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Objective: To analyse clinical information from a cohort of 877 cochlear implant (CI) surgeries performed in patients with inner ear malformations and report our experience with this procedure. Methods: Clinical information on patients with inner ear malformation who underwent CI surgery in our department from January 2010 to April 2017 was analysed. Results: Seven hundred and fifty‐four CI surgeries (86.0%) were uneventful but cerebrospinal fluid gusher occurred during surgery in 118 cases (13.5%). Not including the patients lost to follow‐up, postoperative Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were collected from 13 patients with common cavity deformity, 38 with IP‐I, 11 with hypoplasia, 13 with IP‐III, 285 with IP‐II and 43 with cochlear nerve deficiency. The mean CAP scores were 2.55, 3.39, 3.45, 3.77, 3.98, 2.95 and 2.90, and the mean SIR scores were 1.54, 3.68, 3.72, 3.54, 3.80, 2.31 and 2.32. Patients with common cavity deformity who underwent surgery using the traditional facial recess approach or transmastoid slotted labyrinthotomy approach had significantly better post‐activation scores on the CAP, SIR and Meaningful Auditory Integration Scale/Infant‐Toddler Meaningful Auditory Integration Scale, with no significant differences between the two approaches. Conclusion: The traditional facial recess approach can be successful, and several approaches may be used for electrode insertion and gusher control in patients with a severe deformity, particularly common cavity deformity and IP‐III. A gusher was the most common intraoperative finding and occurred most often in patients with IP‐III. Careful consideration of the type of electrode used is important. [ABSTRACT FROM AUTHOR]

Objectives: Speech materials validated with normal-hearing listeners may not be appropriate for clinical assessment of cochlear implant (CI) users. The aim of this study was to validate list equivalency of the Mandarin Speech Perception (MSP) sentences, disyllables, and monosyllables in Mandarin-speaking CI patients. Design: Recognition of MSP sentences, disyllables, and monosyllables each were measured for all 10 lists. Study sample: 67 adult and 32 pediatric Mandarin-speaking CI users. Results: There was no significant difference between adult and pediatric subject groups for all test materials. Significant differences were observed among lists within each test. After removing one or two lists within each test, no significant differences were observed among the remaining lists. While there was equal variance among lists within a given test, the variance was larger for children than for adults, and increased from monosyllables to disyllables to sentences. Conclusions: Some adjustment to test lists previously validated with CI simulations was needed to create perceptually equivalent lists for real CI users, suggesting that test materials should be validated in the targeted population. Differences in mean scores and variance across test materials suggest that CI users may differ in their ability to make use of contextual cues available in sentences and disyllables. [ABSTRACT FROM AUTHOR]

For deaf individuals with residual low-frequency acoustic hearing, combined use of a cochlear implant (CI) and hearing aid (HA) typically provides better speech understanding than with either device alone. Because of coarse spectral resolution, CIs do not provide fundamental frequency (F0) information that contributes to understanding of tonal languages such as Mandarin Chinese. The HA can provide good representation of F0 and, depending on the range of aided acoustic hearing, first and second formant (F1 and F2) information. In this study, Mandarin tone, vowel, and consonant recognition in quiet and noise was measured in 12 adult Mandarin-speaking bimodal listeners with the CI-only and with the CI+HA. Tone recognition was significantly better with the CI+HA in noise, but not in quiet. Vowel recognition was significantly better with the CI+HA in quiet, but not in noise. There was no significant difference in consonant recognition between the CI-only and the CI+HA in quiet or in noise. There was a wide range in bimodal benefit, with improvements often greater than 20 percentage points in some tests and conditions. The bimodal benefit was compared to CI subjects’ HA-aided pure-tone average (PTA) thresholds between 250 and 2000 Hz; subjects were divided into two groups: “better” PTA (<50 dB HL) or “poorer” PTA (>50 dB HL). The bimodal benefit differed significantly between groups only for consonant recognition. The bimodal benefit for tone recognition in quiet was significantly correlated with CI experience, suggesting that bimodal CI users learn to better combine low-frequency spectro-temporal information from acoustic hearing with temporal envelope information from electric hearing. Given the small number of subjects in this study (n = 12), further research with Chinese bimodal listeners may provide more information regarding the contribution of acoustic and electric hearing to tonal language perception. [ABSTRACT FROM AUTHOR]

Purpose: In this study, the authors aimed to identify speech information processed by a hearing aid (HA) that is additive to information processed by a cochlear implant (CI) as a function of signal-to-noise ratio (SNR). Method: Speech recognition was measured with CI alone, HA alone, and CI + HA. Ten participants were separated into 2 groups; good (aided pure-tone average [PTA]<55dB) and poor (aided PTA ≥ 55 dB) at audiometric frequencies > 1 kHz in HA. Results: Results showed that the good-aided PTA group derived a clear bimodal benefit (performance difference between CI + HA and CI alone) for vowel and sentence recognition in noise, whereas the poor-aided PTA group received little benefit across speech tests and SNRs. Results also showed that a better aided PTA helped in processing cues embedded in both low and high frequencies; none of these cues was significantly perceived by the poor-aided PTA group. Conclusions: The aided PTA is an important indicator for bimodal advantage in speech perception. The lack of bimodal benefits in the poor group may be attributed to the nonoptimal HA fitting. Bimodal listening provides a synergistic effect for cues in both low- and high-frequency components in speech. [ABSTRACT FROM AUTHOR]

Objective: The full benefit of bilateral cochlear implants may depend on the unilateral performance with each device, the speech materials, processing ability of the user, and/or the listening environment. In this study, bilateral and unilateral speech performances were evaluated in terms of recognition of phonemes and sentences presented in quiet or in noise. Design: Speech recognition was measured for unilateral left, unilateral right, and bilateral listening conditions; speech and noise were presented at 0°° azimuth. The ''binaural benefit'' was defined as the difference between bilateral performance and unilateral performance with the better ear. Study sample: Nine adults with bilateral cochlear implants participated. Results: On average, results showed a greater binaural benefit in noise than in quiet for all speech tests. More importantly, the binaural benefit was greater when unilateral performance was similar across ears. As the difference in unilateral performance between ears increased, the binaural advantage decreased; this functional relationship was observed across the different speech materials and noise levels even though there was substantial intra- and inter-subject variability. Conclusions: The results indicate that subjects who show symmetry in speech recognition performance between implanted ears in general show a large binaural benefit. Sumario Objetivo: Se estudiaron los datos audiolóógicos de investigacióón epidemiolóógica basada en una poblacióón de adultos mayores. Se buscaron diagnóósticos especííficos de alteraciones otolóógicas y audiolóógicas que pueden ocasionar hipoacusia. Diseñño: Estudio retrospectivo de registros. Muestra: Se estudiaron tres cohortes etááreas, 474 individuos de 70 a 75 añños (''jóóvenes) y 252 individuos de 85 añños o mayores (''viejos''). Se utilizaron las audiometríías de tonos puros y de lenguaje. Se incluyeron los datos de sus expedientes méédicos. Resultados: Se diagnosticóó hipoacusia conductiva en 6.1% del grupo de ''jóóvenes'' y un 10.3% de los ''viejos''. Se establecieron diagnóósticos especfíícos (otitis media cróónica y otoesclerosis) en cerca de la mitad de los casos. Se diagnosticóó hipoacusia sensorineural no relacionada con la edad ni inducida por ruido en 3.4 y 5.2% de los casos. El 0.4% del grupo de ''jóóvenes'' y el 10% del grupo de ''viejos'' presentaban un reconocimiento del lenguaje severamente afectado, posiblemente relacionado con neuropatíía auditiva por edad. El 3.2% de las personas de 85 añños de edad presentaban sordera funcional bilateral, pero éésta no se presentóó en el grupo de 70--75 añños. Conclusiones: La incidencia de neuropatííaa auditiva probablemente relacionada con la edad aumenta considerablemente de los 70--75 añños a los 85. Existen marcadas diferencias en cuando a hipoacusia que afecta severamente la comunicacióón entre los grupos de ''jóóvenes'' y ''viejos'' en la poblacióón estudiada. [ABSTRACT FROM AUTHOR]

Cochlear implantation is safe for simple case presentations. However, facial nerve (FN) aberrations may impede insertion of the electrode through a round window and increase the risk of iatrogenic FN injury. We developed a novel method of cochlear implantation, a present the case of a child with FN aberrations. Inferior displacement of a horizontal segment of FN lay on the surface of th e promontory, obscuring the round window. The oval window was used and the electrode successfully inserted. No postoperative complications occurred, and significant improvement in speech perception was achieved. This novel approach is suitable for patients with FN aberrations. [ABSTRACT FROM AUTHOR]

Purpose: Due to the specificity of cochlear implantation (CI) programming parameters and outcomes in cochlear nerve deficiency (CND) patients, this study aimed to investigate the correlation between programming parameters and outcomes and further compare the difference between normal and CND groups. Methods: Ninety (95 ears) CND patients (normal cochlea, 39; malformed cochlea, 56) and seventy-nine (81 ears) normal cochlea patients who underwent CI surgery with either Med-El or Cochlear devices were included. The programming parameters and outcomes evaluated by the questionnaires were collected and compared among the normal CND, malformed CND, and normal groups in the two device groups, and their correlation was analyzed. Results: In the CND group, a reduced stimulation rate, higher pulse width, and triphasic pulse were needed in some cases. The stimulus levels of the CND group were significantly higher than that of the normal group (p < 0.05), but the outcomes of the CND group were significantly worse than that of the normal group (p < 0.05), and the stimulus level was significantly correlated with the outcomes (p < 0.05). However, there was no difference between normal and malformed CND groups. The non-auditory response was observed in the CND group, especially the ones with malformations. Conclusion: The CI programming parameters of some CND patients need to be adjusted, and a slower stimulation rate and higher pulse width are required sometimes. CND patients need a higher stimulus level than normal patients but their outcomes are poorer. Non-auditory response should be noticed in CND patients during programming. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to assess the correlation between residual hearing and audiologic outcomes after cochlear implant (CI) surgery in patients with cochlear nerve deficiency (CND). Methods: This retrospective study included 57 patients with CND who underwent CI surgery. Patients were divided into four groups according to hearing level (80–95, 95–110, 110–120, and >120 dB) and three groups according to residual hearing (entire spectrum hearing, partial spectrum hearing, and no spectrum hearing) based on the measured response at each frequency. Auditory performance (categorical auditory performance [CAP], Infant–Toddler Meaningful Auditory Integration Scale [IT‐MAIS]) and speech perception (speech intelligibility rating [SIR] and meaningful use of speech scale [MUSS]) were assessed before and 2 years after the surgery. Results: Forty‐seven (82.5%) patients had complete or total hearing loss (≥95 dB) and 17 (29.8%) had no spectrum hearing before CI surgery. Twenty‐nine (50.9%) patients did not exhibit residual hearing at 4 kHz. All patients demonstrated an improvement in auditory performance and speech perception: the CAP score in the 80–95 dB group was significantly higher than that in the 110–120 and >120 dB groups, and the entire spectrum hearing group showed significantly higher CAP, SIR, and IT‐MAIS scores than the partial spectrum hearing group and significantly higher CAP, SIR, IT‐MAIS, and MUSS scores than the no spectrum hearing group. Conclusion: For patients with CND, residual hearing, especially high‐frequency residual hearing, was poor and postoperative audiologic outcomes were significantly associated with the range of residual hearing. Level of Evidence: 4 [ABSTRACT FROM AUTHOR]

Cochlear ossification (CO) after meningitis can cause profound sensorineural hearing loss (SNHL). Cochlear implantation (CI) is the ideal treatment strategy for CO. To explore the strategy for CI in patients with CO after meningitis. In this retrospective study, the medical records of patients diagnosed with profound SNHL due to CO after meningitis and who underwent CI in our department between September 2010 and September 2021 were collected and reviewed. Their imaging and surgical findings were analyzed. The data of 26 patients with unilateral CI were reviewed. All patients underwent preoperative temporal high-resolution computed tomography (HRCT) and 22 patients magnetic resonance imaging (MRI). The sensitivity of HRCT was 61.5% (10/26), whereas that of MRI was 81.8% (18/22). Combined HRCT and MRI achieved a detection rate of 92.3% (24/26). Twenty-two and four patients underwent complete and partial electrode implantations, respectively. Preoperative temporal bone HRCT and MRI are essential for determining whether a patient is suitable for CI and surgical planning. A false-negative diagnosis is possible when diagnosing CO, but combined HRCT and MRI can improve the positive rate of preoperative diagnosis of CO post meningitis. Early CI is recommended. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to analyse the reasons behind the need for cochlear implant revision surgeries, as well as the rate at which they occur, to reduce the revision surgery rate for non-device failures. We also aimed to elucidate the cumulative survival and device survival rates in different age groups. Methods: This retrospective single cohort study reviewed 4563 cochlear implant surgeries and 119 revision surgeries performed at a tertiary referral hospital in China between 1996 and 2019. Kaplan–Meier curves were used to calculate the cumulative survival and device survival rates. Results: The revision surgery rate was 2.61%. The reasons for revision included device (73.1%) and non-device (26.9%) failures. The most common reasons were hard device (47.1%) and non-device failure (28.6%). The 10- and 20-year cumulative survival rates were 96.8% and 96.7%, respectively. Younger children were more likely to undergo a second surgery. Conclusion: This study is the longest study about revision surgery in China. Cochlear implantation is a reliable treatment. It has a low complication rate in patients with sensorineural hearing loss. Children have a higher revision rate than adults. Doctors should be aware of each complication and perform the appropriate procedure. [ABSTRACT FROM AUTHOR]

Summary: Objective: The purpose of the present study was to investigate tone production performance of native Mandarin Chinese speaking children with cochlear implants and to evaluate the effects of age at implantation and duration of implant use on tone production in those children. Methods: Fourteen prelingually deaf children who had received cochlear implantation and 14 age-matched normal-hearing children participated in the study. Both groups were of native Mandarin Chinese speaking children. One hundred and sixty tone tokens were recorded from each of the children. The total of 4480 tokens (160×28) were then used in the tone perception tests in which seven normal-hearing native Mandarin Chinese speaking adults participated. Results: The tone production of the cochlear implant children showed tremendous individual variability. The group mean performance was 48.4% correct, statistically significantly lower than the group mean performance of 78.0% correct in the normal-hearing controls. The tone confusion matrix analysis revealed that the production of Mandarin tone 2 (the rising tone) was most severely impaired in the cochlear implant children, followed by tone 3 (the low and dipping tone) and tone 4 (the falling tone). The most frequently perceived tone irrespective of the target tone was tone 1 (the high level tone). The tone production performance was negatively correlated with the age at implantation and positively correlated with the duration of implant use. Conclusions: There is a remarkable deficit in tone production in a majority of native tone language speaking, prelingually deaf children who have received cochlear implants. While an increased duration of implant use might facilitate tone production, the age at implantation appears to have a negative effect on tone production in cochlear implant children. Therefore, early implantation might be beneficial to tone production in prelingually deaf children whose native language is a tone language. [Copyright &y& Elsevier]

With the increasing clinical focus on the safety of bilateral cochlear implantation (CI) and the potential risk of bilateral vestibular dysfunction, evaluating vestibular end-organ function in patients with vestibular malformations with accompanying abnormalities has been strongly recommended. To identify the vestibular-evoked myogenic potential (VEMP) values among children with sensorineural hearing loss (SNHL) with vestibular malformation and assess the effectiveness of VEMP testing for inner ear malformations (IEM) diagnosis. This study included 96 children (192 ears), including those with vestibular malformations (48 ears), large vestibular aqueduct syndrome (LVAS) (50 ears), and SNHL without IEM (94 ears; control group). All groups underwent ocular and cervical VEMP (oVEMP and cVEMP, respectively) testing. The response rates, VEMP parameters, and wave characteristics were compared. The cVEMP response rates were 37.5 %, 64 %, and 58.51 % and the oVEMP response rates were 42.86 %, 78.95 %, and 77.27 % in the vestibular malformation, LVAS, and control groups, respectively, and significantly differed between groups (cVEMP: X 2 = 18.228, P< 0.001) (oVEMP: X 2 = 7.528, P = 0.023). Significant inter-group differences were observed for the cVEMP and oVEMP latency and amplitude (P < 0.05). The LVAS group's waveform exhibited a prolonged latency and increased amplitude compared with that of the other groups. Patients with SNHL were highly susceptible to otolith dysfunction, regardless of comorbid vestibular malformation presence. Measuring VEMPs is an effective and rapid evaluation technique for vestibular function and could provide a basis for vestibular rehabilitation training. • Studies of characteristics of VEMPs waveform in severe SNHL and vestibular malformations are limited. • Vestibular-evoked myogenic potential (VEMP) testing is an effective and rapid method for measuring saccule and utricle function. • The cVEMP and oVEMP response rate of the vestibular malformation group was significantly low. • Patients with SNHL were highly susceptible to otolith dysfunction. • Results of VEMP testing could improve the classification of vestibular malformations and allow further characterization of IEM subsets. [ABSTRACT FROM AUTHOR]

Background: Few studies on speech performance of children after cochlear implantation (CI) described isolated large vestibular aqueduct syndrome (LVAS). Objective: To investigate speech developmental trajectories of infants with LVAS after CI, and to compare with those who have structurally normal inner ears. Materials and methods: 1112 infants with congenital severe to profound hearing loss participated in this study. 150 infants in group A were diagnosed with LVAS, 962 infants in group B with structurally normal inner ear. The speech performance was assessed via the Meaningful Use of Speech Scale (MUSS). The evaluations were performed pre-implant, 1, 3, 6, 9, 12, 24, 36, 48 and 60 months after CI. Results: The mean scores of the MUSS improved over a 5-year period after implantation in both groups A and B. The LVAS group presented similar speech developmental trajectory to the non-LVAS group at each assessment interval, except pre-operation. There were significant differences in mean scores between vocalizing behavior and oral communication skills, clarification skills of infants in both two groups. Conclusions and significance: Speech performance of infants with LVAS developed rapidly after CI and was similar to infants with structurally normal inner ear. For infants with isolated LVAS, CI had a significant effect and should be recommended as a therapeutic option. [ABSTRACT FROM AUTHOR]

Abstract Cochlear implant (CI) users have been shown to be more susceptible to the variations in speech production encountered in everyday listening, in which speaking rate, amplitude, duration, and voice pitch information may be quite variable, depending on the production context. Such variations may be further enlarged by the background noise, especially dynamic noise. The limited spectral resolution provided by the CI limits perception of voice pitch, which is an important cue for speech prosody and for tonal languages such as Mandarin Chinese. In this study, the effect of varying speaking rates and styles and background noise on speech understanding was investigated in Mandarin-speaking CI and normal-hearing (NH) listeners. Thirteen (5 male and 8 female, age 19–62 years) Mandarin-speaking, post-lingually deafened adult CI patients using their clinical processors and 9 (5 male and 4 female, age 23–59 years) NH subjects listening to unprocessed speech. Five different types of speech variations, including 3 speaking rates (slow, normal, fast) and 2 speaking styles (emotional, shouted) were presented with two masking noises (speech-shaped steady state noise-SSN or six-talker babble). Speech reception threshold, defined as the signal-to-noise ratio producing 50% correct word-in-sentence recognition using Mandarin Speech Perception materials was measured. NH listeners performed significantly better (16.7 dB) than CI patients across all conditions regardless of speech variations and noise types. CI patients' performance deficit was highly dependent on speech rate and noise type; the deficit was smallest (11.7 dB) when slowly-spoken speech was presented in SSN and largest (20.6 dB) when shouted speech was presented in six-talker speech babble. NH listeners performed significantly better in speech babble than in SSN for all speech variations, while CI patients performed similarly in both noise types. The use of clear and slowly-spoken speech in the laboratory setting may largely underestimate CI patients' performance deficits in real-world listening conditions, where acoustic variations introduced by speech variations and dynamic noise may present additional challenges. [ABSTRACT FROM AUTHOR]

Mandarin is a tonal language, and it is important to preserve lexical tone information in synthesized speech. With natural speech, Chinese cochlear implant (CI) users have difficulty perceiving voice pitch cues important for lexical tone perception; it is unclear whether this difficulty persists in Mandarin synthesized speech. In this study, intelligibility of naturally produced and synthesized Mandarin speech was measured in Chinese CI listeners; intelligibility was also measured in a control group of normal-hearing (NH) listeners. Five synthesized voices were selected to represent different talker genders (male, female, child), speaking rates (normal, slow), and speaking styles (emotional, accent). The data showed that while modern Mandarin text-to-speech (TTS) systems can provide perfect speech intelligibility for NH listeners, overall intelligibility was much poorer for CI than for NH listeners. CI performance was significantly poorer with synthesized speech than with natural speech (p < 0.001). CI listeners were highly sensitive to the “extra-atypical” synthesized emotional and accented speech. Performance with each of the synthesized speech types was significantly correlated with performance with natural speech in CI users (p < 0.01 in all cases). While modern TTS systems offer educational and communication benefits to CI users and hearing-impaired individuals, the selection of synthesized voices should be carefully considered in education applications of TTS for hearing-impaired individuals, especially CI children, since poor intelligibility performance may affect language learning. [ABSTRACT FROM AUTHOR]

One of the many parameters that can affect cochlear implant (CI) users’ performance is the site of presentation of electrical stimulation, from the CI, to the auditory nerve. Evoked compound action potential (ECAP) measurements are commonly used to verify nerve function by stimulating one electrode contact in the cochlea and recording the resulting action potentials on the other contacts of the electrode array. The present study aimed to determine if the ECAP amplitude differs between the apical, middle, and basal region of the cochlea, if double peak potentials were more likely in the apex than the basal region of the cochlea, and if there were differences in the ECAP threshold and recovery function across the cochlea. ECAP measurements were performed in the apical, middle, and basal region of the cochlea at fixed sites of stimulation with varying recording electrodes. One hundred and forty one adult subjects with severe to profound sensorineural hearing loss fitted with a Standard or FLEXSOFTelectrode were included in this study. ECAP responses were captured using MAESTRO System Software (MED-EL). The ECAP amplitude, threshold, and slope were determined using amplitude growth sequences. The 50% recovery rate was assessed using independent single sequences that have two stimulation pulses (a masker and a probe pulse) separated by a variable inter-pulse interval. For all recordings, ECAP peaks were annotated semi-automatically. ECAP amplitudes were greater upon stimulation of the apical region compared to the basal region of the cochlea. ECAP slopes were steeper in the apical region compared to the basal region of the cochlea and ECAP thresholds were lower in the middle region compared to the basal region of the cochlea. The incidence of double peaks was greater upon stimulation of the apical region compared to the basal region of the cochlea. This data indicates that the site and intensity of cochlear stimulation affect ECAP properties. [ABSTRACT FROM PUBLISHER]

Objective To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation. Methods Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12–84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS). Results All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500 Hz, 1000 Hz, 2000 Hz and 4000 Hz) of aided hearing thresholds ranged from 17.5 to 57.5 dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition. Conclusion Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months. [ABSTRACT FROM AUTHOR]

The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation. A total of 545 young children (age 7 to 36 months) with prelingual, severe to profound hearing loss participated in this study. All children received cochlear implantation. Based on whether or not there was a Mondini dysplasia as diagnosed with CT scanning, the subjects were divided into 2 groups: (A) 514 young children with radiologically normal inner ears and (B) 31 young children with Mondini dysplasia. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to assess the children's auditory skills that include vocalization changes, spontaneous alerting to sounds in everyday living environments, and the ability to derive meaning from sounds. The assessment was performed prior to surgery and at 1, 3, 6, 9, 12, 24, and 36 months after implant device switch-on. The mean scores for overall auditory skills were not significantly different between groups A and B at pre-surgery, 1, 12, 24, and 36 months post-surgery, but were significantly different at 3, 6, and 9 months post-surgery. The mean scores for all auditory skills in children with Mondini dysplasia showed significant improvement over time. The mean scores for the three subcategories of auditory skills in children with Mondini dysplasia also showed significant differences at pre-surgery, 1, 3, 6, and 9 months, however, there were no significant differences at 12, 24, and 36 months. Overall, the auditory skills of young children with Mondini dysplasia developed rapidly after cochlear implantation, in a similar manner to that of young children with radiologically normal inner ears. Cochlear implantation is an effective intervention for young children with Mondini dysplasia. [ABSTRACT FROM AUTHOR]

Conclusion: This study indicates that Mandarin-speaking subjects acquire significant benefit from a cochlear implant (CI) and audio processor with the fine structure processing (FSP) coding strategy. It can take adult Mandarin CI users time to become accustomed to their CI and appreciate the benefits. Objectives: This study aimed to determine speech perception outcomes in Mandarin-speaking adults who received the FSP coding strategy. Methods: This was a prospective study. Ten adults who were implanted unilaterally with a PULSARCI100, and received an OPUS 1 or OPUS 2 speech processor, were included in this study. The mean age at implantation was 31.1 years. We determined the benefit to postlingually deafened Mandarin-speaking adults with a severe to profound hearing loss using the tests: monosyllables in quiet, sentences in quiet and the Mandarin Hearing in Noise Test (MHINT). Tone perception was evaluated using a tone perception test. A visual analog scale (VAS) was used to score sound quality and ease of listening. Results: The results showed a significant improvement between first fitting and 6 months in all speech tests and in tone perception. The VAS scores showed a significant improvement over 6 months in sound quality and an immediate benefit in ease of listening. [ABSTRACT FROM AUTHOR]

Abstract: Objective: The purpose of this study was to investigate the auditory performance of infants with isolated Large Vestibular Aqueduct Syndrome (LVAS) after cochlear implantation, compare their performance with those of infants with a normal inner ear, and establish a database of auditory development. Method: 435 infants with congenital severe to profound hearing loss participated in this study. 62 infants in group A were diagnosed with isolated LVAS. 373 infants in group B had a normal inner ear. Infant–Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to evaluate the development of auditory skills. Results: The mean scores for auditory ability showed no significant difference between groups A and B. The mean scores for the three different auditory skills increased significantly over time. The differences were statistically significant in mean scores among the three different auditory skills for group B. Conclusion: Auditory skills of infants with isolated LVAS developed rapidly after cochlear implantation, in a similar manner to those of infants with a normal inner ear. Cochlear implantation is an effective interventional approach and an established therapeutic option for infants with isolated LVAS. [Copyright &y& Elsevier]

Conclusions: In prelingually deaf children with cochlear implants, tone perception and production performance are highly correlated. This result is consistent with the hypothesis that tone perception is the prerequisite for good tone production. Objectives: Previous research has shown remarkable deficits in tone perception and production in native tone language-speaking, prelingually deafened children with cochlear implants. The purpose of the present study was to investigate the relationship between tone perception and production in those children. Methods: Twenty-five prelingually deaf children with cochlear implants participated in the study. All subjects were Advanced Bionics CII/90K users with various lengths of implant use. To evaluate tone perception performance, subjects completed a computerized tone contrast test. For tone production performance, an artificial neural network was used to evaluate the accuracy of tones recorded from each of the 25 subjects. Results: Large individual differences in tone perception and production performance were observed in these subjects. Tone perception accuracy ranged from 50.0 to 96.9% correct (chance performance == 50% correct; mean == 71.0% correct). Tone production performance ranged from 19.4 to 97.2% correct (mean == 52.0% correct). A strong correlation was found between tone perception and production performance in this group of subjects ( r == 0.805). [ABSTRACT FROM AUTHOR]

Abstract: The coarse pitch information in cochlear implants might hinder the development of singing in prelingually-deafened pediatric users. In the present study, seven prelingually-deafened children with cochlear implants (5.4–12.3years old) sang one song that was the most familiar to him or her. The control group consisted of 14 normal-hearing children (4.1–8.0years old). The fundamental frequencies (F0) of each note in the recorded songs were extracted. The following five metrics were computed based on the reference music scores: (1) F0 contour direction of the adjacent notes, (2) F0 compression ratio of the entire song, (3) mean deviation of the normalized F0 across the notes, (4) mean deviation of the pitch intervals, and (5) standard deviation of the note duration differences. Children with cochlear implants showed significantly poorer performance in the pitch-based assessments than the normal-hearing children. No significant differences were seen between the two groups in the rhythm-based measure. Prelingually-deafened children with cochlear implants have significant deficits in singing due to their inability to manipulate pitch in the correct directions and to produce accurate pitch height. Future studies with a large sample size are warranted in order to account for the large variability in singing performance. [Copyright &y& Elsevier]

Incomplete partition type III (IP-III) is a relatively rare inner ear malformation that has been associated with a POU3F4 gene mutation. The IP-III anomaly is mainly characterized by incomplete separation of the modiolus of the cochlea from the internal auditory canal. We describe a 71-year-old woman with profound sensorineural hearing loss diagnosed with an IP-III of the cochlea that underwent cochlear implantation. Via targeted sequencing with a non-syndromic gene panel, we identified a heterozygous c.934G > C p. (Ala31Pro) pathogenic variant in the POU3F4 gene that has not been reported previously. IP-III of the cochlea is challenging for cochlear implant surgery for two main reasons: liquor cerebrospinalis gusher and electrode misplacement. Surgically, it may be better to opt for a shorter array because it is less likely for misplacement with the electrode in a false route. Secondly, the surgeon has to consider the insertion angles of cochlear access very strictly to avoid misplacement along the inner ear canal. Genetic results in well describes genotype-phenotype correlations are a strong clinical tool and as in this case guided surgical planning and robotic execution. [ABSTRACT FROM AUTHOR]