Modified Surgical Technique for Combined Congenital External and Middle Ear Malformations (original) (raw)
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Congenital malformations of the ear
The Indian Journal of Pediatrics, 1992
Tile external ear develops from I and II branchial arches commencing on 38th day of fetal life. The middle ear is formed from the ends of Ist pharyngeal puch and the surrounding mesenchyme, which also is part of the I and II branchial arches. The congenital defects of the external and middle ear usually occurs in combination, and many times with congenital defects of other systems. The cochlear functions i.e. bone conduction is normal in 50% of these cases, thus rehabilitation of these patients with congenital anomalies of external and middle ear is possible. The coexistence of congenital aural atresia with varying degrees of malformation of inner ear may be more frequent than generally assumed. Moderate and severe forms of congenital aural atresia area encountered in about 1 in 10,000 to 20,000 individualJ Tile more severe forms of congenital auricular malformation are always associated with meatal atresia, whereas meatal atresia may, in a few cases be seen in patients with a normal pinna. Atresia of the meatus may be membranous or osseus, membranous atresia is much less common and is characterised by rudimentary cartilagenous canal sep~ated from the middle ear by a dense structure of con
Hearing Results of Surgery for Acquired Atresia of the External Auditory Canal
Otology & Neurotology, 2019
Objective: To evaluate short-and long-term hearing results of surgery for acquired atresia of the external auditory canal (EAC) in a large patient cohort and to define preoperative audiometric conditions useful for patient counseling. Study Design: Retrospective cohort study. Setting: Academic tertiary referral center. Patients: Seventy-eight ears from 72 patients with postinflammatory acquired atresia of the EAC who underwent canal-and meatoplasty were included. Patients with involvement of the ossicular chain, (syndromic) external ear malformations, or congenital aural atresia were excluded. Intervention: Canal-and meatoplasty. Main Outcome Measures: Mean pure-tone averages of thresholds at 0.5, 1, 2, and 3 kHz (PTA 0.5,1,2,3) for air conduction (AC), bone conduction, and air-bone gap (ABG) were calculated preoperatively and at short-term (0.55 yr) and long-term follow-up (>0.55 yr). Additionally, the numbers of ears with a closed ABG 10 dB and 20 dB, and with Social hearing (defined as: AC PTA 0.5,1,2,3 35 dB) were assessed. Results: At short-term follow-up AC PTA 0.5,1,2,3 improved by 18 dB. Social hearing was obtained in 81% of the ears. Postoperatively, 35% of the ears had a closed ABG 10 dB, 83% was closed 20 dB. During follow-up, significant deterioration of 5 to 7 dB occurred for AC thresholds at 0.25, 0.5, and 1 kHz. Conclusions: Surgery for acquired atresia of the EAC is often beneficial. This study suggests overall advantageous surgery when the preoperative indication criteria ABG PTA 0.5,1,2,3 >20 dB and AC PTA 0.5,1,2,3 >35 dB are applied.
Surgical management of conductive hearing loss in children
Otolaryngologic Clinics of North America, 2002
Congenital middle ear anomalies pose a challenge to clinicians because they present in a myriad of ways; however, advances in the diagnosis and treatment of these relatively uncommon ear disorders over the past decade have simplified the care of patients who suffer from them. This article focuses on the evaluation of congenital middle ear anomalies (excluding congenital cholesteatoma and aural atresia) as well as their surgical management.
Cochlear implant and inner ear malformation
International Journal of Pediatric Otorhinolaryngology, 2008
The objective of this retrospective study is to evaluate the efficiency of hyperosmolar therapy for cerebrum spinal fluid (CSF) leakage in cochlear implant (CI) surgery in children with inner ear malformations. Between 1991 and 2006, 490 cochlear implantations were performed in Armand Trousseau Children's Hospital. Thirty-seven patients (7.5%) had inner ear malformation. They were classified as isolated enlargement of the vestibular aqueduct (EVA) (18 cases), incomplete partition (IP) (11 cases), common cavity (CC) (1 case) and variable canal and vestibular malformations (VSCC) (7 cases). A hyperosmolar protocol was applied during surgery to 13 patients after 2003 (Gp) to be compared to the 24 patients without treatment previously to this date (G0). Mean age at implant CI was 8.1 years (1-20 years), mean follow up was 3.9 years (1 month-15 years). Per operative observations were collected for all patients with an empiric method of evaluation of the leakage. A grading using five steps ranged from Grade 0 (no leak) to Grade 4 (gusher). Grading, complications and perceptive results in closed and open set word (Lafon lists) at respectively preoperatively, at 3 and 24 months were gathered and compared between the two groups. Important per operative leak was observed (Grade 4) in 24.3% cases (9/37) of Grade 4, 88.8% of them in G0 (8/9). In 66.6% cases there was a severe dysplasia (CC or IP) (6/9), to be compared to the 21.4% of cases of severe dysplasia with Grade < 3 (6/28) ( p = 0.02). Grade 4 was seen in 45% cases of IP (5/11); it represented 33.3% of the IP in Gp (1/3), and 50% of the IP in G0 (4/8) ( p > 0.05). Grade 4 was seen in 16.6% cases of EVA (3/18); there were no Grade 4 observed in Gp (0/10), and 37.5% cases of EVA in G0 (3/8) ( p = 0.04). Grade 4 was observed in 100% case in CC in the G0 (1/1). Severe complications were misplacement of the electrode in one case (G0), persistent leakage in one case (G0) and meningitis in one case (Gp). Vertigo was observed in 29.7% of cases (11/37) in this population, 72.7% of them in G0 * Corresponding author at: Service d'Oto-rhino-laryngologie et de chirurgie cervico-faciale, Hôpital d'enfants Armand-Trousseau,
Middle Ear Surgery: Pointers and Pitfalls
Careful preoperative selection of patients is one of the most important aspects to avoid complications. First, accurate audiology is essential. Audiological studies can contain inaccuracies as a result of machine, human, or calibration error. The otolaryngologist can use tuning forks judiciously to efficiently check results of the audiology. The 256, 512, and 1024 tuning forks, used in conjunction, can determine the degree of conductive hearing loss. In this way, otolaryngologists can operate on patients who have conductive hearing loss, not nerve hearing loss, which naturally leads to better results. The 256 tuning fork gives a negative result (positive is normal) if the air-bone gap is greater than 15 dB. The 512 tuning fork gives a negative result if the air-bone gap is greater than 25 dB. The 1024 tuning fork is negative if the air-bone gap is greater than 30 to 35 dB. Ideally, all three forks are used in conjunction. However, if only one of the forks can be used, it should be the 512 fork. The 256 fork can be less accurate because it tests low frequencies, which are the same as the ambient noise in an office. More importantly, the 256 fork gives a negative result if the air-bone gap is greater than only 15 dB. A 15 dB air-bone gap should never be an indication for a surgical procedure to improve hearing. Stapedectomy should be performed when the air-bone gap is greater than 25 dB; therefore the 256 test alone indicates surgery more often than is optimal. The 512 tuning fork, on the other hand, tests negative for someone who has an air-bone gap of 25 to 30 dB or greater and who could be helped by a stapedectomy. Because the 1024 tuning fork by itself indicates surgery less often than is optimal, the best surgical indicator is the 512 tuning fork.
Hearing results of stapedotomy and malleo-vestibulopexy in congenital hearing loss
International Journal of Pediatric Otorhinolaryngology, 2009
Aims: To analyze hearing results of surgical treatment of hearing loss associated with the congenital stapes ankylosis with or without malformations of ossicular chain. Study design: Retrospective chart review. Methods: The charts of 1369 stapedotomies performed by senior author (JH) from 1991 to 2006 were reviewed. In 40 cases operative findings were consistent with isolated congenital stapes fixation or associated with middle ear malformations. The modified stapedotomy technique was employed in 33 cases and malleo-vestibulopexy was used in 7 cases. Operative findings were standardized according to Cremers' classification. The outcomes of 40 surgeries were analyzed according to the 1995 AAO-HNS Committee on Hearing and Equilibrium guidelines. High frequency hearing results on 4, 8 and 12 kHz were reported in addition to standard frequencies. Results of stapedotomies and malleo-vestibulopexies were calculated separately. Surgical complications were described. Results: The mean post-operative air conduction (AC) was 33 dB, bone conduction (BC) 22 dB and speech reception thresholds (SRT) 31 dB. Closure of the air-bone gap (ABG) to within 10 dB was achieved in 24/ 40 (60%) of cases. Lack of improvement was observed in 3/40 (8%) patients. In 26/32 (81%) of cases with potential for bilaterally serviceable hearing it was achieved. In 24/40 (60%) of cases symmetrical hearing with interaural difference of less than 10 dB was demonstrated. Conclusion: Significant hearing gain in patients with congenital stapes ankylosis makes surgical treatment a valuable adjunct or an alternative to hearing aids in selected cases. ß
International Congress Series, 2003
The Institute of Physiology and Pathology of Hearing in Warsaw is a reference center for diagnosis, treatment and rehabilitation of hearing and speech disorders in Poland. There is a large amount of clinical material: in the year 2001, almost 4000 hospitalizations, over 38,000 patients in ambulatory follow-up, 48,000 school age children screened in Program ''I can hear'' allow for epidemiological and statistical analysis. This is also a very strong background for the creation of therapeutic strategies and algorithms. In our opinion, reference centers must supervise early detection programs and provide complex therapy for all patients with advanced pathologies. An adequate level of medical service can be secured, thanks to close cooperation of multidisciplinary teams and regular training in most modern diagnostic and therapeutic methods and tools.
Middle Ear Dimensions in Congenital Aural Atresia and Hearing Outcomes After Atresiaplasty
Otology & Neurotology, 2010
To determine if middle ear dimensions in congenital aural atresia (CAA) patients can predict early postoperative audiometric outcomes in order to establish specific parameters that facilitate stratification of surgical candidates. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Twenty-five patients with CAA (28 atretic ears and 22 nonatretic ears) and 12 controls (24 ears). Intervention: Primary repair of congenital aural atresia. Main Outcome Measures: Measure and compare middle ear dimensions in controls, atretic ears, and nonatretic ears in unilateral CAA. Determine correlations between the dimensions and best speech reception threshold during the first postoperative year (SRT-1). Results: The epitympanic depth, medial canal diameter, and the mesotympanic height, area, and estimated volume measurements in atretic ears differ significantly with those in control ears. The mesotympanic length, area, and estimated volume measurements each correlate significantly with SRT-1. Atretic ears with a mesotympanic volume estimate measurement greater than or equal to 42 mm 3 are 24 times more likely to have an SRT-1 of 25 dB or better than those measuring less than 42 mm 3 (odds ratio = 24.5; 95% confidence interval, 2.826Y212.4; Fisher's exact test, p = 0.0022). Conclusion: Middle ear measurements in appropriately selected patients may help predict successful early hearing outcomes after atresiaplasty, thus offering a valuable tool for the surgical decision-making process.
Indication for Surgery in Otosclerotic Patients With Unilateral Hearing Loss
Otology & Neurotology, 2009
The aim of this study is to evaluate postoperative spatial speech discrimination in noise in a group of otosclerotic patients with unilateral hearing loss in a 6-month follow-up. Moreover, an additional objective is to verify if our routine criteria (air-bone gap 930 dB at 250Y2,000 Hz) for surgical indication in such patients can be acceptable. Study Design: Prospective evaluation in 20 patients divided into 2 groups: unilateral otosclerosis and bilateral otosclerosis already successfully operated on 1 side and planned for stapedotomy on the contralateral ear. Setting: Tertiary referral center, University clinic. Patients: Otosclerotic patients with unilateral hearing loss. Intervention: Evaluation of functional outcome. Main Outcome Measure: Spatial test based on speech discrimination in noise. Results: All patients reach postoperative air-bone gap closure within 10 dB. Average postoperative gain in discrimination under noise was 11.5% for unilateral otosclerosis group and 19.3% for the second ear group. Conclusion: Free-field discrimination in noise closely reflects the usual listening conditions in everyday life. Improvement in postoperative free-field discrimination in noise can justify our routine criteria for surgical indication in the patients with unilateral otosclerosis and with bilateral otosclerosis already operated on 1 side. The surgical choice for each patient, apart from audiologic evaluation, is linked to a truly informed consent regarding possible advantages and risks mainly based on an analysis of the real auditory needs of the patient and individual results of the surgeon.