Congenital Middle Ear Anomalies Research Articles

Surgical results of 29 ears with congenital middle ear anomalies; Microscopic vs. endoscopic ear surgery

ObjectiveAlthough congenital middle ear anomalies include various types of ossicular anomalies, all of these can be treated by ossiculoplasty or stapes surgery. Transcanal endoscopic ear surgery (TEES) is a minimally invasive surgical method for middle ear disease with an excellent surgical view that has been widely adopted worldwide. To determine the efficacy of TEES for middle ear anomalies, we describe the surgical results and compare the hearing outcomes between patients treated by TEES and microscopic ear surgery (MES). MethodsA total of 39 ears with congenital middle ear anomalies were treated surgically at the University Hospital of Kochi Medical School between January 2011 and December 2021. In total, 29 ears of 23 patients were included in the study. Demographics, type of anomaly, surgical methods, pre- and postoperative hearing thresholds, and surgical complications were investigated by retrospective chart review. ResultsOf the 29 ears, 11 were treated by MES and 18 were treated by TEES. There were no differences in sex, age, preoperative hearing thresholds, or rate of stapes surgery between the two groups. The mean air-bone gap improvement was 20.6 dB in the MES group and 28.8 dB in the TEES group; these values were not significantly different. The median operation time was not significantly different between the MES and TEES groups (125 and 130 min, respectively). The improvements of air conduction in class 1 and 2 (stapes surgery) and class 3 (ossiculoplasty) cases were also not different between the groups. ConclusionsTEES achieved comparable hearing outcomes to MES without postauricular or endaural incisions. Further, class 1 and 2 anomalies demonstrated hearing improvement similar to class 3 without major complications. Since the surgical field limited around the ossicular chain, coupled with the fact that the middle ear anomaly itself does not exhibit inflammation leading to unfavorable bleeding, TEES is a feasible surgical procedure for all types of congenital ossicular anomalies.

先天性耳小骨奇形の手術

Transcanal endoscopic ear surgery (TEES) is a minimally invasive surgical treatment technique for middle ear diseases and is recently becoming popular around the world. TEES enables accurate surgical manipulation under a wide magnified, and clear surgical view. We describe the results of surgical treatment for congenital middle ear anomalies by comparing the hearing outcomes between patients treated by TEES and those treated by microscopic ear surgery (MES). Congenital middle ear anomalies were safely treated by TEES, with comparable hearing outcome to those after MES. The surgical field is limited in the tympanic cavity, inflammation is usually absent, and there is little bleeding during the operation; therefore, TEES is a feasible surgical approach for the treatment of congenital middle ear anomalies. However, as middle ear anomalies are diverse, preoperative diagnosis is sometimes difficult, and the surgeon must be adequately prepared in advance for various types of procedures.

Congenital Anomalies of the Ossicular Chain: Surgical and Audiological Outcomes.

Objectives:In this study, we aim to analyze audiometric outcomes of middle ear surgery in patients with congenital middle ear anomalies.Methods:In this single center retrospective cohort study, audiological outcomes were extracted from patient files. Patients with a congenital middle ear anomaly treated surgically in a tertiary referral center between June 2015 and December 2020 were included. Pre- and postoperative short- and long-term audiometric data (at ≥3 and ≥10 months respectively) were compared to analyze hearing outcomes.Results:Eighteen ears (15 patients) were treated surgically with an exploratory tympanotomy. At short term follow up statistically significant improvements in air conduction thresholds and air-bone gaps were found. Hearing improved in 94.4% (17/18) of operated ears. Successful outcome, defined as an air-bone gap closure to within 20 dB after surgery, was reached in 44.4% (8/18). Serviceable hearing (air conduction ≤30 dB) was reached in 55.6% (10/18). Negative outcome (any significant deterioration in hearing) occurred in 1 patient: in this ear otitis media occurred during the postoperative course. At long term follow up, available for 50% of the cohort, hearing remained stable in 5 ears, improved in 1 ear and deteriorated in 3, all of which underwent revision surgery. Sensorineural hearing loss due to surgery, or other complications, were not encountered.Conclusion:middle ear surgery was found to be an effective treatment option to improve hearing in this cohort of patients with congenital middle ear anomalies. Surgical goals of obtained gain in air conduction thresholds and serviceable hearing levels were met by most patients without the occurrence of any iatrogenic sensorineural hearing loss.

Multicenter Study of Congenital Middle Ear Anomalies. Report on 246 Ears.

Congenital middle ear anomalies represent a relatively rare condition. This study aimed to describe the characteristics and the surgical outcomes for patients with middle ear anomalies. A multicenter study was conducted of consecutive patients with congenital middle ear anomalies who underwent primary surgical treatment between January 2008 and December 2017. Demographics, surgical procedures, and audiometric data were registered into the institutional database. Hearing changes and postoperative air-bone gap (ABG) were evaluated 1 year after surgery. A total of 246 patients (246 ears) (median age: 14 years, range: 4-75 years old) were included in this study. Anomalies were subdivided using the Teunissen and Cremers classification: 53 ears (22%) were categorized as class I, comprising only stapes ankylosis; 35 ears (14%) as class II, having ossicular chain anomalies with stapes ankylosis; 139 ears (57%) as class III, having ossicular chain anomalies with a mobile stapes-footplate; and 19 ears (8%) as class IV, with aplasia of the oval window. Evaluation of hearing outcomes for 198 ears with more than 1 year of follow-up revealed that good postoperative ABG (≤20 dB) was achieved in 82% of class I, 68% of class II, 74% of class III, and 23% of class IV anomalies. The postoperative ABG in class IV was significantly worse than in class I (P < .001) or class III (P < .01). This study demonstrated that class III anomalies comprised the majority of middle ear anomalies and surgical outcomes for class IV anomalies are unfavorable. 4 Laryngoscope, 131:E2323-E2328, 2021.

Clinical application of transcanal endoscopic ear surgery in the diagnosis and treatment of conductive hearing loss with intact tympanic membrane

Objective:To explore the clinical efficacy of transcanal endoscopic ear surgery in the diagnosis and treatment of conductive hearing loss with intact tympanic membrane. Method:The clinical data of 16 patients with conductive hearing loss with intact tympanic membrane were retrospectively analyzed. They were diagnosed and treated by transcanal endoscopic ear surgery. Result:All patients were diagnosed by exploratory tympanotomy, including 6 cases of congenital middle ear anomalies, 5 cases of congenital cholesteatoma, 2 cases of congenital middle ear anomalies with congenital cholesteatoma, 2 cases of otosclerosis, and 1 case of traumatic ossicular chain disruption. During the tympanic exploration by transcanal endoscopic ear surgery, different methods of hearing reconstruction were applied according to the intraoperative lesions. Among 14 cases（14 ears）, 7 patients underwent reconstruction with partial ossicular replacement prosthesis （PORP）, 5 patients had total ossicular replacement prosthesis （TORP）, and 2 patients had piston. The remaining 2 patients did not undergot ossicular reconstruction. After the operation, the mean air-conductive threshold of 14 patients decreased from （61.7±6.5） dB HL to （29.8±10.7） dB HL （P<0.01） and the mean ABG decreased from（36.8±3.2） dB HL to （10.7±6.9） dB HL （P<0.01）. 1 case of congenital middle ear anomalies with congenital cholesteatoma underwent the lesion resection without ossicular reconstruction. Due to lack of suitable Piston, 1 case of congenital middle ear anomalies with fixed stapes did not perform hearing reconstruction. No serious complications occured after operations. Conclusion:Transcanal endoscopic ear surgery was suitable for the diagnosis and treatment of conductive hearing loss with intact tympanic membrane. It was minimally invasive with low complications, and the patients had a good hearing recovery after ossicular reconstruction.

Efficacy of transcanal endoscopic ear surgery for congenital middle ear anomalies

Efficacy of transcanal endoscopic ear surgery for congenital middle ear anomalies

Congenital Middle Ear Anomalies: Anatomical and Functional Results of Surgery

The aim of this study was to describe the audiometric results following surgery in a consecutive series of pediatric patients with a congenital middle ear disorder. Retrospective chart review was performed for 29 consecutive children who underwent 33 middle ear surgeries for congenital ossicular chain anomaly between 1990 and 2012. Anomalies were classified into four groups according to the Teunissen and Cremers classification. Audiological parameters using four frequency averages (0.5, 1, 2 and 4 kHz) were assessed pre- and postoperatively. Clinical and audiometric follow-up times were, respectively, 49 ± 8 and 35 ± 5 months (mean ± SEM). Fifty-eight percent of all patients achieved an air-bone gap (ABG) ≤20 dB, 62.5% in class I, 50% in class II and 57.9% in class III. The improvement of the mean ABG was 13.6 dB, 19.2 dB for class I, 0.2 dB in class II and 15.4 dB in class III. Overall mean pure-tone averages improved 14.8 dB with 13.9 dB for class I; there was no improvement for class II and 20.2 dB for class III. The sensorineural hearing loss rate was 9%. This pediatric series showed that hearing results depend on type of anomaly. Class I and class III showed better hearing improvement than class II.

Middle ear malformations in identical twins

The majority of the congenital anomalies of middle ear are solitary and a non-hereditary. We report cases of identical twins with congenital incudo-stapedial disconnection. Case 1 was an 8-year-old girl. Hearing impairment was identified at the age of three. She was referred to our university hospital in April 2005. Pure-tone audiogram showed conductive hearing impairments. Computed tomography (CT) revealed the incudo-stapedial disconnections in both ears. The exploratory tympanotomies on the right and left ears were performed in May and July 2005, respectively. The surgical findings showed absence of the long process and presence of the lenticular process of the incus in both ears. After the reconstructions of ossicular chain, the hearing of both ears improved. Case 2 was an 11-year-old girl. The hearing impairment of the right ear was identified in May 2008. She was referred to our university hospital three months later. Pure-tone audiogram showed the conductive hearing impairment in the right ear. CT revealed the incudo-stapedial disconnection in the right ear. The surgery showed the same findings as those of case 1. Anomalies of both cases suggest that the lenticular process of the incus and the stapes originate from a common primordium.

Wideband energy reflectance findings in presence of normal tympanogram in children with Down's syndrome

Objectives The prevalence of middle ear disorders in children with Down syndrome is higher than normal children due to the associated craniofacial abnormalities. The goal of this study is to evaluate middle ear function using wideband energy reflectance at ambient pressure in 14 young children with Down syndrome and matched control group (2½–5 years old; N = 19 ears per group) who each have a normal 226 Hz tympanogram. Methods All children underwent otoscopic examination, hearing screening using play audiometry (500–4000 Hz), and middle ear testing using 226 Hz tympanometry and wideband energy reflectance. The chirp signals for the wideband energy reflectance were presented to the child's ear at 65 dB SPL stimulus level and the recording was done over 220–8000 Hz range. The measured energy reflectance represents the ratio of the sound energy reflected from the tympanic membrane to the incident sound energy transmitted to the middle ear at a specific frequency. Paired Samples t-test was computed for the mean, 95th, 75th, 25th, and 5th percentile data of each frequency of the two groups. Results Despite the presence of normal tympanometric findings in both groups, results revealed abnormal wideband energy reflectance findings in 63% of the children with Down syndrome compared to the normal wideband energy reflectance findings in the control group. The mean energy reflectance ratio of the Down syndrome group was abnormally lower than that of the control at 5700–8000 Hz ( p < 0.0005). The 5th and 95th percentile ratios of the Down syndrome group fell outside the 5th and 95th percentile of the control group ( p < 0.0005). Conclusions Abnormally low energy reflectance ratios above 4000 Hz in the presence of normal tympanograms in the Down syndrome group may suggest associated congenital middle ear anomalies in children with DS. The present findings suggest that wideband energy reflectance has the potential to be of more practical value in children with DS than tympanometry. Further research with a larger number of Down syndrome children will illuminate the potential of wideband energy reflectance in diagnosing middle ear disorders in children with Down syndrome.

Speech recognition in patients after successful surgery for unilateral congenital ear anomalies

It is generally assumed that auditory stimulation since birth is important for the proper development of the central auditory nervous system. Whether auditory deprivation occurs in man and, therefore, whether it may be considered as a contraindication to surgery in unilateral congenital middle ear anomalies and atresias is the subject of the present study. Speech recognition during both monaural and binaural presentation was studied in patients who had successful surgery for a unilateral congenital ear anomaly. In binaural speech recognition tests, the average results of the patient group (N = 13) and a group of subjects with normal hearing proved to be comparable. The average speech recognition score using monaurally presented band-pass filtered speech was 84% +/- 8% and 77% +/- 10% for the unoperated (normal) and operated ears, respectively (statistically significant). Speech-to-noise ratios of -5.6 +/- 0.7 dB and -3.9 +/- 1.6 db were found in the normal and operated ears, respectively (statistically significant). It is concluded that, in general, the speech recognition scores of the operated ears were satisfactory, but poorer than those of the normal ears.

Surgery for congenital anomalies of the middle ear with mobile stapes.

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.

Classification of congenital middle ear anomalies. Report on 144 ears.

The surgical findings in 144 successive ears operated on for congenital conductive hearing loss were analyzed, and the results were evaluated in terms of hearing gain. All the patients underwent middle ear surgery at the University Hospital Nijmegen between 1964 and 1990. A classification system was developed to analyze the findings. Class 1 comprises ears with congenital isolated stapes ankylosis. Class 2 comprises ears with congenital stapes ankylosis in combination with a congenital anomaly of the ossicular chain. Class 3 comprises ears with congenital anomalies of the ossicular chain and at least a mobile stapes footplate. Class 4 comprises ears with aplasia or severe dysplasia of the oval window or round window.

Congenital anomalies limited to the middle ear.

Congenital anomalies of the middle ear are occasionally encountered during surgery for conductive hearing loss and are unexpected in patients with no other deformities. We reviewed 12 such patients operated on at The New York Eye and Ear Infirmary from 1985 through 1989. Nine of the patients (75%) had unilateral conductive hearing loss whereas three (25%) had bilateral symptoms. One had bilateral congenital middle ear anomalies. Three patients (25%) had anomalies limited to the malleus and scutum. Five patients (47%) had agenesis of the oval window. After reconstructive surgery, 72% of patients had hearing improvement ranging from 13 to 38 dB. The etiology of these anomalies is discussed and their evaluation and surgical indications are presented.