Hanneke Bruijnzeel

The disease recurrence rate after the canal wall up (CWU) or canal wall down (CWD) technique in adults

To review which type of cholesteatoma surgery, canal wall up (CWU) or canal wall down (CWD), provides the lowest risk for residual and/or recurrent disease in adults with primary acquired cholesteatoma.

A Systematic Review to Define the Speech and Language Benefit of Early (<12 Months) Pediatric Cochlear Implantation

Objective: This review aimed to evaluate the additional benefit of pediatric cochlear implantation before 12 months of age considering improved speech and language development and auditory performance. Materials and Methods: We conducted a search in PubMed, EMBASE and CINAHL databases and included studies comparing groups with different ages at implantation and assessing speech perception and speech production, receptive language and/or auditory performance. We included studies with a high directness of evidence (DoE). Results: We retrieved 3,360 articles. Ten studies with a high DoE were included. Four articles with medium DoE were discussed in addition. Six studies compared infants implanted before 12 months with children implanted between 12 and 24 months. Follow-up ranged from 6 months to 9 years. Cochlear implantation before the age of 2 years is beneficial according to one speech perception score (phonetically balanced kindergarten combined with consonant-nucleus-consonant) but not on Glendonald auditory screening procedure scores. Implantation before 12 months resulted in better speech production (diagnostic evaluation of articulation and phonology and infant-toddler meaningful auditory integration scale), auditory performance (Categories of Auditory Performance-II score) and receptive language scores (2 out of 5; Preschool Language Scale combined with oral and written language skills and Peabody Picture Vocabulary Test). Conclusions: The current best evidence lacks level 1 evidence studies and consists mainly of cohort studies with a moderate to high risk of bias. Included studies showed consistent evidence that cochlear implantation should be performed early in life, but evidence is inconsistent on all speech and language outcome measures regarding the additional benefit of implantation before the age of 12 months. Long-term follow-up studies are necessary to provide insight on additional benefits of early pediatric cochlear implantation.

Influence of Ossicular Chain Damage on Hearing After Chronic Otitis Media and Cholesteatoma Surgery A Systematic Review and Meta-analysis

IMPORTANCE Physicians should ideally be able to provide patients with chronic otitis media and/or cholesteatoma specific information about postoperative hearing outcome, based on their level of preoperative ossicular chain damage (OCD). OBJECTIVE To identify the influence of preoperative OCD on hearing outcomes in patients after chronic otitis media and/or cholesteatoma surgery. DATA SOURCES PubMed, EMBASE, and the Cochrane Library databases were systematically searched for available evidence, without any constraints, on December 13, 2014, for articles published between January 1, 1975, and December 13, 2014. STUDY SELECTION We reviewed the literature for articles assessing the prognostic value of OCD on postoperative hearing outcome (air-bone gap [ABG] in decibels), using Austin-Kartush criteria or independent OCD classification systems. We assessed relevance and validity using a self-designed critical appraisal tool based on the Cochrane Collaborations risk of bias tool. DATA EXTRACTION Characteristics of study populations and postoperative ABGs in decibels were extracted from all included studies by 4 authors (E.F.B., M.N.G., N.J.K., A.S.H.J.L.). RESULTS The tested hypothesis was formulated before data collection. Primary study outcome was defined as postoperative adult hearing outcomes after COM and/or cholesteatoma surgery defined as mean postoperative ABG. Our search yielded 5661 articles. Nine articles with high relevance were included. Pooled results of studies using the Austin-Kartush criteria showed a significant (P < .001) difference in mean ABG in favor of group B, when comparing group B (patients with malleus present, stapes absent; 11.1 [95% CI, 10.3-11.8] dB) to group C (patients with malleus absent, stapes present; 15.7 [95% CI, 14.6-16.7] dB) and group B to group D (patients with malleus absent, stapes absent; 16.5 [95% CI, 15.2-17.9] dB). Three studies using independent OCD classification criteria found no influence of stapes structure (intact stapes suprastructure, 13.5 [95% CI, 10.3-16.7], 15.1 [95% CI, 11.8-18.3], and 21.9 [95% CI, 15.0-28.8] dB vs absent stapes structure, 12.8 [95% CI, 9.5-16.1], 19.5 [95% CI, 14.9-24.1], and 30.2 [95% CI, 24.7-35.8] dB) on postoperative ABG. One study reported a significant (P = .04) difference in mean ABG between patients with present (18.9 [95% CI, 15.7-22.1] dB) and absent (24.4 [95% CI, 20.2-28.6] dB) malleus. CONCLUSIONS AND RELEVANCE Pooled results of Austin-Kartush studies showed that in patients with COM, with or without cholesteatoma, the malleus status is a significant predictor of postoperative hearing outcome, independent of the stapes condition. Studies reporting on individual ossicle status supported this finding by showing that only malleus condition influenced postoperative hearing outcome. These findings are based on level IV evidence, which indicates the need for future high-level evidence studies.

Systematic Review on Surgical Outcomes and Hearing Preservation for Cochlear Implantation in Children and Adults

Objective The mastoidectomy with facial recess approach (MFRA) is considered the reference standard for cochlear implantation. The suprameatal approach (SMA) was developed more recently and does not require mastoidectomy, which could influence postoperative outcomes. We aim to identify the optimal operative approach for cochlear implantation based on postoperative complications and hearing preservation in children and adults. Data Sources PubMed, EMBASE, Scopus, and Google Scholar. Review Methods Studies comparing MFRA and SMA in children and adults were eligible for inclusion. Original reports with moderate relevance and validity were included. Relevance and validity were assessed with a self-modified critical appraisal tool. This review was reported in accordance to PRISMA guidelines. Results We retrieved 294 citations. Only retrospective nonrandomized studies were identified (level III evidence). Six articles were selected for full-text inclusion and 4 articles for data extraction. No article found a significant difference between MFRA and SMA with respect to postoperative complications in children and adults. One study found a significantly (P < .023) higher pediatric MFRA mastoiditis rate; however, meta-analysis did not indicate an overall effect. Hearing preservation was reported only in adults, and outcomes between techniques did not differ. Conclusion No evidence was noted for lower complication rates or improved hearing preservation between the MFRA and SMA for cochlear implantation in children and adults. Pediatric data were available for children implanted above the age of 24 months only. Level I evidence is needed to resolve the uncertainty regarding differences in postoperative outcomes of pediatric and adult MFRA and SMA.

A systematic review on the surgical outcome of preauricular sinus excision techniques.

Preauricular sinuses are benign congenital malformations of preauricular soft tissues. Complete excision using either sinectomy or supra‐auricular approach is advised to prevent recurrence. Reported recurrence varies between 0 and 42%. We evaluated which surgical technique resulted in lowest complication and recurrence rates.

Preliminary audiologic and peri-operative outcomes of the Sophono™ transcutaneous bone conduction device: A systematic review

OBJECTIVE To delineate the auditory functional improvement and peri-operative outcomes of the Sophono™ transcutaneous bone conduction device. METHODS Eligible articles presenting patients implanted with the Sophono™ were identified through a comprehensive search of PubMed and Embase electronic databases. All relevant articles were reviewed to justify inclusion independently by 2 authors. Studies that successfully passed critical appraisal for directness of evidence and risk of bias were included. RESULTS From a total of 125 articles, 8 studies encompassing 86 patients using 99 implants were selected. Most patients (79.1%) were children. Ear atresia (67.5%) was the most frequently reported indication for Sophono™ implantation. Overall pure tone average auditory improvement was 31.10 (±8.29) decibel. During a mean follow-up time of 12.48 months, 25 patients (29%) presented with post-operative complications from which 3 were deemed as serious implant-related adverse events (3.5%). CONCLUSIONS The Sophono™ transcutaneous bone conduction device shows promising functional improvement, no intra-operative complications and minor post-operative skin related complications. If suitable, the device could be a proposed solution for the rehabilitation of hearing in children meeting eligibility criteria. A wearing schedule must be implemented in order to reduce magnet-related skin complications.

Retrospective complication rate comparison between surgical techniques in paediatric cochlear implantation

To compare paediatric complication occurrence between the Mastoidectomy with Posterior Tympanotomy and the SupraMeatal Approach for cochlear implantation.

Identification of Pure-Tone Audiologic Thresholds for Pediatric Cochlear Implant Candidacy: A Systematic Review

Importance Although current guidelines recommend cochlear implantation only for children with profound hearing impairment (HI) (>90 decibel [dB] hearing level [HL]), studies show that children with severe hearing impairment (>70-90 dB HL) could also benefit from cochlear implantation. Objective To perform a systematic review to identify audiologic thresholds (in dB HL) that could serve as an audiologic candidacy criterion for pediatric cochlear implantation using 4 domains of speech and language development as independent outcome measures (speech production, speech perception, receptive language, and auditory performance). Evidence Review PubMed and Embase databases were searched up to June 28, 2017, to identify studies comparing speech and language development between children who were profoundly deaf using cochlear implants and children with severe hearing loss using hearing aids, because no studies are available directly comparing children with severe HI in both groups. If cochlear implant users with profound HI score better on speech and language tests than those with severe HI who use hearing aids, this outcome could support adjusting cochlear implantation candidacy criteria to lower audiologic thresholds. Literature search, screening, and article selection were performed using a predefined strategy. Article screening was executed independently by 4 authors in 2 pairs; consensus on article inclusion was reached by discussion between these 4 authors. This study is reported according to the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement. Findings Title and abstract screening of 2822 articles resulted in selection of 130 articles for full-text review. Twenty-one studies were selected for critical appraisal, resulting in selection of 10 articles for data extraction. Two studies formulated audiologic thresholds (in dB HLs) at which children could qualify for cochlear implantation: (1) at 4-frequency pure-tone average (PTA) thresholds of 80 dB HL or greater based on speech perception and auditory performance subtests and (2) at PTA thresholds of 88 and 96 dB HL based on a speech perception subtest. In 8 of the 18 outcome measures, children with profound HI using cochlear implants performed similarly to children with severe HI using hearing aids. Better performance of cochlear implant users was shown with a picture-naming test and a speech perception in noise test. Owing to large heterogeneity in study population and selected tests, it was not possible to conduct a meta-analysis. Conclusions and Relevance Studies indicate that lower audiologic thresholds (≥80 dB HL) than are advised in current national and manufacturer guidelines would be appropriate as audiologic candidacy criteria for pediatric cochlear implantation.

Low-Frequency Sensorineural Hearing Loss in Familial Hemophagocytic Lymphohistiocytosis Type 5

Introduction: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased. Case Presentation: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature. Management and Outcome: The progressive nature of FHL disorder may cause bilateral, low-frequency, irreversible sensorineural hearing loss. This type of hearing loss should be considered among the long-term sequelea presenting with FHL5. Discussion: We recommend audiological evaluation at initial FHL5 diagnosis to assess for hearing functions. Follow-up in audiology should be part of the long-term monitoring of patients with FHL5 as hearing loss could develop long after diagnosis.

Incorporating ceiling effects during analysis of speech perception data from a paediatric cochlear implant cohort

Abstract Objective: To compare speech perception between children with a different age at cochlear implantation. Design: We evaluated speech perception by comparing consonant–vowel–consonant (auditory) (CVC(A)) scores at five-year follow-up of children implanted between 1997 and 2010. The proportion of children from each age-at-implantation group reaching the 95%CI of CVC(A) ceiling scores (>95%) was calculated to identify speech perception differences masked by ceiling effects. Study sample: 54 children implanted between 8 and 36 months. Results: Although ceiling effects occurred, a CVC(A) score difference between age-at-implantation groups was confirmed (H (4) = 30.36; p < 0.001). Outperformance of early (<18 months) compared to later implanted children was demonstrated (p <0.001). A larger proportion of children implanted before 13 months compared to children implanted between 13 and 18 months reached ceiling scores. Logistic regression confirmed that age at implantation predicted whether a child reached a ceiling score. Conclusions: Ceiling effects can mask thorough delineation of speech perception. However, this study showed long-term speech perception outperformance of early implanted children (<18 months) either including or not accounting for ceiling effects during analysis. Development of long-term assessment tools not affected by ceiling effects is essential to maintain adequate assessment of young implanted infants.