Stapes Surgery for 121 Patients With Mixed Hearing Loss.

This study aims to determine the benefit of stapes surgery for otosclerosis in 121 patients with a mixed hearing loss and a preoperative bone conduction (BC) threshold >30 dB. Average postoperative air conduction (AC) improved from 61.5 dB to 34.3 dB. Average air-bone gap closed from 27.1 dB to 6.1 dB. Bone conduction improved from 34.3 dB to 28.2 dB, with 38% of patients achieving a postoperative AC of <30 dB. Glasgow Benefit Inventory scores showed significantly increased quality of life postoperatively in the 88 patients who responded to follow-up, with an average score of 56. There was a mean reduction in daily hearing aid use postsurgery of 5.48 hours, with 56% of patients who responded to follow-up questionnaire no longer needing to use one. When assessing suitability for stapes surgery, surgeons should consider that preoperative BC thresholds may be a poor indicator of the true cochlear reserve and therefore the potential for improvement in AC thresholds and quality of life.

Hearing Preservation Outcomes With Standard Length Electrodes in Adult Cochlear Implantation and the Uptake of Electroacoustic Stimulation.

BACKGROUND: Cochlear implantation with preservation of residual low-frequency hearing enables patients to utilize acoustic and electrical stimulation. It is widely accepted that preservation of residual low-frequency hearing is beneficial in both background noise and for music appreciation. The extent to which patients may benefit is not fully understood, but the importance of these concepts is reflected in electrode design developments and also refinement of surgical technique. Greater understanding is needed around factors that may affect hearing preservation. This study reports experience in adults using standard length cochlear implant arrays. OBJECTIVE: The study reviews hearing preservation outcomes using the HEARRING GROUP method for factors such as gender, electrode type, insertion depth, laterality, preoperative hearing level, and time between surgery and audiogram. Furthermore, the study reviews rates of electroacoustic stimulation use in those with postoperative functional residual low-frequency hearing. METHODOLOGY: Retrospective case series. INCLUSION CRITERIA: preoperative ≤ 85 dB HL at 250 Hz and aged ≥ 18 years. The hearing preservation percentages were calculated using the HEARRING group formula S=[1 - ((PTApost - PTApre)/(PTAmax - PTApre))*100]%. Preservation of > 75% was considered complete, 25 to 75% partial, and 1 to 25% minimal. Standardized operative technique with facial recess approach, posterior tympanotomy, and minimally traumatic round window insertion was performed for each implant. RESULTS: Fifty-three implantations in 52 patients met the inclusion criteria. The mean age at implantation was 55.5 years. The average time since the last audiogram was 10 months. The mean average total pre and postoperative pure-tone averages were 92.4 dB, 99.2 dB, respectively, using minimum reporting standards for adult cochlear Implant (CI). Thirty percent demonstrated complete hearing preservation, 35.8% partial hearing preservation, and 20.8% minimal hearing preservation. Overall, mean hearing preservation was 52.9%. Sex, age at implantation, insertion depth, lateral versus perimodiolar electrode, and preoperative hearing level did not statistically significantly affect rates of hearing preservation in our study. There was a statistically significant deterioration in hearing preservation outcomes difference at 3 months compared with 12 months postoperatively. Only two patients within our study out of 17 with functional postoperative hearing went on to use electroacoustic stimulation. CONCLUSION: Hearing preservation varies between patients and postoperative outcomes are difficult to predict. This study adds to existing literature in terms of likelihood of hearing preservation following cochlear implantation. In turn, this improves our ability to counsel patients as to the chances of preserving residual low-frequency hearing postoperatively and their ability to use electroacoustic stimulation.

Stapes Surgery for Profound Hearing Loss Secondary to Otosclerosis.

The objective of this study is to evaluate stapes surgery in patients with otosclerosis and "profound" hearing loss. This means they meet hearing threshold criteria for cochlear implantation (CI). We performed a retrospective study and patient questionnaire. The results from 33 patients (35 ears) were recorded (mean age: 63.6, range: 40-85). The primary outcome measure was hearing thresholds recorded before and after surgery at 0.5, 1, 2, 3, and 4 kHz. Hearing thresholds at 2 and 4 kHz were also analyzed. Glasgow Benefit Inventory (GBI) was used in 21 patients to assess life quality changes. Hearing thresholds improved in 80% of ears (mean improvement, 26.3 dB), were unchanged in 11.4%, and worsened in 8.6%. Mean GBI score was +20.7. Hearing aid use decreased in 23.8% and ceased in 28.6%. One patient subsequently underwent CI. For patients with profound otosclerosis, stapes surgery provides a quantitative improvement in hearing thresholds and improvement in quality of life, with reduced reliance on hearing aids. This avoids CI, auditory rehabilitation, and a change in quality and tonality of sound.

The Epidemiology of Otosclerosis in a British Cohort.

OBJECTIVE: To analyse the epidemiology of otosclerosis in a British cohort collected between 2011 and 2017. DESIGN: Retrospective cohort study. SETTING: Five UK ENT Departments. PATIENTS: Patients with surgically confirmed otosclerosis. MAIN OUTCOME MEASURES: Questionnaire data documented family history of otosclerosis, age of onset, medical history, and information on associated risk factors for 657 patients. Pre and post-surgical pure-tone audiometry was collected for 154 of these patients. RESULTS: The age of onset, incidence of bilateral disease, tinnitus and vertigo, a higher prevalence of women (65%) than men (35%) are similar to those reported previously for otosclerosis cohorts. No association with measles infection was detected. Patients with a family history (40%) have an earlier age of onset and a higher incidence of bilateral disease and vertigo than non-familial subjects. Pedigree analysis is consistent with an autosomal dominant inheritance with reduced penetrance being apparent in 44/91 pedigrees studied. Women who associate their hearing loss with pregnancy have an earlier age of onset than those that do not (p = 6 × 10). CONCLUSIONS: This study confirms that otosclerosis is an early adult onset disease that is more prevalent in women than men with a large minority of patients having a family history of otosclerosis. We report new evidence to support a relationship between pregnancy and otosclerosis progression in a proportion of women. In addition, this is the first study to identify differences in severity between familial and non-familial cases of otosclerosis, highlighting the possibility that more than one etiology may be involved.

Hearing Preservation With Standard Length Electrodes in Pediatric Cochlear Implantation.

OBJECTIVE: Preserving low frequencies following cochlear implantation improves outcomes and allows patients to use a combination of electrical and acoustic stimulation. This importance has been reflected in advances in electrode design and refined surgical techniques. Full insertion of standard length electrodes may be advantageous over shortened electrodes because more electrodes can be activated over time if low frequency hearing loss progresses. Surgeons must counsel patients over this choice but data is lacking regarding the degree and likelihood of hearing preservation achievable with standard length electrodes in children. We report our experience using standard length cochlear implant arrays for hearing preservation in children. METHODS: Retrospective case series. INCLUSION CRITERIA: preoperative hearing ≤85 dB HL at 250 Hz and aged ≤18 years. Hearing preservation percentages are calculated using the HEARRING group formula. (Equation is included in full-text article.)Preservation of > 75% was considered complete, 25 to 75% partial, and 1 to 25% minimal. Patients were implanted with either MED-EL FLEX28 or Cochlear Nucleus CI522. Standardized operative technique with facial recess approach, posterior tympanotomy and minimally traumatic round window insertion. RESULTS: Fifty-two implantations in 27 pediatric patients met inclusion criteria. Mean age at implantation: 9.8 years. Average latest audiogram: 8 months. Mean total pre- and postoperative pure-tone averages were 82.8 and 92.6 dB. Seventeen (33%) ears demonstrated complete hearing preservation, 22 (42%) ears partial hearing preservation, 7 (13%) minimal hearing preservation, and 6 (12%) exhibited no acoustic hearing postoperatively. Mean hearing preservation was 55.5%. CONCLUSION: Hearing preservation is achievable to varying degrees in pediatric cochlear implantation using standard length electrodes though it is difficult to predict preoperatively which children may benefit. This study is among the largest additions to the knowledge base for this patient group.

Evidence of distinct RELN and TGFB1 genetic associations in familial and non-familial otosclerosis in a British population.

Otosclerosis is a common form of hearing loss which typically presents in young adults. The disease has a familial, monogenic form and a non-familial form with a more complex aetiology. A previous genome wide association study identified evidence that variants within RELN are associated with the condition. Other genes in which an association has been reported include BMP2, COL1A1, FGF2, PPP2R5B and TGFB1. However, follow up studies have often failed to replicate initial positive results. The aim of this study was to establish if an association exists between eight single nucleotide polymorphisms (SNPs) in these six previously implicated genes and otosclerosis in a British case-control cohort (n = 748). Evidence of an association between rs1800472 in TGFB1 and otosclerosis was found (p = 0.034), this association was strongest amongst non-familial cases (p = 0.011). No evidence of an association was detected with variants in COL1A1, FGF2, BMP2, and PPP2R5B. No association between variation in RELN and otosclerosis was observed in the whole cohort. However, a significant association (p = 0.0057) was detected between one RELN SNP (rs39399) and otosclerosis in familial patients. Additionally, we identify expression of one RELN transcript in 51 of 81 human stapes tested, clarifying previous conflicting data as to whether RELN is expressed in the affected tissue. Our findings strengthen the association of TGFB1 (rs1800472) with otosclerosis and support a relationship between RELN and familial otosclerosis only, which may explain previous variable replications.

Mutations and altered expression of SERPINF1 in patients with familial otosclerosis.

Otosclerosis is a relatively common heterogenous condition, characterized by abnormal bone remodelling in the otic capsule leading to fixation of the stapedial footplate and an associated conductive hearing loss. Although familial linkage and candidate gene association studies have been performed in recent years, little progress has been made in identifying disease-causing genes. Here, we used whole-exome sequencing in four families exhibiting dominantly inherited otosclerosis to identify 23 candidate variants (reduced to 9 after segregation analysis) for further investigation in a secondary cohort of 84 familial cases. Multiple mutations were found in the SERPINF1 (Serpin Peptidase Inhibitor, Clade F) gene which encodes PEDF (pigment epithelium-derived factor), a potent inhibitor of angiogenesis and known regulator of bone density. Six rare heterozygous SERPINF1 variants were found in seven patients in our familial otosclerosis cohort; three are missense mutations predicted to be deleterious to protein function. The other three variants are all located in the 5'-untranslated region (UTR) of an alternative spliced transcript SERPINF1-012 RNA-seq analysis demonstrated that this is the major SERPINF1 transcript in human stapes bone. Analysis of stapes from two patients with the 5'-UTR mutations showed that they had reduced expression of SERPINF1-012 All three 5'-UTR mutations are predicted to occur within transcription factor binding sites and reporter gene assays confirmed that they affect gene expression levels. Furthermore, RT-qPCR analysis of stapes bone cDNA showed that SERPINF1-012 expression is reduced in otosclerosis patients with and without SERPINF1 mutations, suggesting that it may be a common pathogenic pathway in the disease.

Day-case pediatric middle ear surgery: from myringoplasty to bilateral cochlear implantation.

OBJECTIVE: In the English speaking literature there is very little evidence demonstrating safe and effective tympanomastoid day case surgery in pediatric practice. At a time when there is ongoing pressure for trusts to meet financial government targets and our care provision revolves around a patient centred approach, could otolaryngologists perform more pediatric middle ear surgery on a day case basis? We report our experience with a series of 52 pediatric middle ear day cases. METHOD: Prospective study from a London tertiary referral centre of 52 consecutive children undergoing tympanomastoid surgery. There were 6 categories of surgical procedure, ranging from myringoplasty to cochlear implantation. Post-operative recovery was monitored. The duration of anaesthesia was compared with the length of post-operative ward stay. RESULTS: The correlation co-efficient r=-0.2203, showing that there was no association between length of anaesthetic and duration of post-operative ward stay. CONCLUSIONS: Despite minor post-operative problems including pain, bleeding, and nausea and vomiting all patients in this series were discharged on the day of surgery. For tympanoplasty our series adds weight to already available evidence. It is the first series showing that mastoid surgery, whether combined approach, traditional mastoidectomy or cochlear implantation can be performed safely as day cases in a pediatric population.

Use and abuse of cotton buds.

Ear injuries caused by cotton buds are commonly seen in ear, nose and throat (ENT) practice. We asked 1000 patients attending an ENT referral clinic whether they used cotton buds to clean the ear canal. Of the 325 who responded, 171 said they did. The frequency of use was no higher in those with ear complaints than in those with nose and other complaints. 15-20% of respondents disagreed with the statements that cotton buds can cause infections, wax impaction or perforations. On the evidence of this survey, manufacturers' warnings need to be fortified.