Long-Term Results of the Linear Incision Technique With Tissue Reduction Versus Tissue Preservation for Inserting Bone-Anchored Hearing Implants: The Ongoing Optimization in Bone Implant Surgery.

OBJECTIVE: To compare the long-term outcomes of the linear incision technique with tissue reduction (LIT-TR) and the linear incision technique with tissue preservation (LIT-TP) for inserting bone-anchored hearing implants (BAHIs). STUDY DESIGN: Single-center retrospective cohort study. SETTING: Large general teaching hospital. PATIENTS: A total of 231 adult patients were included between August 2005 and October 2020, with a minimum follow-up time of 6 months. INTERVENTION: The test group received a BAHI using the LIT-TP (N = 147). The control group underwent surgery using the LIT-TR (N = 84). MAIN OUTCOME MEASURES: Soft tissue reactions, skin thickening, postoperative complications (e.g., wound dehiscence), and implant loss were compared between the test and control group. Furthermore, Cochlear Bone Anchored Solutions AB (Mölnlycke, Sweden) and Oticon Medical AB (Askim, Sweden) implants/abutments within the LIT-TP cohort were compared. Validated questionnaires were used to quantify patients' health-related quality of life (HRQoL). RESULTS: Significantly more cases with wound dehiscence and adverse soft tissue reactions (Holgers ≥2) were observed in the LIT-TR cohort (p < 0.001). However, the LIT-TP cohort showed significantly more cases with skin thickening (requiring treatment) within the first 2 years after implantation. There were no differences in implant loss rates, overall soft tissue reactions (Holgers >1), and overall HRQoL between the two patient groups. Significant improvement in the patients' HRQoL after implementation of a BAHI was found in both techniques. The Ponto Wide implant/abutment showed less frequent skin thickening (requiring treatment) and fewer soft tissue reactions compared with the BIA400 implant/abutment. CONCLUSION: This large-scale study demonstrates that the LIT-TP shows excellent long-term outcomes, including a low incidence of implant failure.

Postoperative Patient Reported Outcomes After Cholesteatoma Surgery.

BACKGROUND: Results and success measures of cholesteatoma surgery are generally described using objective data whereas subjective data are mostly lacking. Patients experiences and complaints are becoming more important alongside clinical and audiometric outcome measures in cholesteatoma care. OBJECTIVE: To investigate the course of patient-reported complaints, the impact of complaints, audiometric measures and the stability of audiometric measures, and complaints over time after primary and recurrent/residual cholesteatoma surgery. METHODS: Postoperative patients were prospectively included and divided into primary acquired and recurrent/residual cholesteatoma. The EuroQol 5D (EQ-5D-3L), Otology Questionnaire Amsterdam (OQUA), and the Speech Spatial Questionnaire (SSQ) were completed by 144 patients up to 2 years postoperative. Patient-reported complaints divided in eight separate domains, postoperative hearing and impact on daily life were longitudinally assessed by means of linear mixed models. RESULTS: Hearing loss and tinnitus are the most reported postoperative complaints over time. Patient-reported loss of taste and the impact of all complaints decline over time. All other patient-reported complaints remain stable over time, only itch complaints fluctuate. Primary cholesteatoma patients score significantly higher on hearing loss complaints compared with recurrent/residual patients although they have comparable mean audiometric hearing loss. Furthermore, pure-tone hearing threshold, instead of asymmetric hearing loss, is correlated with the localization domain of the SSQ. CONCLUSION: This study provides important insights in the course of complaints and its impact on daily life after cholesteatoma surgery. Overall, the postoperative patient-reported complaints after cholesteatoma surgery are generally low in the studied population.

A Multinational Cost-Consequence Analysis of a Bone Conduction Hearing Implant System-A Randomized Trial of a Conventional vs. a Less Invasive Treatment With New Abutment Technology.

Background: It is hypothesized that, for patients with hearing loss, surgically placing an implant/abutment combination whilst leaving the subcutaneous tissues intact will improve cosmetic and clinical results, increase quality of life (QoL) for the patient, and reduce medical costs. Here, incremental costs and consequences associated with soft tissue preservation surgery with a hydroxyapatite (HA)-coated abutment (test) were compared with the conventional approach, soft tissue reduction surgery with an all-titanium abutment (control). Methods: A cost-consequence analysis was performed based on data gathered over a period of 3 years in an open randomized (1:1) controlled trial (RCT) running in four European countries (The Netherlands, Spain, France, and Sweden). Subjects with conductive or mixed hearing loss or single-sided sensorineural deafness were included. Results: During the first year, in the Netherlands (NL), France (FR), and Spain (ES) a net cost saving was achieved in favor of the test intervention because of a lower cost associated with surgery time and adverse event treatments [NL €86 (CI -50.33; 219.20), FR €134 (CI -3.63; 261.30), ES €178 (CI 34.12; 97.48)]. In Sweden (SE), the HA-coated abutment was more expensive than the conventional abutment, which neutralized the cost savings and led to a negative cost (SE €-29 CI -160.27; 97.48) of the new treatment modality. After 3 years, the mean cost saving reduced to €17 (CI -191.80; 213.30) in the Netherlands, in Spain to €84.50 (CI -117.90; 289.50), and in France to €80 (CI -99.40; 248.50). The mean additional cost in Sweden increased to €-116 (CI -326.90; 68.10). The consequences in terms of the subjective audiological benefit and Health-related quality of life (HRQoL) were comparable between treatments. A trend was identified for favorable results in the test group for some consequences and statistical significance is achieved for the cosmetic outcome as assessed by the clinician. Conclusions: From this multinational cost-consequence analysis it can be discerned that health care systems can achieve a cost saving during the first year that regresses after 3 years, by implementing soft tissue preservation surgery with a HA-coated abutment in comparison to the conventional treatment. The cosmetic results are better. (sponsored by Cochlear Bone Anchored Solutions AB; Clinical and health economic evaluation with a new Baha® abutment design combined with a minimally invasive surgical technique, ClinicalTrials.gov NCT01796236).

Percutaneous bone-anchored hearing implant surgery: dermatome versus linear incision technique.

The objective of this historical cohort study is to identify if there are differences in soft tissue reactions and skin thickening between implantation of the percutaneous bone-anchored hearing implant (BAHI) using the dermatome or linear incision technique. All adult patients who received a BAHI between August 2005 and January 2013 were selected. One surgeon performed all procedures and only the dermatome and linear incision technique were used. A total of 132 patients/implants were included and significantly more patients with risk factors were seen in the linear incision cohort. A soft tissue reaction Holgers ≥1 was present in 18 patients (40.9 %) in the dermatome compared to 36 patients (40.9 %) in the linear incision group. A Holgers ≥2 was noticed in 9 (20.5 %) and 19 (21.6 %) patients, respectively. Skin thickening was described in 14 (31.8 %) and 11 patients (12.5 %) in, respectively, the dermatome and linear incision cohort, which was a significant difference (p = 0.001). Nevertheless, therapeutic interventions were effective. In conclusion, there was no significant difference in (adverse) soft tissue reactions; however, skin thickening was more present in the dermatome technique. In addition, significantly more patients with risk factors were allocated to the linear incision technique. Based on these results, the linear incision is advocated as preferred technique.

Vestibular deterioration precedes hearing deterioration in the P51S COCH mutation (DFNA9): an analysis in 74 mutation carriers.

OBJECTIVES: To analyze cochleovestibular impairment features in P51S COCH mutation carriers (n = 22) in a new, large Dutch family and to compare the results to those obtained in previously identified similar mutation carriers (n = 52). To evaluate age-related features between progressive hearing and vestibular impairment of all mutation carriers (n = 74). STUDY DESIGN: Family study. METHODS: Regression analysis was performed in relation to age to outline the development of hearing thresholds, speech recognition scores, and vestibulo-ocular reflex time constant as the key vestibular response parameter. RESULTS: Pure tone thresholds, phoneme recognition scores, and vestibular responses of the mutation carriers in the new family were essentially similar to those previously established in all other mutation carriers. Hearing started to deteriorate in all mutation carriers from 43 years of age onwards, whereas deterioration of vestibular function started from age 34. CONCLUSION: Vestibular impairment starts earlier, progresses more rapidly, and, eventually, is more complete than hearing impairment in P51S COCH mutation carriers.

Progression of low-frequency sensorineural hearing loss (DFNA6/14-WFS1).

OBJECTIVE: To assess the audiometric profile and speech recognition characteristics in affected members of 2 families with DFNA6/14 harboring heterozygous mutations in the WFS1 gene that cause an autosomal dominant nonsyndromic sensorineural hearing impairment trait. DESIGN: Family study. SETTING: Tertiary referral center. Patients Thirteen patients from 2 recently identified Dutch families with DFNA6/14 (Dutch III and IV). METHODS: Cross-sectional and longitudinal analyses of pure-tone thresholds at octave frequencies of 0.25 to 8 kHz were performed, and speech phoneme recognition scores were assessed. Progression was evaluated by linear regression analysis with and without correction for presbycusis. RESULTS: All individuals showed low-frequency hearing impairment. The 2-kHz frequency was more affected in the Dutch III family than in the Dutch IV family. Progressive hearing loss beyond presbycusis was found in the Dutch IV family and in 3 individuals in the Dutch III family. Annual threshold deterioration was between 0.6 and 1 dB per year at all frequencies. The speech recognition scores in the Dutch III family showed significantly more deterioration at increasing levels of hearing impairment compared with those in the Dutch IV family. CONCLUSION: Both families showed an autosomal dominant, progressive, low-frequency sensorineural hearing impairment caused by heterozygous WFS1 mutations.

Cross-sectional analysis of hearing threshold in relation to age in a large family with cochleovestibular impairment thoroughly genotyped for DFNA9/COCH.

Hearing threshold was analyzed for each frequency in relation to age in 88 members of a large Dutch family with cochleovestibular impairment caused by a P51S mutation in the COCH gene within the DFNA9 locus (chromosome 14q12-13). The participants in this study were 34 mutation carriers and 54 relatives without the mutation (control subjects). A sigmoidal dose-response curve with a variable slope was used to fit the mutation carriers' threshold-on-age data. Progression started at about 40 years of age and only lasted for some 20 to 25 years; the associated average progression was 2.9 dB/y for all frequencies. However, some hearing impairment was already present before, predominantly at the high frequencies. The mean thresholds in the young mutation carriers (< 33 years of age) were significantly higher (by 4 to 13 dB) than those in age-matched controls at 2 to 8 kHz. Presumably, mutation carriers have a congenital, stable offset threshold (10 to 29 dB) at these frequencies, and develop progression later in life.

Autosomal dominant low-frequency hearing impairment (DFNA6/14): a clinical and genetic family study.

OBJECTIVE: To delineate the phenotype and genotype of an autosomal dominant low-frequency sensorineural nonsyndromic hearing impairment trait in relation to similar traits. STUDY DESIGN: Family study, including retrospective case reviews. SETTING: Tertiary referral center. PATIENTS: Hearing impairment was documented in 11 family members in five generations, 8 of whom were alive and participated in this study. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: Clinical study: medical and otologic history and examination, retrieval of previous audiograms, pure-tone audiometry, and statistical analysis of audiometric data. Genetic study: linkage analysis of blood samples in 18 clinically affected and nonaffected family members. RESULTS: Hearing impairment had been present since early childhood, mainly affecting the low frequencies (mean threshold 45 dB HL at 0.25-1 kHz); speech recognition was hardly affected during the first three decades of life. Higher frequencies became involved with increasing age, thus causing a flat-type audiogram at middle age and down-sloping audiograms after age 60 years. Progression was mild but significant at all frequencies (0.5 dB/year at 0.25 kHz to 1.3 dB/year at 8 kHz) and persisted after correction was applied for normal presbyacusis. The trait was linked to chromosome 4p16.3, in a region comprising both the previously located, closely adjacent DFNA6 and the DFNA14 loci for low-frequency hearing impairment. CONCLUSION: A third family (designated Dutch II) was identified with a low-frequency hearing impairment trait showing linkage to chromosome 4p16.3 (DFNA6/14). The progression of hearing impairment beyond presbyacusis in the current study is unprecedented for DFNA6/14 traits.

Mutations in the WFS1 gene that cause low-frequency sensorineural hearing loss are small non-inactivating mutations.

Hereditary hearing impairment is an extremely heterogeneous trait, with more than 70 identified loci. Only two of these loci are associated with an auditory phenotype that predominantly affects the low frequencies (DFNA1 and DFNA6/14). In this study, we have completed mutation screening of the WFS1 gene in eight autosomal dominant families and twelve sporadic cases in which affected persons have low-frequency sensorineural hearing impairment (LFSNHI). Mutations in this gene are known to be responsible for Wolfram syndrome or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), which is an autosomal recessive trait. We have identified seven missense mutations and a single amino acid deletion affecting conserved amino acids in six families and one sporadic case, indicating that mutations in WFS1 are a major cause of inherited but not sporadic low-frequency hearing impairment. Among the ten WFS1 mutations reported in LFSNHI, none is expected to lead to premature protein truncation, and nine cluster in the C-terminal protein domain. In contrast, 64% of the Wolfram syndrome mutations are inactivating. Our results indicate that only non-inactivating mutations in WFS1 are responsible for non-syndromic low-frequency hearing impairment.