Good Hearing Results Research Articles

Hearing results after ossiculoplasty with incus transposition, cortical bone autografting and bone cement

Background: This study assesses hearing results after ossiculoplasty with incus transposition (IT), cortical bone autograft (CBA) and bone cement (BC).Methods: A total of 21 patients with mild/moderate conductive hearing loss were enrolled in our study. Patients were assigned to each of the three techniques according to length of incus defect. Pre-operative and 6 months post-operative audiograms (PTA) were obtained and results of air bone gap (ABG) were statistically analyzed.Results: A total of 7 patients were included in each technique. BC ABG was decreased from 26.57 (5.06) (mean (SD)) to 10.86 (6.34) with mean gain 15.71 (5.85) For IT group, the mean for pre- ABG was 33.57 with SD 5.13, it became 15.86 (1.86) after 6 months of follow-up with 17.71 (3.40) gain. For CBA, pre- ABG mean was 35.86 (5.67) that finally became 19.14 (2.41) achieving ABG gain 16.71 (6.68). No significant statistical difference between pre-post gain in all three surgical procedures (p-value = 0.80). Regarding side effects in each technique, infections was noted in one case of IT and two cases of CBA, transient infection was found in one case of IT and CBA, and transient vertigo in one case of BC with no significant difference in adverse effects between the three group (P-value= 0.494).Conclusions: Both BC, IT and CBA are reliable techniques for ossiculoplasty with good postoperative hearing results. The choice of the proper technique depends on length of incus defects

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation.

Intravestibular schwannomas (IVS) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the long-term outcomes of CI during the unpredictable evolution of NF2 disease. Retrospective case review. Tertiary referral center. Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients. Periodic radiologic follow-up, tumor resection, and hearing rehabilitation. Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome. Three NF2 patients (mean age at diagnosis, 26.3 ± 3.2 yr) were identified with IVS in the period between 2000 and 2017. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4 ± 1.5 years of follow-up. IVS were removed via a translabyrinthine approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures. Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors.

Cochlear implantation in adults with auditory deprivation: What do we know about it?

In the ENT community, auditory deprivation is frequently considered as a negative prognostic factor for a good hearing outcome of cochlear implantation (CI), even if a growing literature suggests that this is not completely true. The purpose of this study is to evaluate the results of CI in patients with hearing deprivation, to compare them to results from non-deprived patients and then estimate how time of deprivation impacts on CI outcome and how a bilateral deprivation can affect the outcome compared to a unilateral deprivation. Seventy-eight adults with severe to profound post-verbal hearing loss, with and without auditory deprivation history, received CI; audiological results obtained at 3-6-12-24months follow up post CI were analyzed. No differences were founded between patients with unilateral deprivation and patients with no deprivation. Patients with bilateral deprivation seem to have a worse hearing outcome compared to that of those patients with unilateral deprivation or no deprivation at all. Long time deprivation (>15years) seems to have a negative influence on the hearing outcome but results with CI remain excellent. Auditory deprivation should not be considered a contraindication to CI. The duration of auditory deprivation in the implanted ear seems to be a negative prognostic factor only for ears deprived from more of 15years.

Our Experience with Two Handed Endoscopic Tympanoplasty.

Endoscopic tympanoplasty is a minimally invasive procedure and may be an alternative to conventional microscopic tympanoplasty with less operative time, producing similar graft uptake. Endoscopic tympanoplasty has a major disadvantage of single handedness which is overcome by a novel concept of "Two-handed technique" using Khan's Justtach (endoholder). The aim was to study the outcomes of two handed endoscopic tympanoplasty in terms of graft uptake and hearing outcome. Two handed endoscopic tympanoplasty was performed in 20 patients with chronic suppurative otitis media between April 2018-April 2019, in the Department of Otorhinolaryngology, M.G.M. Medical college and MYH hospital, Indore. Tragal cartilage graft via palisading supported with perichondrium was used. Postoperative follow up was done at 3rd month's period, graft status and hearing evaluation was done using Pure tone audiometry. Graft uptake was intact in 19 (95%) out of 20 patients and infection and failure in 1 (5%) case, average preoperative A-B gap was 15-25dB loss (minimal) in 1 (5%) patient, 3 (15%) patients had 25-40dB loss (mild) and 16 (80%) patients had 40-55dB loss (moderate) hearing loss. The post-operative improvement was evaluated in which 6 (30%) patient had 15-25dB loss (minimal), 12 (60%) patients had 25-40dB loss (mild), and 2 (10%) patients had 40-55dB (moderate) conductive hearing loss. We used Wilcoxon Signed Ranks Test for audiological evaluation with a 'p value' of 0.000 making the hearing improvement significant. "Two handed endoscopic tympanoplasty" is an emerging technique which can overcome almost all the major issues related with single handed technique with high degree graft uptake and good hearing outcomes. Endoscope holder is a valuable adjunct to conventional endoscopic surgery.

Trans-mastoid approach for cerebrospinal fluid leak repair.

To describe the technique and discuss the advantages and outcomes of the trans-mastoid approach (TMA) for temporal bone cerebrospinal fluid (CSF) leak. TMA for CSF leak repair is an alternative to middle cranial fossa approach (MCFA) with less morbidity and good outcomes. Persistent CSF leak in the temporal bone whether idiopathic, congenital or acquired, is an indication for surgery. TMA is a valid option for surgery, competing with MCFA in selected cases. Surgical technique consists of a standard mastoidectomy, exposure of the osteodural defect, and repairing it using multiple layers in an inlay and overlay fashion. Outcomes show low recurrence and complication rates with good hearing results. However, long-term follow-up should be made, as recurrences can be delayed.

Utilization of incus with bone cement for total ossicular reconstruction

Background: Total ossicular chain reconstructions are performed to connect mobile stapes footplate and tympanic membrane. Data on the use of incus for total ossicular reconstruction is quite limited in the literature.Objective: The main objective of this study is to describe a novel surgical method that utilizes incus with bone cement to stabilize the ossicular chain for total ossicular reconstruction.Materials and methods: 16 patients who underwent total ossicular reconstruction using our method were included in this study. We evaluated hearing by comparing preoperative and postoperative air-bone gap (ABG), air conduction (AC) and bone conduction (BC). The percentage of patients achieving ABG ≤20 dB was determined.Results: The mean preoperative ABG was 35.3 ± 8.2 and postoperative ABG decreased significantly to 23.7 ± 7.6 (p < .001). The mean preoperative AC (57.5 ± 10) decreased significantly postoperatively to (46.5 ± 13.3)(p = .014). There was not any difference between pre- and post-operative BC. We achieved successful hearing results (ABG ≤ 20dB) in 44% of patients.Conclusion: The use of incus with bone cement stabilization for total ossicular reconstruction seems a feasible option. Good hearing outcomes, and low cost, complication, and extrusion rate may be the main reasons to prefer this method.

Randomized clinical trial to evaluate the outcome of partial canal wall preserved tympanomastoidectomy

Background: The main surgical techniques for the treatment of chronic otitis media so far, all have their characteristic advantages and disadvantages. Otolaryngologists have been trying to find an ideal surgical method for chronic otitis media.Objective: To study and evaluate the outcome of partial canal wall preserved tympanomastoidectomy (PCM) for the treatment of chronic otitis media.Methods: Thirty-nine patients were randomly divided into two groups. Twenty patients underwent PCM and 19 underwent canal wall down tympanomastoidectomy (CWD). All patients had a follow-up period of 5 years.Results: The mean time of healing was 6 weeks in the PCM group and 8 weeks in the CWD group. The cavities in the PCM group were normal or slightly larger than the normal external auditory canal. The patients required cavity cleaning less than once per year in the PCM group and 3–4 times per year in the CWD group. One patient in the PCM group developed a recurrent cholesteatoma located in the attic.Conclusion: The PCM technique showed a low-recurrence rate and good hearing outcomes. Patients had normal or slightly enlarged postoperative external auditory canal. Therefore, PCM is a useful technique for chronic otitis media.

Endoscopic cartilage tympanoplasty: full thickness and partial thickness tragal graft

IntroductionCartilage is the grafting material of choice for certain disorders of the middle ear. The indications for its routine use remain controversial due to the possible detrimental effect on post-operative hearing. ObjectiveThe present study was carried out to report a personal experience with “tragal cartilage shield” tympanoplasty to compare the results, in terms of graft uptake and hearing improvement, of endoscopic cartilage shield technique using either partial thickness or full thickness tragal cartilage for type 1 tympanoplasty and to highlight the tips for single-handed endoscopic ear surgery. MethodsFifty patients with safe chronic suppurative otitis media, assisted at out-patient department from February 2014 to September 2015 were selected. They were randomly allocated into two groups, 25 patients were included in group A where a full thickness tragal cartilage was used and 25 patients included in group B where a partial thickness tragal cartilage was used. Audiometry was performed 2 months after the surgery in all cases and the patients were followed for one year. ResultsOut of the total of 50 patients 39 (78%) had a successful graft take up, amongst these 22 belonged to group A and 17 belonged to the group B. The hearing improvement was similar in both groups. ConclusionThis study reveals that endoscopic tragal cartilage shield tympanoplasty is a reliable technique; with a high degree of graft take and good hearing results, irrespective of the thickness. Furthermore, the tragal cartilage is easily accessible, adaptable, resistant to resorption and single-handed endoscopic ear surgery is minimally invasive, sutureless and provides a panoramic view of the middle ear.

Ossiculoplasty: A Prospective Study on 50 Patients Using Various Graft Materials.

With large number of grafts available for ossiculoplasty, choice becomes difficult. An ideal graft should be safe, easily available, cost efficient, with good hearing results, uptake and low extrusion rates. The ear nose and throat surgeon is still facing the indecision over type of graft to be selected. A prospective studywas conducted in Department of Otorhinolaryngology, Rajindra Hospital, Patiala (August 2012-2014) on 50 patients 15-60years, of either sex withABG > 40dB. Operative procedure planned and type of graft decided intraoperatively. Autografts included remodelled malleus, remodelled incus and tragal cartilage grafts. Synthetic (Teflon) grafts were PORP and TORP. Graft uptake/failure was noted at 1 and 3months. PTA done at 3months postoperatively. Net hearing gain (change in ABG) was calculated for various grafts used and analysed. Mean age was 35.26 ± 13 yrs, male and female 1:1. Most common involved ossicle was incus (100%) followed by stapes (36%) and malleus (34%). Autografts, 23, uptake in 20 (86.95%), failure 3 (13.04%) cases. Synthetic grafts, 27, uptake 18 (66.66%) and failure 9 (33.33%). 'p'value 0.09 (non significant). Mean hearing gain, autografts 14.47 ± 6.54dB and synthetic grafts 14.57 ± 13.12dB. 'p' value 0.976 (non significant). No significant difference seen in mean hearing gain and uptake/failure of autografts and synthetic grafts. Autografts being cost effective are preferred choice.

Long-term hearing results in endoscopic sandwich myringoplasty: An innovative Dhulikhel hospital technique

Background: The endoscope has diagnostic and therapeutic role in different otological surgeries. It has excellent view of images where there are difficult nooks and corners. The use of sandwich cartilage perichondrium in the repair of tympanic membrane peroration has its own benefit in graft uptake and hearing results whether long term or short term. The main purpose of the present study was to evaluate the long-term hearing results in patients who underwent endoscopic sandwich myringoplasty with Dhulikhel Hospital (D-HOS) technique. Materials and Methodology: This was a prospective, cohort study performed among 45 patients who underwent endoscopic sandwich myringoplasty with D-HOS technique using tragal cartilage and perichondrium. The hearing was assessed by comparing pre- with post-operative air-bone gap (ABG) and ABG closure in speech frequencies (0.5 kHz, 1 kHz, 2 kHz, and 4 kHz). Results: Among forty-five patients, 42 (93.3%) had graft uptake in 12 months of follow-up. The postoperative ABG (17.0 ± 8.5 dB) was lesser than preoperative ABG (27.3 ± 12.5 dB) with statistically significant results. Conclusion: Endoscopic sandwich myringoplasty with D-HOS technique is a safe procedure with high-graft uptake rate and good long-term hearing results.

Hearing status and behavioural patterns among school aged children with cleft lip and/or palate

ObjectiveThere is a dearth of studies on long term hearing status and behavioural patterns among cleft lip and/or palate children after their primary lip and palate closure in Malaysia. This study describes the audiology status and behavioural patterns in a group of school aged children with cleft lip and/or palate. MethodA cross sectional study was carried out where caretakers of cleft lip and/or palate were asked to complete the translated Malay language version of Strength Difficulties Questionnaire. The hearing status of the children was analyzed based on recent pure tone audiometric and tympanogram results. The patients’ age, gender, type of cleft pathology, age of palatal surgery and behavioural patterns were examined for their potential relationship with hearing status. ResultsA total of 74 children (148 ears) aged between 7 and 17 years with cleft lip and/or palate were recruited. The result showed 37 ears (25.0%) had hearing loss with majority suffered from mild conductive hearing loss. There were 16 ears (10.8%) that had persistent middle ear effusion. Hearing improvement occurred when palatal repair was performed at the age of less than 1 year old. (p = 0.015) There was no significant relationship between patients' gender, age, type of cleft and history of myringotomy with their hearing status. In terms of behavioural patterns, 16.3% were abnormal for total behavioural score, 39.2% for peer problem and 17.6% for conduct problem. For prosocial behaviour, 16.3% were rated low and very low. There was fair correlation between age and hyperactivity problems (r = 0.44). Patients’ gender, type of cleft pathology, had been teased apart and hearing status was found not related to behavioural problems. ConclusionCleft lip and/or palate patients have a good longterm hearing outcome. Majority had normal hearing and if there is hearing impairment, it is only a mild loss. Early palatal repair surgery before the age of 1 year can significantly reduce the risk of hearing loss. Cleft lip and/or palate patients experienced peer problems. There was no significant correlation between behavioural difficulty and hearing status among school-aged children with cleft lip and palate.

The application of cartilage in middle ear surgery

Summary The aim of this study is to review the application, techniques and procedures of cartilage in the middle ear surgery. Cartilage offers the advantage of higher mechanical stability in tympanoplasty, which is the most common procedure in the middle ear surgery, thus apply to much complicated situation especially for tubal dysfunction and adhesive processes. There are many variations on the shape, size, thickness and technical matters of the cartilage grafts. The past decades and literature have observed excellent graft take rates and good postoperative hearing outcomes for cartilage tympanoplasty and the reservation of perichondrium seems to show better long-term survival. There have been concerns regarding malacia of cartilage struts, as a result, cartilage struts for ossiculoplasty have not gained popularity except for combined application with synthetic materials. Meanwhile, the benefits of cartilage in the reconstruction of posterior canal wall and attic, mastoid obliteration and adhesive otitis media have been demonstrated in the previously published studies. It is well to be reminded that cartilage could be applied in some unusual cases such as vibrant soundbridge, meningoencephalocele.

A Polymer-Based Extended Release System for Stable, Long-term Intracochlear Drug Delivery.

Investigate a new polymer-based drug coating suitability for safe intracochlear delivery and ability to maintain long-term physiologically active levels of the corticosteroid fluticasone propionate. In vitro dissolution study to evaluate release profiles of polymer-coated drug particles and in vivo studies using a guinea pig model to measure perilymph drug concentrations at specific time points after implantation with polymer-coated drug particles and evaluate their effect on hearing function. Polymer-coated fluticasone propionate (FP) particles were surgically implanted in guinea pigs through the round window membrane into the cochlear scala tympani. In the pilot study, pre- and post-op hearing thresholds were conducted on days 7, 14, and 42. In a second study, post-op hearing thresholds were conducted on days 90, 120, and 180. Perilymph drug concentrations were measured on the same time points. In 15 of 16 animals from day 7 through day 90, drug levels were within the targeted range, with no initial burst release detected. Drug was present in all animals on day 90 and was detected in some animals at 120 and 180 days. Hearing was tested and compared with non-implanted ears. Very good hearing preservation was observed in ears implanted with intracochlear particles when compared with contralateral ears. The polymer-based extended release system is effective in providing long-term, stable drug delivery for at least 90 days with good hearing outcomes. The results of this study support the potential for achieving long-term drug delivery with a single intracochlear administration.

Transcutaneous Baha Attract system: Long‐term outcomes of the French multicenter study

Bone conduction implants based on abutment-driven acoustic transmission result in good hearing outcomes; however, skin complications impact the quality of life (QOL) and possibly the viability of the device for many patients. The transcutaneous magnetic Baha® Attract technology was developed with the goal of minimising skin complications. To analyse surgical, auditory and QOL outcomes for patients implanted with the Baha® Attract. Prospective multicentre cohort study. Four French tertiary referral centres. Thirty-two patients implanted with the Baha® Attract, including 25 with conductive and mixed hearing loss and 7 with single-sided deafness. Postoperative follow-up involved the visual analysis of soft tissue adaptation and sound processor magnet strength measurement. The audiometric outcomes were evaluated in quiet and noise, and the QOL was assessed using three different questionnaires. After 12months of use, soft tissue was thinner, and mean magnet strength was significantly decreased (3.7-3.1, P<0.05) relative to measures during surgery. The speech recognition threshold in quiet significantly improved compared to unaided situation (73-44 dB HL respectively, P<0.001) as did functional gain in noise (+2.8). All QOL scores improved, and the APHAB questionnaire score correlated with the audiometric outcomes. The Baha® Attract technology results in significant hearing gain and improves QOL. Skin complications were not observed, although surgeons, audiologists and patients should be aware of soft tissue evolution during the first postoperative year. The reversibility of this implant is a major advantage that allows switching to another system if hearing degrades.

Unilateral congenital malformations of middle ear with intact external ear: a review of 64 cases

To describe the operative findings and surgical results of unilateral congenital middle ear malformations with intact external ear. A retrospective review was performed on 64 patients with unilateral congenital middle ear malformations and intact external ear who underwent exploratory tympanotomy from 2011 to 2016. Demographic data, clinical data, high-resolution computed tomography findings, audiometric data and intraoperative findings were collected. Audiological evaluations before and 6months after surgery were analyzed in 47 patients. The most common malformation were mobile stapes with missing incus long process and stapes suprastructure. The air conduction pure tone average was 58.9 ± 10.5dB HL (range 34.4-78.1dB HL) preoperatively and 28.8 ± 10.6dB HL (range 9.4-55.6dB HL) postoperatively (P = 0.000). Twenty-five cases (53.2%) acquired an air conduction hearing gain exceeding 30dB. Mean air-bone gap (ABG) was 44.5 ± 9.4dB (range 22.5-66.4dB HL) before surgery and 15.6 ± 9.3dB (range 0-35.6dB) after surgery (P = 0.000) for an average gain of 28.8 ± 11.5dB. Thirty-four cases (72.3%) showed a postoperative ABG of less than 20dB, 15 had an ABG within 10dB, and 4 had 0dB ABG after operation. No significant difference was observed for air conduction hearing gain regarding age (P = 0.261) or types of malformations (mobile stapes footplate with or without a suprastructure anomaly, P = 0.058). Unilateral congenital middle ear malformations with intact external ear can be complex and diverse. Functional ossiculoplasty for patients with unilateral congenital middle ear malformations can achieve good hearing outcomes.

Short and Long-Term Outcomes of Titanium Clip Ossiculoplasty.

To report short (∼4 mo) and long-term (>12 mo) audiometric outcomes following ossiculoplasty using a titanium clip partial ossicular reconstruction prosthesis. Case series at a single tertiary referral center reviewing 130 pediatric and adult patients with conductive hearing loss (CHL) secondary to chronic otitis media (n = 121, 93%) or traumatic ossicular disruption (n = 9, 7%) who underwent partial ossiculoplasty from January 2005 to December 2015 with the CliP prosthesis. At both short and long-term follow-up, postoperative air-bone gap (ABG) was significantly improved (18 dB HL, IQ range 13-26, p < 0.0001 and 18 dB HL, IQ range 13-29, p = 0.0002, respectively) when compared with preoperative values (29 dB HL, IQ range 19-37). No significant change in ABG was noted when comparing short and long-term intervals (18 versus 18 dB HL, p = 0.44). Fifty seven percent of cases (51/89) achieved a long-term ABG less than or equal to 20 dB at the time of their last follow-up. The extrusion and displacement rates were 1.5% (2/130), and 0.8% (1/130), respectively. There were no cases of iatrogenic sensorineural hearing loss. Partial ossiculoplasty with the titanium CliP produces good hearing outcomes with a favorable safety profile. At long-term follow-up (minimum of 12 mo), median ABG was 18 dB and remained stable when compared with short-term follow-up. The majority of patients had successful long-term results, with 57% of patients achieving an ABG is less than or equal to 20 dB. Low rates of extrusion (1.5%) and prosthesis displacement off the stapes (0.8%) support the long-term stability of the CliP prosthesis in the middle ear.

Aural Atresia with Cholesteotoma and Its Management Strategies.

The term congenital aural atresia is used to describe failure of external auditory canal to open. Thus it results in conductive hearing loss. There are ways to improve the transmission of sound to the healthy inner ear, we have performed surgery to open the ear canal and restore the natural sound-conducting mechanism to the inner ear. Thus avoiding multiple surgeries. The study was conducted among patients aged between 12 and 24years, our study included 8 patients with aural atresia with cholesteotoma. After a detailed examination of patients were subjected to surgery. In our study with the sample size of eight patients Where, all our patients underwent canal wall down mastoidectomy with simultaneous single stage reconstruction of ossicular chain. All our patients were discharge free and Had subjective improvement in hearing with the post operative period was well accomplished to < 35dB. In our study all eight patients who underwent canal wall down mastoidectomy had good hearing outcome. As to avoid unnecessary recurrence and complications which are usually seen in intact canal wall down mastoidectomies, we suggest canal wall down and single stage reconstruction.

Management of Attic Cholesteatoma While Preserving Intact Ossicular Chain; “Modified Bondy Technique” vs. “Canal Wall Up Mastoidectomy with Tympanoplasty Type I &amp; Scutumplasty”

Background and Objectives The aim of this study is to evaluate the clinical outcomes of two surgical techniques-modified Bondy technique and canal wall up mastoidectomy with tympanoplasty type I and scutumplasty (CWUM/T1)-to remove attic cholesteatoma while preserving ossicular chain intact. Subjects and Method A retrospective study was performed on 23 surgical cases for the attic cholesteatoma with postoperative audiometry data of more than six months after surgery. The patients’ postoperative clinical features and audiometric results were compared between the two surgical groups. Results Out of 23 patients, CWUM/T1 was performed in 13 cases and modified Bondy technique was used in 10 cases. There were no significant differences for the preoperative and postoperative audiograms between the two groups. But air-bone gap increased significantly after CWUM/T1 while it decreased after modified Bondy technique. Three cases with postoperative problems were seen after CWUM/T1 (recurrent cholesteatoma, pars tensa adhesion, recurrent otitis media with effusion). Two cases with postoperative problems were found after modified Bondy technique (mild attic retraction, pars tensa retraction). Conclusion Both surgical techniques seem to be adequate to treat attic cholesteatoma while preserving intact ossicular chain. Given good postoperative hearing results and stability of open cavity against recidivism, the modified Bondy technique seems to be a good choice for the attic cholesteatoma with intact ossicular chain when mastoid is not highly pneumatized. Key words: Audiometry ㆍ Cholesteatoma ㆍ Mastoid ㆍ Otologic surgical procedures

The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder.

Auditory neuropathy spectrum disorder (ANSD) is a condition in which auditory testing reveals normal otoacoustic emissions, but auditory brainstem testing is abnormal or absent and speech discrimination is poor. This constellation of findings ostensibly suggests that the cochlea is healthy and an abnormality of conduction or processing of sound occurs along the nerve fibers. As more is learned about this condition, it is becoming clear that ANSD describes heterogeneous, distinct clinical entities that must be taken into account when devising treatment modalities. Modern auditory testing, genetic testing, and neuroimaging can allow for an accurate understanding of the location of the lesion causing ANSD in the auditory pathway. Contributing causes can include genetic mutations, absent or deficient cochlear nerve, hypoxia and jaundice among others. Hearing aids can be successful in the management of ANSD. Several studies suggest that cochlear implantation can lead to successful hearing outcomes in a subset of this patient population. Auditory neuropathy spectrum disorder represents a relatively rare but important diagnosis for clinicians. Treatment for this condition includes hearing aids and FM systems in more mild cases, and cochlear implants in severe cases. Cochlear implantation for many patients can lead to a good hearing outcomes but the outcome can vary greatly depending on the underlying etiology of ANSD.

Stapes surgery in Sweden: evaluation of a national-based register

The aim of the National Quality Registries is to monitor the outcome of healthcare given to patients. The Swedish Quality register for otosclerosis surgery is one of the nine official national registers for ear, nose and throat diseases in Sweden. Since 2004, surgical and audiological results and patient satisfaction scores have been systematically collected from a majority of the ear, nose and throat clinics performing stapes surgery in Sweden. The results of 1688 patients who underwent primary operations for otosclerosis were evaluated for 24 out of totally 26 clinics performing stapes surgery, between 2004 and 2010. The most common surgical technique reported was stapedotomy accomplished in an overnight stay. A majority of patients experienced improved hearing, and were satisfied with the preoperative counselling. Successful surgery, defined as an ABG closure ≤10 dB HL, was achieved in 69%, improvement in AC by ≥20 dB in 63% and BC not worsened by more than ≥5 dB in 93% of the patients. An overall low incidence of postoperative complications was reported. The outcome for ABG and BC was demonstrated to be independent of the number of operations performed by each clinic. An evaluation of the register and the results from the SQOS revealed that stapes surgery is a safe procedure with good hearing outcomes, low complication rates and a high rate of patient’s satisfaction on a national level.