ABSTRACT
A labyrinthine fistula is a severe complication of middle ear cholesteatoma that can cause profound sensorineural hearing loss and vertigo. However, there is no consensus regarding the transition to postoperative hearing. Although hearing deteriorates gradually with a delay in some cases of labyrinthine fistula, insufficient consideration has been given to this point. We examined perioperative changes in cases of middle ear cholesteatoma with labyrinthine fistulas. We retrospectively reviewed the medical records of 578 patients with middle ear cholesteatoma who underwent tympanoplasty at our hospital between 2016 and 2021. Patients with labyrinthine fistulas were selected; their perioperative bone-conduction hearing was assessed. Fistula depth was determined following the classification reported by Dornhoffer et al. The hearing was compared preoperatively, early postoperatively (3-6 months), and 1 year postoperatively. Forty-eight patients (8.3%) had labyrinthine fistulas. Regarding depth, 21 cases were type I, 14 were type IIa, 3 were type IIb, and 10 were type III. Preoperative bone-conduction hearing was significantly poor in invasion type IIb or deeper cases. Cases with type IIb or deeper fistulas, multiple fistulas, or vertigo deteriorated postoperatively. Type III cases or those with multiple fistulas deteriorated further from the early postoperative period to 1 year postoperatively. Concerning frequency, 500 and 2000 Hz showed a delayed deterioration. This is a valuable report of delayed hearing loss after surgery in patients with a labyrinthine fistula. This change is associated with the labyrinthine fistula's depth and multiple fistulas-this is important during preoperative counseling of patients undergoing surgery.
Subject(s)
Cholesteatoma, Middle Ear , Fistula , Labyrinth Diseases , Postoperative Complications , Tympanoplasty , Humans , Retrospective Studies , Male , Female , Fistula/etiology , Fistula/surgery , Middle Aged , Adult , Cholesteatoma, Middle Ear/surgery , Cholesteatoma, Middle Ear/complications , Labyrinth Diseases/surgery , Labyrinth Diseases/etiology , Labyrinth Diseases/diagnosis , Postoperative Complications/etiology , Tympanoplasty/methods , Hearing Loss, Sensorineural/etiology , Hearing Loss, Sensorineural/surgery , Aged , Young Adult , Bone Conduction/physiology , Vertigo/etiology , Adolescent , Hearing/physiologyABSTRACT
OBJECTIVE: To prospectively evaluate the association between hearing preservation after cochlear implantation (CI) and intracochlear electrocochleography (ECochG) amplitude parameters. STUDY DESIGN: Multi-institutional, prospective randomized clinical trial. SETTING: Ten high-volume, tertiary care CI centers. PATIENTS: Adults (n = 87) with sensorineural hearing loss meeting CI criteria (2018-2021) with audiometric thresholds of ≤80 dB HL at 500 Hz. METHODS: Participants were randomized to CI surgery with or without audible ECochG monitoring. Electrode arrays were inserted to the full-depth marker. Hearing preservation was determined by comparing pre-CI, unaided low-frequency (125-, 250-, and 500-Hz) pure-tone average (LF-PTA) to LF-PTA at CI activation. Three ECochG amplitude parameters were analyzed: 1) insertion track patterns, 2) magnitude of ECochG amplitude change, and 3) total number of ECochG amplitude drops. RESULTS: The Type CC insertion track pattern, representing corrected drops in ECochG amplitude, was seen in 76% of cases with ECochG "on," compared with 24% of cases with ECochG "off" ( p = 0.003). The magnitude of ECochG signal drop was significantly correlated with the amount of LF-PTA change pre-CI and post-CI ( p < 0.05). The mean number of amplitude drops during electrode insertion was significantly correlated with change in LF-PTA at activation and 3 months post-CI ( p ≤ 0.01). CONCLUSIONS: ECochG amplitude parameters during CI surgery have important prognostic utility. Higher incidence of Type CC in ECochG "on" suggests that monitoring may be useful for surgeons in order to recover the ECochG signal and preventing potentially traumatic electrode-cochlear interactions.
Subject(s)
Audiometry, Evoked Response , Cochlear Implantation , Hearing Loss, Sensorineural , Humans , Audiometry, Evoked Response/methods , Cochlear Implantation/methods , Female , Middle Aged , Aged , Male , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/physiopathology , Prospective Studies , Cochlear Implants , Cochlea/surgery , Cochlea/physiopathology , Adult , Hearing/physiology , Audiometry, Pure-ToneABSTRACT
BACKGROUND: Children with cochlear implants (CIs) often lag behind children with normal hearing (NH) in early literacy skills. Furthermore, the development of language skills associated with their emergent literacy skills seems to depend on good auditory access. Supporting language acquisition and early literacy in children with CIs may prevent difficulties in primary school. The use of technology may facilitate auditory and speech recovery in children with CIs, but evidence on computer-based early literacy programs is limited. OBJECTIVE: This study investigates (a) the effects of a computer-based program focusing on the syllabic method on the literacy skills of children with CIs (CIs group), comparing them with the literacy skills of a group of age-matched NH (normal hearing) peers (NHs group); (b) the associations between language and early literacy skills in the NHs group and between language, auditory and early literacy skills in the CIs group. METHOD: Nine prelingually deaf children with CIs (M = 61.11, SD = 6.90) with severe to profound sensorineural hearing loss and nine age-matched NH children participated in the program. Categories of Auditory Performance (CAP) as measures of children's auditory skills were collected. All participants were tested on phonological, morphosyntax (grammatical comprehension and repetition), and early literacy skills (syllable blending and segmentation, syllable and word reading) (T1). Next, all children participated in the computer-based program for 12 weeks. After the program was completed (T2), only early literacy tests were administered to the children. RESULTS: Although, on average, both groups obtained higher scores in all literacy tasks at T2, the CIs group scored lower than the NHs group. In the CIs group, at T2 we found significant improvements in syllable segmentation (p = 0.042) and word reading (p = 0.035). In the NHs group, at T2 we found significant improvements in syllable segmentation (p = 0.034), syllable blending (p = 0.022), syllable reading (p = 0.008), and word reading (p = 0.009). We also found significant associations in both groups between measures of morphosyntax at T1 and measures of early literacy at T2. In addition, for the CIs group, we found significant associations between children's auditory performance at T1 and measures of morphosyntax at T1 and early literacy at T2. CONCLUSION: a computer-based program focused on the syllabic method could support children with CIs in acquiring emergent literacy abilities. The auditory performance of children with CIs seems to influence their morphosyntax and later early literacy skills.
Subject(s)
Cochlear Implants , Literacy , Humans , Male , Female , Child , Deafness/surgery , Child, Preschool , Case-Control Studies , Hearing Loss, Sensorineural/surgery , Language Development , Cochlear Implantation , Reading , SoftwareABSTRACT
A vestibular schwannoma is a benign tumor; however, the schwannoma itself and interventions can cause sensorineural hearing loss. Most vestibular schwannomas are unilateral tumors that affect hearing only on one side. Attention has focused on improving the quality of life for patients with unilateral hearing loss and therapeutic interventions to address this issue have been emphasized. Herein, we encountered a patient who was a candidate for hearing preservation surgery based on preoperative findings and had nonserviceable hearing after the surgery, according to the Gardner-Robertson classification. Postoperatively, the patient had decreased listening comprehension and ability to localize sound sources. He was fitted with bilateral hearing aids, and his ability to localize sound sources improved. Although the patient had postoperative nonserviceable hearing on the affected side and age-related hearing loss on the unaffected side, hearing aids in both ears were useful for his daily life. Therefore, the patient was able to maintain a binaural hearing effect and the ability to localize the sound source improved. This report emphasizes the importance of hearing preservation with vestibular schwannomas, and the demand for hearing loss rehabilitation as a postoperative complication can increase, even if hearing loss is nonserviceable.
Subject(s)
Hearing Aids , Neuroma, Acoustic , Humans , Neuroma, Acoustic/surgery , Male , Middle Aged , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/rehabilitation , Hearing Loss, Sensorineural/etiology , Quality of Life , Hearing Loss/etiology , Hearing Loss/surgery , Hearing Loss/rehabilitation , Postoperative Complications/etiologyABSTRACT
OBJECTIVES: Current evidence supports the benefits of cochlear implants (CIs) in children with hearing loss, including those with auditory neuropathy spectrum disorder (ANSD). However, there is limited evidence regarding factors that hold predictive value for intervention outcomes. DESIGN: This retrospective case-control study consisted of 66 children with CIs, including 22 with ANSD and 44 with sensorineural hearing loss (SNHL) matched on sex, age, age at CI activation, and the length of follow-up with CIs (1:2 ratio). The case and control groups were compared in the results of five open-set speech perception tests, and a Forward Linear Regression Model was used to identify factors that can predict the post-CI outcomes. RESULTS: There was no significant difference in average scores between the two groups across five outcome measures, ranging from 88.40% to 95.65%. The correlation matrix revealed that younger ages at hearing aid fitting and CI activation positively influenced improvements in speech perception test scores. Furthermore, among the variables incorporated in the regression model, the duration of follow-up with CIs, age at CI activation, and the utilization of two CIs demonstrated prognostic significance for improved post-CI speech perception outcomes. CONCLUSIONS: Children with ANSD can achieve similar open-set speech perception outcomes as children with SNHL. A longer CI follow-up, a lower age at CI activation, and the use of two CIs are predictive for optimal CI outcome.
Subject(s)
Cochlear Implants , Hearing Loss, Central , Hearing Loss, Sensorineural , Speech Perception , Humans , Male , Female , Case-Control Studies , Child, Preschool , Child , Retrospective Studies , Hearing Loss, Central/physiopathology , Hearing Loss, Central/surgery , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/physiopathology , Speech Perception/physiology , Treatment Outcome , Cochlear Implantation , Infant , PrognosisABSTRACT
OBJECTIVE: To describe the rare process of osteolytic labyrinthitis, previously referred to as labyrinthine sequestrum, which involves progressive obliteration of the bony and membranous labyrinth with eventual supplantation with soft tissue and, in some cases, bony sequestrum. PATIENTS: Three patients with diverse presentations of osteolytic labyrinthitis from two tertiary care academic medical centers. INTERVENTIONS: Case series report analyzing the relevant clinical, radiologic, pathologic, and surgical data on our patients with osteolytic labyrinthitis and comparing these index cases to the existing literature. MAIN OUTCOME MEASURES: We describe the varying image findings seen in osteolytic labyrinthitis on computed tomography and magnetic resonance imaging. Also, we report successful surgical intervention and hearing rehabilitation with cochlear implantation in patients with osteolytic labyrinthitis. RESULTS: Our three patients presented with profound sudden sensorineural hearing loss and vertigo consistent with labyrinthitis. None of the three patients had a history of chronic otitis media. Imaging workup revealed varying degrees of erosion to the otic capsule bone demonstrating the spectrum of disease seen in osteolytic labyrinthitis. Although two cases showed osteolytic changes to the semicircular canals and vestibule, the first case revealed frank bony sequestrum within the obliterated labyrinth. The three cases were taken for surgical debridement and cochlear implantation. CONCLUSIONS: We propose the new term, osteolytic labyrinthitis-previously referred to as labyrinthine sequestrum-to describe the rare spectrum of disease characterized by destruction of the osseous and membranous labyrinth and potential supplantation with bony sequestrum. Cochlear implantation is a viable option in selected patients with osteolytic labyrinthitis.
Subject(s)
Cochlear Implantation , Labyrinthitis , Humans , Cochlear Implantation/methods , Labyrinthitis/surgery , Labyrinthitis/complications , Labyrinthitis/diagnostic imaging , Male , Female , Middle Aged , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/diagnostic imaging , Hearing Loss, Sensorineural/etiology , Adult , Treatment Outcome , Osteolysis/diagnostic imaging , Osteolysis/surgery , Osteolysis/complications , Aged , Vertigo/surgery , Vertigo/etiology , Vertigo/diagnostic imagingABSTRACT
Objective: To investigate the auditory and speech abilities of children with congenital auditory neuropathy (AN) after cochlear implant (CI), and to analyze the role of genetic testing in predicting the postoperative outcomes of CI in AN patients. Methods: Fourteen children diagnosed with AN by audiological battery test and underwent CI surgery in Xijing Hospital of the Air Force Medical University from 2002 to 2021 were included in this study (9 males and 5 females), with an implantation age of (3.1±1.7) years (mean±standard deviation, the same as follows). The preoperative audiological results and deafness gene results were analyzed. Another 52 children with ordinary sensorineural hearing loss (SNHL) were selected as the control group (30 males and 22 females), with an implantation age of (2.2±0.9) years. The demographic factors such as age and gender were matched with those of the AN group. The modified Category Auditory Performance (CAP-â ¡) and Speech Intelligence Rate (SIR) were used to evaluate the development of postoperative auditory and speech abilities in two groups. The Mandarin Speech Test System was used to test the speech recognition rate of monosyllabic and disyllabic words and sentences. Matlab 2022 software was used to analyze the data. Results: The results of gene in 14 children with AN showed that 6 cases had OTOF gene mutations, 2 cases (siblings) were confirmed to have TNN gene mutations through whole exome sequencing, and the remaining 6 cases were not find any clear pathogenic gene mutations. All subjects underwent CI surgery with electrodes implanted into the cochlea smoothly, and there were no postoperative complications. After surgery, all AN children had improved auditory and speech abilities, but only 64% (9/14) of AN children with CI had auditory ability scores comparable to the control group of SNHL children (including 2 children with TNN gene mutations), and 36% (5/14) of AN children had lower scores than the control group of SNHL children.The average speech recognition rate of two children with TNN gene mutations was 86.5%, and of two children with OTOF gene mutations was 83.2%. Conclusions: AN children achieved varying degrees of auditory and speech abilities after CI, but the postoperative effects varied greatly. Some children achieved similar results as ordinary SNHL children, but there were still some children whose effects were worse than those of ordinary SNHL children. The postoperative efficacy of CI in two children with AN caused by TNN pathogenic genes were comparable to that of ordinary SNHL in children. Genetic testing had certain reference value for predicting the postoperative effect of CI in AN children.
Subject(s)
Cochlear Implantation , Cochlear Implants , Hearing Loss, Central , Hearing Loss, Sensorineural , Humans , Male , Female , Child, Preschool , Hearing Loss, Central/genetics , Hearing Loss, Central/surgery , Hearing Loss, Sensorineural/surgery , Treatment Outcome , Child , Speech PerceptionABSTRACT
OBJECTIVE: To investigate the outcomes of cochlear implantation in patients with TMTC2 -associated sensorineural hearing loss and auditory neuropathy/auditory dys-synchrony. PATIENTS: Adult and pediatric cochlear implant (CI) patients followed in an academic center who tested positive for TMTC2 genetic variant rs35725509. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Speech perception scores in quiet. RESULTS: Ten CI patients were identified with TMTC2 variant rs35725509 out of 157 patients who underwent genetic testing (i.e., 6.3% of patients tested). All demonstrated progressive, bilateral hearing loss with severe-to-profound audiometric thresholds preoperatively. Pre-CI and 1-year post-CI speech recognition percent correct scores were compared. Post-CI speech perception (mean 61.0%, standard deviation 31.4%) was significantly higher than pre-CI speech perception (mean 21.0%, standard deviation 27.0%) ( p = 0.002). Individually, 9 of the 10 subjects experienced significant improvements in speech perception pre- to post-CI ( p < 0.05). Electrically evoked compound action potential measures were available for five patients, and all showed normal electrically evoked compound action potential thresholds. CONCLUSION: Patients with TMTC2 -associated sensorineural hearing loss and auditory neuropathy/auditory dys-synchrony have significantly improved speech perception outcomes with cochlear implantation and should be considered candidates for this intervention if there are no other contraindications.
Subject(s)
Cochlear Implantation , Cochlear Implants , Hearing Loss, Central , Hearing Loss, Sensorineural , Speech Perception , Humans , Hearing Loss, Sensorineural/surgery , Male , Speech Perception/physiology , Female , Adult , Treatment Outcome , Child , Hearing Loss, Central/surgery , Hearing Loss, Central/genetics , Adolescent , Middle Aged , Child, Preschool , Young AdultSubject(s)
Cochlear Implantation , GTP Phosphohydrolases , Hearing Loss, Central , Humans , GTP Phosphohydrolases/genetics , Male , Hearing Loss, Central/genetics , Hearing Loss, Central/surgery , Female , Mutation , Adult , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/genetics , PedigreeABSTRACT
Moderate-to-profound sensorineural hearing loss in humans is treatable by electrically stimulating the auditory nerve (AN) with a cochlear implant (CI). In the cochlea, the modiolus presents a porous bony interface between the CI electrode and the AN. New bone growth caused by the presence of the CI electrode or neural degeneration inflicted by ageing or otological diseases might change the effective porosity of the modiolus and, thereby, alter its electrical material properties. Using a volume conductor description of the cochlea, with the aid of a 'mapped conductivity' method and an ad-hoc 'regionally kinetic' equation system, we show that even a slight variation in modiolus porosity or pore distribution can disproportionately affect AN stimulation. Hence, because of porosity changes, an inconsistent CI performance might occur if neural degeneration or new bone growth progress after implantation. Appropriate electrical material properties in accordance with modiolar morphology and pathology should be considered in patient-specific studies. The present first-of-its-kind in-silico study advocates for contextual experimental studies to further explore the utility of modiolus porous morphology in optimising the CI outcome.
Subject(s)
Cochlear Implants , Spiral Ganglion , Porosity , Humans , Cochlear Nerve , Neurons/physiology , Electric Stimulation , Hearing Loss, Sensorineural/therapy , Hearing Loss, Sensorineural/surgery , CochleaABSTRACT
HYPOTHESIS: Preimplantation word scores cannot reliably predict postimplantation outcomes. BACKGROUND: To date, there is no model based on preoperative data that can reliably predict the postoperative outcomes of cochlear implantation in the postlingually deafened adult patient. METHODS: In a group of 228 patients who received a cochlear implant between 2002 and 2021, we tested the predictive power of nine variables (age, etiology, sex, laterality of implantation, preimplantation thresholds and word scores, as well as the design, insertion approach, and angular insertion depth of the electrode array) on postimplantation outcomes. Results of multivariable linear regression analyses were then interpreted in light of data obtained from histopathological analyses of human temporal bones. RESULTS: Age and etiology were the only significant predictors of postimplantation outcomes. In agreement with many investigations, preimplantation word scores failed to significantly predict postimplantation outcomes. Analysis of temporal bone histopathology suggests that neuronal survival must fall below 40% before word scores in quiet begin to drop. Scores fall steeply with further neurodegeneration, such that only 20% survival can support acoustically driven word scores of 50%. Because almost all cochlear implant implantees have at least 20% of their spiral ganglion neurons (SGNs) surviving, it is expected that most cochlear implant users on average should improve to at least 50% word recognition score, as we observed, even if their preimplantation score was near zero as a result of widespread hair cell damage and the fact that ~50% of their SGNs have likely lost their peripheral axons. These "disconnected" SGNs would not contribute to acoustic hearing but likely remain electrically excitable. CONCLUSION: The relationship between preimplantation word scores and data describing the survival of SGNs in humans can explain why preimplantation word scores obtained in unaided conditions fail to predict postimplantation outcomes.
Subject(s)
Cochlear Implantation , Hearing Loss , Language , Speech Perception , Aged , Female , Male , Middle Aged , Audiometry , Cochlear Implantation/instrumentation , Cochlear Implantation/methods , Deafness/surgery , Hearing Loss/surgery , Hearing Loss, Sensorineural/surgery , Linear Models , Prognosis , Retrospective Studies , Speech Perception/physiology , Temporal Bone/pathology , Treatment Outcome , HumansABSTRACT
Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.
Subject(s)
Cochlear Implantation , Cochlear Implants , Deafness , Hearing Loss, Sensorineural , Hearing Loss , Meningitis , Child , Humans , Male , Female , Cochlear Implants/adverse effects , Cochlear Implantation/adverse effects , Hearing Loss, Sensorineural/epidemiology , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/etiology , Cross-Sectional Studies , Hearing Loss/epidemiology , Hearing Loss/complications , Deafness/epidemiology , Deafness/surgery , Meningitis/complications , DemographyABSTRACT
BACKGROUND: Patients with severe-to-profound hearing loss may benefit from management with cochlear implants. These patients need a referral to a cochlear implant team for further assessment and possible surgery. The referral pathway may result in varied access to hearing healthcare. This study aimed to explore referral patterns and whether there were any socioeconomic or ethnic associations with the likelihood of referral. The primary outcome was to determine factors influencing referral for implant assessment. The secondary outcome was to identify factors impacting whether healthcare professionals had discussed the option of referral. METHODS AND FINDINGS: A multicentre multidisciplinary observational study was conducted in secondary care Otolaryngology and Audiology units in Great Britain. Adults fulfilling NICE (2019) audiometric criteria for implant assessment were identified over a 6-month period between 1 July and 31 December 2021. Patient- and site-specific characteristics were extracted. Multivariable binary logistic regression was employed to compare a range of factors influencing the likelihood of implant discussion and referral including patient-specific (demographics, past medical history, and degree of hearing loss) and site-specific factors (cochlear implant champion and whether the hospital performed implants). Hospitals across all 4 devolved nations of the UK were invited to participate, with data submitted from 36 urban hospitals across England, Scotland, and Wales. Nine hospitals (25%) conducted cochlear implant assessments. The majority of patients lived in England (n = 5,587, 86.2%); the rest lived in Wales (n = 419, 6.5%) and Scotland (n = 233, 3.6%). The mean patient age was 72 ± 19 years (mean ± standard deviation); 54% were male, and 75·3% of participants were white, 6·3% were Asian, 1·5% were black, 0·05% were mixed, and 4·6% were self-defined as a different ethnicity. Of 6,482 submitted patients meeting pure tone audiometric thresholds for cochlear implantation, 311 already had a cochlear implant. Of the remaining 6,171, 35.7% were informed they were eligible for an implant, but only 9.7% were referred for assessment. When adjusted for site- and patient-specific factors, stand-out findings included that adults were less likely to be referred if they lived in more deprived area decile within Indices of Multiple Deprivation (4th (odds ratio (OR): 2·19; 95% confidence interval (CI): [1·31, 3·66]; p = 0·002), 5th (2·02; [1·21, 3·38]; p = 0·05), 6th (2·32; [1·41, 3·83]; p = 0.05), and 8th (2·07; [1·25, 3·42]; p = 0·004)), lived in London (0·40; [0·29, 0·57]; p < 0·001), were male (females 1·52; [1·27, 1·81]; p < 0·001), or were older (0·97; [0·96, 0·97]; p < 0·001). They were less likely to be informed of their potential eligibility if they lived in more deprived areas (4th (1·99; [1·49, 2·66]; p < 0·001), 5th (1·75; [1·31, 2·33], p < 0·001), 6th (1·85; [1·39, 2·45]; p < 0·001), 7th (1·66; [1·25, 2·21]; p < 0·001), and 8th (1·74; [1·31, 2·31]; p < 0·001) deciles), the North of England or London (North 0·74; [0·62, 0·89]; p = 0·001; London 0·44; [0·35, 0·56]; p < 0·001), were of Asian or black ethnic backgrounds compared to white patients (Asian 0·58; [0·43, 0·79]; p < 0·001; black 0·56; [0·34, 0·92]; p = 0·021), were male (females 1·46; [1·31, 1·62]; p < 0·001), or were older (0·98; [0·98, 0·98]; p < 0·001). The study methodology was limited by its observational nature, reliance on accurate documentation of the referring service, and potential underrepresentation of certain demographic groups. CONCLUSIONS: The majority of adults meeting pure tone audiometric threshold criteria for cochlear implantation are currently not appropriately referred for assessment. There is scope to target underrepresented patient groups to improve referral rates. Future research should engage stakeholders to explore the reasons behind the disparities. Implementing straightforward measures, such as educational initiatives and automated pop-up tools for immediate identification, can help streamline the referral process.
Subject(s)
Cochlear Implantation , Cochlear Implants , Hearing Loss, Sensorineural , Hearing Loss , Adult , Female , Humans , Male , Middle Aged , Aged , Aged, 80 and over , Hearing Loss, Sensorineural/surgery , Hearing Loss/surgery , Educational StatusABSTRACT
The difficulty of cochlear implantation in patients with congenital microtia is usually increased due to the vague anatomical marks and facial nerve malformation. The common types of facial nerve malformation include facial nerve bony cover loss, aberrant position, and bifurcation malformation. Bifurcation malformation may obscure the oval window, press against stapes, and bifurcate in the vestibular window while obscuring the round window. It is important to correctly identify the facial nerve and choose a reasonable surgical approach to avoid postoperative complications. This article describes a case of profound sensorineural hearing loss due to facial nerve malformation in our institution. The patient underwent cochlear implantation through the retro-facial approach. There was no facial nerve injury or dysfunction symptoms such as facial paralysis and hemifacial spasm 2 years after the operation, and the cochlear implant works well. The score of the categories of the auditory performanceï¼CAPï¼ questionnaire was 7, and the score of the speech intelligibility ratingï¼SIRï¼ questionnaire was 4. When the round window cannot be exposed through the facial recess approach during surgery, the retro-facial approach is a feasible method. To avoid facial nerve injury, a thin-section CT of the temporal bone should be performed before the middle and inner ear surgery for patients with facial nerve malformation, and the intraoperative facial nerve monitor should be used to clarify the course of the facial nerve to avoid injury.
Subject(s)
Cochlear Implantation , Congenital Microtia , Facial Nerve , Humans , Cochlear Implantation/methods , Congenital Microtia/surgery , Facial Nerve/abnormalities , Facial Nerve/surgery , Hearing Loss, Sensorineural/surgery , Temporal Bone/abnormalities , Temporal Bone/surgeryABSTRACT
PURPOSE: This review aims to provides a comprehensive overview of the latest research progress on IP-III inner ear malformation, focusing on its geneticbasis, imaging features, cochlear implantation, and outcome. METHODS: Review the literature on clinical and genetic mechanisms associated with IP-III. RESULTS: Mutations in the POU3F4 gene emerge as the principal pathogenic contributors to IP-III anomalies, primarily manifesting through inner ear potential irregularities leading to deafness. While cochlear implantation stands as the primary intervention for restoring hearing, the unique nature of the inner ear anomaly escalates the complexity of surgical procedures and postoperative results. Hence, meticulous preoperative assessment to ascertain surgical feasibility and postoperative verification of electrode placement are imperative. Additionally, gene therapy holds promise as a prospective treatment modality. CONCLUSIONS: IP-III denotes X-linked recessive hereditary deafness, with cochlear implantation currently serving as the predominant therapeutic approach. Clinicians are tasked with preoperative assement and individualized postoperative rehabilitation.
Subject(s)
Cochlear Implantation , Ear, Inner , POU Domain Factors , Humans , Cochlear Implantation/methods , Ear, Inner/abnormalities , POU Domain Factors/genetics , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/genetics , Hearing Loss, Sensorineural/congenital , Genetic Diseases, X-Linked/genetics , Genetic Diseases, X-Linked/surgery , Mutation , Vestibular Aqueduct/abnormalitiesABSTRACT
OBJECTIVE: To investigate the assumption that day-case cochlear implantation is associated with lower costs, compared to inpatient cochlear implantation, while maintaining equal quality of life (QoL) and hearing outcomes, for the Dutch healthcare setting. STUDY DESIGN: A single-center, non-blinded, randomized controlled trial in a tertiary referral center. METHODS: Thirty adult patients with post-lingual bilateral sensorineural hearing loss eligible for unilateral cochlear implantation surgery were randomly assigned to either the day-case or inpatient treatment group (i.e., one night admission). We performed an intention-to-treat evaluation of the difference of the total health care-related costs, hospital and out of hospital costs, between day-case and inpatient cochlear implantation, from a hospital and patient perspective over the course of one year. Audiometric outcomes, assessed using CVC scores, and QoL, assessed using the EQ-5D and HUI3 questionnaires, were taken into account. RESULTS: There were two drop-outs. The total health care-related costs were 41,828 in the inpatient group (n = 14) and 42,710 in the day-case group (n = 14). The mean postoperative hospital stay was 1.2 days (mean costs of 1,069) in the inpatient group and 0.7 days (mean costs of 701) for the day-case group. There were no statistically significant differences in postoperative hospital and out of hospital costs. The QoL at 2 months and 1 year postoperative, measured by the EQ-5D index value and HUI3 showed no statistically significant difference. The EQ-5D VAS score measured at 1 year postoperatively was statistically significantly higher in the inpatient group (84/100) than in the day-case group (65/100). There were no differences in postoperative complications, objective hearing outcomes, and number of postoperative hospital and out of hospital visits. CONCLUSION: A day-case approach to cochlear implant surgery does not result in a statistically significant reduction of health care-related costs compared to an inpatient approach and does not affect the surgical outcome (complications and objective hearing measurements), QoL, and postoperative course (number of postoperative hospital and out of hospital visits).
Subject(s)
Cochlear Implantation , Quality of Life , Humans , Cochlear Implantation/economics , Cochlear Implantation/methods , Male , Female , Middle Aged , Adult , Aged , Hearing Loss, Sensorineural/surgery , Hearing Loss, Sensorineural/economics , Ambulatory Surgical Procedures/economics , Hospitalization/economics , Netherlands , Health Care Costs , Hospital Costs/statistics & numerical data , Treatment Outcome , Cost-Benefit AnalysisABSTRACT
Viruses are often implicated as a cause of sensorineural hearing loss (SNHL), particularly sudden cases, including COVID-19. Determining the viral mechanism that leads to hearing loss is necessary for its future prevention and treatment. The 47-year-old woman who is the subject of this case study presented with sudden SNHL following multiple infections of COVID-19. Following a trial of a contralateral routing of sound device, she received a right cochlear implant (CI). Following a period of high performance, additional cases of COVID-19 infection and device failure issues resulted in the explant/reimplant of 1 ear and implantation of the contralateral ear. Despite extensive rehabilitation after these events, the patient continues to experience difficulties in speech understanding, not reaching her initial high levels of right ear performance. Further research is needed to determine the implications of COVID-19 as it relates to SNHL. This case study aimed to highlight the course of treatment and provide insight into the impact of COVID-19 on sudden hearing loss and its relationship to CI performance.
Subject(s)
COVID-19 , Cochlear Implantation , Cochlear Implants , Hearing Loss, Sensorineural , Hearing Loss, Sudden , Humans , Female , COVID-19/complications , Hearing Loss, Sudden/etiology , Hearing Loss, Sudden/virology , Middle Aged , Cochlear Implants/adverse effects , Hearing Loss, Sensorineural/etiology , Hearing Loss, Sensorineural/surgery , SARS-CoV-2 , Hearing Loss, Bilateral/surgery , Hearing Loss, Bilateral/etiologyABSTRACT
Cochlear implantation has become a standard of care for a child diagnosed with bilateral profound sensorineural hearing loss with a structured surgical standard operating procedure. A 3-year-old boy with bilateral profound prelingual sensorineural deafness underwent a Med-EL Sonata Ti100 implant. We faced a peculiar situation intraoperatively after inserting the electrodes and closing the wound. The impedance recording indicated high ground path impedance with short-circuiting of few electrodes. As a bionic implant, its electronic components may at times malfunction both intraoperatively and/or postoperatively; therefore, neural response telemetry (NRT) was invented to check it. By using NRT and a few milliliters of normal saline, we were able to diagnose as well as rectify the malfunctioning of the implant.
Subject(s)
Cochlear Implantation , Cochlear Implants , Hearing Loss, Sensorineural , Child, Preschool , Humans , Male , Cochlea/surgery , Cochlear Implantation/adverse effects , Cochlear Implantation/methods , Cochlear Implants/adverse effects , Hearing Loss, Bilateral/surgery , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/surgery , Saline Solution , Telemetry/methodsABSTRACT
INTRODUCTION: Surgical treatment of Ménière's disease (MD) and deafness aims to treat vertigo and hearing disabilities. Current treatment options like labyrinthectomy and cochlear implantation (CI) have shown acceptable results but are destructive. Less destructive procedures, like the occlusion of the lateral semicircular canal and endolymphatic sac surgery, have been shown to be successful in vertigo control. The combination of both procedures with CI has not been investigated; therefore the objective of this study was to investigate the outcome of this combination in patients with single-sided MD and moderately severe to complete sensorineural hearing loss. METHODS: In this retrospective study, 10 patients with single-sided MD and moderately severe to complete sensorineural hearing loss were included. In all of them, a single-staged surgery, which consisted of CI, endolymphatic sac surgery, and occlusion of the lateral semicircular canal, was performed. The surgery was performed after a failed conservative therapy trial. The clinical outcome was evaluated by the Dizziness Handicap Inventory (DHI) and audiological tests. These were assessed preoperatively, 3 and 6 months after surgery. An MRI with a hydrops sequence was performed to support the clinical diagnosis. RESULTS: After the combined surgery, the mean DHI testing improved significantly from 71 to 30. Mean audiological monosyllabic speech testing outcome with the cochlea implant was 65% at 65 dB. The residual hearing of 2 patients could be preserved after the surgical procedure. CONCLUSION: The combination of occlusion of the lateral semicircular canal, endolymphatic sac surgery, and CI is an efficient low traumatic treatment for patients with a single-sided MD and moderately severe to complete sensorineural hearing loss.
Subject(s)
Cochlear Implantation , Endolymphatic Sac , Hearing Loss, Sensorineural , Meniere Disease , Semicircular Canals , Humans , Meniere Disease/surgery , Male , Middle Aged , Retrospective Studies , Female , Semicircular Canals/surgery , Endolymphatic Sac/surgery , Adult , Aged , Hearing Loss, Sensorineural/surgery , Treatment Outcome , Deafness/surgeryABSTRACT
Nervus intermedius (NI) arises from the superior salivary nucleus, solitary nucleus, and trigeminal tract. It leaves the pons as 1 to 5 roots and travels between the facial and vestibulocochlear nerves before merging with the facial nerve within the internal auditory canal. The mastoid segment of the facial nerve then gives rise to a sensory branch that supplies the posteroinferior wall of the external auditory meatus and inferior pina. This complex pathway renders the nerve susceptible to various pathologies, leading to NI neuralgia. Here, the authors present an unusual intraoperative finding of an atrophic NI in a patient with refractory NI neuralgia and a history of ipsilateral sudden-onset central facial palsy and microvascular decompression for trigeminal neuralgia. The patient underwent NI sectioning via the previous retrosigmoid window and achieved partial ear pain improvement. The gross size of the NI is compared with a cadaveric specimen through stepwise dissection. This case highlights the potential significance of subtle central ischemic events and subsequent atrophy of NI in the pathogenesis of NI neuralgia, as well as the ongoing need to investigate the therapeutic efficacy of nerve sectioning.