Surgery for congenital anomalies of the middle ear with mobile stapes

Summary In a series of 104 patients with congenital middle ear anomalies operated on from 1964 to 1986, 27 cases were found in which the stapes footplate was mobile and the conductive deafness was due to an anomaly in the remaining part of the ossicular chain. In 8 cases the middle ear anomaly was caused by discontinuity of the ossicular chain owing to congenital malformation. In the other 19 ears, epitympanic fixation of the ossicular chain was observed, whether or not in combination with malformation of the stapes, incus or malleus. Ossicular chain reconstruction produced an improvement of at least 15 dB in 6 of the first 8 cases with discontinuity of the ossicular chain. The mean gain was 31 dB. Epitympanotomy with exposure of the fixed ossicle and if necessary ossicular chain reconstruction led to an improvement of at least 15 dB in 12 of the 19 ears, with a mean gain of 28 dB. Correspondence to: E. Teunissen     

Allograft stapes surgery for conductive hearing loss in patients with ossicular chain anomalies

An allograft stapes was used during surgical intervention for conductive hearing loss due to ossicular malformations in 11 ears of seven patients. The external auditory canal and tympanic membrane were normal in all ears. The surgical findings for the ossicles were stapes fixation (7 ears), stapes fixation with discontinuity of the incudostapedial joint (in both ears of one patient), a deformed incus and stapes crura compressed by the facial nerve (one ear in which the stapes was not fixed) and an anomaly of the incus and stapes combined with a dermoid cyst (in one ear in which the stapes was also not fixed). In all ears, the following procedures were performed: stapedectomy followed by sealing the oval window with a vein graft and placing an allograft stapes between the oval window and the lenticular process of the incus or the handle of the malleus. The allograft stapes was placed with its capitulum on the oval window in all cases, and fibrin glue was used for stabilizing the seal and the allograft stapes. The indications for stapedectomy for conductive hearing loss due to ossicular chain anomalies and the utility of allograft stapes are discussed.     

Hereditary isolated ossicular anomalies in two generations of patients

We present herein a report of an isolated form of ossicular anomaly that affected two generations of patients. Two female patients, a mother and daughter, were admitted with complaints of conductive hearing loss, with no other anomalies and no history of ear infection. Surgical exploration revealed identical ossicular anomalies: the complete absence of the long process of the incus and fixation of the stapes. This anomaly can be considered to have been inherited in an autosomal-dominant or X-linked-dominant manner. To date, two reports have described isolated forms of congenital ossicular anomalies. Our findings suggest that isolated congenital anomalies can be inherited.     

Reconstruction of the entire ossicular conduction mechanism

OBJECTIVES/HYPOTHESIS: Stapes fixation combined with fixation, absence, or malformation of the malleus-incus complex requires an uncommon surgical reconstruction and offers a unique combination of challenges and hazards. This situation may occur in the presence of severe tympanosclerosis, otosclerosis, congenital ossicular malformations, and revision surgery for either stapedectomy or chronic ear disease. In previous reports, this procedure has been grouped with total ossicular reconstruction without much distinction. However, the challenges unique to this problem deserve special consideration. The present report offers a treatment plan for a group of patients requiring reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate. STUDY DESIGN: Retrospective review. METHODS: Three thousand three hundred fifty (3350) charts of patients requiring total ossicular replacement prostheses (TORPs) were reviewed. Of this group of patients, only 21 of 3350 patients from 1977 to 1999 required TORP placement and removal of the stapes footplate. The patients were followed for an average period of 50 months. RESULTS: Hearing results indicated an overall improvement in the air-bone gap of 10 dB, with 52% achieving an air-bone gap of less than 20 dB. Of the 21 cases, 5 revision surgeries were performed. Three were performed because of a displaced TORP (14.2%). and 2 were performed because of extruded TORPs (9.5%). CONCLUSIONS: Reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate can be successfully achieved with improvement of the air-bone gap of less than 20 dB. Hearing results and extrusion rates are comparable to reported results of TORP placement on a mobile footplate. Successful stapedectomy and simultaneous ossicular chain reconstruction can be performed as a single or staged procedure. Special attention is paid to avoid intrusion of the prosthesis into the vestibule.     

Computed tomography evaluation of the inner ear as a diagnostic,counselling and management strategy in patients with congenital sensorineural hearing impairment

The value of computed tomography (CT) of the petrous bone in the investigation of congenital sensorineural hearing impairment has been questioned. We have conducted a study to establish the usefulness of CT of the temporal bone in the evaluation and management of a consecutive series of unselected adolescent patients with congenital sensorineural hearing impairment of greater than 50 dB HL. Seventy-one patients (142 ears) were identified and images reviewed to establish the incidence of inner ear malformations. Fifteen ears were found to be abnormal in eight patients (seven bilateral and one unilateral abnormality). Three patients had Mondini abnormalities and one of these also had dilatation of the lateral semicircular canals. There were five patients with dilatation of the vestibular aqueduct. One patient had a unilateral dysplasia of the middle and external ear. A variety of incidental intracranial abnormalities were also discovered. We conclude that CT does have a valuable role in the management of SNHI.     

Congenital stapes ankylosis: study of 28 cases and surgical results

OBJECTIVE: The objective of this study was to analyze functional results after stapes surgery in patients with congenital nonprogressive conductive deafness resulting from an isolated fixation of the stapes according to age and surgical procedure. STUDY DESIGN: The authors conducted a retrospective case series from March 1993 to December 2003 in patients from two tertiary referral centers. METHODS: Twenty-eight patients were operated on by stapedotomy or partial stapedectomy using Teflon stapes prostheses. The median age at surgery was 14.2 years (range, 8.3-29.1 years). Main outcome measures were clinical and audiometric evaluation before and after surgery. Mean air conduction (MAC) and bone conduction (MBC) thresholds were recorded at 0.5, 1, 2, and 4 kHz. The evaluation of functional outcome was based on the MAC gain, the MBC comparison, and the mean postoperative and residual air-bone gaps. RESULTS: The median preoperative MAC was 50 dB (range, 19.0-65.0 dB) with a 35.0 dB median dB air-bone gap. With a mean follow up of 19 months, postoperative hearing improvement was statistically significant: median gain of 32.5 dB (P<.001) and median residual air-bone gap of 3.5 dB. The MBC was also statistically improved with median pre- and postoperative MBC of 11.5 and 6.5 dB, respectively (P<.001). Results were not dependent on the age group or type of surgery (stapedotomy or partial stapedectomy). No perceptive hearing loss was observed despite one gusher case. CONCLUSION: Surgical treatment of isolated congenital stapes ankylosis allows good functional results regardless of age or type of surgery.     

软带BAHA在先天性中外耳畸形患者的应用

目的观察软带BAHA(Bone-anchored hearing aids,骨锚式助听器))在双侧先天性中外耳畸形患者的听力改善效果,探索其适应症。方法比较6例佩戴软带BAHA前后的双侧先天性中外耳畸形患者听力情况,包括日常交流情况、纯音(声场)测听或条件反射测听。结果 6例患者或家属反映佩戴BAHA后,日常交流情况明显改善,纯音(声场)测听或条件反射测听气导阈改善30.2dB。结论软带BAHA对双侧先天性中外耳畸形患者是一种简单、有效的听力解决方案。     

先天性耳聋147例临床分析

目的 对147例先天性耳聋的临床资料进行分析,提高对先天性耳聋的认识。方法 收集自1990年2月—2002年4月的先天性耳聋的临床资料,从临床特征、影像学检查、诊断与治疗进行了讨论分析。结果 聋哑、听力下降、说话延迟、行走不稳是先天性耳聋的重要临床特征;Mondini发育异常是最常见的先天性耳聋,占33％;颞骨高分辨率CT检查对先天性耳聋的诊断有重要价值。助听器是目前主要的治疗手段,部分病人可以考虑电子耳蜗植入。结论 提高对先天性耳聋的认识,早期发现,保护利用残余听力,早期开始语训。     

Bone conductive implantation in asymmetric hearing loss (AHL)

Abstract

Background: Bone conductive implants (BCI) represent one possible solution for rehabilitation of single-sided deafness (SSD).

Aims: The aim of the present study was to verify the efficacy of bone conduction implantation in subjects with unilateral severe-to-profound hearing loss and contralaterally impaired hearing, that is, asymmetric hearing loss (AHL), and to compare it with known BCI indications for SSD.

Material and methods: Twenty-one subjects received BCI for either SSD or AHL. All of the subjects underwent a battery of audiological and subjective tests, Data were collected and statistically evaluated within and between the SSD group and the AHL group.

Results: A PTA threshold gain was observed in AHL patients along with improved values in speech audiometry in quiet and noise. The two visual analogue scale evaluations (QoL and QoS) and the GBI showed significantly better scores in AHL patients compared to SSD patients.

Conclusions: BCI provided improvement for auditory or speech recognition in AHL subjects, as compare to SSD. From these findings, it is possible to predict a positive role of BCI for some audiological aspects of AHL subjects that are generally not present or not detectable in SSD cases.     

完壁式上鼓室进路鼓室探查术治疗外伤性听骨链中断

目的:评估完壁式上鼓室进路鼓室探查术治疗外伤性听骨链中断的临床疗效.方法:10例(耳)颞骨骨折和耳外伤而鼓膜完整的患者,颞骨CT轴位示锤砧关节不规整,冠状位示砧镫关节不连续.虚拟耳镜示锤砧关节分离,砧镫关节脱位,听骨链中断.手术经颞线下开放上鼓室,保留完整的外耳道后上壁,充分暴露上、后鼓室和听骨链,检查并行听骨链成形术.结果:砧骨长脚骨折1耳,将砧骨改型后行锤-镫连接术;锤砧和砧镫关节脱位6耳,砧骨完全脱落3耳,行砧骨复位双关节固定术.10耳均行上鼓室进路鼓室探查术,术后语频听力平均提高25～50 dB.结论:完壁式上鼓室进路鼓室探查术治疗外伤性听骨链中断疗效满意.     

Bilateral congenital cholesteatomas associated with ossicular anomalies: a case report

A case of bilateral congenital cholesteatomas, which occurred in a 15-year-old male was reported. His family had been aware of his hearing impairment since he was 3 years old. He had experienced neither otalgia nor otorrhea. Tympanograms were A type bilaterally, and an audiogram showed conductive hearing loss of about 60 dB on both sides. High resolution CT of a horizontal section revealed soft tissue density occupying the space medial to the body of the incus in the right ear, while the middle ear space appeared to be completely clear on the left side. The membranous cholesteatomas on both sides were accompanied by ossicular anomalies with hypoplasia of the long process of incus and the superstructure of the stapes. This case constitutes the seventh case report of bilateral congenital cholesteatomas in the literature. The literature is reviewed and the pathogenesis of congenital cholesteatomas associated with ossicular anomalies is discussed.     

Cochlear implant performance in children deafened by congenital cytomegalovirus—A systematic review

Background

Congenital cytomegalovirus (cCMV) infection is a major cause of sensorineural hearing loss in children.

Objective of review

The objective of this systematic review was to compare performance in paediatric cochlear implant users with SNHL caused by cCMV compared to non‐cCMV implantees.

Type of review

Systematic review

Search strategy

PubMed, EMBASE and the Cochrane databases were searched from inception up to 15 May 2017 for children, cochlear implant, performance and their synonyms.

Evaluation methods

Titles, abstracts and full texts were screened for eligibility. Directness of evidence and risk of bias were assessed. From the included studies, study characteristics and outcome data (speech perception, speech production, receptive language and auditory performance of cCMV groups and non‐cCMV groups) were extracted.

Results

A total of 5280 unique articles were screened of which 28 were eligible for critical appraisal. After critical appraisal, 12 studies remained for data extraction. Seven of 12 studies showed worse performance after cochlear implantation in cCMV children compared to non‐cCMV children. Worse performance in cCMV children was attributed to cCMV‐related comorbidities in six of these studies. Available data on asymptomatic cCMV children compared to non‐cCMV children did not reveal an unfavourable effect on cochlear implant performance.

Conclusions

The available evidence reveals that cCMV children often have worse cochlear implant performance compared to non‐cCMV children, which can be attributed to cCMV related comorbidities. We urge physicians to take into account the cCMV related comorbidities in the counselling of paediatric CI users deafened by cCMV.     

Hearing Loss in the Nonocular Stickler Syndrome Caused by a COL11A2 Mutation

Objective Evaluation of hearing impairment as a feature of the nonocular Stickler syndrome (type II) linked to COL11A2. Study Design Family study. Methods General, orthopaedic, ophthalmologic, and otorhinolaryngologic examinations were performed on 15 affected persons in a Dutch family. Audiograms were obtained and/or retrieved from elsewhere. Cross‐sectional and longitudinal analyses were conducted on the hearing threshold (sensorineural component) in relation to the patient's age to evaluate whether hearing impairment was progressive. Results Mixed hearing loss, i.e., including a substantial air‐bone gap of up to 20 to 60 dB, was present in six cases, concomitantly with a submucous or overt cleft palate in five of them. The audiograms in 14 evaluable cases showed the following types of threshold: U‐shaped (n = 3), flat (n = 2), flat or gently (downward) sloping (n = 3), gently sloping (n = 3), or steeply sloping (n = 3). Cross‐sectional analysis did not reveal any significant effect of age on sensorineural hearing impairment. Conclusion In contrast to the classic Stickler syndrome (type I) with high myopia, this nonocular type shows a high prevalence of sensorineural hearing impairment. The mean sensorineural hearing threshold in our patients was about 40 dB HL (95% CI,15–65 dB) and was liable to increase (presumably by presbycusis) by several tens of decibels at the highest frequencies. Given the tendency for otitis media to develop in many of these patients, appropriate otologic care is of major importance.     

单纯中耳畸形的临床分析

目的 :分析单纯中耳畸形的临床和听力学特征 ,手术探查明确畸形形式 ,避免和减少对该病的误诊、误治。方法 :9例 11耳单纯性中耳畸形患者均行鼓室探查术 ,部分行听骨链重建术 ,其中 ,行镫骨切除加钢丝结绨组织栓人工听骨重建 4耳 ,前庭开窗加人工听骨重建 2耳 ,砧镫关节松解术 1耳 ,高分子材料TORP重建听骨1例 ,蜗窗不明确的 3耳 ,仅做鼓室探查。结果 :术中发现砧骨畸形最多 (10 / 11) ,豆状突细小或缺失 ,砧骨游离 ,长突缺失、变细或变粗 ;镫骨缺如 (2 / 11) ;其他 (7/ 11)包括镫骨不完整或游离、固定、前后脚融合 ;砧镫关节粘连(1/ 11) ;面神经和前庭窗无法辨认 (4 / 11) ;鼓岬表面凹凸不平 ,无法辨认圆窗 (3/ 11)。 3例并发其他全身异常。听骨重建后患者听力满意 ,纯音测听示低频比高频提高明显 ,言语频率平均提高 2 4dB。无并发症发生。结论 :单纯先天中耳畸形最终确诊靠鼓室探查术 ,一定条件下镫骨切除或前庭开窗加钢丝结绨组织栓人工听骨重建术可满意提高听力     

Hearing in patients with congenital deformity of the inner ear

Congenital abnormalities of the bony labyrinth and internal auditory meatus range from complete absence to minor anomalies compatible with normal cochlea function. A ‘follow-up’ survey to assess the hearing of 56 patients with abnormalities of the inner ear shown by tomography was made. The hearing levels are discussed in relation to the tomographic appearances and comparison made with other series of inner ear abnormalities demonstrated both radiographically and histologically.