Performance with an Auditory Brainstem Implant and Contralateral Cochlear Implant in Pediatric Patients.

OBJECTIVE: To assess bimodal auditory performance in children with a cochlear implant (CI) and contralateral auditory brainstem implant (ABI). METHODS: This is a retrospective case review performed at a tertiary referral center. Four patients with cochlear nerve deficiency initially underwent cochlear implantation but were not benefiting from their devices and underwent ABI in the contralateral ear. The main outcome measures included age-appropriate speech perception and production assessments. RESULTS: Three subjects performed better on their auditory perception assessments using both of their devices than with either device alone. One subject had only preliminary outcomes, but subjectively performed best with both devices. CONCLUSIONS: We observed continued improvement in CI performance over time, even if no benefit was evident before the decision for ABI. This could suggest that ABI and CI have a synergistic effect, or it could simply be the adaptive ability of the developing brain to utilize the signals coming from these devices. There is preliminary evidence to support choosing the ear contralateral to the CI for an ABI in a pediatric patient with bilateral cochlear nerve deficiency.

Electrically Evoked Auditory Event-Related Responses in Patients with Auditory Brainstem Implants: Morphological Characteristics, Test-Retest Reliability, Effects of Stimulation Level, and Association with Auditory Detection.

OBJECTIVE: This study aimed to (1) characterize morphological characteristics of the electrically evoked cortical auditory event-related potentials (eERPs) and explore the potential association between onset eERP morphology and auditory versus nonauditory stimulation; (2) assess test-retest reliability of onset eERPs; (3) investigate effects of stimulation level on onset eERPs; and (4) explore the feasibility of using the onset eERP to estimate the lowest stimulation level that can be detected for individual stimulating electrodes in patients with auditory brainstem implants (ABIs). DESIGN: Study participants included 5 children (S1 to S5) and 2 adults (S6 to S7) with unilateral Cochlear Nucleus 24M ABIs. Pediatric ABI recipients ranged in age from 2.6 to 10.2 years (mean: 5.2 years) at the time of testing. S6 and S7 were 21.2 and 24.6 years of age at the time of testing, respectively. S6 and S7 were diagnosed with neurofibromatosis II (NF2) and implanted with an ABI after a surgical removal of the tumors. All pediatric subjects received ABIs after being diagnosed with cochlear nerve deficiency. The lowest stimulation level that could be detected (behavioral T level) and the estimated maximum comfortable level (C level) was measured for individual electrodes using clinical procedures. For electrophysiological measures, the stimulus was a 100-msec biphasic pulse train that was delivered to individual electrodes in a monopolar-coupled stimulation mode at stimulation levels ranging from subthreshold to C levels. Electrophysiological recordings of the onset eERP were obtained in all subjects. For studies evaluating the test-retest reliability of the onset eERP, responses were measured using the same set of parameters in two test sessions. The time interval between test sessions ranged from 2 to 6 months. The lowest stimulation level that could evoke the onset eERP was defined as the objective T level. RESULTS: Onset eERPs were recorded in all subjects tested in this study. Inter- and intrasubject variations in morphological characteristics of onset eERPs were observed. Onset eERPs with complex waveforms were recorded for electrodes that evoked nonauditory sensations, based on feedback from subjects, as well as for electrodes without any indications of nonauditory stimulations. Onset eERPs in patients with ABIs demonstrated good test-retest reliability. Increasing stimulation levels resulted in increased eERP amplitudes but showed inconsistent effects on response latencies in patients with ABIs. Objective and behavioral T levels were correlated. CONCLUSIONS: eERPs could be recorded in both non-NF2 and NF2 patients with ABIs. eERPs in both ABI patient groups show inter- and intrasubject variations in morphological characteristics. However, onset eERPs measured within the same subject in this study tended to be stable across study sessions. The onset eERP can potentially be used to estimate behavioral T levels in patients with ABIs. Further studies with more adult ABI recipients are warranted to investigate whether the onset eERP can be used to identify electrodes with nonauditory stimulations.

Cochlear implantation for a child with cochlear nerve deficiency: parental perspectives explored through narrative.

OBJECTIVE: The objective of this study was to explore, from the parents' perspectives, decision-making regarding a cochlear implant (CI) for their child when a favourable outcome is less likely because of abnormal neurophysiology. DESIGN: The primary research method of this single case study was qualitative interviewing drawing on a narrative approach to elicit the parents' perspectives about their experiences over time. Each parent was interviewed separately, but thematic analyses were undertaken both within and across interviews in order to identify overlaps and differences. STUDY SAMPLE: Participants included the parents of a five-year old child with severe-profound hearing loss, cochlear nerve deficiency, and bilateral common cavities who had received a CI at the age of 18 months. RESULTS: Four themes were identified across the four narrative stages that emerged from the parents' accounts of their experiences regarding their daughter's CI. Themes included hope and despair, questioning professionals' motivations, does deafness need a cure, and bringing the child into the family. Although these themes emerged from both parents' accounts, each parent expressed different perspectives and insights within them. CONCLUSIONS: Findings highlight the central place of parental needs and perspectives in decision-making regarding a CI, particularly in the context of uncertain outcomes.

Electrophysiologic and behavioral outcomes of cochlear implantation in children with auditory nerve hypoplasia.

OBJECTIVES: Hypoplasia of the auditory nerve (AN) refers to significant narrowing of the VIIIth cranial nerve which could compromise stimulation of the nerve by electrical pulses delivered from a cochlear implant (CI), thereby hindering activity in other parts of the auditory pathways. To compensate, high current levels or increased charge may be required to elicit auditory perception causing current to spread to other cranial nerves and potentially resulting in unwanted myogenic responses. Deficits in central auditory activity could reduce perception of speech and language. In the present study, we measured auditory brainstem responses in children with and without hypoplasia of the AN to answer the following questions. In children with hypoplastic ANs, (a) can CI stimulation evoke typical patterns of activity from the AN and brainstem?, (b) do brainstem responses change with CI experience?, (c) are evoked responses dependent on the size of the AN pathway?, and (d) does auditory development measured by behavioral tests of speech perception develop more slowly than in peers with normal AN diameter? DESIGN: Of 807 children using CIs in our program, 20 (2.5%) were identified as having AN hypoplasia using high-resolution computed tomographic scan and/or magnetic resonance imaging. An age-matched control group of children using CIs with normal AN diameter were recruited to compare electrophysiological and behavioral measures. Radiologic imaging was used to measure the diameter of the internal auditory canal (IAC), auditory nerve canal (ANC), and AN. Electrophysiological testing of the evoked compound action potential and auditory brainstem response was performed at CI activation and every 3 mo after initial testing up to 2 yr. Peak latency and waveform morphology were compared between study and control groups. Tests of speech perception and discrimination were attempted every 12 mo after device activation up to 10 yr. RESULTS: : Hypoplastic AN was identified as moderate to critical stenosis of the IAC, ANC, and AN. Initial electrically evoked compound action potential responses were mostly absent in children with AN hypoplasia. In the time window when electrically evoked auditory brainstem responses would be expected, some responses included single amplitude peaks at normal wave eV latencies, but the majority were abnormal, with peaks at atypical latencies or with no observable wave peaks. All evoked responses were inconsistent over time and did not reflect a typical pattern of auditory brainstem development. Speech perception scores were significantly poorer in the study group compared with controls and did not improve with CI experience. The type of abnormal evoked waveform response was independent of IAC, ANC, or AN diameter and also independent of behavioral outcome measures. CONCLUSIONS: : Evoked responses recorded in CI children with AN hypoplasia indicate a high incidence of nonauditory activity with CI use. The range of abnormal responses was not predicted by the severity of the hypoplastic AN or associated structures. This, along with poorer auditory development compared with peers with normal AN diameters, suggests that children with hypoplasia of the AN are poor candidates for cochlear implantation.

[Vibrotactile neurofeedback training with the Vertiguard®-RT-system. A placebo-controlled double-blinded pilot study on vestibular rehabilitation]. / Vibrotaktiles Neurofeedbacktraining mit dem Vertiguard®-RT-System. Placebokontrollierte Doppelblind-Pilot-Studie zur Rehabilitation von Gleichgewichtsstörungen.

BACKGROUND: The success of neurofeedback training for vestibular rehabilitation depends largely on the type of feedback signal as well as on the specific parameters. Ideal training should be based on the individual balance deficits in patients in everyday situations. The present study is therefore aimed at investigating the therapeutic outcome of a new vibrotactile neurofeedback system for the first time in a pilot study. PATIENTS AND METHODS: A total of 36 patients performed daily vibrotactile neurofeedback training (for 10 days) based on a sway analysis on the first day. The reduction of body sway and vertigo symptom scale (VSS) scores were calculated after the training and compared with those of the placebo group. RESULTS: All five patient groups (characterized by different vestibular disorders) which performed the training with the correct feedback signal showed reduced body sway and VSS score (n=30). This effect was not visible in the placebo group. CONCLUSIONS: Individualized vibrotactile neurofeedback training as presented here with the Vertiguard® system appeared to improve balance during daily activities in all patient groups investigated, but not in controls. Future studies should investigate the efficacy of this new method in a larger sample as well as its long term effects.

Psychotherapy in dizziness: a systematic review.

BACKGROUND: About 30-50% of complex dizziness disorders are organically not sufficiently explained or related to a psychiatric disorder. Of patients with such dizziness disorders, 80% are severely impaired by dizziness in their daily and working lives; nevertheless, they are often not diagnosed or treated adequately. OBJECTIVES: This review aims to give a systematic overview of psychotherapeutic approaches and their efficacy regarding the treatment of dizziness that is medically not sufficiently explained or related to a psychiatric disorder. METHODS: A systematic literature search was conducted in Medline, PSYNDEX and PsycINFO. Included in this systematic review were (randomised) controlled trials ((R)CTs) concerning psychotherapy in patients with dizziness, medically not sufficiently explained or associated with a psychiatric disorder. If possible, Hedges' g was used to express the effect sizes (ES) of the treatment. Heterogeneity was assessed using the Q statistic. In addition, the quality of the studies was rated. RESULTS: Three (R)CTs were included. All studies used cognitive-behavioural treatment methods in combination with relaxation techniques or vestibular rehabilitation. All studies suggested that psychotherapy may provide improvement. The mean ES in the treatment groups was 0.46 (95% CI 0.05 to 0.88) for dizziness related outcome, 0.10 (-0.44 to 0.64) for anxiety and 0.17 (-0.24 to 0.58) for depression whereas in the control groups the mean dizziness related ES was -0.04 (-0.44 to 0.37), anxiety related ES was -0.03 (-0.43 to 0.38) and depression related ES was -0.02 (-0.42 to 0.38). The quality of the studies was average. Sample sizes were small, however, and there was a lack of long term studies. CONCLUSION: This systematic review provides some preliminary evidence that psychotherapy may be effective in patients with dizziness that is medically not sufficiently explained or due to a psychiatric disorder. The results should be replicated in larger samples and follow-up RCTs.

Brain stem responses evoked by stimulation with an auditory brain stem implant in children with cochlear nerve aplasia or hypoplasia.

OBJECTIVES: The inclusion criteria for an auditory brain stem implant (ABI) have been extended beyond the traditional, postlingually deafened adult with Neurofibromatosis type 2, to include children who are born deaf due to cochlear nerve aplasia or hypoplasia and for whom a cochlear implant is not an option. Fitting the ABI for these new candidates presents a challenge, and intraoperative electrically evoked auditory brain stem responses (EABRs) may assist in the surgical placement of the electrode array over the dorsal and ventral cochlear nucleus in the brain stem and in the postoperative programming of the device. This study had four objectives: (1) to characterize the EABR by stimulation of the cochlear nucleus in children, (2) to establish whether there are any changes between the EABR recorded intraoperatively and again just before initial behavioral testing with the device, (3) to establish whether there is evidence of morphology changes in the EABR depending on the site of stimulation with the ABI, and (4) to investigate how the EABR relates to behavioral measurements and the presence of auditory and nonauditory sensations perceived with the ABI at initial device activation. DESIGN: Intra- and postoperative EABRs were recorded from six congenitally deaf children with ABIs, four boys and two girls, mean age 4.2 yrs (range 3.2 to 5.0 yrs). The ABI was stimulated at nine different bipolar sites on the array, and the EABRs recorded were analyzed with respect to the morphology and peak latency with site of stimulation for each recording session. The relationship between the EABR waveforms and the presence or absence of auditory electrodes at initial device activation was investigated. The EABR threshold levels were compared with the behavioral threshold (T) and comfortably loud (C) levels of stimulation required at initial device activation. RESULTS: EABRs were elicited from all children on both test occasions. Responses contained a possible combination of one to three peaks from a total of four identifiable peaks with mean latencies of 1.04, 1.81, 2.61, and 3.58 msecs, respectively. The presence of an EABR was a good predictor of an auditory response; however, the absence of the EABR was poor at predicting a site with no auditory response. The morphology of EABRs often varied with site of stimulation and between EABR test occasions. Postoperatively, there was a trend for P1, P3, and P4 to be present at the lateral end of the array and P2 at the medial end of the array. Behavioral T and C levels showed a good correlation with postoperative EABR thresholds but a poor correlation with intraoperative EABR thresholds. CONCLUSIONS: The presence of an intraoperative EABR was a good indicator for the location of electrodes on the ABI array that provided auditory sensations. The morphology of the EABR was often variable within and between test sessions. The postoperative EABR thresholds did correlate with the behavioral T and C levels and could be used to assist with initial device fitting.

Auditory neuropathy: unexpectedly common in a screened newborn population.

Auditory neuropathy, or dyssynchrony, is defined by an abnormal or absent auditory brainstem response but intact otoacoustic emissions or cochlear microphonics. It is associated with impaired hearing on behavioural pure-tone audiometry, absent acoustic reflexes, and poor speech perception, particularly in noisy environments. These results suggest a disorder of inner hair-cell and or eighth-nerve function. We describe a case-note survey of patients with and without auditory neuropathy, using data from the local newborn hearing screening programme collected prospectively from 2002 to 2007. During this period, 45 050 infants were screened with otoacoustic emissions, 30 patients were diagnosed with suspected severe to profound hearing loss (16 males, 14 females), and 12 of those 30 had auditory neuropathy (six males, six females). Mean gestational age was 33 weeks 1 day in the auditory neuropathy group and 35 weeks in the non-auditory neuropathy group. The most significant risk factors for auditory neuropathy were hyperbilirubinaemia (p=0.018), sepsis (p=0.024), and gentamicin exposure (p=0.024). Children with auditory neuropathy comprise a subgroup of patients with hearing impairment involving different pathologies most commonly associated with the risk factors related to admission to neonatal intensive care units. Improvement is possible with maturity, at least in a minority.

Tratamiento rehabilitador de los trastornos del equilibrio. Diseño de un protocolo general con la posturografía dinámica computerizada / Rehabilitation treatment of the balance: design of a general protocol with dynamic computerized posturography

La posturografía dinámica (PD) es un método ampliamente difundido para el estudio del equilibrio. Su principal utilidad es el diseño de estrategias terapéuticas basadas en la rehabilitación vestibular. En determinados pacientes y con apoyo de otros elementos de rehabilitación la PD favorece la compensación del deterioro secundario a determinadas entidades patológicas que afectan al sistema vestibular periférico o central. Normalmente se emplean estrategias individualizadas por cada paciente, y aunque esto es lo más correcto, consideramos necesario proponer unas pautas globales que faciliten la programación de la rehabilitación y la comparación de los resultados obtenidos por distintos autores

The dynamic posturography (PD) is an amply diffused method for the study of balance. Its principal utility is the design of therapeutic strategies based on the vestibular rehabilitation. In determined patients and with the help of other elements of rehabilitation the PD favour the compensation of the degradation of the secondary balance to determinated pathologic entities that affect the vestibular peripheric or central system. Normally individualized strategies for each patient are employed and although this is the more correct, we consider necessary to propose global measures that facilitate the rehabilitation program and the comparison of the results of different authors

[Clinical and audiological findings in children with auditory neuropathy]. / Klinische und audiologische Befunde von Kindern mit auditorischer Neuropathie und ihre Versorgung mit einem Cochlea-Implantat.

BACKGROUND: Auditory neuropathy is a disorder characterised by preservation of outer hair cells function with normal otoacoustic emissions (OAEs), but with absent auditory brainstem responses (ABR). Perisynaptic synchronisation disorder is one of the possible pathogenesis underlying auditory neuropathy. In this paper we describe the clinical presentation and audiological findings in pediatric auditory neuropathy and its management. PATIENTS AND METHODS: 9 children with auditory neuropathy could be included in the study. An audiological evaluation was performed in all children including behavioural audiometry, measurement of the OAEs as well as electrocochleography (ECoG) and ABR recordings. Children who failed to get any benefit from conventional amplification received a cochlear implant. Prior to implantation the responses to electrical stimuli were examined with the promontory test and with the electrically evoked ABR. RESULTS: One child showed auditory neuropathy only on one side with normal hearing thresholds on the contralateral ear. Another child had normal hearing thresholds after the follow up period. Four children received a hearing aid. But variable hearing reactions were observed. Thus in three cases a CI is planned. In three children cochlea implantation was done. Following implantation a remarkable improvement in hearing/speech capabilities with the CI compared to conventional hearing aids were observed in all three cases. Beside, these three children developed open set speech discrimination and are using now oral language for communication. CONCLUSIONS: Auditory neuropathy is a disorder which presents with different clinical and audiological findings. Thus the management of this disorder must be an individual one. In light of our findings we support the use of cochlear implants as an option for children with auditory neuropathy in cases where conventional amplification does not work sufficiently.

Vestibular rehabilitation decreases fall risk and improves gaze stability for an older individual with unilateral vestibular hypofunction.

BACKGROUND AND PURPOSE: Partial or total unilateral vestibular loss is the third most common cause of peripheral vestibular dysfunction. Dysfunction of one or both of the vestibular mechanisms can manifest physically as abnormalities of posture, balance, and/or visual acuity. This case report describes physical therapy examination and individualized intervention with vestibular rehabilitation for a patient with unilateral vestibular hypofunction. CASE DESCRIPTION: The patient was an 80-year-old male with electronystamographically confirmed unilateral vestibular loss of 98.3%. He demonstrated altered balance and gaze stability classifying him as having an increased risk for falling. OUTCOMES: After 5 weeks of individualized vestibular rehabilitation, the patient significantly decreased his fall risk from 11 to 20 of 24 on the Dynamic Gait Index. His gaze stability also improved from a 4 to 1 line disparity with dynamic visual acuity testing. The patient also had a decrease in perceived disability on the Dizziness Handicap Inventory from 30/100 at evaluation to 12/100 at discharge. CONCLUSION: Individualized vestibular rehabilitation decreased fall risk and improved gaze stability for a patient with significant unilateral vestibular hypofunction.

[Diagnosis and therapy of auditory synaptopathy/neuropathy]. / Diagnostik und Therapie der auditorischen Synaptopathie/Neuropathie.

Pathological auditory brainstem responses (lack of responses, elevated thresholds and perturbed waveforms) in combination with present otoacoustic emissions are typical audiometric findings in patients with a hearing impairment that particularly affects speech comprehension or complete deafness. This heterogenous group of disorders first described as "auditory neuropathy" includes dysfunction of peripheral synaptic coding of sound by inner hair cells (synaptopathy) and/or of the generation and propagation of action potentials in the auditory nerve (neuropathy). This joint statement provides prevailing background information as well as recommendations on diagnosis and treatment. The statement focuses on the handling in the german language area but also refers to current international statements.

Speech perception in individuals with auditory neuropathy.

PURPOSE: Speech perception in participants with auditory neuropathy (AN) was systematically studied to answer the following 2 questions: Does noise present a particular problem for people with AN? Can clear speech and cochlear implants alleviate this problem? METHOD: The researchers evaluated the advantage in intelligibility of clear speech over conversational speech in 13 participants with AN. Of these participants, 7 had received a cochlear implant. Eight sentence-recognition experiments were conducted to examine the clear speech advantage in 2 listening conditions (quiet and noise) using 4 stimulation modes (monaural acoustic, diotic acoustic, monaural electric, and binaurally combined acoustic and electric stimulation). RESULTS: Participants with AN performed more poorly in speech recognition in noise than did the normal-hearing, cochlear-impaired, and cochlear implant controls. A significant clear speech advantage was observed, ranging from 9 to 23 percentage points in intelligibility for all listening conditions and stimulation modes. Electric stimulation via a cochlear implant produced significantly higher intelligibility than acoustic stimulation in both quiet and in noise. Binaural hearing with either diotic acoustic stimulation or combined acoustic and electric stimulation produced significantly higher intelligibility than monaural stimulation in quiet but not in noise. CONCLUSIONS: Participants with AN most likely derive the clear speech advantage from enhanced temporal properties in clear speech and improved neural synchrony with electric stimulation. Although the present result supports cochlear implantation as one treatment choice for people with AN, it suggests that the use of innovative hearing aids may be another viable option to improve speech perception in noise.

Open set speech perception with auditory brainstem implant?

OBJECTIVE: Only a small percentage of auditory brainstem implant (ABI) recipients treated for neurofibromatosis type 2 (NF2) have proved capable of identifying words using only the sound from the ABI. Recently, the ABI was applied to a series of patients with no cochlear nerve or with cochlear disorders that could not benefit from a cochlear implant (i.e., cochlear nerve aplasia or posttraumatic avulsion) or whose benefit was or would be severely compromised. A significant number of these patients have proven capable of understanding speech, including effortless telephone use. In the present study, a series of psychophysical tests were administered to determine the cause of the difference in performance between tumor (T) and nontumor (NT) ABI patients. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Twenty patients with ABIs participated in the investigation. Ten were NF2 patients and 10 NT subjects. Patient ages ranged from 24 to 61 years. Eleven were males and nine females. INTERVENTION: Auditory rehabilitation in auditory disconnection caused by cochlea or cochlear nerve disorders. RESULTS: There was a significant correlation between modulation detection thresholds and speech understanding and a significant difference in modulation detection between T and NT patients. CONCLUSIONS: The difference in modulation detection between the two groups suggests a difference in the survival of specific cells in the cochlear nucleus that support modulation. The pattern of results indicates a separate pathway of auditory processing that is specialized for modulated sounds, and that pathway is critical for speech understanding. In NF2 patients, the tumor and surgery may selectively damage this pathway, resulting in poor speech recognition with prosthetic stimulation.

Drop attacks and vertical vertigo after transtympanic gentamicin: diagnosis and management.

Drop attacks represent a significant problem during the natural course of Meniere's disease. They are characterized by a sudden fall to the ground without loss of consciousness. Diagnosis is clinical and based on the typical description of the patient. Involvement of vertical canal is possible during Meniere's disease and also after gentamicin application. Treatment of drop attacks is still a matter of discussion; most cases have a benign course with spontaneous remission and no treatment is necessary. In severe cases, aggressive treatment (surgical or pharmacological) is necessary. A case of drop attack associated with vertical vertigo is presented. Vestibular tests were performed in order to assess the involvement of inner ear. Caloric test and ice water test reveal no response. Vestibular Evoked Myogenic Potentials are present even after high doses of gentamicin. Drop attacks and vertical vertigo can occur after transtympanic gentamicin and can be well managed with high doses of local gentamicin.

Efficacy of vestibular rehabilitation on chronic unilateral vestibular dysfunction.

OBJECTIVE: To assess the efficacy of vestibular rehabilitation exercises on patients with chronic unilateral vestibular dysfunction. DESIGN: Prospective study. SETTING: Physical Medicine and Rehabilitation Clinic and Otolaryngology Clinic of a tertiary referral hospital. SUBJECTS: One-hundred and twenty-five patients with unilateral chronic vestibular dysfunction were included in the study. INTERVENTIONS: Eight-week, two-staged (clinic and home) vestibular rehabilitation programme with components of Cawthorne-Cooksey and Norre exercises was applied. MAIN OUTCOME MEASURES: Dizziness Handicap Inventory (DHI) and visual analogue scale (VAS) were completed three times (at the beginning, end of the second week and end of the treatment). RESULTS: Data for 112 patients in the first stage and 93 patients in the second stage were evaluated because of insufficient compliance of the other patients. The mean DHI score was decreased from 50.42 +/- 24.12 points to 21.21 +/- 15.97 points (p < 0.001) at the end of first two weeks, and to 19.93 +/- 19.33 points at the end of the whole treatment. The mean VAS score was decreased from 5.87 +/- 2.27 to 2.02 +/- 1.75 (p < 0.001) at the end of second week, and to 1.51 +/- 1.29 at the end of eighth week. In respect to both VAS and DHI scores, improvement was noted in 67 patients (77.4%). Age, gender and disability level had no predictive value about therapy outcome. CONCLUSIONS: There was a fast recovery in the supervised exercise session, whereas there was no significant difference in the home exercise session. These findings suggest that either supervised exercise is better than home exercise or that 10 supervised sessions are sufficient to get the end result.

Auditory neuropathy/dys-synchrony: diagnosis and management.

Auditory brainstem responses (ABRs) and otoacoustic emissions (OAEs) are objective measures of auditory function, but are not hearing tests. Normal OAEs reflect normal cochlear outer hair cell function, and an ABR indicates a synchronous neural response. It is quite possible for a patient to have normal OAEs but absent or grossly abnormal ABR and a behavioral audiogram that is inconsistent with either test. These patients, who may constitute as much as 10% of the diagnosed deaf population, have auditory neuropathy/dys-synchrony (AN/AD). To diagnose AN/AD accurately, ABRs are obtained in response to condensation and rarefaction clicks to distinguish cochlear microphonics (CM) from neural responses. Appropriate management is confounded by variation among patients and changes in auditory function in some patients over time. Recommendations for management include visual language exposure through methods such as American Sign Language (ASL), Cued Speech, or baby signs, and closely following patients.

Electrical stimulation of the auditory nerve via cochlear implants in patients with auditory neuropathy.

Auditory neuropathy (AN) is a term used to describe an auditory disorder in which there is evidence of normal outer hair cell function (otoacoustic emissions and/or cochlear microphonics) and poor function of the auditory nerve (absent or highly distorted auditory brain stem response starting with wave I). Many of these patients have evidence of generalized peripheral nerve disease, leading to an assumption that the peripheral portion of the auditory nerve is the most likely site of lesion. A small group of these patients has received cochlear implants, and the majority of them achieve average to above-average performance. Although this outcome may seem incongruous with neural disease, average performance by patients with AN may be a result of the reintroduction of neural synchrony by electrical stimulation and/or the fact that most deaf patients have poor nerve survival. Although cochlear implants are promising for deaf patients with AN, more study of the disorder is needed.