Patient and clinician experiences with the multidisciplinary single-day cochlear implant selection (MSCS) protocol.

PURPOSE: This study assessed the MSCS (Multidisciplinary Single-day Cochlear Implant Selection) protocol with a primary focus on sustaining or enhancing patient satisfaction throughout the cochlear implant selection process. MATERIALS AND METHODS: Following the implementation of the new selection protocol, where all selection appointments take place on the same day, we surveyed 37 individuals who underwent the process. Twenty adhered to the standard procedure, while 17 followed the MSCS protocol. We also gathered feedback from seven out of eight involved healthcare providers. This method enabled us to evaluate the protocol's effectiveness in maintaining patient satisfaction and ensuring staff contentment with care delivery within a condensed timeframe. RESULTS: Patient responses showed slight variations in average scores without statistical significant differences, indicating comparable satisfaction between the MSCS pathway and the standard protocol. The majority of patients preferred the MSCS protocol, with none of the MSCS participants opting for appointments spread over multiple days. Healthcare practitioners of the CI center also displayed similar or increased satisfaction levels with the MSCS protocol. CONCLUSION: The adoption of the MSCS in daily clinical care has led to a decrease in patient appointment times without sacrificing patient satisfaction. Additionally, the majority of individuals actively choose the MSCS protocol. Among those who have directly experienced it, there is unanimous preference for the consolidated appointments over spreading them across multiple days. Professionals within the CI team express equal satisfaction with both the new and old protocols, indicating that the reduction in patient time does not diminish overall satisfaction.

Pilot study of a multidisciplinary single-day cochlear implant selection protocol.

PURPOSE: This study aimed to explore and introduce the potential of a MSCS (Multidisciplinary Single-day Cochlear Implant Selection) protocol. The primary objectives of this pilot were to reduce the duration between referral and surgery, minimize hospital visits and decrease the time healthcare professionals dedicate to the cochlear implant (CI) selection process. MATERIALS AND METHODS: We established a pilot program at the CI center of the Erasmus MC, a tertiary referral center in the Netherlands, with the goal of improving and shorten the selection process. We evaluated our pilot, including 15 CI candidates, and conducted a retrospective analysis for time and cost savings. RESULTS: The results showed that the pilot of the MSCS protocol significantly reduced the length of the CI selection phase (84 days vs 1; standard intake vs MSCS protocol) and the number of hospital visits (6 vs 2 visits; standard vs MSCS protocol), resulting in less travel time and lower costs for the CI candidates. The total time of professionals spend on patients was also reduced with 27 %. CONCLUSION: This study highlights the potential benefits of the MSCS protocol in terms of reducing the burden on patients and healthcare providers and improving the efficiency of the CI selection process.

Evaluation of subclasses for T4-classified squamous cell carcinoma of the external auditory canal.

BACKGROUND: T4-classified squamous cell carcinoma (SCC) of external auditory canal (EAC) can potentially involve different anatomical structures, which could translate into different treatment strategies and survival outcomes within one classification. Our aim is to evaluate the clinical added value of T4-subclasses proposed by Lavieille and by Zanoletti. METHODS: Retrospective data, including patients with primary operated cT4-classified EAC SCC, was obtained from 12 international hospitals. We subclassified according to the T4-subclasses. The treatment strategies, disease-free survival (DFS) and overall survival per subclass were calculated. RESULTS: A total of 130 T4-classified EAC SCC were included. We found commonly used treatment strategies per subclass according to Lavieille and the DFS seems also to differ per subclass. Subclass according to Zanoletti showed comparable treatment strategies and survival outcomes per subclass. CONCLUSION: Our study suggests that the subclass according Lavieille might have added value in clinical practice to improve care of T4-classified EAC SCC.

A retrospective analysis of hearing after cholesteatoma surgery: the bony obliteration tympanoplasty versus canal wall up and canal wall down without mastoid obliteration.

OBJECTIVE: To evaluate and compare the hearing outcome after the bony obliteration tympanoplasty (BOT), canal wall up (CWU) without mastoid obliteration and canal wall down (CWD) without mastoid obliteration in a large patient cohort. As the aeration of the middle ear is associated with hearing outcome, we hypothesized that the post-operative hearing after the BOT may be better compared to CWU and CWD without obliteration. METHODS: This is a retrospective cohort study on all adult patients who underwent the BOT, CWU without obliteration or CWD without obliteration for primary or revision cholesteatoma between January 2003 and March 2019 with audiological follow-up at our institution. Pre-operative, short-term post-operative and long-term post-operative hearing tests were analyzed and potential factors influencing post-operative hearing were assessed. RESULTS: 626 ears were included. We found no significant differences between the short-term and long-term post-operative audiometry. The pre-operative air-bone gap (ABG) was the factor with the largest effect size on change in air-bone gap (ABG) between pre- and post-operative. When stratifying for this factor along with the type of ossicular chain reconstruction to account for differences at baseline, no significant differences in post-operative ABG were found between BOT and non-obliteration CWU and CWD. CONCLUSION: In this large retrospective cohort study, we found no significant differences in post-operative ABG between the BOT and the non-obliteration CWU and CWD. A solid comparison of hearing between groups remains very challenging as hearing outcome seems to be dependent on many different factors. Hearing outcome seems to be no additional argument to choose for BOT over non-obliteration surgery.

Revision surgery for chronically discharging mastoid cavities: mastoid obliteration with canal wall reconstruction versus non-obliteration surgery.

PURPOSE: To evaluate the surgical results of revision canal wall down (CWD) surgery for chronically discharging mastoid cavities and to compare the non-obliteration approach to mastoid obliteration with canal wall reconstruction. METHODS: This is a retrospective cohort study. All adult patients (≥ 18 years) who underwent revision surgery for chronically draining mastoid cavities between January 2013 and January 2020 were included. Primary outcome measures included the dry ear rate, complications and postoperative hearing. RESULTS: 79 ears were included; 56 ears received revision CWD with mastoid obliteration and posterior canal wall reconstruction and 23 ears received CWD without mastoid obliteration. The dry ear rate at the most recent outpatient clinic visit (median 28.0 months postoperative) was significantly higher in the obliteration group with 96.4% compared to 73.9% for the non-obliteration group (p = .002). There were no differences in audiological outcome and incidence of complications between the two techniques. CONCLUSION: We show that in our study population revision CWD surgery with mastoid obliteration and posterior canal wall reconstruction is superior to revision CWD surgery without mastoid obliteration in the management of chronically discharging mastoid cavities. In the obliteration group, a dry ear was achieved in 96.4% as this was 73.9% in the non-obliteration group. We found no differences in audiological outcome and in incidence of complications between the two techniques.

Treatment Outcome of the Bony Obliteration Tympanoplasty Versus Nonobliterative Techniques in Cholesteatoma Surgery: A Retrospective Analysis.

OBJECTIVE: In cholesteatoma surgery, obliteration of the mastoid and epitympanic space (bony obliteration tympanoplasty, BOT) is an increasingly used technique with low recurrent and residual cholesteatoma rates. While factors as the postoperative hearing level and infection rate are important for the patient as well, these outcome parameters are not frequently reported on in current literature. The objective of this study is to evaluate the recurrent and residual cholesteatoma rates of the BOT technique and nonobliterative canal wall up (CWU) and canal wall down (CWD) mastoidectomy in a large patient cohort. Secondary objectives were to evaluate the infection rate and hearing outcome for all three techniques. DESIGN: Retrospective cohort study. SETTING: Single-center study. PATIENTS: All 337 adult patients (≥18 yrs) who underwent primary or revision cholesteatoma surgery between January 2013 and March 2019 were included. MAIN OUTCOME AND MEASURES: Recurrent cholesteatoma rates, residual cholesteatoma rates, postoperative infections and other complications, hearing outcome. RESULTS: The estimated combined rate of recurrent and residual cholesteatoma at 5 years follow-up was 7.6% in the BOT group, 34.9% in the CWU group, and 17.9% in the CWD group. The postoperative infection rate in the different groups ranged from 4.3% to 4.9%. The median gain in AC threshold level varied from 0.0 dB in the BOT and CWD group to 3.8 dB in the CWU group. CONCLUSIONS: We show that cholesteatoma recurrence rates after the BOT technique in our clinic are significantly lower compared to CWU surgery. There were no differences in infection rate and no clinically relevant differences in postoperative hearing between the BOT, CWU, and CWD technique.

Uniform Registration Agreements on Cholesteatoma Care: A Nationwide Consensus Procedure.

BACKGROUND: To coordinate and align the content for registration of cholesteatoma care. METHODS: Systematic Delphi consensus procedure, consisting three rounds: two written sessions followed by a face-to-face meeting. Before this procedure, input on important patient outcomes was obtained. Consensus was defined as at least 80% agreement by participants. Hundred-thirty-six adult patients who had undergone cholesteatoma surgery and all ENT surgeons of the Dutch ENT Society were invited. The consensus rounds were attended by ENT surgeons with cholesteatoma surgery experience. Feasibility and acceptability of outcome measures and reporting agreements were assessed in round 1 by 150 ENT surgeons. In round 2 definitions were narrowed and context information to interpret outcome measure were questioned. In round 3, the results, amendments, and the open-ended points were discussed to reach agreement. RESULTS: Most important outcome measures are: 1) the presence or absence of a cholesteatoma in the first 5 years after surgical removal of cholesteatoma, 2) hearing level after surgical removal of cholesteatoma, and 3) the documented assessment of patient's complaints with a validated patient reported outcome measures questionnaire (PROM). Furthermore, consensus was reached on the registration of cholesteatoma type (residual/recurrent), localization of cholesteatoma, and reporting of the presence of cholesteatoma in the follow-up. CONCLUSION: Consensus was reached on the content and method of registration of cholesteatoma care based on patient's and ENT surgeons input. Three outcome measures were defined. National agreements on the method and content of registration will facilitate monitoring and feedback to the ENT surgeon about the cholesteatoma care.

Evaluation of the modified Pittsburgh classification for predicting the disease-free survival outcome of squamous cell carcinoma of the external auditory canal.

BACKGROUND: Squamous cell carcinoma (SCC) of the external auditory canal (EAC) is a rare disease, which is commonly classified with the modified Pittsburgh classification. Our aim was to evaluate the predictive performance of this classification in relation to disease-free survival (DFS). METHODS: We examined retrospective data from a nationwide Dutch cohort study including patients with primary EAC SCC. These data were combined with individual patient data from the literature. Using the combined data, the predictive performances were calculated using the c-index. RESULTS: A total of 381 patients were included, 294 for clinical and 281 for the pathological classification analyses. The c-indices of the clinical and the pathological modified Pittsburgh classification predicting DFS were 0.725 (0.668-0.782) and 0.729 (0.672-0.786), respectively. CONCLUSION: The predictive performance of the modified Pittsburgh classification system as such appears to be acceptable to predict the DFS of EAC SCC. Other factors need to be added to a future model to improve the predicted performance.

Single-Stage Mastoid Obliteration in Cholesteatoma Surgery and Recurrent and Residual Disease Rates: A Systematic Review.

IMPORTANCE: The ideal surgical treatment of cholesteatoma has been subject to discussion for years because both traditional surgical techniques (canal wall down [CWD] and canal wall up [CWU] tympanoplasty) have their own advantages and disadvantages. A more recently propagated surgical approach, to combine the CWD or CWU tympanoplasty technique with obliteration of the mastoid and epitympanum, is showing promising results. OBJECTIVE: To systematically review the literature on recurrent and residual cholesteatoma rates after single-stage CWU and CWD tympanoplasty with mastoid obliteration. EVIDENCE REVIEW: A systematic search of literature was performed to identify relevant publications in multiple electronic databases. The initial search was conducted in December 2016 and was updated in July 2017. Each study was reviewed by 2 independent reviewers on predetermined eligibility criteria. The methodological quality was determined using the methodological index for nonrandomized studies (MINORS) scale, and the relevance to the current topic was determined using a 4-criterion checklist. FINDINGS: The searches identified a total of 336 potentially relevant publications; 190 articles were excluded based on title and abstract. The full-text articles of the remaining 146 citations were assessed for eligibility, resulting in 22 articles. After assessing these remaining articles for methodological quality and relevance to the current topic, another 8 studies were excluded, and a total of 13 studies (1534 patients) were included. Of the 1534 patients who underwent CWD or CWU tympanoplasty with mastoid obliteration, the rate of recurrent disease was 4.6%, and the rate of residual disease was 5.4%. In CWU tympanoplasty with mastoid obliteration, these rates were 0.28% and 4.2%, respectively and in CWD tympanoplasty with mastoid obliteration, 5.9% and 5.8%, respectively. CONCLUSIONS AND RELEVANCE: We show the recurrent and residual disease rates after either CWU or CWD tympanoplasty with mastoid obliteration to be qualitatively similar to, if not better than, previously reported rates of for nonobliterative techniques. In this study, the lowest recurrent and residual rates were reported when combining the CWU tympanoplasty with mastoid obliteration, on average 0.28 and 4.2%, respectively.

Phenotype analysis of an Australian DFNA9 family with the 1109N COCH mutation.

OBJECTIVES: We studied the clinical characteristics of an Australian family with an autosomal dominant sensorineural hearing impairment (DFNA9) caused by an I109N mutation in COCH. METHODS: Retrospective analyses of audiometric data from 8 mutation carriers of an Australian DFNA9 family with the I109N COCH mutation were performed. Cross-sectional hearing levels related to age, age-related typical audiograms, and speech recognition scores related to age and to the level of hearing impairment were investigated. Data were compared to those obtained in previously identified DFNA9 families with P51S, V66G, G87W, G88E, I109T, and C542F COCH mutations. RESULTS: Deterioration of hearing in the I109N mutation carriers started before the age of 40 years. The audiometric characteristics of the I109N mutation carriers are essentially similar to those previously established in I109T mutation carriers and, to a lesser extent, in P51S, G87W, and G88E mutation carriers. CONCLUSIONS: The phenotype associated with the I109N COCH mutation is largely similar to that associated with the I109T, P51S, G87W, and G88E mutation carriers. However, subtle differences seem to exist in terms of age of onset and rate of progression.

Vestibular impairment in a Dutch DFNA15 family with an L289F mutation in POU4F3.

Vestibular examination (electronystagmography with rotatory chair and caloric tests) was performed on 18 carriers and 1 phenocopy carrier in a Dutch family with autosomal dominant nonsyndromic DFNA15. This is the second DFNA15 family worldwide to have a novel L289F mutation in POU4F3. Vestibular involvement appeared to be present in 2 affected individuals according to their medical history. Vestibular examination results in an extended subset of L289F POU4F3 mutation carriers varied from normal to areflexia. DFNA15 is the third cochleovestibular disorder, after DFNA9 and DFNA11, in the autosomal dominant nonsyndromic hearing impairment.

Audiometric characteristics of a Dutch family linked to DFNA15 with a novel mutation (p.L289F) in POU4F3.

OBJECTIVE: To report on the audiometric characteristics of a large Dutch family linked to DFNA15 with a novel mutation (p.L289F) in POU4F3 (OMIM 602460). DESIGN: Clinical investigation. SETTING: Tertiary referral center. PATIENTS: Family members from a large 5-generation pedigree with sensorineural hearing impairment segregating as an autosomal dominant trait. MAIN OUTCOME MEASURES: Cross-sectional and longitudinal analyses of pure-tone audiometric data, and cross-sectional analyses of speech audiometry data. RESULTS: Overall, a flat to gently downsloping audiometric configuration was observed with a progression rate of approximately 0.8 dB/y across most frequencies. Speech recognition scores remained fairly good in relation to age and hearing level compared with a group of patients with presbycusis. Interindividual variability was observed in terms of subjective onset age and audiometric configuration. Two mutation carriers, who reported vestibular symptoms, underwent vestibular examination and showed hypofunction of the vestibular labyrinth. CONCLUSIONS: The audiometric phenotype of the Dutch family linked to DFNA15 with a novel mutation in POU4F3 is comparable to that observed in the original Israeli family linked to DFNA15. Relatively good speech recognition scores suggest outer hair cell involvement. DFNA15 may represent a cochleovestibular disorder.

Phenotype description of a novel DFNA9/COCH mutation, I109T.

OBJECTIVES: This is a report of the audiological and vestibular characteristics of a Dutch DFNA9 family with a novel mutation, I109T, in the LCCL domain of COCH. METHODS: From the family with the novel I109T COCH mutation, audiometric data were collected and analyzed longitudinally. Results were compared to those obtained in previously identified P51 S, G88E, and G87W COCH mutation carriers. Special attention was also given to a comparison of age-related features such as progressive hearing loss and vestibular impairment. RESULTS: A novel mutation (I109T) in COCH segregates with hearing impairment and vestibular dysfunction in the present family. Pure tone thresholds, phoneme recognition scores, and vestibular responses of the I109T mutation carriers were essentially similar to those previously established in P51S, G87W, and G88E mutation carriers. Deterioration of hearing in the I109T mutation carriers started at 43 years of age, and vestibular function deteriorated at least 7 years later. CONCLUSIONS: The phenotype associated with the novel COCH (I109T) mutation is largely similar to that associated with P51S and G88E mutation carriers. However, subtle differences in terms of onset age and rate of progression seem to exist.

Vertical corneal striae in families with autosomal dominant hearing loss: DFNA9/COCH.

PURPOSE: Investigation of a possible association between vertical corneal striae and mutations in the COCH gene, observed in four DFNA9 families with autosomal dominant hearing loss and vestibular dysfunction. DESIGN: Prospective case series. METHODS: Ophthalmologic examinations with photography of the cornea after instillation of fluorescein were performed in 98 family members with 61 mutation carriers of four DFNA9 families at the Radboud University Nijmegen Medical Centre. Families 1 and 2 harbor the Pro51Ser mutation, and families 3 and 4 harbor the Gly88Glu and the Gly87Trp mutation, respectively. Statistical analysis was performed to find an association between the vertical corneal striae and the COCH mutation for each family and to test whether the four families were different in this respect. RESULTS: The vertical corneal striae were exclusively visible after instillation of fluorescein. They caused minor problems, as dry eye symptoms, and were not present in the general Dutch ophthalmologic population. The striae were present from an age of 47 years in 32 individuals, of whom 27 individuals had a COCH mutation. Statistical analysis on the striae and the COCH mutations showed a significant association in families 1, 2, and 3 (P = .0006), but not in family 4 (P = .63). CONCLUSIONS: Data analysis demonstrated a significant association between vertical corneal striae and the Pro51Ser and Gly88Glu mutations in the COCH gene in DFNA9 families 1, 2, and 3 with cochleovestibular dysfunction. Our findings suggest that the vertical corneal striae and cochleovestibular dysfunction may be caused by the same COCH mutations.

Clinical characteristics of a Dutch DFNA9 family with a novel COCH mutation, G87W.

The present study aims to report audiological and vestibular characteristics of a Dutch DFNA9 family with a novel mutation, G87W, in the LCCL domain of COCH. From the family with the novel G87W COCH mutation audiometric data were collected and analyzed longitudinally. Results were compared with those obtained in previously identified P51S COCH mutation carriers (n = 74) and with those obtained in G88E mutation carriers. Special attention was also given to a comparison of age-related features, such as progressive hearing loss and vestibular impairment. A novel mutation (G87W) in COCH is indicative of hearing impairment and vestibular dysfunction in the present family. Pure-tone thresholds, phoneme recognition scores, and vestibular responses of the G87W mutation carriers were essentially similar to those previously established in the P51S and G88E mutation carriers. Deterioration of hearing and vestibular function in the G87W mutation carriers started at the age of 43 years. Remarkably, similar to G88E mutation carriers, the proportion of patients over 40 years of age who developed complete vestibular areflexia was significantly lower for the G87W mutation carriers than for the P51S mutation carriers. In conclusion, the phenotype associated with the novel COCH (G87W) mutation is largely similar to that associated with the P51S and G88E mutation carriers. However, subtle differences in terms of onset age and rate of progression seem to exist.

The phenotype of the first otosclerosis family linked to OTSC5.

OBJECTIVE: To report the audiometric and radiologic findings in the first otosclerosis family linked to OTSC5. STUDY DESIGN: A clinical investigation of a family linked to OTSC5, including analyses of audiometric data and of high-resolution computed tomography (CT) images of the temporal bones from genetically affected family members. SETTING: Tertiary referral center. PATIENTS: Family members from a four-generation pedigree with otosclerosis segregating as an autosomal dominant trait. MAIN OUTCOME MEASURE(S): Pre-surgery pure tone audiometric data. Classification of otosclerotic foci on high-resolution spiral CT images of the temporal bones of genetically affected individuals. RESULTS: Audiometric data showed a considerable degree of phenotypic variability. Cross-sectional regression analysis did not disclose any clear age dependence of threshold-related data. Systematic differences between mean parameter values relating to the thresholds in the best or the worst ear were found. High-resolution CT images revealed a fenestral otosclerotic focus in seven of nine (78%) clinically affected individuals and cochlear foci in one of these seven patients. CONCLUSION: The phenotype of OTSC5 seems to be variable. Additional long-term audiometric data are needed to construct age-related typical audiograms, which may also facilitate the comparison between phenotypes of the different otosclerosis loci. The detection rate of otospongiotic foci in our study group is similar or lower compared with previous reports on CT data in consecutive otosclerosis patients who had stapes replacing surgery.