scholarly journals Scala vestibuli cochlear implant supported by 3D modeling of the inner ear

2021 ◽  
Author(s):  
Clemens Holzmeister ◽  
Alexandros Andrianakis ◽  
Peter Kiss ◽  
Ulrich Moser ◽  
Matthias Graupp
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Surgical Planning ◽  
Present Report ◽  
Hearing Threshold ◽  
Pure Tone Audiometry ◽  
Electrode Placement ◽  
3D Segmentation ◽  
Brainstem Response ◽  
Scala Vestibuli

SummaryPatients with scala tympani (ST) ossification present a distinct surgical challenge. Three-dimensional (3D) segmentation of the inner ear offers accurate identification of ossification and surgical planning of the cochleostomy to access the scala vestibuli. The scala vestibuli placement of cochlear implantation electrode is an alternate solution in these patients and is well supported by the literature.The present report describes a case of cochlear implantation in the scala vestibuli assisted by 3D segmentation of the cochlea for a patient with ossification in the ST and reviews the relevant literature. Clinical presentation of a 45-year-old Austrian female who was referred with a history of sudden sensorineural hearing loss 2 years ago in the right ear, confirmed by pure tone audiometry (PTA) and acoustically evoked auditory brainstem response (ABR). 3D segmentation of the inner ear identified the extent of ossification in the ST and assisted in the surgical planning of cochleostomy drilling anterior-superior to the round window to access the scala vestibuli for the electrode placement. Postoperative computed tomography (CT) to confirm the electrode placement in the scala vestibuli and PTA was performed to assess the hearing threshold following the cochlear implantation. Postoperative CT confirmed the full insertion of a flexible electrode. The hearing threshold measured by PTA was ≤ 40 dB across all frequencies tested. Review of the literature identified a total of 13 published reports on cochlear implantation electrode placement in scala vestibuli in cases with ossification in the ST.

Role of Electrically Evoked Auditory Brainstem Response in Cochlear Implantation of Children With Inner Ear Malformations

2008 ◽  
Vol 29 (5) ◽  
pp. 626-634 ◽  
Author(s):  
Ana H. Kim ◽  
Paul R. Kileny ◽  
H. Alexander Arts ◽  
Hussam K. El-Kashlan ◽  
Steven A. Telian ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Brainstem Response

scholarly journals Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Birgul Gumus ◽  
Armagan Incesulu ◽  
Mehmet Ozgur Pinarbasli
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
External Ear ◽  
Ear Canal ◽  
External Ear Canal ◽  
Brainstem Response ◽  
Visual Disturbances

Background.Keratitis-ichthyosis-deafness (KID) syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature.Case Report.This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR), and the postoperative follow-up was conducted using pure-tone audiometry.Conclusion.Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

scholarly journals Auditory neuropathy: A case report and review of literature

2014 ◽  
Vol 19 (2) ◽  
pp. 135-139
Author(s):  
Ali Imam Ahsan ◽  
Rashedul Hasan ◽  
Nasimul Jamal
Keyword(s):  
Inner Ear ◽  
Hearing Threshold ◽  
Otoacoustic Emission ◽  
Auditory Brainstem ◽  
Normal Function ◽  
Auditory Neuropathy ◽  
Speech Discrimination ◽  
Brainstem Response ◽  
Missed Diagnosis ◽  
Audiologic Evaluation

Auditory Neuropathy (AN), also known as Auditory Dyssynchrony(AD) is a very often missed diagnosis in clinical practice. In AN, sound enters the inner ear normally but the transmission of signals from inner ear to brain is impaired. Due to the lack of advanced audiological test facilities in Bangladesh, mostly AN remains undiagnosed. Here we described a 25 years old male, presented with difficulty in understanding speech for last 10 years. On audiologic evaluation, bilateral moderate sensorineural hearing loss was found on Pure tone audiogram (PTA). Speech discrimination scores were poor and disproportionate to his hearing threshold. Presence of Otoacoustic emission (OAE) revealed the normal function of cochlea. Conversely, absence of Auditory brainstem response (ABR) confirmed the definite dysfunction at auditory neural pathway and hence, the diagnosis of AN was confirmed. Since AN is an underdiagnosed condition in Bangladesh, otolaryngologists should be aware of this clinical entity. All the suspected cases should be referred to specialized centers, where advanced audiological facilities are available. DOI: http://dx.doi.org/10.3329/bjo.v19i2.17638 Bangladesh J Otorhinolaryngol 2013; 19(2): 135-139

scholarly journals A Ten-Year Review of Audiological Performance in Children with Inner Ear Abnormalities after Cochlear Implantation in Singapore

2019 ◽  
Vol 2019 ◽  
pp. 1-8
Author(s):  
Sok Yan Tay ◽  
Rosslyn Anicete ◽  
Kun Kiaang Henry Tan
Keyword(s):  
Inner Ear ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Hearing Threshold ◽  
Special Schools ◽  
Inner Ear Malformation ◽  
Ear Malformations ◽  
Clinical Records ◽  
To Receive

Objectives. To evaluate children with inner ear malformations following cochlear implantation (CI) in a tertiary pediatric hospital in Singapore to identify factors influencing outcomes after CI. Methods. This is a retrospective cohort study of children aged 0 to 18 years, who had CI between 2000 and 2013. Demographic information, data on risk factors, type of inner ear malformation (IEM), age at implantation, speech pre- and postimplantation, and duration of follow-up were collected from clinical records. Operative details and audiological outcomes were also analyzed. Results. A total of 70 children underwent 83 CI surgeries. The mean age of the patients was 4.05 ± 3.17 years (range 1–18 years). Twenty patients (28.57%) had abnormal CT scan findings. CSF gusher occurred in 15 out of 26 CI (57.69%) in the group with IEM. Nine out of twenty patients (45.00%) had poor IT-MAIS scores prior to implantation. The average preoperative IT-MAIS score for children with anomalous inner ear anatomy was 14.1. The older CI patients, 3/20 (15.00%), mean age 8.33 years (range 7–10 years), were mostly referred for persistently unclear speech following hearing aids. Eleven patients (55.00%) had good speech and aided hearing threshold within speech limits after CI and were eligible for reintegration into mainstream schools. Five patients (25.00%) had improvement in speech but continued to receive education in special schools. Four patients (20.00%) had poor progress after surgery. Conclusion. The presence of absent cochlear nerve, electrode folding, and underlying neurological disorders seemed to be associated with poorer outcomes.

scholarly journals Peripheral and central auditory assessment in among the elderly

2016 ◽  
Vol 19 (5) ◽  
pp. 839-849
Author(s):  
Alessandra Giannella Samelli ◽  
Carla Gentile Matas ◽  
Camila Maia Rabelo ◽  
Fernanda Cristina Leite Magliaro ◽  
Natália Paião Luiz ◽  
...  
Keyword(s):  
Elderly People ◽  
Social Life ◽  
Hearing Threshold ◽  
Pure Tone Audiometry ◽  
The Elderly ◽  
Daily Routine ◽  
Elderly Persons ◽  
Hearing Thresholds ◽  
Significant Difference ◽  
Brainstem Response

Abstract Introduction: Presbycusis can affect different portions of the auditory system, causing impacts of varying degrees of seriousness on the daily routine of elderly persons. It is essential that the extent of the deficit as well as the degree of handicap is evaluated, so that the hearing of the elderly can be effectively rehabilitated, improving their quality of life. Purpose: To characterize the peripheral and central hearing of elderly individuals and assess their auditory handicaps. Methods: A cross sectional observational study was performed. We evaluated 83 elderly persons (60-85 years; 33 men, 50 women) with normal hearing or sensorineural hearing loss. Individuals were divided into 3 groups according to the 3 to 6kHz hearing thresholds: G1 - mean of 0 to 39 dBHL (80 ears); G2 - mean of 40 to 59 dBHL (48 ears); G3 - mean of 60 to 120dBHL (38 ears). All individuals responded to the Hearing Handicap Inventory for the Elderly (HHIE), and underwent Pure Tone Audiometry, Auditory Brainstem Response (ABR) and Long Latency Response (P300) evaluation. Results: Men had higher auditory thresholds at frequencies from 500 to 12,000Hz (with a statistical difference between 2-8 kHz) and also significantly greater latencies for ABR components. There was no difference between genders for the P300 evaluation. Comparison between groups showed: a statistically significant difference for age; greater ABR wave latencies and interwave intervals; that questionnaire scores worsened as hearing threshold declined; and similar P300 latencies. Conclusions: Elderly people have impairment throughout the auditory pathway (peripheral and central). The P300 was less accurate at identifying the losses that come with age. The HHIE demonstrated negative effects on the social life of elderly people, agreeing with the hearing thresholds found.

scholarly journals Identification of Binding Partners of Deafness-Related Protein PDZD7

2018 ◽  
Vol 2018 ◽  
pp. 1-10 ◽  
Author(s):  
Haibo Du ◽  
Rui Ren ◽  
Panpan Chen ◽  
Zhigang Xu ◽  
Yanfei Wang
Keyword(s):  
Inner Ear ◽  
Knockout Mice ◽  
Binding Proteins ◽  
Hearing Threshold ◽  
Auditory Brainstem ◽  
Pdz Domains ◽  
Deafness Gene ◽  
Binding Partners ◽  
Brainstem Response

PDZD7 is an important deafness gene, whose mutations are associated with syndromic and nonsyndromic hearing loss. PDZD7 contains multiple PDZ domains that are essential for organizing various proteins into protein complex. Several PDZD7-binding proteins have been identified, including usherin, ADGRV1, whirlin, harmonin, SANS, and MYO7A, all belonging to USH proteins. Here, we report the identification of novel PDZD7-binding partners through yeast two-hybrid screening using the first two PDZ domains of PDZD7 as bait. Eleven proteins were identified, most of which have not been reported as PDZD7-binding partners before. Among the identified proteins, ADGRV1, gelsolin, and β-catenin have been shown to play important roles in hearing, whereas the functions of other proteins in the inner ear remain elusive. We confirmed the expression of one candidate PDZD7-binding protein, CADM1, in the mouse inner ear and evaluated the auditory function of Cadm1 knockout mice by performing auditory brainstem response (ABR) measurement. Unexpectedly, Cadm1 knockout mice show normal hearing threshold, which might be explained by the possible compensation by its homologs that are also expressed in the inner ear. Taken together, our work identified several novel PDZD7-binding proteins, which will help us to further understand the role of PDZD7 in hearing transduction.

Dexamethasone Is One of the Factors Minimizing the Inner Ear Damage from Electrode Insertion in Cochlear Implantation

2016 ◽  
Vol 21 (3) ◽  
pp. 178-186 ◽  
Author(s):  
Hyun Soo Cho ◽  
Kyu-Yup Lee ◽  
Hongsoo Choi ◽  
Jeong Hun Jang ◽  
Sang Heun Lee
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Hearing Threshold ◽  
Hearing Preservation ◽  
Control Group ◽  
Hearing Level ◽  
Hearing Thresholds ◽  
The Difference ◽  
Caloric Response ◽  
Inner Ear Damage

The aim of this study was to investigate the efficacy of preoperative and intraoperative steroid administration for inner ear protection in cochlear implantation (CI). Nineteen subjects who underwent CI were included in the study, and 10 subjects were enrolled as controls (steroid-administered group, n = 19; control group, n = 10). Dexamethasone (dexamethasone sodium phosphate, 5 mg/ml) was systemically administered preoperatively (1 ml) and topically applied during CI (0.5 ml). The extent of hearing preservation (HP) after CI and the change in the bithermal caloric response were evaluated. Hearing level was calculated using mean thresholds [(250 Hz + 500 Hz + 1,000 Hz + 2,000 Hz)/4]. Preoperative hearing thresholds were similar in the steroid-administered and control groups (100.92 ± 12.60 vs. 103.29 ± 14.39 dB, p = 0.650). The mean thresholds significantly increased in both groups after surgery (108.46 ± 14.08 dB, p = 0.006, for the steroid-administered group; 117.50 ± 6.34 dB, p = 0.027, for the control group), and the difference between the groups was also significant (p = 0.027). The postoperative shift in the hearing thresholds at frequencies of 500 and 1,000 Hz was significant in the steroid-administered group and that at the frequencies of 500, 1,000 and 2,000 Hz was significant in the control group. However, the extent of the shift in hearing threshold levels at each frequency was not significantly different between the groups. Preservation of hearing thresholds was compared between the groups, and there were significantly more subjects with complete and partial HP in the steroid-administered group than in the control group (p = 0.008). The preoperative caloric response was maintained after CI in the steroid-administered group. This study suggests that the perioperative use of a steroid could minimize the inner ear damage after CI.

Surgical considerations during cochlear implantation: the utility of temporal bone computed tomography

2021 ◽  
pp. 1-8
Author(s):  
Beomcho Jun ◽  
Sunwha Song
Keyword(s):  
Computed Tomography ◽  
Temporal Bone ◽  
Cochlear Implantation ◽  
Round Window ◽  
Three Dimensional ◽  
Electrode Placement ◽  
Posterior Tympanotomy ◽  
Electrode Insertion ◽  
Window Approach ◽  
Bone Structures

Abstract Objective This paper describes the construction of portals for electrode placement during cochlear implantation and emphasises the utility of pre-operative temporal bone three-dimensional computed tomography. Methods Temporal bone three-dimensional computed tomography was used to plan portal creation for electrode insertion. Results Pre-operative temporal bone three-dimensional computed tomography can be used to determine the orientation of temporal bone structures, which is important for mastoidectomy, posterior tympanotomy and cochleostomy, and when using the round window approach. Conclusion It is essential to create appropriate portals (from the mastoid cortex to the cochlea) in a step-by-step manner, to ensure the safe insertion of electrodes into the scala tympani. Pre-operative three-dimensional temporal bone computed tomography is invaluable in this respect.

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