /ki/-/gi/, /to/-/do/, /si/-/sti/ y /so/-/zo/. La transición de la formante en las consonantes y las pausas entre los pares de CV fueron prolongados. Se probaron las habilidades de rima, segmentación y combinación utilizando palabras a una tasa normal del habla y con prolongación del flujo del habla. Muestra: Se compararon catorce adultos con neuropatía auditiva con un grupo pareado en su percepción del habla acústicamente procesada, de pacientes con implante coclear. Resultados: El grupo de AN distinguió los pares CV a tasa de velocidad baja, en particular con modificaciones en la duración de las consonantes. Las habilidades de reconocimiento fonológico se deterioraron en los adultos con AN pero mejoraron con la prolongación del intervalo de tiempo inter-silábico. Conclusiones: Un programa de rehabilitación auditiva debe considerar las modificaciones temporales del lenguaje, el entrenamiento del procesamiento auditivo temporal y el uso de equipos con esquemas innovadores de procesamiento de la señal. Las modificaciones verbales así como las imágenes visuales parecen ser prometedoras estrategias compensatorias para remediar las afecciones en las habilidades del procesamiento fonológico. [ABSTRACT FROM AUTHOR]

Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these patients, and 49 patients tried cochlear implants. Approximately 15% reported some benefit from hearing aids for language learning, while improvement in speech comprehension and language acquisition was reported in 85% of patients who were implanted. Approximately 5% (13/260) of the total population developed normal speech and language without intervention. Patients were diagnosed at our laboratory (n=66) or referred from other sites (n=194), and all showed absent/grossly abnormal auditory brainstem responses (ABR), often ‘ringing’ cochlear microphonics, and the presence or history of otoacoustic emissions. Etiologies and co-existing conditions included genetic (n=41), peripheral neuropathies (n=20), perinatal jaundice and/or anoxia and/or prematurity (n=74). These patients comprise 10% or more of hearing impaired patients; their language acquisition trajectories are generally unpredictable from their audiograms. Sumario Se resumen los resultados de las pruebas y los datos del tratamiento de 260 pacientes con diagnóstico de Espectro de desórdenes de la Neuropatía Auditiva (ANSD). En 85 de estos pacientes se probó el uso de auxiliares auditivos y 49 pacientes recibieron un implante coclear. Aproximadamente 15% reportaron algún beneficio con los auxiliares auditivos para la adquisición del lenguaje mientras que el 85% de los que recibieron un implante reportaron una mejoría en la comprensión y la adquisición del lenguaje. Aproximadamente 5% (13/260) de la población total desarrolló lenguaje normal sin intervención. Los paciente fueron diagnosticados en nuestro laboratorio (n=66) o referidos de algún otro lado (n=194) y todos mostraron ausencia o anormalidad importante de los potenciales evocados (ABR), frecuentemente con una microfónica coclear “timbrante” y con presencia o historia de emisiones otoacústicas. La etiología o las condiciones co-existentes incluidas fueron: genéticas (n=41), neuropatías periféricas (n=20), ictericia perinatal y/o anoxia y/o prematurez (n=74). Estos pacientes representan 10% o más de los pacientes con hipoacusia; su trayectoria en el proceso de adquisición del lenguaje es generalmente impredecible a partir de sus audiogramas. [ABSTRACT FROM AUTHOR]

Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these patients, and 49 patients tried cochlear implants. Approximately 15% reported some benefit from hearing aids for language learning, while improvement in speech comprehension and language acquisition was reported in 85% of patients who were implanted. Approximately 5% (13/260) of the total population developed normal speech and language without intervention. Patients were diagnosed at our laboratory (n=66) or referred from other sites (n=194), and all showed absent/grossly abnormal auditory brainstem responses (ABR), often ‘ringing’ cochlear microphonics, and the presence or history of otoacoustic emissions. Etiologies and co-existing conditions included genetic (n=41), peripheral neuropathies (n=20), perinatal jaundice and/or anoxia and/or prematurity (n=74). These patients comprise 10% or more of hearing impaired patients; their language acquisition trajectories are generally unpredictable from their audiograms. Sumario Se resumen los resultados de las pruebas y los datos del tratamiento de 260 pacientes con diagnóstico de Espectro de desórdenes de la Neuropatía Auditiva (ANSD). En 85 de estos pacientes se probó el uso de auxiliares auditivos y 49 pacientes recibieron un implante coclear. Aproximadamente 15% reportaron algún beneficio con los auxiliares auditivos para la adquisición del lenguaje mientras que el 85% de los que recibieron un implante reportaron una mejoría en la comprensión y la adquisición del lenguaje. Aproximadamente 5% (13/260) de la población total desarrolló lenguaje normal sin intervención. Los paciente fueron diagnosticados en nuestro laboratorio (n=66) o referidos de algún otro lado (n=194) y todos mostraron ausencia o anormalidad importante de los potenciales evocados (ABR), frecuentemente con una microfónica coclear “timbrante” y con presencia o historia de emisiones otoacústicas. La etiología o las condiciones co-existentes incluidas fueron: genéticas (n=41), neuropatías periféricas (n=20), ictericia perinatal y/o anoxia y/o prematurez (n=74). Estos pacientes representan 10% o más de los pacientes con hipoacusia; su trayectoria en el proceso de adquisición del lenguaje es generalmente impredecible a partir de sus audiogramas. [ABSTRACT FROM AUTHOR]

Auditory neuropathy is a hearing disorder characterized by abnormal or absent auditory brainstem responses, and the presence of otoacoustic emissions and/or cochlear microphonics, indicating normal functioning of the outer hair cells. Here, subjects with auditory neuropathy, with near-normal hearing to moderate hearing loss, were tested using the TEN(HL) test for diagnosis of dead regions and also using psychophysical tuning curves (PTCs). Results for the majority of subjects met the TEN(HL)-test criteria at one or more frequencies (often at several or all frequencies). However, the PTCs did not show shifted tips. Hence, the positive results of the TEN(HL) test should not be interpreted as indicating the presence of dead regions. Rather, it appears that high thresholds in noise are caused by poor processing efficiency, perhaps associated with loss of neural synchrony. [ABSTRACT FROM AUTHOR]

The objectives of this study were to (a) estimate the prevalence of auditory dys-synchrony in Mysore, a city of one million population in Southern India and, (b) present the results of audiological testing of this clinical population as well as the relationship between these figures. A register-based study design was employed wherein the results of audiological tests of all patients who visited the Department of Audiology, All India Institute of Speech and Hearing between January 2000 and December 2003 were reviewed. Results showed that the prevalence of auditory dys-synchrony was around 1 in 183 in individuals with sensory neural hearing loss. Behavioural thresholds and speech identification scores were variable. Around 60% of the individuals had no measurable speech identification scores. There was no relation between the hearing thresholds and speech identification scores or between otoacoustic emissions and speech identification scores. These results indicate that auditory dys-synchrony is not an extremely rare disorder. [ABSTRACT FROM AUTHOR]

The primary goal of this study was to compare the characteristics of the post-implant evoked potentials with pre-implant evoked potentials in patients with auditory neuropathy (AN) or dys-synchrony. AN is typically characterized by sensorineural hearing loss, reduced speech perception, abnormal temporal processing, and unusual patterns of results with various objective audiological tests. In some cases, these patients may be appropriate candidates for a cochlear implant. In this article, we highlight evoked potential findings in two children diagnosed with AN who were provided with multichannel cochlear implants. Preoperative, interoperative and postoperative evoked potential measures show that the restoration of neural synchrony may occur at multiple levels of the auditory pathways in patients with AN when appropriate diagnostic tests, cochlear implantation and rehabilitation are provided. [ABSTRACT FROM AUTHOR]

Objective: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. Design: Narrative literature review. Results: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure. Conclusion: Early CI is recommended if bacterial meningitis causes profound sensorineural hearing loss (SNHL). CI in cochleovestibular malformation requires pre-operative imaging to plan surgical technique, choice of electrode, and to anticipate complication. Children with congenital severe to profound SNHL should undergo early bilateral simultaneous implantation, preferably before 12 months of age, except those with auditory neuropathy spectrum disorder who should be implanted after one year. Soft surgical technique should be deployed in an attempt to preserve any residual hearing. Otitis media with effusion is not a contraindication to cochlear implantation, but active or recurrent acute otitis media requires resolution of infection with grommet insertion pre-operatively. Device failure in CI recipients requires a stepwise audiological, medical, radiological, and device integrity assessment to determine the need for reimplantation. [ABSTRACT FROM AUTHOR]

Objective: The individual outcome after cochlear implantation in children with auditory synaptopathy/neuropathy (AS/AN) is difficult to predict. A tool for preoperative assessment would be helpful for counseling parents. This study evaluates the outcome after CI in children with AS/AN and with sensorineural hearing loss (SNHL), and correlates it with the preoperative ECochG results in order to find specific parameters of prognostic value., Design: The improvement of auditory behavior after CI was retrospectively assessed using the LittlEARS questionnaire and quantified in a score (LS). This score was correlated with the CAP/SP ratio in the preoperative ECochG. The score was further correlated with the patient's age six months following CI., Study Sample: Nine children with AS/AN were compared to nine children with SNHL., Results: Both groups showed a significant improvement in LS following CI. There was a significant positive correlation between the CAP/SP ratio and the improvement in LS in all children. The correlation between age and LS was significantly negative in the SNHL group and positive in the AS/AN group., Conclusion: All children with AS/AN and SNHL benefit to a similar extent from CI. The preoperatively assessed CAP/SP ratio has a prognostic value for the development of auditory behavior following CI.